Module 02: Cardiovascular

Pericardial Diseases: Surgical Diseases

of the Pericardium & Tumors of the Heart
Emmanuel C. San Pedro, M.D. | 08/19/25 | Asynchronous | Surgery

TABLE OF CONTENTS →​3. Pulmonary artery

→​4. Left atrium
Learning Objectives 1 IV.​ Specific Cardiac 7 →​5. Right atrium
I.​ The Pericardium 1 Tumors →​6. Parietal pericardium
A.​Layers of the 1 A.​Myxoma 7 →​7. Heart covered by the visceral pericardium
Pericardium B.​Metastatic 8
II.​ Diseases of the 1 Cardiac A. LAYERS OF THE PERICARDIUM
Pericardium Neoplasms
A.​Acute Pericarditis 1 Summary/Key Points 9 ●​ The pericardium is composed of the visceral and parietal
B.​Constrictive 3 Review Questions 9 pericardium
Pericarditis Rationale 10 ●​ Visceral pericardium
III.​ Overview of the 6 References 10 →​ Also known as the epicardium
Tumors of the Heart →​ Intimately attached to the heart, atrium, and ventricles
A.​Primary Cardiac 6 →​ Monocellular in layer
Neoplasms
●​ Parietal pericardium
B.​Metastatic Cardiac 6
Neoplasms →​ Tough, fibrous layer
C.​Symptoms of 6 →​ Produces pericardial fluid (15 mL to 50 mL)
Cardiac ■​ Not detectable by physical examination or by any imaging
Neoplasms ●​ Visceral and parietal pericardium fuse over the great vessels and
D.​PE Findings 6 pulmonary veins
E.​Imaging Studies 6
F.​Treatment 6 II. DISEASES OF THE PERICARDIUM
G.​General Principles 7
in Tumor Excision A. ACUTE PERICARDITIS
●​ Acute inflammation of the parietal and visceral pericardium
LEARNING OBJECTIVES
ETIOLOGY
1.​ To refresh knowledge in anatomy and physiology of the
pericardium ●​ Infectious
2.​ Recognize the clinical presentation of common surgical →​ Bacterial, viral, tuberculous, or fungal infections
diseases of the pericardium ●​ Inflammatory
3.​ Identify important ancillary examinations in the evaluation of →​ Autoimmune (lupus erythematosus), scleroderma, rheumatoid
diseases of the pericardium arthritis
4.​ Formulate diagnosis and management of common surgical ●​ Cardiac
diseases of the pericardium →​ Myocarditis
5.​ Recognize the clinical presentation of common cardiac tumors →​ Post pericardiotomy syndrome
6.​ Identify important ancillary examinations in the evaluation of ■​ In cases wherein you need to open the pericardium during
cardiac tumors surgery
7.​ Formulate diagnosis and management of cardiac tumors ■​ Acute pericarditis and effusion of the pericardium is a
common occurrence after the procedure
I. THE PERICARDIUM
●​ Neoplastic
●​ Protective cover of the heart →​ Metastatic, primary cardiac malignancy, tumor invasion or
→​ Prevents rapid cardiac enlargement direct extension of tumor into the pericardium
→​ Adapts to slow increase in pericardial volume ●​ Metabolic
→​ Prevents rapid exsanguination from penetrating injuries by →​ Uremia, hypothyroidism
promoting blood clot formation inside the pericardial sac ●​ Radiation injury
●​ Responsible for the production of fluid lubricant (pericardial fluid) ●​ Idiopathic (80%)
→​ In 80% of cases, etiology is unidentifiable

PATHOPHYSIOLOGY
●​ Compressive effect of pericardial fluid to heart
→​ Causes decrease in diastolic volume
●​ Affects: Right atrium → right ventricle → left ventricle
→​ Affects the right atrium first, then the thicker right ventricle,
then the thickest left ventricle

Figure 1. The Layers of the Pericardium

Figure 1. The Layers of the Pericardium Figure 2. Pathophysiology of Acute Pericarditis

●​ The figure above was not discussed by the lecturer but the Figure 2. Pathophysiology of Acute Pericarditis
original image was found with the labelled parts below:
●​ Causes collapse of the RA, RV, and LV
→​1. Superior vena cava
→​This causes a decrease in stroke volume
→​2. Aorta

TG18: Taguba, Agustin, Bambalan, Burog, Mendoza, Ong, Parugrug, Samson, Santos, Te
02.14 CG9: Chan, Aguinaldo, Alvarez, Dela Cruz, Garganera, Ngo, Pena, Santillana, Sarmiento, Tan V2 Page 1 of 10
 ●​ Decreased stroke volume leads to decreased of cardiac →​ Evolves in 4 stages
output →​ Only 50% of patients with pericarditis experience all 4 stages
●​ If the cycle is unabated, it will lead to shock Table 1. ECG stages of acute pericarditis
●​ The compensation of the body would be a sympathetic surge,
STAGE WAVE MORPHOLOGY
including:
→​Tachycardia ●​ ST-segment elevation with concave upward ST
→​Vasoconstriction Stage I segments
●​ In the early phases of acute pericarditis and tamponade, there ●​ Noted on all leads except V1
is an increase in the heart rate before there is hypotension
●​ ST segments return to baseline with T-wave
Stage II
flattening

Stage III ●​ T-wave inversion without Q-wave formation

Stage IV ●​ Characterized by ECG normalization

RADIOLOGIC FINDINGS
●​ Chest radiograph
→​ Imaging to detect effusion can include chest radiograph
→​ Cardiac size can be normal (problem for x-ray)
■​ Acute and rapid accumulation can cause tamponade
without apparent increase in cardiac shadow
→​ Enlarged cardiac shadow
■​ In cases where pericardial effusion is increasing to
moderate or massive, there may be enlargement of the
cardiac shadow

Figure 3. Progression into Ventricular Collapse via Pericardial Effusion

Figure 3. Progression into Ventricular Collapse via Pericardial

Effusion
●​ This illustration shows the normal appearance of the
pericardium and the pericardial fluid
→​Pericardial fluid is usually not identifiable by any imaging
●​ In moderate pericardial effusion, it might affect first the right
atrium
●​ In more than moderate or large pericardial effusion, the
fluid starts to compress not only on the atrium but also on the
ventricle
→​This causes a collapse in the volume of the right ventricle
and the left ventricle, resulting in a decrease in the
stroke volume
Figure 4. Typical picture of massive pericardial effusion, wherein there is
characteristic water-bottle cardiac appearance
CLINICAL FINDINGS
●​ 2D Echo
●​ Symptoms
→​ Important imaging of acute pericarditis include 2D echo
→​ Chest pain
→​ Detects presence and degree of effusion
■​ Described as
→​ Indicates thickness of pericardium
○​ Substernal
■​ Tells you if pericardium is thickened or not
○​ Inspiratory
→​ Indicates presence of tamponade
■​ Worsened by supine position
■​ Early signs would be RA wall diastolic collapse, followed
■​ Relieved by leaning forward
by RV wall diastolic collapse
→​ Dyspnea, orthopnea
■​ Also includes dilated inferior vena cava (IVC) without
●​ Physical examination
inspiratory collapse
→​ Findings are non specific
→​ In diastole, the atrium and right ventricle collapses because of
→​ Pericardial friction rub
the pressure
→​ Tamponade
■​ In patients with significant amount of pericardial fluid that
goes into tamponade, you have Beck’s triad:
○​ Hypotension
○​ Distended neck veins
○​ Muffled heart sounds
●​ Work up
→​ Directed by clinical impression
→​ Blood works are non specific
■​ Elevated ESR
■​ Elevated white blood cell (WBC) counts
■​ Positive bacterial cultures
■​ Increased viral titers
■​ Cardiac enzymes may be elevated
○​ Troponin I may be elevated in viral or idiopathic acute
pericarditis, but in many cases, it may be normal
●​ ECG Figure 5. 2D echo imaging that shows pericardial effusion, noting the heart
→​ Can be diagnostic (encircled) and the shadow surrounding the heart is fluid (red arrow)

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●​ Other imaging modalities ●​ Percutaneous insertion and most sedation
→​ Include chest CT scan and MRI favorable under echo-guidance
■​ Can show fluid density around the heart, but it will not tell Advantages
you if patient is going into tamponade
●​ Less prone to clogs
●​ Able to obtain pericardium for
●​ Less invasive
biopsy
●​ Permits repeated drainage
●​ Less prone to myocardial injury
●​ Permits repeated drainage

Disadvantages

●​ Cannot obtain pericardium for

biopsy
●​ Prone to myocardial injury since the ●​ Invasive: subxiphoid incision down
heart moves as the needle is being to pericardium
inserted (especially the right
ventricle)

B. CONSTRICTIVE PERICARDITIS
GENERAL CHARACTERISTICS
●​ There is constriction or restriction of the heart by the pericardium
which is usually inflamed or thickened
●​ Thickened fibrotic pericardium impede normal diastolic filling
●​ Involves the parietal pericardium and visceral pericardium
Figure 6. CT scan of patient with acute pericarditis ●​ No race predilection
WORK UP TO ESTABLISH ETIOLOGY OF PERICARDITIS ●​ More common in males compared to females
●​ Age
●​ In the management of acute pericarditis, it is very important to
→​ Range: 8 to 70 years old
establish the etiology
→​ Median: 61 years old
●​ Can be determined by performing the following:
→​ Fluid examination ETIOLOGY
■​ Cytology ●​ Similar etiology to acute pericarditis
■​ Cell count ●​ All causes of pericarditis may lead to constrictive pericarditis
■​ Protein →​ Idiopathic (46%)
■​ LDH ■​ No known antecedent cause, presumably viral
■​ Glucose →​ Post cardiothoracic surgery (37%)
→​ Pericardial biopsy →​ Radiation therapy (9%)
MANAGEMENT AND TREATMENT →​ Infectious (most usual cause in the Philippines)
■​ Bacterial
Management
○​ Mycobacterium tuberculosis is one of the most notorious
●​ Management of acute pericarditis is concerned with treating the agents in causing constrictive pericarditis (CP). It is also
underlying cause the leading cause of CP in the Philippines (uncommon in
→​ Problem is that 80% would be idiopathic, so we will not know the US, and European countries)
what to treat the patient with ○​ Post-suppurative pericarditis of any cause
→​ Treatment of some common etiologies are listed in Table 2 (Pneumococcal, Staphylococcus, Streptococcus, E. coli)
Table 2. Treatment of some common etiologies ■​ Viral
Cause Management ○​ Coxsackievirus
○​ Hepatitis
Bacterial/purulent pericarditis Antibiotics
○​ Adenovirus
TB pericarditis Anti-TB/Anti-Koch’s regimen ○​ Echovirus
Post pericardiotomy syndrome, viral,
Short course NSAIDs (5-7 days) CHRONIC CONSTRICTIVE PERICARDITIS
post-MI, idiopathic

Indications for Surgery

●​ Drain
→​ Consider draining when:
■​ Patient is in tamponade
■​ Suspecting suppurative pericarditis due to bacteria or TB
■​ Unresponsive to medical treatment
●​ Need to establish etiology
→​ Patient is not responding to therapy
Draining Methods
●​ 2 methods to drain:
→​ Pericardiocentesis Figure 7. Development of chronic CPP
■​ Using a needle ●​ Starts as acute pericarditis which causes pericardial effusion
■​ Safer by ultrasound guidance →​ It then causes fibrin deposition around the visceral and
→​ Pericardiostomy parietal pericardium
■​ Tube pericardiostomy ■​ Leads to pericardial organization then chronic fibrotic
Table 3. Pericardiocentesis vs. Tube Pericardiostomy scarring of the pericardium and calcification
Draining Method ●​ Thickened, fibrotic, calcified, inelastic, fused pericardium restricts
movement and diastolic filling of the heart
Pericardiocentesis Tube Pericardiostomy

●​ Involves inserting a smaller bore ●​ Create a small incision

tube/needle (fr 14 and below) ●​ Involves a bigger tube (fr 28-36)
●​ Done under local anesthesia ●​ Done under local anesthesia with

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 →​ Produced due to the sudden cessation of ventricular filling
early in diastole
Work Up
●​ Blood tests
→​ May be non specific
●​ Brain natriuretic peptide
→​ One of the important things to request to differentiate chronic
pericarditis from heart failure or cardiomyopathy
■​ In constrictive pericarditis it is less than <150 ng/L
■​ In restrictive cardiomyopathy it is elevated at >650 ng/L
●​ Chest x-ray
→​ You can appreciate severe pericardial calcification in 20-30%
of cases
→​ Cardiac silhouette may appear normal
■​ Minimal pericardial effusion unlike in acute pericarditis
which presents with massive effusion
→​ Can show dilated superior vena cava
Figure 8. Pathophysiology of chronic CP →​ Pleural effusions (usually bilateral)
●​ With limited diastolic filling of the RV, there is decrease in the ■​ An effect of constrictive pericarditis
stroke volume →​ Finding of pulmonary edema is rare
→​ Like in cardiac tamponade, this consequently decreases
cardiac output and manifests as easy fatigability and
exertional dyspnea
●​ Also, with the limited diastolic filling of the RV, there will be an
increase in RV pressure
→​ Leads to an elevated right atrial pressure and elevated
central venous pressure (CVP) of more than 10-30 mmHg
●​ This causes:
→​ Hepatomegaly
→​ Ascites
→​ Peripheral edema
→​ Increased blood volume
■​ Especially in the venous system
→​ Distended neck veins
Figure 9. Chest X-ray showing calcification around the cardiac shadow
History
Figure 9. Chest X-ray showing calcification around the cardiac
●​ There is slow development of symptoms shadow
→​ Dyspnea
●​ In the lateral view (right image), you can appreciate a rim of
■​ Most common presenting symptom calcification around the right ventricle
■​ Occurs in virtually all patients
●​ Echocardiography
→​ Other symptoms:
→​ Not sensitive
■​ Easy fatigability
→​ Not a reliable technique to visualize the pericardium
■​ Bipedal edema
→​ Pericardium can be echo-dense
■​ Ascites
●​ Echo findings can include:
■​ Hepatic congestion (chronic passive congestion of the liver)
→​ Thickened pericardium
○​ Manifests as nausea, vomiting, and jaundice
→​ Early rapid diastolic filling
■​ Chest pain
→​ Trans-tricuspid velocity show an opposite pattern to the
○​ Only found in a minority of cases unlike in acute
trans-mitral velocity during inspiration
pericarditis
■​ As the velocity of blood increases across the tricuspid
General PE Findings valve, its velocity is decreased in the mitral valve during
●​ In the early stages: inspiration
→​ None or subtle changes ●​ High-resolution CT scan and fast MRI
●​ In the late stages: →​ Procedures of choice for imaging of the pericardium
→​ Ill with marked muscle wasting and cachexia →​ Evaluates thickness of pericardium accurately
→​ Unexplained jugular venous distention ■​ 1-2 mm: normal
→​ Production of pleural effusion ■​ 3-4 mm: thickened
→​ Manifestations of chronic passive congestion (CPC) of the ■​ 4 mm: supports constrictive pericarditis
liver ●​ Findings suggesting impaired right ventricular filling can be seen
■​ Jaundice by CT scan includes:
■​ Liver enlargement →​ Dilatation of the vena cava, hepatic vein, and right atrium
■​ Ascites →​ Presence of ascites
Cardiovascular PE Findings →​ Hepatosplenomegaly

