Congenital Heart Disease

Part 2: Complex Lesions

Carole A. Warnes MD, FRCP, FACC
CVBR

©2017 MFMER | 3645208-1

Question 1

A 40-yr old man with repaired tetralogy of Fallot at age 12 yrs.

is referred after an episode of syncope. A Holter shows
episodes of non sustained VT. o/e he is in SR, normal JVP,
has a parasternal heave & at the LSB has a soft ejection
systolic murmur Grade 2/6 and a diastolic decrescendo
murmur Grade 2/4.
His ECG shows SR with RBBB, QRS duration 182 msec.
The most likely residual hemodynamic problem is:

1
 Question 1

1. Residual pulmonary stenosis

2. Residual ventricular septal defect
3. Residual pulmonary hypertension
4. Aortic regurgitation
5. Pulmonary regurgitation

QUESTION 2

• A 40 yr old woman comes for evaluation of progressive

dyspnea and 2 episodes of pre-syncope when walking
quickly. She notices a purple discoloration in her fingers.
She has a history of a heart murmur as a child but had no
intervention.
• Her CXR is shown.
• Which of the following is the most likely diagnosis ?

2
 QUESTION 2

1. Pulmonary stenosis
2. Tetralogy of Fallot
3. Eisenmenger syndrome
4. Ebstein’s anomaly
5. Congenitally corrected
transposition

Question 3

A 52-yr old man presents with increasing dyspnea over

the last 3 yrs with occasional dizziness. He was diagnosed
with some type of congenital heart disease in childhood
but had no follow up after being told he was “fine”.
His ECG is shown.

The most likely diagnosis is:

3
 Question 3

Question 3

1. Ebstein’s anomaly
2. Congenitally corrected transposition
3. Eisenmenger syndrome
4. Tetralogy of Fallot
5. Tricuspid atresia

4
 Question 4
• A 45 yr old woman is seen
for severe dyspnea. She is on
furosemide for peripheral edema.
3 years ago she had device closure
of a secundum ASD & her
symptoms did not improve.

• o/e She has sinus tachycardia.

She has a loud 1st sound and a
soft 1/6 systolic murmur at the LSE.

Question 4
Her CXR is shown
The most likely diagnosis is
1. Ebstein’s anomaly
2. Tricuspid atresia
3. Eisenmenger VSD
4. Tetralogy of Fallot
5. Eisenmenger PDA

10

5
 Congenital Heart Disease
Part 2: Complex Lesions
Carole A. Warnes MD, FACC
CVBR

No disclosures
©2017 MFMER | 3645208-11

11

Learning Objectives

• Recall the anatomy, pathophysiology, clinical presentation,

complications, & management of Eisenmenger syndrome
• Recognize the anatomy, pathophysiology, clinical presentation
& management of tetralogy of Fallot.
• Describe the anatomy, pathophysiology, clinical presentation
& treatment of d- & l- transposition.
• Identify the anatomy, pathophysiology, clinical presentation &
treatment of single ventricle & post-operative complications of
the Fontan procedure.

12

6
 COMPLEX CONGENITAL HEART DISEASE

13

Right to Left
shunt

Tetralogy of Eisenmenger
Fallot syndrome

PH

PS
VSD

14

7
 CYANOTIC CHD ADULTS

Low PAp High PAp

• PS + ASD • Eisenmenger syndrome

• Tetralogy
• Single ventricle • Single ventricle
• Ebstein’s anomaly

LOUD MURMUR NO LOUD MURMUR

15

CYANOTIC CHD ADULTS

• Have erythrocytosis,
not polycythemia
• WBC normal, platelets usu low
• Excess erythrocytosis →
•  blood viscosity
• Symptoms – headache,
poor concentration

16

8
 CYANOTIC CHD ADULTS
Most pt have stable erythropoiesis & need a high Hb
If Hb  20 g/dL & Hct  65 - do not phlebotomize

George Washington in 1799

17

Erythrocytosis
Phlebotomies
Iron deficiency
Microcytosis
ex capacity
 blood viscosity

 blood
viscosity
 symptoms

18

9
 ADULT CYANOTIC HEART DISEASE

Phlebotomy
• Symptomatic hyperviscosity – Hct > 0.65
( provided not dehydrated )
• If symptoms & Hct < 0.65 (suspect iron deficiency)
• Always replace fluid

19

ADULT CYANOTIC HEART DISEASE

Iron deficiency

• Reduced RBC survival time

• Reduces RBC O2 carrying capacity
• RBCs become rigid,  blood viscosity
• Cause commonly phlebotomy

Treatment

• Ferrous sulfate once daily

• D/C when Hct rises (often within 1 wk)

20

10
 CYANOTIC CHD ADULTS

Hemostatic problems
•  prothrombin time
•  APTT
•  coagulation factors
•  platelets, abnormal platelet function

