OBSTRUCTIVE DEFECTS

●​ Blood flow in heart meets an area of anatomic narrowing (stenosis) ➡ obstruction to blood flow
●​ Pressure in the ventricle and great arteries before obstruction is increased; pressure in areas beyond
obstruction is decreased.
●​ Location of narrowing near the valve:
-​ Valvular: site of valve itself
-​ Subvalvular: narrowing in vent below valve (ventricular outflow tract)
-​ Supravalvular: narrowing in great art above valve.
●​ (+) pressure load on ventricle- decrease CO.
●​ s/sx CHF
●​ Mild obstruction: asymptomatic
●​ Severe stenosis: hypoxemia (rare)
COARCTATION OF AORTA
●​ The aorta is narrowed near the insertion of the ductus arteriosus
●​ Increased pressure proximal to the defect
●​ Causes high BP & bounding pulses in arms; weak or absent femoral pulses, and cool lower extremities
with low BP
Signs of CHF in Infants:
-​ Conditions can deteriorate rapidly.
-​ Older kids may complain of dizziness, headache, fainting and epistaxis from hypertension.
●​ Patients at risk for ruptured aorta, aortic aneurysm, or stroke.

​ ​ ​ ​ ​ ​ ​ Treatment: non-surgical
-​ Balloon angioplasty. (Usually effective)
-​ Surgical: Does not require bypass since defect is
outside pericardium.
-​ Post-op complication is hypertension
-​ Usually done before age 2 years.
-​ Risk of recurrence.

AORTIC STENOSIS
●​ Narrowing of aortic valve usually malformed in BI- rather than TRIcuspid valve.
●​ Causes increased resistance in the left ventricle, decreased Co, L ventricular hypertrophy and
pulmonary vascular congestion.
●​ L ventricular wall is hypertrophied >> increased pulmonary vascular resistance and pulmonary
hypertension .
●​ LVh >> decreased coronary artery perfusion and increase risk of MI.
●​ Clinical Manifestations:
-​ Signs of decreased CO: faint pulses, hypotension, poor feeding, tachycardia.
-​ Murmur ; exercise intolerance.
-​ Chest pain, dizziness with standing.
●​ Treatment:
-​ Balloon angioplasty to dilate the valve.
-​ Surgery: Konne procedure (valve replacement.
-​ May require repeat procedures.
​ ​ ​ ​ ​ ​ ​ ​ PULMONARY STENOSIS
​ ​ ​ ​ ​ ​ ​ ​
●​ Pulmonary valve is stenosed
●​ Narrowing at entrance to pulmonary
artery>> R ventricular hypertrophy and
decreased pulmonary blood flow.
●​ Extreme form of PS: Pulmonary atresia
(total fusion of the commissures and no
blood flow to lungs.)
●​ PS >> RVH, R ventricular failure >> R atrial
pressure increases and may reopen
foramen ovale.
●​ Shunts unoxygenated blood to L atrium >>
systemic cyanosis
●​ May lead to CHF
●​ Often have PDA as well
●​ Cardiomegaly on CXR

TREATMENT:
-​ Balloon angioplasty to dilate the valve.
-​ Surgical treatment: Brock Procedure
(Bypass to do valvotomy)
-​ Usually can repair with catheterization.

DEFECTS OF DECREASED PULMONARY BLOOD FLOW

• Obstruction of pulmonary blood flow +anatomic defect (ASD/ VSD) between R & L side
of heart
• Difficulty of blood exiting R heart via pulmonary artery → increase R side pressure > L pressure →
desaturated blood shunt R to L → desaturated blood in systemic circulation
• Hypoxemia, usually cyanotic

TETRALOGY OF FALLOT
• Involves four heart defects:
– Ventricular Septal Defect
– Pulmonary stenosis
– Right ventricular hypertrophy
– Overriding aorta
• Hemodynamics vary widely
– Depends on extent of pulmonic valve stenosis & size of VSD
– If VSD is large, pressures are equal in R and L ventricles.
Blood is shunted in the direction of the least resistance
(pulmonary or systemic vascular resistance)
• PVR is > than systemic vascular resistance, shunt will be R to L
Clinical manifestations:
– “TET SPELLS” or “blue spells” with acute episodes of cyanosis and hypoxia
– Anoxic after feeding or with crying.
RISK of emboli, LOC, sudden death, seizures
– Repairs: usually indicated when Tet spells and hypercyanotic spells increase
• Stage 1: Blalock or modified Blalock shunt >> blood to pulmonary arteries from L or R subclavian
artery
• Complete repair: usually in 1st year of life.
Repair of VSD, resect stenosed area, and patch R ventricular outflow