●​ Elevated jugular venous pressure

●​ Distension of neck veins
●​ Sinus tachycardia
●​ Blood pressure can be normal or low
●​ Apical impulse is often impalpable
→​ Due to the thickened pericardium
●​ Heart sounds may be distant or muffled
●​ Pericardial knock

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 radiographs for patients with acute pericarditis.
3.​ Constrictive pericarditis involves the _________________
& ______________________.
4.​ T/F: Pericardial fluid is easily identified through the use
of ultrasound.
5.​ T/F: The most common presenting symptom of chronic
constrictive pericarditis is chest pain.
Answer Key: 1Square Root Sign, 2F, 3Parietal & Visceral Pericardium, 4F, 5F

TB PERICARDITIS
●​ Common type of constrictive pericarditis in the Philippines
●​ Prophylactic pericardiectomy (established case of TB)
→​ Recommended once diagnosed
→​ Acute TB pericarditis invariably goes into constrictive
pericarditis
■​ Note that in our setting, the patient usually comes late
○​ Do not wait for the pericardium to thicken and cause
Figure 10. High-resolution CT Scan
constrictive pericarditis
Figure 10. High-resolution CT Scan ○​ Do it when there is less adhesion to the heart and
●​ CT scan showing the heart in the middle
pericardium is still soft
→​Surrounded by thickened pericardium which is constricting →​ Easier dissection
the heart ●​ Perform pericardiectomy once patient presents with signs of
→​There is some amount of pericardial fluid constrictive pericarditis
●​ ECG
→​ No specific ECG patterns
■​ Inverted T waves
■​ Low voltage ECG
■​ Atrial fibrillation in long term cases (nonspecific)
→​ Normal ECG findings
●​ Cardiac catheterization
→​ Not routine
■​ But can help you diagnose chronic constrictive pericarditis
→​ Done by inserting a catheter into the right atrium through the
femoral vein
●​ Elevated venous pressure (10 to 20 mm Hg)
●​ Square root sign (pathognomonic)
●​ Equalization of pressures in the different cardiac chambers (from
RA-RV-PA-pulmonary capillary wedge)

Figure 12. Thickened pericardium that was removed after a pericardiectomy

Figure 12. Thickened pericardium that was removed after a

pericardiectomy
●​ Pericardium is removed from the phrenic nerve on the right
side to the phrenic nerve on the left side
●​ This, in turn, frees the following structures:
→​Ventricle
→​Roots of the aorta
→​Pulmonary artery

Figure 11. Square root sign (arrow) finding in constrictive pericarditis

Treatment
●​ Pericardiectomy
→​ Parietal pericardium has to be removed
■​ Visceral pericardium doesn’t have to be removed
→​ Approach
■​ Median sternotomy (preferred): splitting the sternum at
the middle
■​ Anterolateral thoracotomy
■​ Video assisted thoracoscopic surgery
○​ Gaining popularity in doing pericardiectomy
→​ Parietal pericardium is dissected off the epicardium
■​ From phrenic nerve to phrenic nerve of the opposite side
■​ From left to right pulmonary arteries
→​ Priority: free the ventricles
→​ Atrial and SVC dissection is optional
Figure 13. Gross appearance of thickened pericardium
ACTIVE RECALL Figure 13. Gross appearance of thickened pericardium
1.​ What is the pathognomonic sign for constrictive
●​ Above, the pericardium is about 6 mm in thickness
pericarditis that can be seen in cardiac catheterization?
●​ Below, the ventricles can be observed
2.​ T/F: Cardiac size is always abnormal in chest

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 ●​ Primary cardiac neoplasms are rare (0.001-0.3% incidence)
●​ Most are benign (75%)
→​ Most common benign primary cardiac neoplasm is myxoma
(30-50%)
→​ Other benign primary cardiac neoplasms
■​ Lipoma
■​ Papillary fibroelastoma
■​ Rhabdomyoma
■​ Fibroma
■​ Hemangioma
■​ Teratoma
■​ Lymphangioma
●​ 25% are malignant
→​ Majority of primary malignant cardiac neoplasms are
Figure 14. Pericardiectomy sarcomas and can be from any of the following:
■​ Blood vessel: angiosarcoma
Figure 14. Pericardiectomy
■​ Heart: rhabdomyosarcoma
●​ Left side: part of the diaphragm ■​ Connective tissue: fibrosarcoma
●​ Right side: part of the aorta ■​ Other sarcomas: leiomyosarcoma, liposarcoma
●​ Note the calcifications underneath the pericardium
→​ 1-2% are lymphomas
●​ The problem with pericardiectomy is that you have to be very
careful in removing the pericardium because it might cause B. METASTATIC CARDIAC NEOPLASMS
ventricular injury, atrial injury, or worse, coronary artery injury
●​ More common than primary cardiac neoplasms [!]
●​ Results: →​ Found in 4-12% of patients dying of cancer
→​ Massive diuresis expected after correcting hemodynamic
C. SYMPTOMS OF CARDIAC NEOPLASMS
abnormalities
→​ Response rate: 95% and up ●​ Mostly asymptomatic
→​ Mortality rate: 5% or less ●​ Non-specific symptoms:
→​ Dyspnea
Case Pericardiectomy →​ Fever
→​ Malaise
→​ Weight loss
→​ Arthralgias
→​ Dizziness

LOCAL EFFECTS TO THE HEART

●​ Heart failure
●​ Pulmonary hypertension
Pericardiectomy ●​ Dyspnea
●​ Note: This section starts at 25:38 of the lecture on Surgical ●​ Orthopnea
Diseases of the Pericardium. You may also watch it by ●​ Edema
scanning the QR code above.
●​ The surgeon is using a cautery, and is carefully dissecting off RELATED TO EMBOLIC EPISODES
the pericardium away from the heart ●​ Tumor can also embolize to different parts of the body
●​ Objective: to slowly and carefully peel off the pericardium →​ Neurologic: stroke
away from the ventricles
→​ Gastrointestinal: superior mesenteric artery occlusion
●​ Normally, the pericardium is not attached to the ventricles
→​They are fused only at the back of the heart (i.e., at the (SMAO)
pulmonary veins and the roots of the great arteries) →​ Peripheral: arterial occlusion of lower or upper extremity
●​ The video shows that, slowly, the heart is being freed from (acute limb ischemia)
the thickened pericardium
●​ Dr. San Pedro usually does sharp and blunt dissection D. PE FINDINGS
during these procedures ●​ Murmurs suggestive of mitral stenosis or insufficiency
→​Using a cautery might cause arrhythmias (usually seen in left atrial myxomas)
●​ However, in this case, this is probably a monopolar type of ●​ Heart failure
cautery that prevents the induction of arrhythmias
→​ Neck vein distension
III. OVERVIEW OF THE TUMORS OF THE HEART →​ Bibasilar crackles
●​ Systemic embolization
A. PRIMARY CARDIAC NEOPLASMS
→​ Vascular occlusion: pulses, cold, pale extremity, stroke, SMAO

E. IMAGING STUDIES
2D ECHO
●​ Visualize tumor and its relationship to the cardiac structures:
→​ Chamber
→​ Wall
→​ Valves
→​ Pericardium
●​ Views
→​ Transthoracic
→​ Transesophageal (TTE)

HIGH RESOLUTION CT SCAN / FAST MRI

●​ Excellent definition of posterior cardiac structures

F. TREATMENT
Figure 15. Primary cardiac neoplasm tree
●​ For benign tumors: Excision is the treatment of choice

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 →​ Avoid important cardiac structures
→​ Reconstruct/repair any involved cardiac structures after
excision (e.g., valve repair, atrial septum reconstruction)
●​ For malignant tumors:
→​ Multimodiality therapy, including:
■​ Excision
■​ Adjuvant therapy (must give)
■​ Radiation (must give)
→​ Metastatic
■​ Seldom excised
■​ Poor outcome

G. GENERAL PRINCIPLES IN TUMOR EXCISION

EVALUATION OF STRUCTURES
Figure 17. Gross appearance of an excised myxoma
●​ Evaluate the structures that might be injured during excision
→​ Coronary arteries TYPES
■​ Difficult to repair but possible ●​ Sporadic Myxoma
→​ Conduction system →​ Usually found in the 5th to 6th decade of life
■​ Not possible to repair ●​ Familial Myxoma
■​ If you injured the main conduction system of the heart, →​ Autosomal dominant
patient may need to be on permanent pacemaker →​ Usually found < 30 years of age
→​ Valve apparatus →​ Syndrome includes:
■​ Repair/replaced ■​ Freckles
→​ Ventricular wall ■​ Pigmented nevi
■​ Limited resection ■​ Nodular adrenal cortical disease
■​ You can only excise so much without limiting its function ■​ Mammary myomatous fibroadenomas
→​ Atrial wall
CLINICAL FINDINGS
■​ Forgiving
●​ Asymptomatic, until the tumors:
POOR PROGNOSIS →​ Embolize (in 40 to 50% of patients)
●​ Do not attempt excision for malignant tumors with →​ Obstruct the mitral orifice when too large, causing:
extra-cardiac spread ■​ Mitral stenosis syndrome
→​ We do not recommend excision for patients with limited ■​ Sudden cardiac death (if total occlusion)
survival
DIAGNOSIS
■​ Poor medical condition
■​ Weeks to few months median survival Imaging Studies
■​ Advanced age ●​ 2D Echo
IV. SPECIFIC CARDIAC TUMORS ●​ MRI or CT Scan

A. MYXOMA
●​ True benign neoplasm of the heart
●​ Found in the left atrium (75%) > right atrium > ventricles
●​ Do not metastasize but they embolize
●​ Do not invade
●​ With tendency to recur

PATHOLOGY

Figure 16. Gross appearance of a myxoma within the heart

●​ Polypoid
●​ 1 to 2 cm stalk attached to the atrial septum
●​ Size is 0.5 to > 10 cm
Figure 18. Myxoma on 2D Echo
●​ Slow growing
●​ Endothelium covered mass Figure 18. Myxoma on 2D Echo
●​ Myxomatous stroma with large stellate cells mixed with ●​ A myxoma can be found in the left atrium, attached to the
fusiform or multinucleated cells atrial septum

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 Take Note! B. METASTATIC CARDIAC NEOPLASMS
●​ The following images were taken from the video in slide 21 of ●​ Found in 4 to 12% of autopsies performed for neoplastic disease
Dr. San Pedro’s PPT and were sourced from ●​ Most common sources
cardiologyboardreview.org. Watch the video embedded in the →​ Lung cancer
slide in order to see the movement of the myxoma in relation →​ Breast cancer
to the other heart structures. →​ Melanoma
→​ Lymphoma
●​ Widespread metastasis is the rule
●​ Multifocal involvement of the heart is more common than solitary
involvement (spares cardiac valves)
CLINICAL MANIFESTATIONS
●​ Unexplained hemorrhagic pericardial effusion
●​ Bizarre cardiac shadow on x-ray

IMAGING STUDIES
●​ 2D echo
→​ Show involvement of cardiac structures
●​ Biopsy obtained by surgery
→​ VATS (Video-Assisted Thoracoscopic Surgery)
→​ Subxiphoid tube pericardiostomy
Figure 19. Myxoma on the left atrium abutting the mitral valve on echocardiography ■​ Patients with massive pericardial effusion
→​ Median sternotomy

TREATMENT
●​ Excision is rarely possible
●​ Main treatment: chemotherapy / radiotherapy (limited response)
●​ Poor outcome

Case Excision of atrial myxoma

Figure 20. Myxoma obstructing the mitral valve orifice on echocardiography with Atrial Myxoma
Doppler ●​ Note: Please watch the full video by scanning the QR code
Histology above
●​ Most common of benign primary cardiac tumors
●​ Foundation of diagnosis would be on histology of the following: ●​ Over 75% occur in left atrium
→​ Surgically removed embolus ●​ Only 8% originate in ventricles
→​ Resected tumor from the heart ●​ Can result in obstructive blood flow symptoms (e.g.
shortness of breath, fatigue, fainting, dizziness, or heart
TREATMENT failure symptoms)
●​ Surgical excision, once diagnosed ●​ Case: 60 y/o woman with sudden left arm paralysis
→​ Approach: →​Workup included echocardiogram showing a large tumor
(refer to Figure 22)
■​ Median sternotomy
■​ Care observed in handling the heart to avoid embolism
during the procedure
■​ Right atrial approach
○​ Open the heart through the right atrium, then through the
right atrium you open the atrial septum
■​ Myxoma, along with the stalk and the septum where stalk is
attached, is excised
■​ Atrial septum is reconstructed afterwards
Figure 22. Echocardiogram showing a large tumor (yellow arrow)
→​Patient was taken to surgery and placed on
cardiopulmonary bypass, and the right atrium was isolated
and opened
■​ Foramen ovale was identified (refer to Figure 23)

Figure 21. Procedural pictures of myxoma excision Figure 23. Foramen ovale
→​An incision was made into the atrial septum to locate