Beware anticoagulation

21

NEED AIR
FILTERS ON
ALL IV LINES

22

11
 ADULT CYANOTIC CHD : Renal function

• Hypercellular congested glomeruli

• Segmental sclerosis
• Thickened basement membrane
• Reduced GFR
• Proteinuria

High uric acid levels 2 o to low • Hyperuricemia

fractional uric acid excretion
> urate overproduction

23

ADULT CYANOTIC CHD

Renal function : caution

• Angiography
• NSAID’s
• Gentamicin

Risk of
renal failure

24

12
 ADULT CYANOTIC CHD

Neurological Scoliosis

• CVA, epilepsy • 0.03 – 6% in general

population
• Brain abscess
• 3 – 19% in adult CHD
• 3 x more common
in cyanotics

25

CYANOTIC CHD ADULTS

• Anesthesia, minor surgery

• Pregnancy, contraception

26

13
 CYANOTIC CHD : ADULTS

• Venesection
• Thrombosis / hemorrhage
• Renal problems / gout Should be followed
• Scoliosis / arthropathy
regularly
• Gallstones
in an ACHD center
• Angiography – care
• Anesthesia, minor surgery
• Pregnancy, pill

27

EISENMENGER SYNDROME

• Large shunt (usually VSD) produces

pulmonary HTN & irreversible
pulmonary vascular disease.

• Shunt reverses R → L
causing cyanosis

• Symptoms: dyspnea
syncope
hemoptysis
stroke

28

14
 EISENMENGER SYNDROME

Blue blood to toes Differential cyanosis

29

EISENMENGER SYNDROME
Physical exam

• Cyanosis and clubbing

• ‘a’ wave in JVP
• RV heave, palpable P2
• Ejection click
• Little or no systolic murmur
• Loud P2
• Diastolic murmur of PR

30

15
31

EISENMENGER SYNDROME

• Beware
ERA : Bosentan / Ambrisentan : 6 min walk
• Hemoptysis
• Syncope PDE5 inhib : Sildenafil / tadalafil : 6 min walk
• Non-cardiac Prostacyclin : 6 min walk
surgery Improve PA pressure
• No vasodilators ? Length of life

32

16
 19-Year-Old Student

• Eisenmenger VSD
• 2 episodes R-sided numbness
Transient – both in hot weather
• Hb 19.8 g, Hct 58
• Otherwise fairly normal life
• Ability index 2

33

Phlebotomy ?
CT scan → Angiogram
12 mm aneurysm ant. communicating A.

“Clip under hypotensive

anesthesia “

CP1020283-20

34

17
 Learning Objectives

Recognize the anatomy, pathophysiology,

clinical presentation & management
of tetralogy of Fallot.

35

TETRALOGY OF FALLOT

• Large subaortic VSD

• RVOT obstruction (± PV stenosis)
• Overriding aorta
• RVH
RVp = LVp
RVOT obstruction determines
clinical presentation

36

18
37

TETRALOGY OF FALLOT

• Mild RVOT obstrn →

• “Acyanotic Fallot”
Stenotic • “Pink tet”
pulmonary Ventricular
valve septal defect
• Infundibular hypertrophy 
Stenotic
RVOT •  age =  cyanosis

38

19
 TETRALOGY OF FALLOT

39

TETRALOGY OF FALLOT

• Right aortic arch = 25%

• Anomalous cor A = 2-10%
• LAD from RCA or
right sinus of Valsalva
• Secundum ASD = 15%

* Di George syndrome (velocardiofacial syndrome) : 22q.11 deletion

AUTOSOMAL DOMINANT
•
40

20
 AORTIC ARCH

Right aortic arch

Normal left aortic arch with tetralogy

41

TETRALOGY OF FALLOT : Surgical repair

Simple Complex

• Resect RVOT muscle • RVOT patch

• Close VSD • Transannular patch
• ± pulm valvotomy • Excision of pulm valve
• RV to PA conduit

42

21
43

TETRALOGY OF FALLOT

RV dysfunction
• Late repair
• Residual RV outflow gradient
• Residual VSD
• Severe pulmonary regurgitation

Worse prognosis if QRS >180 msec

44

22
 POST-OP TETRALOGY OF FALLOT

95% have RBBB

Wider QRS correlates with worse RV and VT

45

POST- OP TETRALOGY OF FALLOT

QRS duration 186 msec

46

23
 TETRALOGY OF FALLOT

47

TETRALOGY OF FALLOT

Beware arrhythmias after repair

48

24
 TETRALOGY OF FALLOT
Usually an underlying hemodynamic abnormality

• Pulmonary stenosis MOST COMMON

PROBLEM
• Residual VSD
IS PULMONARY
REGURGITATION
• Aortic regurgitation

49

TOF : ? Pulmonary valve replacement

PVR is reasonable with at least 2 of following :