TRICUSPID ATRESIA
●​ Failure of tricuspid valve to develop
●​ No communication from R atrium to R ventricle
●​ Blood flows thru an ASD or a patent FO to L side of the heart thru a VSD to R ventricle to lungs
●​ Often associated with PS and TGS.
●​ Complete mixing un02 and 02 blood in L side of the heart ➡ systemic desaturation, pulmonary
obstruction ➡ decrease pulmonary blood flow.
●​ At birth: presence of patent FO (or ASD) is required to permit blood flow across septum into L atrium.
-​ PDA allows blood flow to the pulmonary artery for oxygenation.

●​ Manifestations: cyanosis, tachycardia, dyspnea,

hypoxemia, clubbing
●​ At risk for bacterial endocarditis, brain abcess,
stroke
●​ Treatment: NB – pulmonary blood flow depends on
PDA
​ ​ ​ ​ ​ ​ ​ ​ – Continuous infusion of PGE 1 until Sx
– Palliative: shunt (pulmonary-to- systemic art
anastomosis)
– Pulmonary artery banding
– Modified Fontan procedure
 MIXED DEFECTS
●​ Survival on postnatal period depends on mixing of blood from pulmonary systemic circulation within
cardiac chambers
●​ Blood is mixed from pulmonary and systemic circulations within the heart chambers >> Relative
desaturation of blood in systemic blood flow
●​ Cardiac output decreases because of volume load on ventricle
●​ Signs of desaturation, cyanosis, and CHF, but variable depending on anatomy
●​ Degree of cyanosis not always visible & signs of CHF
– TGA: severe cyanosis in 1st day of life → CHF (later)
– Truncus arteriosus: severe CHF 1st few weeks of life and mild desaturation
HYPOPLASTIC LEFT HEART SYNDROME

●​ L eft side of the heart is underdeveloped

●​ Left ventricle is small and aortic atresia
●​ Most blood flows across patent foramen ovale to R atrium – to R ventricle and out the pulmonary
●​ Descending aorta receives blood from the PDA to supply the systemic circulation
●​ PDA closure >> rapid deterioration and CHF

●​ Treatment: Keep ductus open with Prostaglandin E infusion

●​ Surgical Treatment:
– Norwood procedure to create a new aorta using the main pulmonary artery and creation of large
ASD
– Bidirectional Glenn Shunt at 6-9 months age to reduce volume load on the R ventricle
– Modified Fontan procedure, similar to Tricuspid atresia repair
– Transplant may be an option for some parts. Mortality rate is very high (30%- 50%)
​ ​ ​ ​ ​
TRANSPOSITION OF THE GREAT VESSELS
●​ Pulmonary artery leaves the L ventricle and the aorta exits from the R ventricle
●​ No communication between the systemicand pulmonary circulations
●​ Must have PDA or Septal defect to permit blood flow
​ ​ ​ ​ ​ ​ ​
​ ​ ​ ​ ​ ​ ​ Surgical Treatment:
-​ Arterial Switch procedure to resect and
reanastomose great vessels.

-​ Coronary arteries have to be reimplanted to supply myocardial circulation.

-​ Other procedures are possible- depending on the defect.

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION

●​ Rare defect
●​ Furosemides, Thiazides, Spirinolactone
●​ Fluid restriction
●​ Sodium restriction
-​ Decrease cardiac demands
●​ Bed rest, treat infection
●​ Preserve body temperature; reduce effort of breathing (semi-fowlers)
●​ Sedate an irritable child
-​ Improve tissue oxygenation & decrease O2 consumption
●​ O2 vasodilator
●​ Cool humidified O2
 ACQUIRED CARDIOVASCULAR DISORDERS

CONGESTIVE HEART FAIULRE

●​ Inability of the heart to pump and adequate amount of blood to the systemic circulation at normal filling
pressures to meet the metabolic demands of the body
●​ Due to structural deformity in children (septal defects) → increased blood volume & pressure in the
heart → MYOCARDIAL FAILURE
●​ Can occur with cardiomyopathy, dysrythmia, severe electrolyte imbalances
●​ Could also be due to excess demands on a normal cardiac muscle (sepsis / severe anemia)