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 tumor ■​ Bacterial/purulent pericarditis: antibiotics
■​ As the atrial septum was opened further, the atrial ■​ TB pericarditis: Anti-TB/Anti-Koch’s regimen
myxoma could be seen in the left atrium ■​ Post pericardiotomy syndrome, viral, post-MI, idiopathic:
→​Careful technique in opening the atrial septum allows short course NSAIDs (5-7 days)
normal tissue to be resected along with the tumor, ●​ Constrictive Pericarditis
preventing its recurrence →​ Infectious is the most usual cause (in the Philippines)
■​ Circumferential dissection around the atrial septum
■​ Bacterial (Mycobacterium tuberculosis, Pneumococcal,
where the myxoma was attached was performed
○​ This base of attachment was used as a handle to Staphylococcus, Streptococcus, E. coli)
remove the gelatinous tumor ■​ Viral (Coxsackievirus, Hepatitis, Adenovirus, Echovirus)
■​ Excised specimen shows a shiny white atrial septum →​ TB Pericarditis is a common type of constrictive pericarditis in
attached to the gelatinous myxoma tumor (refer to the Philippines.
Figure 24) ■​ Prophylactic pericardiectomy is recommended once it is
diagnosed
●​ Primary cardiac neoplasms are rare (0.001–0.3%); 75% are
benign (most common = myxoma), 25% malignant (mostly
sarcomas; angiosarcoma is most common). Metastatic cardiac
tumors are far more common, found in 4–12% of cancer deaths.
→​ Often asymptomatic or nonspecific (dyspnea, fever, malaise);
may cause local cardiac effects (heart failure, pulmonary
hypertension), systemic embolization (stroke, SMA occlusion,
acute limb ischemia), or mimic valvular disease (e.g., mitral
Figure 24. Atrial septum attached to the gelatinous myxoma tumor
stenosis murmur from LA myxoma).
→​Left atrium was then carefully inspected to ensure no
→​ 2D echo (TTE/TEE) is first-line; CT/MRI for detailed anatomy
residual tumor was left behind
(esp. posterior structures). Benign tumors → surgical excision;
→​Patch closure of the septal defect created by resecting the
tumor was performed using the patient’s own pericardium malignant tumors → multimodality therapy, but prognosis is
→​Finally, right atrium incision was closed prior to separation poor, and excision is not recommended if extra-cardiac spread
from cardiopulmonary bypass exists.
●​ Myxomas are a true benign neoplasm and are most commonly
ACTIVE RECALL found in the left atrium. They do not metastasize or invade, but
they embolize and they have a tendency to recur.
1.​ T/F: Chronic pericarditis invariably goes into constrictive
pericarditis. →​ There are two types of myxomas, Sporadic (5th to 6th
2.​ Which of the following is the most common malignant decade) and Familial (autosomal dominant, <30 years old).
primary cardiac tumor? →​ They are often asymptomatic, unless they embolize or they
A.​Lymphoma become too big and obstruct the mitral orifice.
B.​Angiosarcoma →​ They are diagnosed with 2D echo or MRI/CT scan and with
C.​Rhabdomyoma histology of the embolus or resected tumor.
D.​Fibrosarcoma
→​ Treatment is through surgical excision via median sternotomy
3.​ T/F: Metastatic cardiac neoplasms present with a bizarre
cardiac shadow on x-ray. with a right atrial approach.
4.​ T/F: Sporadic myxomas are commonly found in the 6th to ●​ Metastatic cardiac neoplasms commonly arise from: lung cancer,
7th decade of life. breast cancer, melanoma, and lymphoma. Widespread
5.​ Which of the following statements is false regarding metastasis is the rule.
myxomas? →​ Clinical manifestations include unexplained hemorrhagic
A.​They are polypoid in shape pericardial effusion and bizarre cardiac shadow on x-ray.
B.​They possess a 1 to 2 cm stalk attached to the atrial
→​ Imaging studies include 2D echo and biopsies obtained by
septum
C.​They are slow-growing surgery
D.​They are an epithelium-covered mass →​ The main treatment is chemotherapy or radiotherapy.
Answer Key: 1F, 2B, 3T, 4F, 5D REVIEW QUESTIONS
SUMMARY & KEY POINTS 1. Which of the following is not true of the parietal
pericardium?
●​ The pericardium is a protective cover of the heart. It is composed
of the visceral (epicardium) and parietal pericardium. The A.​It is a tough fibrous layer, intimately attached to the heart.
parietal pericardium is responsible for producing pericardial fluid. B.​It is responsible for producing pericardial fluid.
●​ Acute pericarditis C.​It fuses over the great vessels and pulmonary veins together with
→​ Acute inflammation of the parietal and visceral pericardium the visceral pericardium.
→​ The compressive effect of pericardial fluid to the heart causes D.​The pericardial fluid it produces is not detectable by physical
decrease in diastolic volume. examination or imaging.
→​ Affects the RA → RV → LV. 2. Which of the following statements on acute pericarditis is
→​ Clinical findings false?
■​ Symptoms: chest pain, dyspnea, orthopnea A.​ECG can be diagnostic.
■​ Physical examination: pericardial friction rub, tamponade B.​Beck’s triad is exhibited in patients with a significant amount of
(hypotension, distended neck veins, muffled heart sounds) pericardial fluid.
■​ Blood work-up are non specific: elevated ESR, elevated C.​2D echo indicates the presence of tamponade.
WBC, bacterial cultures, viral titers, cardiac enzymes D.​Chest pain is relieved by supine position and worsened by
■​ ECG can be diagnostic and evolves in 4 stages leaning forward.
→​ Radiologic findings
3. [True or False] Post-cardiothoracic surgery is the most
■​ Chest radiograph: massive pericardial effusion shows common etiology for constrictive pericarditis in the
water-bottle cardiac appearance Philippines.
■​ 2D echo: detects presence and degree of effusion,
4. Modified T/F Question
thickness of pericardium, presence of tamponade
■​ CT scan and MRI: shows fluid density around heart [Statement A] Myxomas are more commonly found in the right
→​ Management
atrium, followed by the left atrium, then the ventricles.
[Statement B] Surgical excision of a myxoma typically involves the
■​ Treatment of underlying cause
right atrial approach.

02.14 Pericardial Diseases: Surgical Diseases of the Pericardium & Tumors of the Heart Page 9 of 10
A.​Statement A is true; Statement B is false 7.​ [B] — Pericardiectomy has a response rate of 95% and up, and
B.​Statement B is true, Statement A is false a mortality rate of 5% or less.
C.​Both statements are true 8.​ [B] — The patient’s condition is chronic constrictive
D.​Both statements are false pericarditis. It begins as acute pericarditis with pericardial
5. A 55-year-old male with a history of metastatic renal cell effusion, then fibrin deposition, organization, fibrosis, and
carcinoma presents with new-onset dyspnea and peripheral calcification. The thickened, fibrotic, calcified, inelastic, fused
edema. Echocardiography reveals a mass in the right atrium. pericardium limits diastolic filling, raising venous pressures (JVP,
Which of the following statements is most accurate hepatomegaly, ascites, edema) and reducing cardiac output
regarding his cardiac tumor? (dyspnea, easy fatigability).
A.​It is most likely a primary cardiac sarcoma. 9.​ [B] — High-resolution CT scan and fast MRI are procedures of
B.​Metastatic cardiac neoplasms are rarer than primary tumors. choice for imaging of the pericardium and evaluating its
C.​Surgical excision is curative in most cases. thickness accurately. The other choices are correct
D.​Cardiac metastasis occurs in a significant proportion of patients
REFERENCES
dying of cancer.
REQUIRED REFERENCES
6. What is the main treatment for metastatic cardiac
neoplasms? [Lecture]
San Pedro. E., Surgical Diseases of the Heart, ASMPH 2028
[Lecture]
San Pedro. E., Tumors of the Heart, ASMPH 2028
A. Chemotherapy / radiotherapy
B. Excision SUPPLEMENTARY REFERENCES
C. Median sternotomy [Trans] ASMPH 2027. 05.13: Pericardial Diseases: Surgical Diseases of the
D. Medical therapy Pericardium & Tumors of the Heart. [Trans]

7. Modified T/F Question IMPORTANT LINKS

[Statement A] The response rate of pericardiectomy is 90% and up. Errata Tracker: [tinyurl.com/YL7ModuleErrataTrackerDrive]
[Statement B] The mortality rate of pericardiectomy is 5% or less. V2 Errata Sheet: [tinyurl.com/YL7V2ErrataSheet]
A.​Statement A is true; Statement B is false Evaluations: [tinyurl.com/YL7TransEvals]
YL7 Trans Map: [tinyurl.com/2028YL7TransMap]
B.​Statement B is true, Statement A is false
C.​Both statements are true FREEDOM SPACE
D.​Both statements are false
8. A patient presents with slow development of dyspnea, easy ‘

fatigability, bipedal edema, and ascites. On exam, there is

distension of the neck veins, hepatomegaly, and a
pericardial knock. Which of the following best explains the
condition?
A.​Acute pericarditis with pericardial effusion causing tamponade
B.​Thickened, fibrotic, calcified pericardium restricting diastolic filling
of the ventricles
C.​Left ventricular systolic dysfunction leading to pulmonary
congestion
D.​Valvular heart disease causing chronic passive congestion of the
liver
9. The following are true regarding chronic constrictive
pericarditis, except?
A.​Echocardiography findings may show thickened pericardium early
rapid diastolic filling
B.​High-resolution X-ray and MRI are the procedures of choice for
imaging the pericardium and evaluating its thickness
C.​Testing for Brain natriuretic peptide can help differentiate chronic
pericarditis from heart failure or cardiomyopathy
D.​Cardiac catheterization may be helpful in the diagnosis but it is
not a routine procedure
Answer Key
1A, 2D, 3F, 4B, 5D, 6A, 7B, 8B, 9B

RATIONALE TO REVIEW QUESTIONS

1.​ [A] — The parietal pericardium is a tough fibrous layer that
follows after the visceral pericardium. The visceral pericardium or
epicardium is intimately attached to the heart, atrium, and
ventricles.
2.​ [D] — Chest pain is worsened by supine position and is relieved
by leaning forward. Statements A, B, and C are true.
3.​ [F] — Infectious causes (bacterial or viral) is the most common
etiology of constrictive pericarditis in the Philippines.
4.​ [B] — 75% of myxomas are found in the left atrium, followed
by the right atrium, then the ventricles.
5.​ [D] — Metastatic cardiac neoplasms are far more common than
primary tumors, being present in 4–12% of cancer-related
deaths. Primary cardiac sarcomas are rare. Surgical excision of
metastatic tumors is seldom done due to poor outcomes and
widespread disease. Thus, the correct choice highlights that
secondary cardiac involvement is more frequent than
primary cardiac tumors.
6.​ [A] — The mainstay treatment for metastatic cardiac neoplasms
is chemotherapy or radiotherapy. Excision is rarely possible.

02.14 Pericardial Diseases: Surgical Diseases of the Pericardium & Tumors of the Heart Page 10 of 10
 Module 02: Cardiovascular

Pediatric Cardiac Catheterization

Dr. Dexter Cheng | 08/22/25 | Asynchronous | Surgery

TABLE OF CONTENTS B. DIAGNOSTIC CARDIAC CATHETERIZATION

I.​ Cardiac 1 C.​Stenting of 6
Catheterization Ductus
A.​What is Cardiac 1 Arteriosus and
Catheterization? Other Vascular
B.​Diagnostic Cardiac 1 Structures
Catheterization D.​Stent of 6
II.​ Scope of Pediatric 2 Pulmonary
Interventional Artery Stenosis
Cardiac E.​PDA Devices 7
Catheterization F.​Patent Ductus 7
A.​Creating an ASD 2 Arteriosus
B.​Relieving 3 G.​Summary 10
Obstruction Summary/Key Points 10
Review Questions 10
Rationale 11
References 11

I. CARDIAC CATHETERIZATION
A. WHAT IS CARDIAC CATHETERIZATION
●​ Invasive procedure that uses small catheters in an artery or vein
which is advanced into the chambers and great vessels of the
heart
→​ In adults, enter through various blood vessels Figure 2. Angiogram of the left coronary artery with a dilated coronary artery fistula
→​ In pediatrics, predominantly enters through the femoral that drained into the right atrium; fistula was occluded with a bunch of implanted
coils (bottom left corner)
artery and vein but sometimes the internal jugular vein
●​ Performed in a cardiac catheterization lab
●​ Used to be primarily diagnostic

Figure 3. Right and left pulmonary arteries are shown with an abnormal pattern in
the right (Note: ultrasounds in the 70s were not as accurate)

Figure 1. Site of cardiac catheterization

Figure 4. Pulmonary arteriovenous malformation (AVM) in the right lower lung field

TG9: Chan, Aguinaldo, Alvarez, Dela Cruz, Garganera, Ngo, Pena, Santillana, Sarmiento, Tan
02.24 CG18: Taguba, Agustin, Bambalan, Burog, Mendoza, Ong, Parugrug, Samson, Santos, Te V2 Page 1 of 11
 →​Cross the small patent foramen ovale with a catheter
→​Inflate the balloon on the left side
→​Then, pull that balloon very hard and quickly across the
atrial septum to tear it open
■​ This creates a defect and therefore allowing the
oxygenated blood to go from LA to RA

