Pediatric Cardiology Board Review

Chapter 11. Surgical Palliation and Repair of Congenital Heart Disease

Nathaniel Taggart

   1. A 4-month-old child with a large membranous ventricular septal defect (VSD) develops complete heart block shortly after surgical repair. A suture “bite” placed too deep in which of the following sites is most likely responsible for the heart block?

A. Posterior-inferior rim of the VSD

B. Anterior-superior rim of the VSD

C. Right ventricular myocardium

D. Superior right atrial free wall

E. Inferior right atrial free wall

   2. Which of the following surgical interventions carries the highest risk of pulmonary vascular obstructive disease among patients with tetralogy of Fallot?

A. Central shunt

B. Potts shunt

C. Classic Blalock–Taussig (BT) shunt

D. Modified BT shunt

E. VSD closure with pulmonary valvectomy

   3. A 2-month-old female with Down syndrome undergoes successful repair of a balanced complete atrioventricular septal defect (AVSD). While discussing her long-term prognosis with her parents, you state that which of the following is the most common indication for re-operation after repair of AVSDs?

A. Residual VSD

B. Residual atrial septal defect (ASD)

C. Left ventricular outflow tract (LVOT) obstruction

D. Tricuspid regurgitation

E. Mitral regurgitation

   4. Which of the following is the strongest predictor for developing mitral valve regurgitation after the repair of AVSDs?

A. Presence of a pre-operative mitral valve cleft

B. Pre-operative severe mitral regurgitation

C. Use of an annuloplasty ring on left atrioventricular (AV) valve annulus

D. Post-operative left ventricular (LV) enlargement

E. Post-operative LVOT obstruction

   5. You perform a cardiac catheterization on a 12-month-old child with pulmonary atresia, VSD, and confluent pulmonary arteries status post placement of a 3.5 mm modified right-sided BT shunt at 1 week of age. His systemic arterial saturation is 62% on room air. Body surface area is 0.5 m2. The RPA diameter is 8 mm and the LPA diameter is 9 mm just proximal to their first lobar branches. There is stenosis of the RPA proximal to the BT shunt insertion, measuring 6 mm in diameter. There are no significant aortopulmonary collateral arteries.

On the basis of the calculated Nakata index, which of the following is the best intervention at this time?

A. Revision of the BT shunt

B. Placement of the left BT shunt

C. Place an RV to PA conduit and leave VSD open

D. VSD closure, placement of an RV to PA conduit, and RPA patch repair

E. Takedown of BT shunt and placement of a bidirectional cavopulmonary anastomosis.

   6. A 3-year-old patient undergoes aortic valve replacement with a tissue bioprosthesis. Two days later, he develops complete heart block with no propagation of electrical activity through the AV node. He is successfully resuscitated and taken to the catheterization laboratory. Angiography is most likely to reveal which of the following?

A. Normal coronary artery anatomy

B. Obstruction of the right coronary artery

C. Obstruction of the left main coronary artery

D. Obstruction of the left anterior descending coronary artery

E. Obstruction of the left circumflex coronary artery

   7. What surgical procedure is demonstrated by the angiogram in Figure 11.1.?

Figure 11.1.

A. BT shunt

B. Classic Glenn anastomosis

C. Bidirectional cavopulmonary anastomosis

D. Extracardiac modified Fontan anastomosis

E. Unifocalization procedure

   8. Which of the following operations carries the highest risk of post-operative sinus node dysfunction?

A. Atrial switch (Mustard) procedure

B. Repair of truncus arteriosus

C. Repair of tetralogy of Fallot

D. Repair of a large muscular VSD

E. Mitral valve mechanical prosthesis replacement

   9. You are meeting with the family of a 1-month-old child with hypoplastic left heart syndrome prior to stage I of the hybrid Norwood procedure. Which of the following best describes the role of the interventional pediatric cardiologist in this procedure?

A. Balloon dilation of the pulmonary arteries

B. Stenting of the coarctation of the aorta

C. Endoluminal pulmonary artery banding

D. Stenting of the ductus arteriosus

E. Aortic balloon valvuloplasty

 10. A 3-month-old patient is undergoing repair for tetralogy of Fallot and severe pulmonary stenosis. After initiation of cardiopulmonary bypass, using bicaval and aortic cannulation, the surgeon notes progressive left heart distention. This is most likely due to the presence of which of the following?

