Cardiac Congenital Disorders

Question 1

Eisenmenger’s syndrome features

Answer 1

Severe pulmonary arterial hypertension due to elevated pulmonary vascular resistance
Causes right to left intracardiac shunt or greater artery shunting
Systemic arterial desaturation

Question 2

Features of TOF

Answer 2

• VSD
• Right ventricular outflow obstruction
• Overriding aorta
• Right ventricular hypertrophy

Question 3

Features of coarctation of aorta

Answer 3

Narrowing of aorta at aortic isthmus, or rarely, in descending thoracic or abdominal aorta
Associated with Turner’s syndrome
Systolic overload i.e. LVH
Chronic upper body systemic hypertension
Difference in pulses above and below coarctation of aorta
Aneurysms, pseudoaneurysm, dissection

Question 4

Features of ASD

Answer 4

Left to right intracardiac shunt
Right heart enlargement
Pulmonary arterial hypertension (in minority of patients)
Systolic ejection murmur over 2nd left ICS sternal border
Wide and fixed splitting of second heart sound

Question 5

Prior to surgery of ASD, what is important to evaluate?

Answer 5

Severe PAH - closure is contraindicated

Question 6

When is ASD closure indicated?

Answer 6

When pulmonary-to-systemic blood flow (shunt) ratio (Qp/Qs) > 1.5:1

Question 7

VSD features

Answer 7

Harsh holosystolic murmur, best heard over left sternal border
Palpable thrill
Evidence of LVH and RVH

Question 8

When is closure of VSD indicated?

Answer 8

Left to right shunt is moderate to large
Pulmonary-to-systemic flow > 1.5:1

Contraindicated in Eisenmenger’s syndrome and severe PAH

Question 9

Cardiac features of Marfan’s syndrome

Answer 9

Progressive aortic root dilatation and aortic dissection
Serial echos required

Question 10

Features of PDA

Answer 10

Signs and symptoms of pulmonary hypertension and heart failure

Question 11

Main locations for ASD

Answer 11

• Fossa ovalis (ostium secundum)
• Inferior portion of atrial septum (Ostium primum)
• Superior portion of atrial septum (sinus venosus)

Question 12

Associated conditions for TOF

Answer 12

DiGeorge syndrome
Down syndrome

Question 13

Clinical findings of TOF

Answer 13

Cyanosis - depending on severity of RVOTO
Tet spells
Relief of symptoms when squatting (increased SVR)
Harsh systolic ejection murmur (caused by RVOTO)
Single S2
RV heave and systolic thrill
Features of heart failure

Question 14

X-ray features of TOF

Answer 14

Boot shaped heart
Normal or decreased pulmonary vascular markings

Question 15

Features of transposition of great vessels

Answer 15

Anatomical reversal of aorta and pulmonary artery

Question 16

Clinical findings of transposition of great vessels

Answer 16

Cyanosis - not affected by exertion or supplemental oxygen
Tachypnoea
Single, loud S2
No murmur
Diminished femoral pulses

Question 17

CXR findigns of TGV

Answer 17

“Egg on a string” appearance
Increased pulmonary vascular markings

Question 18

Features of tricuspid valve atresia

Answer 18

Absent or rudimentary tricuspid valve, results in no blood flow between RA and RV
ASD, VSD - only way for patient to have intertrial and interventricular communications
RV hypoplasia

Question 19

Clinical features of tricuspid valve atresia

Answer 19

Central cyanosis
Tachypnoea
Holosystolic murmur at lower left sternal border
Single S2
Jugular venous distension with prominent A wave
Diminished peripheral pulses

Question 20

Features of Ebstein anomaly

Answer 20

Malformed tricuspid valve leaflets that are displaced into right ventricle with subsequent tricuspid valve regurgitation right heart enlargement
Interatrial communication
Conduction disorders

Question 21

Clinical findings of Ebstein anomaly

Answer 21

Depending on severity of abnormality
Mild apical displacement –> asymptomatic presentation throughout adulthood
Moderate –> cyanosis or heart failure in infancy or childhood
Severe –> in utero heart failure –> death

Loud S1
Widely split S1 and S2
Holosystolic murmur at left sternal border
Digital clubbing

Question 22

ECG and CXR findings of ASD

Answer 22

ECG - RAD, RBBB, RVH
CXR features of enlarged R atrium and ventricle, increased pulmonary vasculature, dilated main pulmonary artery, small aortic knob

Question 23

Features of PFO

Answer 23

Variant of cardiac anatomy in which foramen ovale remains patent beyond 1 year of age
Results in embolisms

Question 24

Features of patent ductus arteriosus

Answer 24

Failure of ductus arteriosus to completely close postnatally
Causes of persistent communication between aorta and pulmonary artery –> left to right shunt –> volume overload of pulmonary vessels

Question 25

Clinical findings of PDA

Answer 25

Bounding peripheral pulses
Wide pulse pressure
Heaving, laterally displaced apical impulse
Loud continuous murmur best heart in left infraclavicular region and loudest at S2 - if large PDA

Question 26

Investigation findings of coarctation of aorta

Answer 26

ECG - signs of LVH
X-ray - cardiomegaly and increase pulmonary vascular markings
Figure of 3 sign
Rib notching

Question 27

Features of pulmonary valve stenosis

Answer 27

Obstruction of blood outflow from right ventricle into pulmonary arteries during systole
Causes RV outflow obstruction –> RVH

Question 28

Clinical findings of pulmonary valve stenosis

Answer 28

Systolic murmur best heard over second left ICS at sternal border
S2 wide splitting