Figure 7. Balloon Atrial Septostomy as seen in Fluoroscopy

Figure 7. Balloon Atrial Septostomy as seen in Fluoroscopy

Figure 5. Left ventricular angiogram showing a large ventricular septal defect;
septum (white) and the defect above it ●​ The balloon has already been inflated on the left
●​ A sudden hard pull across the atrial defect will cause the
II. SCOPE OF PEDIATRIC INTERVENTIONAL CARDIAC balloon to come to the RA
CATHETERIZATION ●​ Balloon is pulled so hard you can see the whole heart and
●​ Creating atrial septal defect (ASD) for obligatory R to L or L to R body shake with the tug
shunt lesions ●​ This procedure is only good for infants with thin atrial
●​ Relieving obstruction — balloon angioplasty septal walls
→​Usually done in children under 1 month old
●​ Stenting of the ductus arteriosus and other vascular structures
●​ Occlusion or closure of congenital heart defects Blade Septostomy and Balloon Atrioseptoplasty
→​ Either patent ductus arteriosus (PDA), ventricular septal defect ●​ If child is diagnosed late and the septum has thickened, then
(VSD), or ASD we would use a blade to cut the septum intermittently in different
A. CREATING AN ASD parts
●​ Accomplished through the following steps:
BALLOON ATRIAL SEPTOSTOMY (BAS)
→​ The blade is pulled down to slice the atrial septum
→​ Once the cut is made, the blade is collapsed to prevent the
cutting of unnecessary structures
■​ Dangerous thing to do inside the heart
→​ The septum will be cut several times
→​ After which, the septum with the cuts is stretched with a
balloon
■​ Balloon atrioseptostomy is performed which creates a hole
of 7 to 8 mm in diameter

Figure 8. Angiogram of an open blade (inferior portion) cutting through the atrial
septum (left)

Figure 6. Balloon Atrial Septostomy (for Transposition of the Great Arteries)

Figure 6. Balloon Atrial Septostomy (for Transposition of the

Great Arteries)
●​ Right ventricle (RV) is connected to the aorta and the left
ventricle (LV) is connected to the pulmonary artery
→​The oxygenated blood is stuck on the left side through the
pulmonary arteries and pulmonary veins
●​ Procedure

02.24 Pediatric Cardiac Catheterization Page 2 of 11

 B. RELIEVING OBSTRUCTION
PULMONARY BALLOON VALVULOPLASTY

Figure 9. Balloon atrioseptoplasty (top), with inflation of the balloon (bottom)

Stent of the Atrial Septum
●​ Sometimes, when the septum is very thick and there are no
blades available, a stent is used

Figure 11. Pulmonary balloon valvuloplasty done across the pulmonary valve

Figure 11. Pulmonary balloon valvuloplasty done across the

pulmonary valve
●​ A catheter is positioned in the right ventricle
●​ An angiogram is done, showing a thickened pulmonary valve
with a narrow jet of contrast through the narrowing of the
pulmonary valve into the pulmonary artery
●​ The contrast flows to the dilated pulmonary artery

Figure 10. Stent of the atrial septum

Figure 10. Stent of the atrial septum

●​ Upper left image: stent with contrast injected into the left
atrium, showing that it is the left side of the heart
●​ Lower left image: stent positioned across the atrial septum
●​ Upper right image: stent being inflated
●​ Lower right image: stent is fully inflated with no restrictions to
flow

Figure 12. Inflation of a balloon for pulmonary valve valvuloplasty

02.24 Pediatric Cardiac Catheterization Page 3 of 11

Figure 12. Inflation of a balloon for pulmonary valve
valvuloplasty
●​ A wire and balloon are placed across the defect
●​ Once the balloon is across, it is inflated
●​ A waist can be seen developing across the body of the
balloon
→​Indicating the location of the narrowing (stenosis) of the
pulmonary valve
●​ When the waist disappears, it indicates that the pulmonary
valve has been stretched open (relief of stenosis)

Figure 13. Proper blood flow through the opened valve

Figure 13. Proper blood flow through the opened valve

●​ Pressure in the right ventricle is rechecked by taking another
angiogram
→​Better flow of contrast indicates an opened pulmonary
valve
→​The pulmonary valve can never be fully opened, but the
obstruction can be maximally relieved
Figure 15. Balloon inflated across the aortic valve
AORTIC BALLOON VALVULOPLASTY
Figure 15. Balloon inflated across the aortic valve
●​ Procedure done for aortic valve stenosis
→​Balloon is inflated across the aortic valve
●​ Access is through the right carotid
→​As it is easier to do in infants
●​ Similarly, the balloon is inflated until the visible waist
disappears

Figure 16. LV angiogram with a positive aortic valve stenosis

Figure 16. LV angiogram with a positive aortic valve stenosis

●​ In older patients, access is through the femoral artery
●​ Catheter is crossed through the LV, doing an LV angiogram,
and confirming presence of a stenotic aortic valve

Figure 14. Aortic balloon valvuloplasty

02.24 Pediatric Cardiac Catheterization Page 4 of 11

 Figure 17. Balloon inflation in aortic balloon valvuloplasty

Figure 17. LV angiogram with a positive aortic valve stenosis

●​ The balloon is inflated across aortic valve
→​There is a visible waist that disappears once the balloon is
full
→​Relief of stenosis
●​ Other visible catheter is from the femoral vein
→​Pacing wire that recreates ventricular tachycardia
■​ So that the balloon is not ejected
●​ Passed in through the right ventricle

Figure 20. Balloon inflation in aortic balloon valvuloplasty

Figure 20. Balloon inflation in aortic balloon valvuloplasty

●​ The balloon is inflated across the aortic valve.
→​There is a visible waist that disappears once the balloon is
full
→​Relief of stenosis
●​ Other visible catheter is from the femoral vein
→​Pacing wire that recreates ventricular tachycardia (VT)
■​ VT is intentional so that the balloon is not ejected
→​Passed in through the right ventricle
Figure 18. Post-balloon valvuloplasty aortic root angiogram

Figure 18. Post-balloon valvuloplasty aortic root angiogram

●​ Aortic root angiogram is also conducted to ensure that there
is no aortic valve insufficiency caused by the procedure

ACTIVE RECALL
1.​ Pediatric cardiac catheterization predominantly enters
through the:
A.​Femoral artery and vein
B.​External iliac vein
C.​Internal jugular vein
D.​Popliteal artery
2.​ T/F: Blade septostomy is performed when the pulmonary
valve is thickened.
3.​ In balloon valvuloplasty, what does the visible “waist” on
the inflated balloon represent?
Answer Key: 1A, 2F, 3The site of valve stenosis (narrowing)

AORTIC STENOSIS BALLOON VALVOPLASTY

Figure 21. Post-balloon valvuloplasty aortic root angiogram.

Figure 21. Post-balloon valvuloplasty aortic root angiogram.

●​ An aortic root angiogram is done to make sure that a severe
aortic valve insufficiency has not been created.
Figure 19. LV angiogram with a positive aortic stenosis.
COARCTATION OF THE AORTA BALLOON DILATATION
Figure 19. LV angiogram with a positive aortic stenosis.
●​ In older patients, we do not come in from the carotid, but we
can use the femoral artery.
●​ The catheter pathway:
→​Crossed into the left ventricle → LV angiogram → Stenosis
in the aortic valve.

Figure 22. Angiograms of the coarctation of the aorta

Figure 22. Angiograms of the coarctation of the aorta
●​ Left angiogram: shows the descending aorta showing a
narrowing or a coarctation

02.24 Pediatric Cardiac Catheterization Page 5 of 11

●​ Middle angiogram: shows the balloon being inflated ●​ Stenosis or coarctation can also occur in the pulmonary arteries
→​As it is being inflated, there is a waist, demonstrating the →​ Figures below show arteries post surgery
coarctation site →​ There was a valve pulmonary conduit and severe stenosis
→​The coarctation site is stretched by the balloon ■​ Procedure
●​ Right angiogram: marked resolution of the coarctation ○​ Stenosis had to be dilated with a balloon because stent
→​It is still there, but most of it has been relieved could not fit
→​There is still some recoil, but the adult patient only needed ○​ After predilation, stent was positioned in left pulmonary
medical management to control hypertension artery
post-procedure ○​ Stent was inflated
○​ Final angiogram shows good flow and after year, the
C. STENTING OF DUCTUS ARTERIOSUS AND OTHER stent was further dilated to accommodate the growth of
VASCULAR STRUCTURES the child
COARCTATION OF THE AORTA STENT IMPLANTATION ●​ Scope of pediatric interventional cardiac catheterization:
1.​ Creating ASD for obligatory R to L or L to R shunt lesions
2.​ Relieving obstruction – balloon angioplasty
3.​ Stenting of the ductus arteriosus and other vascular
structures
4.​ Occlusion or closure of defects

Figure 23. Pre-stenting of a severe coarctation of the aorta. Figure 26 and 27. Examples of Pulmonary Artery Stents

Figure 23. Pre-stenting of a severe coarctation of the aorta. CARDIOLOGICAL HISTORY (PEDIATRICS)
●​ If the coarctation is quite severe, (as pointed by the arrows), a ●​ Though Dr. Porstman introduced the technique of transcatheter
covered stent can be placed. closure of PDA in 1976, the procedure was complicated and
required a large cannulation access
→​ The patient closed was an adult with patent ductus arteriosus,
but it required a 24 french sheath cut down into the femoral
artery
→​ This method is not done in children because it is too big
●​ Rashkind and Cuaso developed a device for closure of patent
ductus arteriosus in the mid 70’s
→​ The device was a small umbrella that attached to the PDA by
tiny hooks at the end of the umbrella arms
→​ Transcatheter closure of patent ductus arteriosus
■​ A three-pronged hook device was used to successfully
Figure 24. Stent being positioned for severe coarctation of the aorta (left) and a
partially inflated stent (right) close the PDA in a 3.5 kg infant
→​ Dr. Cuaso is a Filipino physician who is still practicing locally
Figure 24. Stent being positioned for severe coarctation of the
aorta (left) and a partially inflated stent (right)
●​ A stent is being positioned.
→​Right image: once it crosses the coarctation, it is partially
inflated
→​Its position is again checked prior to being inflated

Figure 25. Fully-inflated stent in severe coarctation of the aorta

Figure 25. Fully-inflated stent in severe coarctation of the aorta.

●​ Once fully-inflated, it stretches Figure 28. The First Transcatheter
→​Because of the stent, there is no more or very little recoil
→​Right image: coarctation site has been dilated and there is
good flow
●​ This aortic stent implantation was done in an older child but
after about five years, the stent had to be redilated because
of the patient’s growth

D. STENT OF PULMONARY ARTERY STENOSIS

02.24 Pediatric Cardiac Catheterization Page 6 of 11

 F. PATENT DUCTUS ARTERIOSUS

Figure 29. Small Umbrella Attached to the Ductus by Tiny Hooks at the end of the
Umbrella Arms

Figure 30. Three Pronged Hook Device used to close PDA

E. PDA DEVICES Figure 32. Closure of Patent Ductus Arteriosus with ADO

●​ Developed by Amplatz
●​ Now they are called “Amplatz-like Duct Occluders” (ADO)
●​ There are three types:
→​ Type 1
→​ Type 2
→​ Type 3

Link
Figure 32. Closure of Patent Ductus Arteriosus with ADO
●​ Takes place in a special room called catheterization laboratory
→​Where minimally invasive and non-surgical procedures are
performed
●​ Duct occluder is delivered to the correct place in the heart
through a catheter
→​A small plastic tube used to access the heart and place the
occluder
●​ The physician deploys the occluder to expand within the
defect, closing off the PDA

Figure 31. ADOs Type 1 (top), Type 2 (left), Type 3 (right)

Figure 33. Angiogram of the aorta with PDA

Figure 33. Angiogram of the aorta with PDA

●​ Occluder device is placed across a large ductus arteriosus
●​ Device is positioned and opened across the ductus
●​ When it is fully opened, it will be pinched which will make the
device sit in place

02.24 Pediatric Cardiac Catheterization Page 7 of 11

 ●​ The rims of the ASD must then be present for the device to sit
properly

Amplatz Septal Occluder Placement

Figure 34. Total occlusion of the PDA

Figure 34. Total occlusion of the PDA

●​ When the physician is satisfied with the device’s position, said
device is released
●​ A repeat angiogram is then done to check for the total
occlusion of PDA within the first five minutes after the
deployment

ACTIVE RECALL
1.​ What device is used to close PDA?
2.​ T/F: In aortic balloon valvuloplasty, the balloon shows a
“waist” at the stenotic site that disappears once fully
inflated.
3.​ Who developed a device for the closure of the patent Figure 37. Demonstration of how an ASD is closed
ductus arteriosus in the 70s.
Figure 37. Demonstration of how an ASD is closed
Answer Key: 1Amplatz-like Duct Occluders, 2T, 3Rashkind and Cuaso
●​ The Amplatz Septal Occluder Placement typically occurs in a
ASD CLOSURE special room called a catheterization laboratory, where many
●​ Follows a similar principle to that of the PDA closure minimally invasive non-surgical procedures are performed.
●​ The pioneer was the Amplatz Septal Occluder ●​ The septal occluder is delivered through a catheter, which is a
small plastic tube used to access the heart and place the
occluder.
●​ The physician deploys the occluder discs on either side of the
defect, closing off the hole.

Take Note!
●​ Dr. Cheng repeatedly refers to Amplatz with that spelling, but
upon searching online, it is constantly referred to as
Amplatzer.

Figure 35. Amplatz Septal Occluder

Steps
1.​ From a small catheter, the left atrial disc is opened
2.​ Once the same disc is fully opened, the central waist is
positioned to sit inside the ASD
3.​ The right-sided disc is opened, pinning the device across the
ASD
→​ This allows the device to pinch rims and hold itself up into
Amplatz Septal Occluder Placement
place
Take Note!
●​ Follow the link above to watch the animation included in the
lecture.