A. Persistent left superior vena cava

B. VSD

C. Patent foramen ovale

D. Aortic valve regurgitation

E. Aortopulmonary collaterals

 11. A 17-year-old boy who had complete repair of a partial AVSD at 15 months of age presents with progressive shortness of breath. He has a 2/6 systolic crescendo–decrescendo murmur that is less prominent with Valsalva. Chest x-ray reveals mild cardiomegaly and mildly increased pulmonary vascularity. Which of the following is the most likely cause of his symptoms?

A. Mitral regurgitation

B. Primary pulmonary hypertension

C. LVOT obstruction

D. Mitral stenosis

E. Residual ASD

 12. A 2-month-old girl is found to have a moderate-large perimembranous VSD with left-to-right shunt. She has mild left ventricular and left atrial enlargement and an estimated right ventricular systolic pressure of 38 mm Hg by Doppler echocardiography. She is growing appropriately and has no respiratory distress with feedings. In the absence of heart failure, by what age should she have surgical repair of her VSD?

A. 3 months

B. 12 months

C. 18 months

D. 2 years

E. 3 years

 13. A 2-week-old infant is found to have anomalous left coronary artery from the pulmonary artery (ALCAPA). Which of the following pre-operative findings is most closely associated with post-operative mortality and need for late re-operation?

A. Mitral insufficiency

B. Tricuspid insufficiency

C. Aortic valve insufficiency

D. Pulmonary valve insufficiency

E. Pulmonary valve stenosis

 14. What is the most common long-term complication in repaired Scimitar syndrome?

A. Atrial arrhythmia

B. Right ventricular systolic dysfunction

C. Pulmonary vein obstruction

D. IVC–RA junction stenosis

E. Pulmonary hypertension

 15. A 3-year-old child has complex single ventricle, bilateral superior venae cavae, and interrupted IVC with azygous continuation to the right SVC. Initial palliation consisted of placement of bilateral bidirectional cavopulmonary anastomoses. Post-operatively his oxygen saturation is 87%. Now his saturation is 75%. Which of the following is most likely to have contributed to his progressive desaturation?

A. Erythrocytosis

B. Intrapulmonary shunting

C. Decreased chest wall compliance

D. Increased coronary sinus drainage

E. Increased pulmonary vascular resistance

 16. You are asked to evaluate a 4-year-old child who recently moved to the USA from Russia. He has tricuspid atresia, normally related great arteries and pulmonary stenosis. At 3 weeks of age, he had a modified BT shunt. His height and weight are in the 15th percentile. The left ventricular impulse is slightly overactive. S1 is normal, S2 is single, and there is a 2/6 continuous murmur at the base of the heart. The liver is 1 cm below the right costal margin. The hemoglobin is 17 g/dL. The data in Table 11.1 are obtained at the time of cardiac catheterization.

Table 11.1 Cardiac Catheterization Data

An angiogram reveals normal size and distributed pulmonary arteries. An echocardiogram reveals an LV ejection fraction of 60%. Which of the following would you recommend?

A. Perform a bidirectional Glenn and takedown of the BT shunt

B. Delay any operative intervention until the hemoglobin reaches 19 g/dL

C. Perform an atriopulmonary connection and closure of the ASD and takedown of the BT shunt

D. Perform an extracardiac fenestrated Fontan and takedown of the BT shunt

E. Perform an extracardiac nonfenestrated Fontan and takedown of the BT shunt

 17. You are asked to evaluate a 4-year-old child who recently moved from Russia. He has tricuspid atresia, normally related great arteries and pulmonary stenosis. At 9 months of age, he had a classic Glenn anastomosis. An echocardiogram reveals normal left ventricular systolic function. The data in Table 11.2 are obtained at the time of cardiac catheterization.

Table 11.2 Cardiac Catheterization Data

Which of the following is true?

A. The patient is not a candidate for a modified Fontan operation.

B. The right lower pulmonary vein should be allowed to drain to the systemic venous side after modified Fontan operation.

C. Completion of a lateral tunnel nonfenestrated Fontan will result in complete separation of arterial (oxygenated) and venous (deoxygenated) circulations.

D. Perfusion of the right lung with hepatic effluent blood should improve systemic arterial oxygen saturation.

E. The Glenn anastomosis should be left intact and a modified BT shunt should be placed from the left subclavian artery to the right pulmonary artery

 18. A 1-day-old infant is diagnosed with tricuspid atresia, normally related great arteries and a large ASD. Oxygen saturation is 60% to 65% on room air. On examination, the child is cyanotic but well-perfused. Chest x-ray shows diminished pulmonary vascularity. Which of the following will this patient most likely require as the first palliative intervention?