●​ RIGHT SIDED HEART FAILURE

– RV unable to pump blood to Pulmonary Artery → increased pressure in RA and in the
systemic venous circulation
– Systemic venous HPN → Hepatosplenomegaly
●​ LEFT SIDED HEART FAILURE
– LV unable to pump blood to the systemic circulation → increased LA pressure and pulmonary
vv → congestion in lungs → increased pulmonary pressure → pulmonary edema → pulmonary
HPN

●​ Signs and symptoms of CHF

– Each side of the heart depends on the adequate function of the other
– Failure of one chamber affects the opposite chamber
●​ If not corrected, it may lead to cardiac damage → inadequate CO → decreased supply to the kidneys
→ Na and H2O resorption → hypervolemia, increased workload on the heart, pulmonary and systemic
congestion

CONGESTIVE HEART FAILURE IN CHILDREN

●​ Impaired myocardial function
– Tachycardia, fatigue, weakness, restless, pale, cool extremities, decreased BP, decreased urine
output
●​ Pulmonary congestion
– Tachypnea, dyspnea, respiratory distress, exercise intolerance cyanosis, wheezes
●​ Systemic venous congestion
●​ Peripheral and periorbital edema, weight gain, ascites, hepatomegaly, neck vein distention,

CHF TREATMENT
Goals:
●​ Improved cardiac function
– Digitalize – Digoxin
●​ Increased CO, decreased size and venous pressure, relieve edema
●​ Lanoxin (Pedia) – more rapid in onset
– Oral / IV doses x 24 hours followed by maintenance dose (BID) to maintain blood levels (Digitalizing
Dose)
–ACE Inhibitors (Capoten / Enalapril)
 ●​(-) normal function of R-A system in kidney
●​Blocks conversion of AI to AII (Vasodilator)
●​Captopril – can be given smaller doses
●​Remove accumulated Fluid and Sodium
– Diuretics – mainstay of treatment to eliminate excess H2O and salt
– Bidirectional glenn
●​ Keep hct and blood viscosity within acceptable limits (hydrate)
●​ Monitor for anemia, Fe supplementation, blood transfusion
●​ Respiratory infection or reduce pulmonary function can worsen hypoxemia
– Aggressive pulmonary hygiene
– CPT, antibiotics
– O2
ENDOCARDITIS (BACTERIAL INFECTIVE ENDOCARDITIS)
●​ BE, IE or subacute bacterial endocarditis (SBE)
●​ Infection in valves and endocardium
●​ Sequelae of sepsis in child with cardiac disease of congenital anomaly
●​ Affects children with valvular abnormalities, prosthetic valves, recent heart surgery with invasive lines
and RHD with valve involvement, drug abuse
●​ Staph aureus, strep viridians (most common), candida albicans, gram negative bacteria
●​ Enter blood system thru: dental (most common), UTI, cardiac catheterization, surgery, etc.
●​ Organism in endocardium → vegetations (verrucae) → fibrin deposits → platelet thrombi → invade
adjacent tissues (mitral/aortic valves) → breaks off and embolize elsewhere (spleen, kidney, CNS) →
death

• Diagnostics
– Based on clinical manifestations
– Blood c/s: definitive diagnosis
– ECG/CXR (cardiomegaly)
– Increased ESR, increased WBC, anemia, microscopic hematuria
– 2D-echo – vegetations, valve function
• Clinical manifestation
– Insidious onset, unexplained fever (low grade, intermittent)
– Anorexia, malaise, weight loss
– Extracardiac emboli
– Splinter h’ge – thin black lines under nails
– Osler nodes – red, painful, intradermal nodes on pads of phalanges
– Laneway lesions – painless h’ges on panes and soles
– Petechiae on oral mucous membrane
– May be present: CHF, dysrythmia, new murmur
• Therapeutic management
– High dose antibiotics: Penicillin, ampicillin, methicillin, cloxacillin, streptomycin, or gentamycin
 – IV/IM x 4 weeks at least
– Amphoterecin or flucytosine for fungal infections
– Treat 2-8 weeks. If antibiotics is unsuccessful >> CHF develops, vulvular damage
– Should be instituted immediately
– Blood c/s periodically to evaluate responseto antibiotics
– Prophylaxis before dental procedures, bronchoscopy, T&A, surgeries, childbirth
– Prophylaxis: 1 hour before procedures (IV) or may use PO in some cases
– Family dentist should be advised of existing heart problems