ASD AMPLATZ CLOSURE

Ultrasound View

Figure 36. An ASD with a larger left atrial disc and a smaller right atrial disc

Figure 36. An ASD with a larger left atrial disc and a smaller
right atrial disc
●​ Note the pink structures which represent the tissues being
sandwiched between the discs

Figure 38. Ultrasound of atrial disc (left) and its closure (right)

02.24 Pediatric Cardiac Catheterization Page 8 of 11

 Figure 38. Ultrasound of atrial disc (left) and its closure (right) VENTRICULAR SEPTAL DEFECT (VSD) CLOSURE
●​ In viewing the video, the right atrial disc can be seen being
released, sandwiching the device across the atrial defect and
pinning the rims between the two device discs
●​ The left atrial disc opens into the left atrium

Fluoroscopic View

Figure 41. Repair of VSD device closure

Figure 41. Repair of VSD device closure

●​ Left image
→​Uses a catheter to go from the IVC crossing into the RV and
then into the LV
→​The device opens in the LV
→​The wing is pulled against the ventricular septal defect
and attaches to the other septum
●​ Right image
→​Once in place, it will open the right-sided wings of the
device, allowing the device to sit across the ventricular
septum
→​Once it is in place, release the device and leave it there
→​Then, the catheter leaves

Hybrid Muscular VSD Closure

Figure 39. Real-time fluoroscopic view of ASD closure

Figure 39. Real-time fluoroscopic view of ASD closure

●​ A: Opening of the left disc in front of the defect
●​ B & C: Opening of the right disc behind the defect
●​ D: releasing the occluder

●​ General procedure of Amplatzer Closure:

→​ Open the left sided disc and pull it against the septum
→​ Release the right-sided disc
Figure 42. Fluorescent view of deployment of disk for VSD
→​ Occlusion of the defect is then checked on ultrasound and
fluoroscopic views Figure 42. Fluorescent view of deployment of disk for VSD
→​ Once satisfactory, the occluder is released to secure the disc ●​ Note the left and right side disks being deployed
in place
●​ Uses fluoroscopy and ultrasound to guide the device closure
Fluoroscopic View
→​ Septum can be seen via ultrasound
●​ Once fully deployed and satisfied with the position, the device is
unscrewed from the cable and and the device is released
Per Ventricular VSD Closure

Figure 40. Embolized ASD device

●​ Sometimes complications may occur
→​ ASD device embolization
■​ Can occur due to it being too small or improperly
positioned
●​ Retrieval of the ASD Amplatz is seen in Figure 40
→​ Device is moved to the right atrium of the heart
→​ It moves around until it finds itself positioned along the main
pulmonary artery (MPA), from where it is snared and pulled
→​ Device is then pulled down and out of the heart through the
Figure 43. Fluorescent view of deployment of disk for VSD
IVC
→​ Device is further pulled back slowly into the sheath, and the Take Note!
catheter is pulled out
●​ Dr. Cheng constantly mentions ASD closure when discussing
●​ If it cannot be removed through this method, the patient is the per ventricular VSD closure slide. Note that VSD involves
immediately brought in for emergency surgical removal of the ventricles and the interventricular septum, which is what’s
the ASD device as well as for the closure of the ASD focused in the slide/figure.
→​ Rare but catastrophic occurrence that requires immediate
removal of the device from the heart

02.24 Pediatric Cardiac Catheterization Page 9 of 11

 ●​ This shows the left atrial disk opening the left atrium then and was inflated. The stent can be further dilated to
releasing of the right-sided atrial disk accommodate the growth of the child.
●​ The device is sandwiched across the atrial defect and ●​ PDA devices, now called Amplatz-like Duct Occluders (ADO),
maintains the rims between the two device disks come in three types: Type 1, Type 2, and Type 3. These devices
are used to close a Patent Ductus Arteriosus (PDA) through a
G. SUMMARY minimally invasive, non-surgical procedure performed in a
●​ Scope of pediatric interventional cardiac catheterization: catheterization laboratory. The occluder is delivered via a
→​ Creating ASD for obligatory R to L or L to R shunt lesions catheter into the heart, where the physician deploys it across the
→​ Relieving obstruction using balloon angioplasty ductus, allowing it to expand and seal the defect. Once
→​ Stenting of the ductus arteriosus and other vascular structures positioned, the device is released, and a follow-up angiogram
■​ Namely, the coarctation and PAS confirms successful and complete closure of the PDA
→​ Occlusion or closure of defects ●​ ASD closure uses an Amplatz Septal Occluder. This occluder
■​ Primary PDA, ASD, and VSDs is delivered through a catheter and involves opening discs on
either side of the defect. The occluder pinches the rims of the
ACTIVE RECALL defect and holds itself in place, effectively closing off the defect.
●​ ASD Amplatz Closure can be viewed through an ultrasound and
1.​ Identify the device used to occlude an ASD.
fluoroscopic view. In either, the occluder can be seen being
2.​ T/F: If percutaneous retrieval of an embolized ASD device
fails, the next step is immediate surgical removal and opened and left behind in the area where it is needed.
closure of the ASD. →​ Sometimes, it can be embolized, which is a compilation, so its
3.​ T/F: Hybrid Muscular VSD Closure uses fluoroscopy and retrieval is attempted until the device can be moved to the
ultrasound to guide the device closure. main pulmonary artery, where it can be snared and pulled
Answer Key: 1Amplatz Septal Occluder, 2T, 3T down and out of the heart. If it cannot be removed, it must be
immediately brought in for emergency surgical removal as well
SUMMARY & KEY POINTS
as for the closure of the ASD.
●​ Cardiac catheterization is an invasive procedure that uses small ●​ Ventricular septal defect (VSD) closure starts with a catheter
catheters in an artery or vein which is advanced into the entering the heart through the IVC, where it crosses to the RV
chambers and great vessels of the heart and then into the LV. Then, the device opens inside the LV, where
→​ In pediatrics, it is predominantly entered through the femoral it is pulled against the VSD and attaches to the other septum.
artery and vein and occasionally through the internal jugular Once in place, the right-sided wing of the device opens, allowing
vein it to sit in place across the ventricular septum, and it is left there.
→​ The scope of pediatric interventional cardiac catheterization
REVIEW QUESTIONS
include the creation of ASD for obligatory shunt lesions,
relieving of obstruction, stenting of ductus arteriosus and other 1. All of the following are included in the scope of pediatric
vascular structures, and the occlusion or closure of defects interventional cardiac catheterization EXCEPT:
●​ The following are the steps in doing pediatric interventional A.​Occlusion or closure of congenital heart defects
cardiac catheterization: B.​Relieving obstruction
→​ Creating ASD for obligatory R to L or L to R shunt lesions C.​Coronary artery bypass grafting (CABG)
→​ Relieving obstruction – balloon angioplasty D.​Creating ASD for obligatory R to L or L to R shunt lesions
→​ Stenting of the ductus arteriosus and other vascular structures 2. Which of the following statements best explains why a
→​ Occlusion or closure of defects pacing wire is used during aortic balloon valvuloplasty?
●​ Balloon atrial septostomy is a procedure mainly done in infants
A.​To monitor the electrical activity of the heart during the procedure
under 1 month old with thin atrial septal walls to create or
B.​To intentionally induce ventricular tachycardia and stabilize the
enlarge a hole in the atrial septum by inflating a balloon in the left
balloon
atrium and pulling it forcefully across the septum.
C.​To detect complications such as arrhythmias after the procedure
→​ Blade septostomy is performed when the atrial septum is
D.​To provide a backup for defibrillation if cardiac arrest occurs
thickened and diagnosed late; it involves making multiple cuts
with a blade before using a balloon to stretch and enlarge the 3. Which of the following is TRUE about ASD closure?
septal opening. A.​The device used is an implanted stent
→​ When the septum is very thick and blades are unavailable, a B.​Pronged hooks are necessary to close the defect
stent is placed across the atrial septum to maintain an open C.​Amplatz occluders may embolize
passage and ensure adequate blood flow. D.​A balloon is necessary to stretch the defect
●​ Balloon valvuloplasty relieves valve stenosis by inflating a 4. [True or False] In pulmonary balloon valvuloplasty, the
balloon across the narrowed valve until the waist disappears. In pulmonary valve can be fully opened to restore normal
pulmonary valvuloplasty, access is via the right ventricle; anatomy.
angiogram shows the obstruction, and post-inflation imaging 5. [True or False] The transcatheter closure of PDA requires a
confirms improved flow, though the valve cannot be fully opened. small cannulation access.
In aortic valvuloplasty, access is through the right carotid in
6. During balloon atrial septostomy, the balloon is inflated on
infants or femoral artery in older patients; an LV angiogram which side of the heart?
confirms stenosis, and a pacing wire may be used to stabilize the
A.​Right atrium
balloon. A post-procedure aortic root angiogram ensures relief
B.​Left atrium
and checks for complications like insufficiency.
C.​Right ventricle
●​ Balloon valvuloplasty and stenting are used to relieve
D.​Left ventricle
obstructive heart lesions. In aortic stenosis, a balloon catheter
introduced via the femoral artery is inflated across the narrowed 7. In VSD closure, which chamber does the device first open
valve, eliminating the “waist” and restoring flow, with pacing used in?
to stabilize the balloon. In coarctation of the aorta, balloon A.​Right ventricle
dilatation widens the narrowed site, though some recoil may B.​Left ventricle
remain. For more severe cases, stent implantation provides a C.​Right atrium
durable solution by preventing recoil and maintaining good flow, D.​Pulmonary artery
but in children, stents may require redilatation as they grow. 8.The following are within the scope of pediatric interventional
●​ The procedure for the treatment of pulmonary artery cardiac catheterization EXCEPT:
stenosis included dilating the stent with a balloon. After
A.​Creating VSD for obligatory R to L or L to R shunt lesions
predilation, the stent was positioned in the left pulmonary artery

02.24 Pediatric Cardiac Catheterization Page 10 of 11

B.​Relieving obstruction using balloon angioplasty
C.​Stenting of the ductus arteriosus and other vascular structures
D.​Occlusion or closure of defects
9.Which of the following statements best describes the closure
of a Patent Ductus Arteriosus (PDA) using an Amplatz-like
Duct Occluder (ADO)?
A.​The procedure requires open-heart surgery with direct placement
of the device.
B.​The occluder is delivered through a catheter in a catheterization
laboratory and expands to seal the ductus.
C.​The device is swallowed and absorbed into the bloodstream to
reach the ductus.
D.​The occluder is placed externally over the chest to compress the
PDA.
Answer Key
1C, 2B, 3C, 4F, 5F, 6B, 7B, 8A, 9B

RATIONALE TO REVIEW QUESTIONS

1.​ [C] — CABG is a surgical procedure, not done via interventional
catheterization in children.
2.​ [B] — During aortic balloon valvuloplasty, a pacing wire is
introduced through the right ventricle to induce rapid ventricular
pacing. This reduces cardiac output and movement, preventing
the balloon from being ejected while it is inflated across the
stenotic valve.
3.​ [C] — Only this statement applies to ASD closure. Stents are Join pawprints :D (threat)
used for coarctation and stenosis. Pronged hooks are used to
close PDA in infants. Finally, balloons are used to relieve
obstruction such as in balloon valvuloplasties.
4.​ [FALSE] — The pulmonary valve can never be fully opened after
valvuloplasty. The goal of the procedure is to relieve the
obstruction as much as possible. A complete restoration of
normal anatomy is not achievable, but significant improvement in
blood flow can be obtained.
5.​ [FALSE] — The transcatheter closure of PDA was complicated
and required a large cannulation access.
6.​ [B] — The balloon is inflated in the left atrium so it can be pulled
across the atrial septum to enlarge the opening by stretching or
tearing the septal tissue.
7.​ [B] — Via a catheter, the device enters the heart through the IVC
to the RV and then into the LV, where the device finally opens.
Then, the device is put in the VSD and left there.
8.​ [A] — Should be creating ASD, not VSD, for obligatory R to L or
L to R shunt lesions
9.​ [B] — The occluder is delivered through a catheter in a
catheterization laboratory and expands to seal the ductus.
REFERENCES
REQUIRED REFERENCES
Pediatric Cardiac Catheterization by Dr. Dexter Cheng taken from:
[Lecture]
https://ateneo.instructure.com/courses/56021/pages/of-closing-shunt
s-and-opening-blockages-2?module_item_id=2806939
IMPORTANT LINKS
Errata Tracker: [tinyurl.com/YL7ModuleErrataTrackerDrive]
V2 Errata Sheet: [tinyurl.com/YL7V2ErrataSheet]
Evaluations: [tinyurl.com/YL7TransEvals]
YL7 Trans Map: [tinyurl.com/2028YL7TransMap]
FREEDOM SPACE

TG9 AND FRIENDS: SUPER CLUTCH *JETT KNIVES* FOR THE

ACE !!
https://youtu.be/v1oG6j_beL8

02.24 Pediatric Cardiac Catheterization Page 11 of 11

 Module 02: Cardiovascular

Surgical Management of Congenital

Heart Disease
Carlo Martin Garcia, MD, FPATACSI | 08/21/2025 | Asynchronous | Surgery

●​ Infant Xenotransplant: The Case of Baby Fae (1984, Loma

TABLE OF CONTENTS
Linda, CA, USA)
Learning Objectives 1 VI.​ The Heart-Lung 6 →​ A historic and well-publicized procedure forty years ago
I.​ Introduction 1 Machine
■​ First heart transplant in a child involved a baboon heart
II.​ Classification of 1 VII.​ Common 7
Congenital Heart Complex Lesions →​ Baby Fae was a case of hypoplastic left heart syndrome (end
Disease A.​Tetralogy of 7 stage when she was born)
A.​Traditional 1 Fallot (ToF) →​ At that time, there was not any kind of repair available yet for
Classification B.​Transposition of 8 such babies and the only resort was transplantation
B.​Operative 1 the Great →​ However, the baby did not survive beyond one to two months
Classification and Arteries (TGA)
due to acute rejection
Analysis VIII.​ Definitive vs 9
III.​ Shunt Lesions 2 Palliative II. CLASSIFICATION OF CONGENITAL HEART DISEASE
A.​Atrial Septal 2 Treatments
Defect (ASD) IX.​ Summary 10 A. TRADITIONAL CLASSIFICATION
B.​Ventricular Septal 2 A.​Shunt Lesions 10 ●​ Gives us an idea of the pathophysiology of a lesion or condition
Defect (VSD) B.​Obstructive 10 →​ For example, if you have a ventricular septal defect, you put it
C.​Patent Ductus 3 Lesions
as “non- cyanotic heart disease, ventricular septal defect”
Arteriosus C.​Tetralogy of 10
D.​Indications and 3 Fallot or “cyanotic heart disease, tetralogy of fallot”
Timing for Closure D.​Transposition of 10 ●​ Cyanotic
of Shunt Lesions the Great →​ R to L shunting of deoxygenated blood
IV.​ Obstructive 4 Arteries ■​ Deoxygenated blood in systemic circulation
Lesions Summary/Key Points 10 ●​ Non-cyanotic
A.​Aortic Coarctation 4 Review Questions 10 →​ Heart failure = pulmonary congestion
B.​Pulmonary Valve 5 Rationale 11
Stenosis References 11 Nice!
V.​ Contraindications 6
for Surgery of ●​ If you are in the clinic, you look at your charts and you see on
Congenital Heart the diagnostic sheets the diagnosis of your patients. You’ll see
Disease if you have a congenital heart case and whether it is cyanotic
A.​Eisenmenger 6 or non-cyanotic
Syndrome
B. OPERATIVE CLASSIFICATION AND ANALYSIS
LEARNING OBJECTIVES ●​ More practical classification which leads surgeons to analyze
1.​ To enumerate surgical interventions for the common congenital these lesions
heart lesions encountered in a pediatric cardiac service. ●​ Shunt Lesions
2.​ To relate principles of surgical management to pathophysiology →​ Determine the level of the shunt
of congenital heart disease. ■​ Shunt: hole or communication between the systemic (left
side) and the pulmonary (right side) circulation
I. INTRODUCTION
→​ Atrial vs Ventricular vs Post- aortic valve
●​ In the surgical management of congenital heart disease, heart ■​ Can either be atrial septal defect (ASD), ventricular septal
transplantation can be called the final frontier defect (VSD), or patent ductus arteriosus, as commonly
→​ Unfortunately we do not have that in the Philippines, but in seen in neonates
more resource-rich countries, they have been conducting it for ○​ Patent Ductus Arteriosus: post-aortic defect or shunt
two to three decades even in children ■​ Gives an idea on the severity and natural history of the
●​ The recent cutting edge research on heart transplantation is disease
somehow reminiscent of how heart transplantation in children ●​ Obstructive Lesions
started →​ Are the lesions obstructing the left or right ventricle?