A. Balloon atrial septostomy

B. Modified BT shunt placement

C. Pulmonary artery banding

D. Damus–Kaye–Stansel (DKS) anastomosis

E. DKS anastomosis with pulmonary artery banding

 19. A neonate is diagnosed with tricuspid atresia and d-transposition of the great arteries (d-TGA). On examination, you note poor perfusion and a loud, harsh ejection-type murmur. Oxygen saturation is 85% on room air. Which of the following is the best initial palliation for this patient?

A. Surgical enlargement of VSD

B. Modified BT shunt

C. Pulmonary artery banding

D. DKS anastomosis with modified BT shunt

E. DKS anastomosis with pulmonary artery banding

 20. A 2-year-old male undergoes successful surgical valvotomy for a stenotic dysplastic pulmonary valve resistant to balloon dilation. Post-operative echocardiography documents mild valvular regurgitation with a predicted gradient across the valve of 10 mm Hg. What is the likelihood that this child will need re-intervention on his pulmonary valve within the next 10 years?

A. 95%

B. 80%

C. 50%

D. 20%

E. 5%

 21. A 4-year-old child has pulmonary atresia and intact ventricular septum. She had placement of a BT shunt as a neonate. Her pulmonary artery trunk diameter is 10 mm. Pulmonary arteriolar resistance is 2.5 WU*m2. Her hemoglobin is now 20 g/dL. Tricuspid valve Z-score is −4. Which of the following is the best next step in this patient’s management?

A. Partial exchange transfusion with a goal hemoglobin of 15 g/dL

B. Routine follow-up until symptoms develop

C. Bidirectional cavopulmonary anastomosis with takedown of the BT shunt

D. ASD closure, tricuspid valve repair or replacement, and RV outflow tract reconstruction

E. Modified (extracardiac) Fontan and BT shunt takedown

 22. A 3-month-old infant has pulmonary atresia with intact ventricular septum and status a post-modified right BT shunt placement. His oxygen saturation is 70% on room air. An angiogram documents confluent pulmonary arteries with valvular atresia. The right ventricle is tripartite but diminutive. The tricuspid valve is well developed with moderate regurgitation and an annulus Z-score of −2.0. There is no evidence of right ventricle–dependent coronary circulation. Which of the following is the best next treatment choice for this patient?

A. Left-sided modified BT shunt placement

B. Central shunt placement

C. RV outflow reconstruction with BT shunt takedown

D. Unifocalization procedure with RV outflow reconstruction

E. Bidirectional cavopulmonary anastomosis with BT shunt takedown

 23. The angiogram in Figure 11.2. is performed in a 4-month-old boy with pulmonary atresia with intact ventricular septum and a large patent ductus arteriosus. Pulmonary arteriolar resistance is 3 WU*m2.

Figure 11.2.

Which of the following operations is best for this patient at this time?

A. Pulmonary valvotomy, modified BT shunt placement

B. PDA ligation, bidirectional Glenn anastomosis

C. PDA ligation, RV to PA conduit placement

D. PDA ligation, pulmonary valvotomy with a bidirectional Glenn

E. PDA ligation, bidirectional Glenn anastomosis, RV to PA conduit placement

 24. A 3-year-old child has pulmonary atresia with VSD. He has a history of hypoplastic central pulmonary arteries and multiple major aortopulmonary collateral arteries (MAPCAs), with multiple surgeries including a central shunt and bilateral unifocalization procedures. He undergoes reconstruction of the central confluence, placement of RV–PA conduit, ligation of two MAPCAs, and VSD closure. When cardiopulmonary bypass is discontinued, his blood pressure is 84/60 mm Hg on multiple inotropic agents. Arterial blood oxygen saturation is 87% on 100% inhaled oxygen. Right ventricular systolic pressure is 69 mm Hg by direct measurement. TEE demonstrates patency of the conduit. Which of the following is the best next step?

A. Placement of ECMO until hemodynamics improve

B. Re-opening the VSD

C. Takedown of RV–PA conduit and re-placement of a central shunt

D. Replacement of the RV–PA conduit with a larger conduit

E. Treatment with nitric oxide to improve pulmonary vascular resistance

F. Removal of the MAPCA ligatures

 25. A neonate with prenatally diagnosed truncus arteriosus is born at 38 weeks gestation via uncomplicated vaginal delivery. On his post-natal echocardiogram, the pulmonary arteries arise from a common trunk and are unobstructed with increased, laminar flow. The aortic arch is right-sided. The truncal valve is quadricuspid with moderate regurgitation. There are no other complicating factors. Which of the following is the best initial surgical intervention for this infant?