Mild or greater RV or LV dysfunction
Severe RV diln: RVEDVi > 160 mL/m2 (N= 108 mL/m2)
RVESVi > 80 mL/m2 (N= 48 mL/m2)
RVEDV > 2 x LVEDV
RVsp > 2/3 systemic pressure
Progressive in ex capacity

Stout KK, Circ 2018

50

25
 TETRALOGY OF FALLOT

Replace pulmonary valve BEFORE

irreversible RV dysfunction

The worse the RV, the worse is

risk for VT and sudden death

Higher risk – those with late repair

51

TETRALOGY OF FALLOT
Residua & sequelae

• SVT
• VT and sudden death
• RVOT aneurysm at patch site
• BE on aortic valve
• Reoperation for PS ± PR
• Aortic dilatation and AR

52

26
 SURGICALLY- CREATED SHUNTS

• Used in cyanotic heart disease with

low pulmonary blood flow (e.g. Tetralogy of Fallot )
• Improves oxygenation and makes small PA’s grow

53

SURGICALLY- CREATED SHUNTS

• Used in cyanotic heart disease with

low pulmonary blood flow (e.g. Tetralogy of Fallot )
• Improves oxygenation and makes small PA’s grow
• Usually performed through a lateral thoracotomy
• All have potential for complications

54

27
 CLASSIC GLENN

SVC

RPA

Seldom used now because of propensity for

pulmonary a - v fistulae

55

BIDIRECTIONAL GLENN
Bi Directional Cavo – Pulmonary Shunt

• Improves oxygenation
• Does not volume load
ventricle like arterial shunt

56

28
 Brachiocephalic
Blalock-Taussig R common trunk (innominate
Shunt carotid artery artery)

R subclavian artery

R pulmonary artery

Aorta

Pulmonary trunk

57

MPA
R
Asc
SVC Ao
L

SVC
RPA
LPA Potts

Desc
Ao
Waterston

58

29
 PROBLEMS WITH SURGICAL SHUNTS

• Distortion of the PAs – kink, thrombose, occlude

• Large shunt produces pulm HTN & pulm vascular
disease
• Large shunt → volume overload of LV

No heart – lung transplant if lateral thoracotomy

59

Learning Objectives

Describe the anatomy, pathophysiology,

clinical presentation & treatment of
d- & l- transposition.

60

30
61

BULBOVENTRICULAR LOOP

• d-loop – morphologic RV on right

• l-loop – morphologic RV on left
Aorta follows the loop, i.e.
• d-TGA – aorta anterior & to the right
• l-TGA – aorta anterior & to the left

62

31
 d - TRANSPOSITION

Ao PA

RV LV

63

d - TRANSPOSITION

AV

PV

RV

LV

Aorta anterior and

RV is on the right to the right

64

32
 TRANSPOSITION OF THE GA’s
All had surgery by adulthood

• Arterial switch
• Mustard, Senning

65

Arterial Switch Procedure

Aorta Coronary
Aorta arteries
Cut in implanted to
arterial
PA PA pulmonary
Coronary
trunks orifices root
* sewn over
* Pulm
Aortic
root
root Aortic Pulm
root root

Morph Morph Morph Morph

RV LV RV LV

66

33
 Mustard Operation

LV
RV

67

D-TGA after MUSTARD

68

34
 TRANSPOSITION AFTER MUSTARD PROCEDURE

RV failure
TR

69

TRANSPOSITION OF THE GA’s

Problems after atrial baffle

• Atrial arrhythmias
• Junctional
• Flutter
• Baffle obstruction BEWARE
• Baffle leak ( ASD ) P/M

70

35
 Congenitally- corrected
Normal transposition

Ao Ao
PT

RA PT LA RA LA

RV LV RV
LV

71

CORRECTED TRANSPOSITION

The AV valve belongs to the

appropriate ventricle
TV belongs to RV
MV belongs to LV

The TV is on the left side,

i.e. in the systemic circulation

72

36
 ATRIOVENTRICULAR CONNECTIONS
Normal CC-TGA

RA RA
LA LA PA
Ao

RA LA

LV RV
RV LV
RV
LV

Concordance Discordance (Pictures courtesy Dr. W.D. Edwards)

73

CONGENITALLY CORRECTED TGA (L-TGA)

Ao
Ao

RV
RV

RV

74

37
 CORRECTED TRANSPOSITION

Associated lesions
• VSD
• PS
• Left ( tricuspid or systemic ) A-V valve regurgitation
BEWARE systemic ventricular dysfunction
Complete heart block