RHEUMATIC FEVER (RF)

RHEUMATIC HEART DISEASE (RHF)
Rheumatic Fever (RF)
●​ Inflammatory disease occurs after Group A Beta- haemolytic streptococcal throat infection
●​ Self-limiting
– Affects joints, skin, brain, serious surfaces, and heart
Risk factors:
●​ Age and sex: (5-15 years old) female
●​ Housing and socioeconomic status
●​ Season: rainy season
●​ Genetic predisposition
Clinical Manifestations
●​ Acute febrile-like illness (2-3 weeks after streptococcal throat infection)
●​ Non-specific
– Fever
– Joint pain
– Loss of appetite
– Muscle ache
●​ Specific
– Joints (swelling and pain of larger joints like knee, ankle, elbow and wrist occurring in rapid
succession – “migratory arthritis”)
– Heart (causes inflammation of the whole layers of the heart – PANCARDITIS) – Palpitation, chest
pain, shortness of breathe, leg swelling, etc.
– Skin & subcutaneous tissues (non-itching macular rash and painless mobile nodules over joints and
spines)
– Central Nervous System (a late manifestation) – abnormal movements of the limbs with muscle
weakness and emotional labiality.
Diagnostic approach:
Modified Jones Criteria
●​ 2 major or 1 major + 2 minor manifestations + strep infection
– MAJOR
●​ Carditis – tachycardia out of proportion to degree of fever
– Cardiomegaly, murmur, muffled heart sounds
– Pericardial friction rub, pericardial pain, changes in ECG
– Involves endocardium, pericardium, and myocardium » Most commonly the mitral valve
●​ Polyarthritis
– Arthritis is reversible and migrates, especially in large joints (knees, elbow, hips, shoulders,
wrists)
– Swollen, hot, red, painful joints, after 1-2 days → affects different joints
-​ MAJOR
– Erythma marginatum – rash
●​ Transitory, non pruritic
●​ Trunk and proximal portion of extremities
●​ Red macule with clear center wavy, well-dermacated border
– Subcutaneous nodules
 ●​ Small nontender nodules
●​ Bony prominences – hands, feet, elbows, scalp, scapulae, vertebrae
●​ Persistent indefinitely after onset of the disease and resolve with no resulting damage

– St. Vitus Dance – The Fifth Manifestation (Sydenham’s Chorea)