Figure 1. Breakthrough in Heart Xenotransplantation

●​ Currently, scientists are trying to transplant pig hearts into
baboons
●​ The problem with heart transplantation is the lack of donors Figure 2. Algorithm for operative classification and analysis of congenital heart
diseases
→​ Cutting edge research right now is focused on minimizing or
shortening that transplant list by expanding the donor pool.
■​ If that donor pool includes non humans, then so be it

TG10: Alba, Agatep, Atienza, Cobar, Dela Merced, Fernandez, Lumagbas, Luz, Noble, VIllarama, Wong
02.25 CG1: Antonio, Busog, De Asis, Garcia, Gomez, Mahusay, Meily, Namoro, Romo, Sing Bengco, Tuazon, Valmores V2 Page 1 of 11
Figure 2. Algorithm for operative classification and analysis of
congenital heart diseases
●​ In an operative classification and analysis, we have to
determine the interactions between the shunt lesion and the
obstructive lesion
→​It tells you whether you have reduced or increased
pulmonary blood flow or reduced systemic blood flow
→​Recall: pathophysiology and fluid dynamics
■​ Shunt: connect one chamber or circulatory system to
another
■​ Obstructions: block outflow from one system to the
other
→​Interactions between systems provide a clearer
understanding of the disease pathophysiology of the Figure 4. 2D echo findings in a patient with ASD; red box shows the
disease communication between the right and left atria
■​ Can either lead to heart failure or circulatory collapse
●​ Through analyzing the shunts or obstructions, we can ●​ 2D echo findings:
determine whether the patient is in →​Shows the four chambers of the heart
→​Cyanosis: cardiac output coming from the heart is not ■​ The probe is placed above the anterior chest wall
oxygenated (“blue blood” or “blue babies”) ■​ The right ventricle is found under the breastbone
→​Heart Failure: there is reduced cardiac output, a more ○​ The right atrium drains into the right ventricle
chronic event ■​ The left chambers of the heart are located posteriorly
■​ Clinical Manifestation: congested lungs →​ASD is big at roughly 2 cm
○​ Due to the damming of the blood back to the lungs →​Right-sided chambers are noticeably enlarged
→​Circulatory Collapse: acute event wherein there is zero ■​ In a normal heart, the LV is usually larger than the RB
cardiac output; a true surgical emergency where you have ■​ In Figure 4, the right ventricular cavity and contour is
to alleviate the lesion’s effects on the heart larger than the left ventricular cavity and contour
■​ Recall: Right-sided enlargement occurs due to the shunt
III. SHUNT LESIONS connecting the LA to RA
A. ATRIAL SEPTAL DEFECT (ASD) ●​ ASD: refers to a communication between the right and left atria
→​ Shunt occurs from the LA to the RA
Case Case 1 ●​ Pressure gradient
●​ 18 year old girl with palpitations, occasional shortness of →​ The LA has a higher pressure than the right atrium, creating a
breath pressure gradient (LA>RA)
●​ Physical examination findings: ■​ Normal left atrial pressure: 10-15 mmHg
→​Normal Vital signs, regular rhythm
■​ Normal right atrial pressure (also called central venous
→​Clear breath sounds, no signs of pulmonary congestion
→​Systolic murmur on the left upper parasternal area
pressure or CVP): 5-8 mmHg
■​ Corresponds to the location of the pulmonary valve →​ Blood usually flows from the LA to the RA due to higher
pressure in the LA
●​ Shunting of oxygenated blood:
→​ Oxygenated blood moves from the LA to the RA and then into
the RV
■​ The increased blood volume on the right side causes
right-sided heart enlargement
→​ Blood from the RV also flows into the lungs, flooding them with
already oxygenated blood
B. VENTRICULAR SEPTAL DEFECTS

Case VSD
●​ 5-year-old boy, short stature, poor weight gain, obvious
shortness of breath on exertion
●​ Physical examination findings:
→​Normal vital signs, regular heart rhythm
→​Clear breath sounds
■​ No signs of pulmonary congestion
Figure 3. Cardiac X-ray findings in a patient with ASD; pink circle points to the →​Systolic murmur heart over the left upper parasternal area
apex, yellow arrow points to a bump on the pulmonary artery and blue arrow
points to the right side
●​ Chest X-ray findings:
→​No left ventricular enlargement
■​ Apex (pink circle): represents the left ventricle
■​ If there is LV enlargement, the apex should be displaced
to the left side of the chest
→​No cardiac waist
■​ A normal heart shows a depression in the area of the
pulmonary artery and aorta, known as the cardiac waist
■​ In Figure 2, the yellow arrow highlights the bump on the
pulmonary artery, indicating an abnormality
→​Normal right atrium and ventricle (blue arrow)
■​ Almost normal contour
Figure 5. Cardiac X-ray findings in a patient with VSD; red arrow points to the
■​ If bulging is present, it indicates right ventricular
pulmonary artery knob and yellow arrow points to the displaced apex
enlargement
●​ Chest X-ray findings:
→​Apex is displaced towards the left (yellow arrow)
→​The heart is grossly enlarged
■​ Comparing the heart size to the rest of the thoracic
cavity, you can see that it is more than 50% in width
→​Suggests left ventricular enlargement

02.25 Surgical Management of Congenital Heart Disease Page 2 of 11

 →​Pulmonary artery is exaggerated (red arrow)
■​ Cardiac waist appears smaller because knob has
enlarged
→​Right side is not much bigger than normal

Figure 6. 2D echo findings in a patient with VSD; yellow box shows the
communication between the right and left ventricles
●​ 2D echo findings: Figure 8. Comparison between PSA, VSD, and ASD in terms of heart
→​Left ventricle is dilated failure and pulmonary congestion
→​Because of the shunt, instead of the oxygenated blood
Figure 8. Atrial vs ventricular vs post-aortic valve defects
going straight into the aorta and systemic circulation, some
of it goes back to the right side ●​ There is more heart failure in VSD due to the left side
■​ Finds its way back to the lungs and back in to the left having high pressure (around 120 mmHg)
side ●​ 120 mmHg of systemic pressure is pumped into the VSD
■​ Goes in circles and into the lungs, causing damage to the lungs
→​Ultimately results in volume-loading the left side →​Imagine a high pressure pumping chamber pushing blood
causing the heart to get strained into the right side
■​ This is ultimately what causes heart failure →​That pressure being is being transmitted into the lungs
○​ For every amount of blood that goes into the heart, a →​The lungs become congested earlier, and they become
lot of it circulates within the heart and lungs hypertensive earlier
○​ Just a fraction of it goes out into the systemic →​Causes pressure and volume overload
circulation →​Degree of congestion would be dependent on how big the
○​ The heart ends up working double-time defects are
■​ In a 1 cm defect in VSD vs 1 cm defect in ASD, you are
COMPARISON BETWEEN VSD AND OTHER SHUNT LESIONS more likely to have worse heart failure in VSD than ASD
●​ In ASD, there is almost passive flow
→​Pressure is around 15-20 mmHg
→​Pumps while the blood passively flows to the right side
→​This only causes volume overload
●​ Important to remember: [!]
→​ASD presents with less heart failure than VSD
→​VSD presents with earlier heart failure and pulmonary
congestion than ASD

C. PATENT DUCTUS ARTERIOSUS (PDA)

Case Patent Ductus Arteriosus

●​ 2-month-old boy, failure to thrive, difficulty in milkfeeding
→​Usual presentation in neonates with heart failure due to
too much effort being required to feed
●​ Physical examination findings:
Figure 7. Atrial vs ventricular vs post-aortic valve defects →​Normal vital signs, regular heart rhythm
■​ Patients may present with tachycardia
Figure 7. Atrial vs ventricular vs post-aortic valve defects →​ Crackles in bilateral lungs
→​Continuous systolic-diastolic murmur [!]
●​ Comparing ASD vs VSD, the degree of pulmonary congestion
■​ Machinery-type of murmur
is different
●​ The lecturer presented the following questions: ●​ Remnant of the neonatal circulation where there’s a
→​When does pulmonary congestion present earlier? ASD or communication between the aorta and pulmonary arteries
VSD? →​ This is usually large
→​Where is there more pulmonary congestion? ASD or VSD?
●​ During the antenatal period (baby is still fetus inside the uterus),
→​Where is the severity of heart failure increased? ASD or
VSD? patent ductus arteriosus is almost as big as the aorta
→​ PDA progressively decreases in size towards term and
eventually closes in a normal neonate
●​ Recall in VSD:
→​ During systole, high pressure blood is transmitted to the
right side lungs
→​ During diastole, the aortic valve is closed → LV relaxes →
pressure in LV decreases
●​ If there is a PDA after the aortic valve:
→​ LV contracts → high systolic pressure blood (120 mmHg)
goes to the pulmonary arteries via the PDA
→​ LV relaxes → diastolic pressure (80 mmHg) goes into
pulmonary circulation via the PDA
→​ Thus, you hear the continuous systolic-diastolic
(machinery-like) murmur
●​ During both systole and diastole, there is a big shunt that wreaks
havoc on the pulmonary arteries

02.25 Surgical Management of Congenital Heart Disease Page 3 of 11

 →​ Pulmonary arteries have 80-120 mmHg of pressure in the →​ Whatever the age, as long as they are symptomatic,
entire cardiac cycle → pulmonary arteries cannot handle this operate
much pressure ●​ The earlier the operation for symptomatic babies, the better the
●​ Patients with PDA present with heart failure and pulmonary outcome
congestion during the first few months of life ●​ Get a prosthetic patch material (usually Gore-Tex or
pericardium) and sew it on to the hole
D. INDICATIONS AND TIMING FOR CLOSURE OF SHUNT
LESIONS →​ Under a full cardiopulmonary bypass with an open heart

●​ Timing of surgery is dictated by pathophysiology of the lesion

●​ All mentioned surgeries for shunt lesions will effectively occlude
the shunts
→​ Over the next few weeks to months → dramatic improvement
improvement in heart failure symptoms
→​ Signs that heart failure has resolved:
■​ Size of the ventricles or the chambers will gradually
decrease
■​ Symptoms will gradually decline
Table 1. Indications and timing for simple shunt lesions
Figure 10. VSD repair using a prosthetic patch
Shunt Lesion Indication Timing
PATENT DUCTUS ARTERIOSUS
Arterial Septal Defect Shunt ratio >2:1 School age
●​ Require immediate closure in newborn or infancy
Ventricular Septal
Shunt ratio >2:1 Infancy to preschool age
→​ Don’t want lungs to be permanently damaged
Defect ●​ Has worst effect on lungs and heart failure out of the three
Patent Ductus simple shunts
Immediate closure in newborn or in infancy
Arteriosus →​ PDAs are post-aortic valve shunt lesions
●​ Closed by a cardiologist using endovascular devices
ATRIAL SEPTAL DEFECTS
→​ They string wires through the baby’s groin or carotid artery
●​ Most benign kind of simple shunt to plug the device to the PDA
●​ Usually presents as benign murmur
→​ Patient is usually asymptomatic: benign
→​ Incidentally found on PE
●​ Usually found not as early as other defects
→​ Found during school age
→​ Not necessary to close shunt if:
■​ ASD and VSD are very small
■​ Shunt fraction <2:1
●​ Based on imaging and echo if shunt fraction >2:1
→​ Close the ASD because lung damage can develop over time
●​ ASDs are usually closed during school age when they present
Figure 11. Device closure of PDA (left) and surgical closure for PDA
(right)
●​ If babies are too small for the device or there are no access
points, they are referred to surgeons for open repair
→​ Approached through the left chest, then locate the PDA
between the aorta and pulmonary arteries
→​ Marker: recurrent laryngeal nerve wrapped around the
PDA
■​ Vagus nerve arches around the ductus arteriosus as the
recurrent laryngeal nerve
■​ Need to preserve these structures during surgery
→​ Closed by ligation (tying it off) or splitting it in half or
across
Figure 9. ASD repair using a pericardial patch
IV. OBSTRUCTIVE LESIONS
●​ Easiest to close
→​ Done under cardiopulmonary bypass ●​ Refer to Figure 1 for the classifications of CHDs
●​ The heart is arrested and a pericardial patch is sewed on ●​ Depending on the location, obstructive lesions can:
→​ Pericardial patch is used because it is easily accessible →​ Either reduce or increase the pulmonary blood flow
■​ The patient’s own pericardium can be used and sewed →​ Reduce systemic blood flow
onto defect ●​ They can cause either cyanosis, heart failure or circulatory
→​ Dramatic improvement in heart failure symptoms over the next collapse
few weeks to months ●​ The most common obstructive lesions encountered in pediatric
●​ Signs of improvements and resolution of heart failure: service:
→​ Size of ventricles and chambers gradually decrease →​ Coarctation of the aorta
→​ Symptoms decline →​ Pulmonary valve stenosis