75

CONGENITALLY CORRECTED TGA

RA LA

LV

RV

76

38
77

CONGENITALLY CORRECTED TRANSPOSITION

Most common lesion

associated with dextrocardia

78

39
 CORRECTED TRANSPOSITION
Conduction

• > 75% pt have varying degrees of A-V block

when all ages included
• Inversion of right & left bundles causes septal
activation from right to left, so Q waves
• Absent in left precordial leads ( V5,V6 )
• Present in right precordial leads ( II, III, avF )

79

CONGENITALLY CORRECTED TGA

Q waves inferior leads

80

40
 CONGENITALLY CORRECTED TGA

81

Atrioventricular Connections
Single Ventricle

82

41
 TRICUSPID ATRESIA

ASD

Tricuspid atresia
Rudimentary RV

83

FONTAN OPERATION
For complex cyanotic disease
• With normal PA size

• With normal PA pressure

e.g. tricuspid atresia, single ventricle

Diverts all blue blood from vena cavae, RA to PA

– many modifications

Goal is to separate blue & red blood

84

42
 CLASSIC FONTAN OPERATION

85

COMPLICATIONS OF CLASSIC FONTAN

Atrial arrhythmias
• Beware Fontan
obstruction
• RA thrombus
* always TEE before DC
version

Meticulous anticoagulation

86

43
 EXTRA – CARDIAC FONTAN

87

FONTAN OP. – OTHER COMPLICATIONS

• Pacemaker
• Residual shunts (cyanosis)
• Obstruction at anastomosis: (elevated JVP, edema, hepatomegaly)
• Reoperation
• Ventricular failure
• Hepatic congestion / cirrhosis / HCC *
• Protein-losing enteropathy

88

44
 FONTAN OPERATION : PLE

• Relates to  systemic venous pressure with 

retrograde pressure in thoracic duct causing
lymphangiectasia

• Dilated lymph vessels lose protein in gut

• > 10% pt develop PLE – pleural effusions,

edema, ascites

•  alpha 1 anti-trypsin concentration in stool,

low serum protein, albumin

89

PEARLS

• Cyanotic patients : need high Hb – no phlebotomy

• Any neuro disturbance – scan head & r/o iron deficiency
• No loud murmurs with Eisenmenger syndrome
• Repaired TOF - think pulmonary regurgitation.
If RV big - beware VT
• D - transposition after Mustard: RV failure + TR
• L - transposition: heart block, systemic AV valve
regurgitation (TR) & heart failure

90

45
 GOOD LUCK!
91

Questions and discussion

92

46
 Question 1

A 40-yr old man with repaired tetralogy of Fallot at age 12 yrs.

is referred after an episode of syncope. A Holter shows
episodes of non sustained VT. o/e he is in SR, normal JVP,
has a parasternal heave & at the LSB has a soft ejection
systolic murmur Grade 2/6 and a diastolic decrescendo
murmur Grade 2/4.
His ECG shows SR with RBBB, QRS duration 182 msec.
The most likely residual hemodynamic problem is:

93

Question 1

1. Residual pulmonary stenosis

2. Residual ventricular septal defect
3. Residual pulmonary hypertension
4. Aortic regurgitation
5. Pulmonary regurgitation

94

47
 QUESTION 2

• A 40 yr old woman comes for evaluation of progressive

dyspnea and 2 episodes of pre-syncope when walking
quickly. She notices a purple discoloration in her fingers.
She has a history of a heart murmur as a child but had no
intervention.
• Her CXR is shown.
• Which of the following is the most likely diagnosis ?

95

QUESTION 2

1. Pulmonary stenosis
2. Tetralogy of Fallot
3. Eisenmenger syndrome
4. Ebstein’s anomaly
5. Congenitally corrected
transposition

96

48
 Question 3

A 52-yr old man presents with increasing dyspnea over

the last 3 yrs with occasional dizziness. He was diagnosed
with some type of congenital heart disease in childhood
but had no follow up after being told he was “fine”.
His ECG is shown.

The most likely diagnosis is:

97

Question 3

98

49
 Question 3

1. Ebstein’s anomaly
2. Congenitally corrected transposition
3. Eisenmenger syndrome
4. Tetralogy of Fallot
5. Tricuspid atresia

99

Question 4
• A 45 yr old woman is seen
for severe dyspnea. She is on
furosemide for peripheral edema.
3 years ago she had device closure
of a secundum ASD & her
symptoms did not improve.

• o/e She has sinus tachycardia.

She has a loud 1st sound and a
soft 1/6 systolic murmur at the LSE.

100

50
 Question 4
Her CXR is shown
The most likely diagnosis is
1. Ebstein’s anomaly
2. Tricuspid atresia
3. Eisenmenger VSD
4. Tetralogy of Fallot
5. Eisenmenger PDA

101

51