●​ St. Vitus Dance(aka, chorea) reflects CNS involvement
●​ Definition: Chorea refers to sudden, aimless movements of extremities, involuntary
facial grimaces, speech disturbances, emotional lability and muscle weakness
●​ Worse with anxiety and relieved by rest​
– MINOR
– Arthralgia
– Fever
LABORATORY
●​ Increased ESR, CRP
– Supporting evidence of antecedent group A Strep Infection
●​ Throat c/s, rapid Ag test
●​ ASO titer (most reliable – 80% children)
●​ AntiDNAse. ESR, CRP
●​ ECG, CXR – evidence of heart involvement
TREATMENT
●​ Goals
– Eradication of haemolytic strep
– Prevention of permanent cardiac damage
– Palliation of other symptoms
– Prevention or recurrence of RF
●​ Prevention of RHD
– Treatment of streptococcal tonsillitis / pharyngitis
●​ Penicillin G – IM x 1
●​ Penicillin V – oral x 10 days
●​ Sulfa – oral x 10 days
●​ Erythromycin (if allergic to above) – oral x 10 days
– Treatment of recurrent RF
– Same as above
●​ Salicylates (ASA) – control inflammatory process esp. joints, dec fever and discomfort
●​ Bed rest – during febrile phase but need not be strict
●​ Should be followed medically x 5 years at least
NURSING CONSIDERATIONS
●​ Encourage compliance with drug
– Encourage adherence to tx’c plan
– (+) poor compliance: monthly injections
●​ Facilitate recovery from illness
●​ Provide emotional support
●​ Prevent disease
●​ Interventions during homecare
– Provide rest and adequate nutrition
– Once fever is over, resume moderate activities, improve appetite
– Carditis: most disturbing and frustrating manifestation
– Gradual
– Mistaken for nervousness, clumsiness, inattentiveness
– Limits physical activity
– Stress parents and teachers: illness is temporary and disappears in time
RHEUMATIC HEART DISEASE (RHD)
●​ Most common complication of RF
●​ Damage to valves leading to stenosis or regurgitation with resultant hemodynamic disturbance.
KAWASAKI DISEASE
●​ Mucocutaneous LN syndrome
●​ Acute systemic vasculitis
●​ < 5 y/o (Peak: toddlers)
●​ Self limiting but 20% children without treatment develop cardiac disease
●​ Etiology: unknown
●​ Area involved: CVS
– Initially: Inflammation arterioles, venules, capilliaries → formation coronary artery aneurysm
– Death: result of coronary thrombosis or severe scar formation & stenosis of main coronary artery
– Myocardial infarction from thrombosis
●​ No specific diagnostic test
– IRRITABILITY – hallmark of Kawasaki Disease
– Persists in 2 weeks
– Help family adjust to the disorder
– Educate family
– Help family cope with effects of the disorder
– Prepare child and family for surgery
●​ Surgical Interventions
– Open-Heart
– Closed heart procedures
– Staged procedures
– Prepare child and family for procedures
●​ Postoperative Care of the Child
– Monitor vital signs and A/V pressures
– Intraarterial monitoring of BP
– Intracardiac monitoring
– Respiratory needs
​ – Rest, comfort and pain management
– Fluid management
– Progression of activity
●​ Postoperative Complications
– CHF – due to excessive pulmonary blood flow or fluid overload
– Hypoxia – inadequate pulmonary blood flow / respiratory problems
– Dysrhythmias
• Early post op period
●​ Due to electrolyte imbalance (hypokalemia) & surgical intervention to septum / myocardium
●​ ECG pattern, apical pulses x 1 minute
• Postoperative Complications
– Cardiac tamponade
●​ Compression of heart by blood and other effusion (clots) in pericardial sac → restricts normal heart
movement
●​ Rising and equalizing RA and LA pressures, narrowing pulse pressure, tachycardia, dyspnea,
apprehension, abrupt stop to chest tube drainage from mediastinal tubes
●​ 2-D echo to confirm the diagnosis
●​ Treatment: Pericardiocentesis
●​ Postoperative Complications
– Decreased cardiac output syndrome
– Signs of Shock: decreased BP, decreased pulse pressure, cool extremities, metabolic acidosis,
oliguria
– Aggressively treated with IV inotropes (dopamine,dobutamine, milrinone)​ ​ ​
​ – Decreased peripheral perfusion
 ●​ Capillary refill time, skin color, warm/cold extremities, pulses (strong/weak)
– Pulmonary changes
●​ Areas of atelectasis common after Sx
TEST OF CARDIAC FUNCTION
●​ Echocardiography (2D Echo)
– Enables visualization and measurements of turbulence, accentuation, direction, and decreases/
increases in blood flow, along with pressure gradients
– Uses high-frequency sound waves obtained by a transducer to produce an image of cardiac structure
– Most useful in diagnosis of CHD in critically ill babies
●​ Angiography
– Assess route of blood flow in heart & major blood vessels
●​ Chest X-ray
– Used to evaluate for cardiomegaly, pulmonary vascular marking, and pulmonary venous congestion
– Also rules out pulmonarydisease
●​ Electrocardiogram (ECG/EKG)
– Electrical activity of the heart
– Not used for diagnosis but provide data on abnormal electrical activity that warrants the need for
additional evaluation
●​ Arterial Blood Gasses (ABG)
– Helpful in differentiating between cardiac and pulmonary pathologies
– Important when the child is at risk or complications such as acidosis and shock
Cardiac Catheterization
●​ Most invasive diagnostic procedure
●​ Radio opaque catheter inserted through peripheral blood vessel into the heart
●​ Performed to evaluate heart valves, heart function and blood supply, or heart abnormalities
●​ Used also for treatment (when combined with angioplasty)
●​ Complications:
– Infective endocarditis
– bleeding/ bruising
– Changes in circulation on cath side
●​ Pre-op care
– History and physical examination
– Laboratory works (ECG, 2D Echo, CBC)
– NPO
– Preprocedural teaching

●​ Post-op care
– Monitor VS
– Monitor extremity distal to the catheter insertion site
– Leep leg immobilized
– Measure I & O
– Check for bleeding at insertion site