VENTRICULAR SEPTAL DEFECTS A. AORTIC COARCTATION

●​ Patients come in earlier since a greater fraction of patients ●​ Most common obstructive lesion
become symptomatic at a younger age ●​ Obstruction just after the aortic arch
→​ Closed during infancy and preschool age if shunt ratio >2:1 ●​ Causes:
●​ Once you have an indication surgery in VSD: →​ Heart failure
→​ Open heart surgery ■​ Left ventricle cannot pump blood efficiently due to the tight
→​ Put the patient on a heart-lung machine obstruction
■​ The LV becomes dysfunctional overtime

02.25 Surgical Management of Congenital Heart Disease Page 4 of 11

 ■​ Most common presentations of heart failure in children: ●​ In very severe cases, an arch repair (purple) must be done, or a
○​ Failure to thrive prosthetic patch (pink) must be placed
○​ Pulmonary congestion: blood goes back into the →​ A lot of times, these are done under cardiopulmonary bypass
pulmonary circuit when the LV can’t eject it ■​ Unlike the simple repair
→​ Distal malperfusion ●​ After repair, usually, the effect on the patient is dramatic
■​ If the obstruction is really tight →​ Heart strain is relieved because blood freely flows down the
■​ Not enough blood from the aortic arch down to the aorta
extremities →​ Upper extremity hypertension is relieved
○​ Leads to a difference in the blood pressure of the upper →​ Better perfusion of the organs in the lower part of the body
and lower extremities
B. PULMONARY VALVE STENOSIS
●​ The most common obstructive heart lesion
→​ Obstruction of the right side or pulmonary circulation
→​ Pulmonary valve is narrow
●​ RV tries to pump blood into the pulmonary circuit but it is
obstructed by a narrow PV → blood dams back to the RA
→​ Unoxygenated blood goes through the foramen ovale (an
ASD) and makes its way into the left side
→​ This unoxygenated blood is pumped back to systemic
circulation
■​ Cyanosis
Figure 12. Coarctation of the aorta
●​ Causes the following:
Figure 12. Coarctation of the aorta →​ Cyanosis
■​ Usually caused by an obstruction plus a shunt lesion
●​ Left: Aortic arch (blue), aortic valve (red), location of LV
that reverses its flow due to the obstruction
(green)
●​ Middle: Innominate artery (yellow), left carotid (purple), left ■​ Happens when right-sided unoxygenated blood finds its
subclavian (black) way into the left chambers and gets ejected into the
●​ Coarctation usually happens at the isthmus (pink) systemic circuit (unoxygenated blood coming out of the
→​Called the isthmus of the aorta aorta)
→​Where the ductus arteriosus joins the aorta ■​ If not relieved and baby survives
→​Narrowing of the area is caused by the tissue of the
○​ Results in a blue baby and with other cyanotic
ductus retracting when it closes during the newborn
period complications
●​ Right: L & R Pulmonary arteries, aortic arch, coarctation (or ○​ Does not result in sudden death
narrowing) of the aorta, left ventricle →​ Right ventricular failure
■​ Worst complication that could happen
COARCTATION REPAIR ■​ RV is not as strong as the LV
○​ Progresses into dysfunction earlier than the LV if there is
an obstruction
■​ Due to the problems the RV has with pumping blood due to
the small opening
■​ Long-time cyanosis → eventually RV failure (not as strong
as the LV) → progresses into dysfunction earlier than the
LV) because it cannot push the blood through the very small
opening

Case Case 4
●​ A 2-week-old baby, cyanosis on crying
→​Lips and fingers turn blue when he cries
●​ PE findings:
→​Normal vital signs
→​Regular rhythm
→​Clear lung sounds
→​Systolic murmur heard at the pulmonary valve area

Figure 13. Coarctation Repair

●​ Heart lung machine is not used for simple coarctations like this
●​ Procedure
→​ Left chest of the baby is opened Figure 14. Chest X-ray of a 2-week-old baby with Pulmonary Valve Stenosis
→​ Narrowed segment of the aorta is chopped off (blue arrow)
●​ X-ray findings:
→​ Everything is mobilized and repaired (black arrow)
→​Enlarged right side
■​ A simple repair →​LV is not as displaced or enlarged
■​ The most common repair →​Cardiac waist is exaggerated
■​ Done through the left chest without cardiopulmonary ■​ Pulmonary artery bump is not seen
bypass ○​ Due to PV stenosis upstream → the pulmonary artery
fails to develop normally → loss of the pulmonary
artery bump due to maldevelopment

02.25 Surgical Management of Congenital Heart Disease Page 5 of 11

 A. EISENMENGER SYNDROME

Figure 15. Echocardiogram showing right-sided obstruction

●​ Image A: Poor opening of valve due to pulmonary stenosis
→​RV: Right Ventricle
→​MPA: Main Pulmonary Artery
●​ Image B: Angiogram where contrast contours show
intraluminal or intrachamber image of PV stenosis where the Figure 17. Eisenmenger syndrome
RV is dilated
●​ Image C: Angiogram showing wasting or narrowing at the RV ●​ By having a big VSD, over time the systemic pressure is
outflow tract (blue arrow); right-sided obstruction transmitted to the pulmonary circuit and to the blood vessels of
→​Patients often present with cyanosis the lungs
■​ Cyanosis can only happen when there is a shunt lesion ●​ Over time, pulmonary arterioles become hypertrophied and
that accompanies stenosis fibrosed → eventually leads to pulmonary hypertension
→​There is always a shunt lesion
→​ Becomes irreversible after it sets in
■​ Babies are always born with at least an ASD that
●​ Patching the VSD in a patient with pulmonary hypertension → RV
eventually closes
○​ E.g., a patent foramen ovale that later closes will not be able to pump blood into the lungs and into pulmonary
circulation due to the RV being a weak chamber
→​ RV can only pump ~30 mmhg, which is not enough to
overcome the resistance of the pulmonary circuit
→​ Patching the VSD → circulatory collapse
●​ Thus, never try to operate on a patient with Eisenmenger
syndrome, and do not attempt to close the shunt
●​ Do not aim for total correction of anatomy in patients with
Eisenmenger syndrome since it will lead to either table death or
death in the ICU after repair
→​ Other palliative procedures can be done, but NEVER total
correction of anatomy
VI. THE HEART-LUNG MACHINE
●​ Modern open heart repairs/intracardiac repairs
→​ How is it done now?
Figure 16. Open heart surgery for pulmonary valve stenosis
■​ Patients are hooked up to the heart-lung machine
●​ Open heart surgery ■​ Venous blood from the RA or the vena cava are sucked into
→​Open the RV→ look for the PV→ open the PV with knife or
the heart-lung machine pump
scissors → attempt reconstruction to make leaflets normal
■​ Oxygenator in the machine infuses oxygen into the
→ augment the narrowing with the use of a patch
→​Under cardiopulmonary bypass deoxygenated blood
■​ Pump delivers oxygenated blood back into the aorta
V. CONTRAINDICATIONS FOR SURGERY OF CONGENITAL ■​ Effectively bypasses both the heart and lungs, which can be
HEART DISEASES (CHD)
arrested
●​ When do you not repair? ○​ Allows for repair of any anatomic problems inside the
→​ In CHDs, there is always a timeframe when you can and when heart
you can or should repair
■​ Beyond this timeframe, the damage to either the lungs or
heart can be so severe that even repairing the anatomy will
not result in recovery
→​ Shunt lesions
■​ Left-sided blood finds its way to the right-side → either
causes volume loading or pressure loading of pulmonary
circuit
■​ Pulmonary arteries are not designed to take on high
pressures Figure 18. Living donor cross-circulation

○​ Pulmonary BP is only around 20-25/10-15 mmHg, Figure 18. Living donor cross-circulation
usually less than 1⁄3 of normal systemic BP
●​ First open-heart surgery was done in the 1950s, on children
○​ Normal systemic BP is 120/80 mmHg
→​Used a living heart-lung machine (left image)
○​ Pulmonary arterial pressure is only around 20-25/10-15 ■​ A relative (mom or dad) of the child who share the same
mmHg blood type to prevent rejection
■​ As such, when the pulmonary arteries are subjected to ●​ Table mortality is around 50%
volume or, worse, systemic pressure overload → severe ●​ Arterial blood from the donor is sucked and pumped into the
irreversible pulmonary hypertension happens arterial blood circulation of the patient
●​ Venous blood from the patient is sucked and pumped into the
●​ Other contraindications: neglected ASD, VSD, PDA
venous circulation of the donor
●​ The donor’s lungs will oxygenate the blood and can be
pumped into the patient again
●​ Before only non-intracardiac repairs were done:
→​Ligation of Persistent Ductus Arteriosus
→​Coarctation repair without a heart-lung machine

02.25 Surgical Management of Congenital Heart Disease Page 6 of 11

 Figure 21. Common complex lesions
●​ Doc mentioned to always remember this diagram as it will
help us in analyzing congenital lesions in the wards [!]
●​ Three main presentations of heart lesions:
→​Cyanosis
■​ Chronic
→​Heart failure
■​ Chronic
→​Circulatory collapse
■​ Most dangerous
■​ Acute

A. TETRALOGY OF FALLOT

Case Case 5

Figure 19. Heart lung machine ●​ 8 month baby boy with cyanosis that worsens on crying
●​ PE findings:
Figure 19. Heart lung machine →​HR: 130
→​RR: 30
●​ Invented in the 1960’s
→​BP: 90/60
●​ Table mortality is around 20-40% or 1/3rd of the previous
→​Clear breath sounds
statistic
→​Systolic murmur
●​ Parts of a heart lung machine:
→​Slight clubbing on fingers and toes
→​Pump
●​ Salient features:
→​Membrane oxygenator
→​8 months is almost at the end of infancy
●​ Venous blood from the patient is sucked out and pumped into
→​Normal vital signs for age
the oxygenator
→​Systolic murmur
●​ Oxygenated blood is pumped back into the patient
→​Clubbing
■​ Result of chronic cyanosis

Figure 22. X-ray of case 5

Figure 20. Modern heart lung machine
●​ CXR findings:
→​Left ventricle is uplifted (pointed arrow)
Figure 20. Modern heart lung machine ■​ Indication of right ventricle enlargement
●​ Equipped with monitoring systems →​Exaggerated cardiac waist
●​ More compact oxygenator ■​ Indicative of pulmonary artery dysplasia
●​ Multiple pump heads ○​ Underdeveloped pulmonary artery
→​Allows for parallel circuits up to 4 at once →​Boot-shaped heart or “Coeur en sabot”
■​ Pathognomonic for Tetralogy of Fallot
■​ Sabot means clog in english
ACTIVE RECALL
1.​ Which of the following is NOT a shunt lesion?
A.​Atrial Septal Defect
B.​Ventricular Septal Defect
C.​Patent Ductus Arteriosus
D.​Pulmonary Valve Stenosis
2.​ T/F: Living donor cross-circulation uses the donor as the
heart-lung machine
3.​ What are the two traditional classifications of congenital
heart disease?
Answer Key: 1D, 2T, 3 Cyanotic and Non- cyanotic

VII. COMMON COMPLEX LESIONS

Figure 23. Tetralogy of Fallot

●​ First described by Frenchman Louis Arthur Etienne Fallot
●​ Four anatomic defects:
→​ (A big) VSD
■​ Main defect
→​ Pulmonary stenosis
■​ Caused by the VSD in the right ventricular outflow tract
→​ Right ventricle hypertrophy
■​ RV has trouble pumping blood due to pulmonary stenosis
→​ Overriding aorta
Figure 21. Common complex lesions ■​ A consequence of the VSD

02.25 Surgical Management of Congenital Heart Disease Page 7 of 11

●​ The first two defects (in bold) are intrinsic, while the last two MODIFIED BLALOCK-TAUSSIG SHUNT
just follow
PATHOPHYSIOLOGY OF TOF

Figure 26. Modified Blalock-Taussig Shunt

Figure 26. Modified Blalock-Taussig Shunt

●​ A bypass graft is placed on the subclavian artery or right
about the innominate artery and is connected to the
Figure 24. Tetralogy of Fallot pathophysiology pulmonary circuit
→​From high pressure systemic circulation to the low pressure
Figure 24. Tetralogy of Fallot pathophysiology pulmonary circulation
→​Relieves cyanosis by taking blood from the systemic
●​ Shunting lesion: VSD
●​ Blockage of pulmonary outflow tract
circulation and increasing pulmonary blood flow
●​ Blood in the right ventricle tries to go through the pulmonary ●​ Remember that surgery for Tetralogy of Fallot is not just to
valve but is shunted to the aorta correct cyanosis
●​ Unoxygenated blood in the artery causes cyanosis in the →​More importantly, it is to save the RV from failure and
baby myocardial dysfunction
●​ This is usually a temporizing procedure
●​ Results in severe cyanosis depending on the size of the →​After a few months, total correction will be done
pulmonary valve outflow
●​ “Modified” because of the graft
●​ Effects on the heart:
→​ Original shunt was made of native artery
→​ PV stenosis strains the RV causing it to fail over time
●​ The main objective of the shunt: relieve cyanosis by increasing
■​ RV cannot pump across the narrowed PV for so long
pulmonary blood flow
→​ Effects of cyanosis
→​ Reduced flow to the pulmonary arteries due to obstruction Uses
■​ The main pulmonary artery fails to develop compared to the ●​ Temporizing (usually)
aorta →​ Relieves cyanosis in a very sick child
○​ The main pulmonary artery is tiny compared to the aorta ■​ Allows the heart and other parts of the body to recover
○​ Normal = 1:1 in size ■​ Operate a few months later to do total correction
TOTAL CORRECTIVE SURGERY ●​ Palliative (sometimes)
→​ Relieves cyanosis when RV has already failed and dilated
→​ Will be the last operation the child will have
Indications
●​ Emergency or severe hypercyanotic “Tet” spells
●​ No physiologic reserve for total corrective surgery →
temporize
→​ Happens in very tiny infants (e.g., born 3 pounds) who do not
have enough pulmonary blood flow to survive corrective
surgery
Figure 25. Tetralogy of Fallot surgical management →​ Total corrective surgery may be done once the infant grows
●​ Total correction bigger and stronger
→​ Patch the VSD to relieve the outflow tract obstruction ●​ Very small pulmonary arteries → temporize
●​ The following factors complicate ToF patients: →​ Because there is PV stenosis, there is obstruction of blood
→​ Timing into the pulmonary circuit, reduced blood flow causes
→​ Extent underdeveloped arteries
→​ Other palliative measures →​ Total corrective surgery may cause the RV to fail some more
●​ Done as early as possible in infancy to avoid RV failure because the pulmonary arteries are still narrow
●​ When the child’s health is unfavorable, temporary shunts must be ■​ Thus we try to increase pulmonary blood flow in the hopes
created to assist the child’s growth prior to surgery of making the pulmonary arteries develop
→​ Examples of unfavorable conditions that make it difficult for an →​ Observe the pulmonary arteries for a few months and see if
infant to survive a total repair: they do enlarge
■​ Too sick ■​ When they enlarge, a total corrective surgery is done
■​ Pulmonary arteries are too small ●​ Myocardial dysfunction → palliate
■​ Ventricular squeeze is too weak →​ Seen in RV failure or cardiomyopathy where both RV and LV
→​ Example of temporary shunt: Modified Blalock-Taussig have quit because of any other complication like the stress of
Shunt heart failure
■​ Weak ventricular squeeze and won’t survive total repair
→​ For relief of cyanosis only → easier passing away
→​ This will probably be the patient’s last operation

