Adult Congenital Surgery

Question 1

What is primary cause of death for adult patients with cyanotic lesions

Answer 1

Arrhythmia followed by heart failure

Question 2

What does Fontan conversion involve

Answer 2

Take down of the previously created venous connection and creation of an extra cardiac cavopulmonary connection with a conduit.

Question 3

List surgeries that may require RVOT reconstruction

Answer 3

Tetralogy of Fallot 
Double outlet right ventricle 
Pulmonary atresia
Truncus arteriosus 
Arterial switch prcoedure

Question 4

What is Ebsteins Anomaly procedure

Answer 4

Tricuspid valve repair or replacement
ASD
Arrhythmia (Maze)

Question 5

What are conduit options for RVOT

Answer 5

Percutaneous approach 
Pulmonary homograft
aortic homograft
bovine jugular vein graft
bioprosthetic valve 
mechanical prosthtetic valve

Question 6

What are options for primary or secondary coartation of the aorta

Answer 6

coarctation resection and end to anastomosis
Onlay patch enlargement with tissue or posthetic patch
Synthetic tube interposition
Percutaneous approach can also be used.

Question 7

What are the features of Scimitar syndrome

Answer 7

ASD
Right ventricle pulmonary vein draining the entire right lung into the IVC
Hypoplastic pulmonary parenchyma
Angiogram demonstrating “fir-tree” appearance
Anomalous pulmonary arterial supply
Dextroposition secondary to hypoplastic right lung

Question 8

List treatment options for HOCM

Answer 8

Cardiac transplantation 
Left ventricular myectomy 
Septal reduction with ETOH ablation 
Mitral valve replacement 
Dual chamber pacing

Question 9

What are indications for repairing coarctation in an adult

Answer 9

Non-invasive pressure difference of > 20 mmHg between upper and lower limbs regardless of symptoms but with upper limb hypertension (> 140/90mmHg in adults)
Patholoical blood pressure response during exercise
significant LVH

Question 10

What are class IIa and IIb indications for adult coarction surgery

Answer 10

independent of pressure gradient, hypertensive pt with > 50% aortic narrowing relatiev to the aortic diameter at the level of diaphragm level

Question 11

What are indications for interventions for right ventricular outflow tract obstruction

Answer 11

RVOT at any level regardless of symtpoms when doppler peak is > 64mmHg provided RV function is normal and no valve substitute is requires
PS vavlotomy should be the intervention of choice
if systolic RV is > 80mmhg

Question 12

What are possible reasons for Fontan Revision

Answer 12

Pt present with extreme right atrial enlargment
right pulmonary vein compression
Arrhythmia
Protein-losing enteropathy
Systemic venous pathway obstruction
semilunar and atrioventricular valve dysfunction