A. BT shunt placement within the first week of life

B. Truncal valve repair by 1 month of age

C. Complete repair by 1 month of age

D. Bidirectional cavopulmonary anastomosis at 4 months of age

E. Pulmonary artery banding by 6 months of age

 26. Which of the following is the most common indication for re-operation in a patient with a history of truncus arteriosus repair?

A. Neo-aortic valve regurgitation

B. Neo-aortic valve stenosis

C. RV–PA conduit failure

D. Residual VSD

E. Branch pulmonary artery stenosis

 27. An asymptomatic 1-year-old child is referred for a murmur. Her blood pressure is 104/56 mm Hg (left arm) and 84/50 mm Hg (left leg). The echocardiogram confirms discrete coarctation of the aorta. Assuming the patient remains asymptomatic without progression of upper extremity hypertension, when would you recommend surgical repair?

A. Age 2 to 3 years

B. Age 5 to 7 years

C. Age 12 to 14 years

D. Only operate when systolic pressure gradient > 40 mm Hg

E. Only operate when she develops moderate or worse left ventricular hypertrophy

 28. You are discussing the mortality risks to parents of a newborn with hypoplastic left heart syndrome. They ask about the risk of dying during the various stages of the surgical Norwood procedure. You explain that current data suggest that the highest risk of mortality is during which of the following periods?

A. Prior to stage 1 palliation

B. Between stage 1 and stage 2 palliation

C. During stage 2 palliation

D. Between stage 2 and stage 3 palliation

E. During stage 3 palliation

 29. One day after a hybrid Norwood palliation procedure, a 7-day-old term neonate develops progressive cyanosis and metabolic acidosis. He is tachypneic with an oxygen saturation of 65% on room air. His heart rate is 160/minute, blood pressure is 65/42 mm Hg. His chest x-ray demonstrates increased pulmonary vascular markings. Which of the following best explains his worsening clinical status?

A. Pulmonary hypertension

B. PDA closure/occlusion

C. PA branch obstruction

D. Restrictive ASD

E. Hypovolemia

 30. Which of the following is a goal of stage 1 surgical palliation (Norwood) for hypoplastic left heart syndrome?

A. Restriction of coronary artery blood flow

B. Creation of a nonrestrictive VSD

C. Establishment of an unrestricted source of pulmonary blood flow

D. Relief of ductal-dependent systemic blood flow

E. Unrestricted right-to-left atrial level shunt

 31. Which of the following is the most common short-term complication of arterial level repair of d-TGA?

A. Supravalvular pulmonary stenosis

B. Supravalvular aortic stenosis

C. Neo-aortic root dilation

D. Neo-aortic valve regurgitation

E. Coronary artery occlusion

 32. While being weaned off cardiopulmonary bypass following an arterial switch procedure for d-TGA, a neonate has persistent hypotension and poor left ventricular function with regional wall motion abnormalities. Which of the following best explains this patient’s clinical status?

A. Presence of ASD/PFO

B. Cerebral air embolism

C. Severe pulmonary regurgitation

D. Kinking or occlusion of coronary arteries

E. Global dysfunction due to cardiopulmonary bypass

 33. A neonate is found to have d-TGA with VSD and ASD. The VSD is nonrestrictive, but there is severe left ventricular outflow obstruction. The patient’s oxygen saturation is 68% on room air. What is the most appropriate initial intervention for this patient?

A. Jatene arterial switch with Lecompte maneuver

B. Mustard operation

C. LV outflow tract balloon dilation

D. PDA stent

E. BT shunt

 34. A 2-year-old child with L-TGA, large membranous VSD with outlet extension, and history of critical pulmonary stenosis has been palliated with a modified BT shunt in the neonatal period and a bidirectional cavopulmonary anastomosis at 6 months of age. His oxygen saturation is 70%. He has good biventricular size and function, and no AV valve straddling. He is presenting for surgical intervention. Which of the following is a reasonable alternative to a modified Fontan operation for this patient?

A. Closing the VSD to the pulmonary valve

B. Closing the VSD to the aortic valve (Rastelli-type repair) and placement of a pulmonary conduit

C. Closing the VSD to the pulmonary valve and baffling IVC to the tricuspid valve

D. Closing the VSD to the aortic valve, placement of a pulmonary conduit, and baffling of IVC to the tricuspid valve

E. Closing the VSD to the aortic valve, placement of a pulmonary conduit, and baffling of IVC to the mitral valve

 35. You are performing an echocardiogram on a ductal-dependent newborn infant in the neonatal ICU. You diagnose double-outlet right ventricle with normally related great arteries and large doubly committed VSD, severe coarctation of the aorta, mild subaortic stenosis, and parachute mitral valve which straddles the VSD. What is the most appropriate initial surgery for this patient?