B. TRANSPOSITION OF GREAT ARTERIES

●​ Most common cyanotic heart lesion presenting in newborns
or neonates
●​ Parallel pulmonary and systemic circulation

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 →​ Recall: Normal circulation is in series
●​ Occurs when:
→​ RV blood goes into the aorta instead of pulmonary artery
→​ LV blood goes into the pulmonary arteries instead of the aorta

Figure 29. Heart of a Neonate

Figure 29. Heart of a Neonate

●​ Blue circle: Aorta
●​ Very small heart, as big as a walnut or a small atis
Figure 27. Parallel Circulation in TGA
VIII. DEFINITIVE VS. PALLIATIVE TREATMENTS
Figure 27. Parallel Circulation in TGA
●​ Palliative treatment
●​ RV venous blood is pumped back into the aorta → cyanosis
→​ Means only palliating a symptom of the defect, not doing a
●​ LV receives oxygenated blood from the lungs → LV pumps it
total correction
back into the lungs
→​ Done for the following reasons:
●​ Patients usually survive if they have an interatrial septum ■​ The patient is just too sick
where mixing of oxygenated and deoxygenated blood happen ■​ The heart and the lungs are just too far gone in terms of
●​ Babies with this disease are usually born with ~80% O2 sat their physiologic status
●​ There is a need for early correction of parallel circulation but →​ Eisenmengerization
not just because of the cyanosis ■​ When there is irreversible pulmonary hypertension
→​ RV is not capable of supporting systemic circulation Table 2. Definitive vs. Palliative Treatments
■​ RV was designed to pump blood to the pulmonary
DEFINITIVE PALLIATIVE
circulation which is only about 1⁄3 of the pressure of the
systemic circulation ASD Closure –
→​ LV weakens over time VSD Closure PA banding (only done in Eisenmengerization)
■​ Despite the LV being very strong, pumping blood into the
PDA Closure PA banding (only done in Eisenmengerization)
pulmonary circuit that is low pressure
■​ LV is not used to pumping systemic pressures anymore Tetralogy Total Repair BT shunting
■​ Possibility of total correction has been missed TGA Arterial Switch Atrial Switch
○​ The timing of total correction is very
●​ Pulmonary artery banding (PA Banding)
essential/important
→​ It is a physiologic intervention that causes a surgical
ARTERIAL SWITCH OPERATION pulmonary stenosis to prevent or reduce blood flow from
●​ This operation is done routinely when presented with TGA the VSD into the lungs
→​ Complex but satisfying procedure ■​ Like a physiologically partial VSD closure, as you reduce
→​ The surgeon switches out the arteries in the wrong position the pulmonary blood flow from the VSD
including the coronary arteries →​ Watch if the patient is able to recover partially
■​ Consider doing definitive repair if: the patient’s lungs
recover and lung pressure goes down because of the
intervention
→​ A lot of times done for very sick and small infants who may
not be able to recover from total repair via open-heart surgery
(i.e., preterm infants)
■​ A quick PA banding is done first to reduce pulmonary
blood flow and control heart failure
■​ The surgeon then waits for the patient to grow a bit until the
patient is ready for a total correction of a VSD
→​ Remove the surgical stenosis once a full correction of VSD
Figure 28. Arterial Switch Operation
is done by patching, resecting, or excising the band
●​ Aim for total repair within 1 month of life ]

→​ Total correction in such a critically ill neonate

→​ Outcomes for the arterial switch operation are best when done
within the first month of life
●​ When switched only after the first month, LV will show signs
of deconditioning
→​ LV is used to pumping into the lower pressure of the
pulmonary circulation
→​ LV struggles because it is suddenly pumping against the
systemic pressure (around 90 mmHg systolic)
●​ Patient can live as long as a normal person

Figure 30. Pulmonary Artery Banding

02.25 Surgical Management of Congenital Heart Disease Page 9 of 11

 IX. SUMMARY ■​ Most common obstructive heart lesion, causing right-sided
obstruction
●​ Main takeaway: early detection and surgical intervention is
■​ Due to the narrowed pulmonary valve forcing the blood
key [!]
back into the RA, there is a right-to-left shunting through the
→​ These would result in the best outcomes
foramen ovale and unoxygenated blood going out of the
A. SHUNT LESIONS systemic circulation
●​ Congenital heart diseases are mostly shunt lesions [!] ●​ The heart-lung machine helped reduce table mortality during
●​ The pathophysiology of shunt lesions is that they cause cardiac operations
pulmonary congestion due to the left-to-right shunt ●​ Three main presentations of heart lesions:
→​ Pulmonary congestion is accompanied by heart failure →​ Cyanosis
■​ Blood is shunted back into the lungs instead of forward to →​ Heart failure
the systemic circulation →​ Circulatory collapse
●​ Always aim for early repair in infancy or early childhood [!] ●​ ASD shunts oxygenated blood and creates right-sided heart
→​ To avoid pulmonary congestion and heart failure enlargement.
→​ PDA: neonate to infancy ●​ VSD causes earlier and more severe heart failure than ASD
■​ Within first 6 months (repair very early) because high LV pressure (120 mmHg) drives blood into the
→​ VSD/ASD: early childhood lungs, leading to congestion and pulmonary hypertension. ASD
■​ Repair beyond childhood can lead to permanent lung only produces passive low-pressure flow (15–20 mmHg),
damage causing mainly volume overload with milder heart failure.
●​ Irreversible Pulmonary Hypertension in neglected shunt ●​ PDA creates continuous high-pressure shunting (80–120
lesions mmHg), leading to early heart failure and pulmonary congestion
→​ The possibility of repair will be reduced in infants.
→​ Worse outcomes ●​ Tetralogy of Fallot is the most common cyanotic heart lesion in
older children.
B. OBSTRUCTIVE LESIONS
→​ X-ray finding: boot-shaped heart, aka coeur-en-sabot
●​ Right-sided obstruction → cyanosis →​ 4 anatomic defects:
●​ Left-sided obstruction → heart failure and circulatory ■​ Pulmonary stenosis
collapse ■​ Overriding aorta
→​ Left ventricle cannot eject blood as efficiently ■​ Septal defect (Ventricular): main intrinsic defect
→​ If the left-sided obstruction is acute and severe → ■​ Hypertrophy of RV
circulatory collapse →​ Treatment:
■​ The patient (usually infants and babies) would lose ■​ Total corrective surgery
circulation and arrest ■​ Modified Blalock-Taussig Shunt
●​ Early repair in infancy or childhood to avoid permanent damage ●​ Transposition of Great Arteries (TGA) is a life-threatening
to the lungs or heart congenital heart defect where the aorta and pulmonary artery
→​ Coarctation of Aorta: repair early during infancy (within 6 are switched, leading to two separate and parallel blood
months) circulation systems that prevent oxygenated blood from reaching
→​ Pulmonary Stenosis/Atresia: repair within infancy the body. It is the most common cyanotic heart lesion present in
■​ Even if they survive infancy without repair and are cyanotic, newborns or neonates.
complications of right ventricular failure are increased as →​ Arterial Switch Operation is done routinely when presented
the patient ages and grows with TGA and must be done within the first month of life.
●​ Right ventricular dysfunction in neglected right-sided ●​ Pulmonary artery banding is an intervention that causes a
obstructions surgical pulmonary stenosis to prevent or reduce blood flow from
→​ Can still be fatal if not repaired timely manner, or even if the VSD into the lungs
cyanosis was addressed ●​ Early detection and surgical intervention are key to the best
●​ Left ventricular dysfunction in neglected left-sided obstructions outcomes
→​ Can also be a fatal end effect ●​ Always aim for early repair in infancy or childhood to avoid
C. TETRALOGY OF FALLOT permanent damage to the lungs or heart
●​ One of the most common cyanotic lesions encountered in REVIEW QUESTIONS
older Children 1. [True or False] In the traditional classification of analysis, it
●​ Early repair in infancy to prevent RV muscle dysfunction is important to determine the level of shunt of a lesion.
→​ Repair within the first year of life 2. Compared to VSD, what does an ASD usually cause?
●​ Modified Blalock-Taussig shunt
A.​Earlier and more severe pulmonary congestion
→​ Temporising procedure in very sick, small infants
B.​Continuous high-pressure shunting into the lungs
■​ Less commonly, done in patients who need to be palliated
C.​Low-pressure, passive flow with mainly volume overload
and will not survive any total correction
D.​Heart failure in the first few months of life
D. TRANSPOSITION OF GREAT ARTERIES
3. [True or False] In operating on a neonate presenting with
●​ Most common cyanotic heart disease presenting in the switched aorta and pulmonary artery, the critical period for
neonatal period doing an Arterial Switch Operation is within 1 year of life.
●​ Neonatal repair within the 1st month of life ( <1 month) to 4. Modified T/F Question
prevent LV de-conditioning
[Statement A] Simple shunt lesions ideally closed around school
→​ Outcomes are progressively worse if not done within 1st
age
month of life [Statement B] ASD and VSD surgical repair is indicated with a
SUMMARY & KEY POINTS shunt ratio >2:1
A.​Both are true
●​ Simple shunt lesions include:
B.​Both are false
→​ Atrial Septal Defect
C.​Statement A only is true
→​ Ventricular Septal Defect
D.​Statement B only is true
→​ Patent Ductus Arteriosus
●​ Common obstructive lesions in pediatrics: 5. Which is an intrinsic defect of Tetralogy of Fallot?
→​ Coarctation of Aorta A. An interatrial hole
→​ Pulmonary valve stenosis B. Pulmonary valve narrowing

02.25 Surgical Management of Congenital Heart Disease Page 10 of 11

C. Right ventricle hypertrophy
D. Overriding aorta
6. [True or False] Pulmonary artery banding is an intervention
that causes a surgical pulmonary stenosis to prevent or
reduce blood flow from the ASD into the lungs.
7. In pulmonary valve stenosis, why is the RV more prone to
failure compared to the LV?
A. The RV pumps at higher pressures compared to the LV
B. The LV is not that affected by the pulmonary circulation
C. Anatomically, the RV is weaker and cannot sustain high
pressures for so long
D. RV has a lower oxygen demand than the LV
8. Modified T/F Question
[Statement A] For TGA, the definitive treatment is Total Repair
while the palliative treatment is Atrial Switch.
[Statement B] For Tetralogy, the definitive treatment is Total Repair,
‘

while the palliative treatment is BT shunting.

A. Both are true
B. Both are false
C. Statement A only is true
D. Statement B only is true
Answer Key
1F, 2B, 3F, 4D, 5B, 6F, 7C, 8B

RATIONALE TO REVIEW QUESTIONS

1.​ [False] — It should be under the operative classification and
analysis
2.​ [B] — In ASD, left-to-right shunting happens at atrial pressures
(15–20 mmHg), producing volume overload but not the severe,
high-pressure damage seen in VSD or PDA. VSD causes earlier
and worse pulmonary congestion due to 120 mmHg LV pressure,
while PDA produces continuous high-pressure shunting, leading
to heart failure early in infancy.
3.​ [False] — The critical period of doing an Arterial Switch
Operation on a neonate presenting with Transposition of Great
Arteries is within 1 month of life.
4.​ [D] — Only ASDs are closed during school age. VSDs must be
closed at infancy to preschool, while PDAs must be closed as
soon as they are discovered during infancy or as a newborn.
5.​ [B] — narrowing of the pulmonary valve is stenosis. Together
with a big VSD, they make up the intrinsic defects in TOF. ASD,
RVH, and overriding aorta are not intrinsic defects but the latter
two are part of TOF.
6.​ [False] — Pulmonary artery banding is an intervention that
causes a surgical pulmonary stenosis to prevent or reduce blood
flow from the VSD into the lungs
7.​ [C] — RV is not as strong as the LV, which progresses into
dysfunction earlier than the LV because it cannot push the blood
through the very small opening for sustained periods
8.​ [B] — For TGA, both the definitive and palliative treatments are
Atrial Switch Operation.
REFERENCES
REQUIRED REFERENCES
[Lecture] Basic Concepts in the Surgical Management of Congenital Heart
Disease by Carlo Martin Garcia, MD, FPATACSI, August 21, 2025
IMPORTANT LINKS
Errata Tracker: [tinyurl.com/YL7ModuleErrataTrackerDrive]
V2 Errata Sheet: [tinyurl.com/YL7V2ErrataSheet]
Evaluations: [tinyurl.com/YL7TransEvals]
YL7 Trans Map: [tinyurl.com/2028YL7TransMap]
FREEDOM SPACE

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