A. Patch VSD to aorta, repair coarctation

B. Repair coarctation, mitral valve annuloplasty, and chord lengthening

C. Repair coarctation only

D. Norwood palliation with Sano shunt

E. Patch VSD to aorta, repair coarctation, resection of subaortic stenosis

 36. What is the surgical procedure of choice for patients with Taussig–Bing anomaly?

A. Pulmonary artery banding with enlargement of the VSD

B. Arterial switch with baffle closure of the VSD to the neo-aorta

C. Systemic to pulmonary (BT) shunt

D. Patch closure of the VSD

E. Aortopulmonary anastomosis and pulmonary artery banding

 37. A term neonate with a harsh systolic murmur at birth is found to have double-inlet left ventricle with a hypoplastic subaortic right ventricle, a restrictive bulboventricular foramen, and severe subaortic stenosis. Prostaglandin-E1 infusion is started. A subsequent echocardiogram documents a large ductus arteriosus. Which of the following is the most appropriate initial operation for this child?

A. Enlargement of the VSD

B. Pulmonary artery banding

C. Bidirectional cavopulmonary anastomosis

D. PDA stent placement and banding of the PAs (hybrid Norwood)

E. Aortopulmonary anastomosis (DKS) with BT shunt

 38. A 5-year-old female is found to have a murmur during a routine physical examination. Echocardiogram reveals a secundum ASD with continuous right-to-left shunt. Which of the following factors would be the strongest indication for surgical repair rather than percutaneous closure of the ASD?

A. Patient’s age

B. Deficient anterior septal rim

C. History of dyspnea on exertion

D. Moderate tricuspid valve regurgitation

E. Estimated pulmonary artery pressure = 40 mm Hg

 39. A child with tricuspid atresia has been palliated with a right-sided bidirectional cavopulmonary anastomosis. He is found to anomalous connection of the right lower pulmonary vein to the inferior vena cava. Placement of an extracardiac Fontan conduit at this time would most likely result in which of the following?

A. Thrombosis of the conduit

B. Infarction of the right lower lobe of the lung

C. Persistent cyanosis due to right-to-left shunt

D. Increased pulmonary blood flow due to right-to-left shunt

E. Pulmonary hypertension due to elevated left atrial pressure

 40. A 31-year-old female with a history of coarctation of the aorta has a chest x-ray performed for chronic cough (see Figure 11.3.).

Figure 11.3.

Which of the following interventions was she most likely to have previously undergone?

A. Percutaneous balloon dilation of coarctation

B. Percutaneous coarctation stent placement

C. Subclavian flap repair of coarctation

D. Synthetic patch repair of coarctation

E. Coarctation resection and end-to-end reanastomosis

 41. A 17-year-old male presents with a history of heart surgery. He states that he had an operation on his pulmonary valve, but he is uncertain about the specifics. As part of his evaluation, the electrocardiogram in Figure 11.4. is obtained.

Figure 11.4.

On the basis of these findings, which of the following interventions is he most likely to have undergone?

A. Balloon pulmonary valvuloplasty

B. Right ventricular outflow tract patch enlargement

C. Pulmonary valve replacement

D. Surgical ASD repair

E. Bicaval cannulation

 42. Which of the following is the most frequently recognized post-operative complication after an atrial switch operation for d-TGA?

A. SVC baffle obstruction

B. IVC baffle obstruction

C. Tricuspid valve regurgitation

D. Mitral valve regurgitation

E. Tricuspid valve stenosis

 43. A term infant is found to have arterial blood oxygen saturations in the mid-80s despite 100% inhaled oxygen. An echocardiogram reveals Type 2 truncus arteriosus. Over the next 48 hours, oxygen saturations increase to the high 80s on room air. Which of the following interventions is the best next step for this patient?

A. Subambient oxygen prior to BT shunt placement

B. Pulmonary artery banding within the first month of life

C. Pulmonary artery banding between 6 and 12 months of life

D. Complete repair within the first month of life

E. Complete repair between 6 and 12 months of life

 44. A 3-month-old child presents with congestive heart failure and is diagnosed with a large VSD in addition to the anomaly that is shown in Figure 11.5. In addition to repair of the VSD, which of the following interventions best addresses his coexisting anomaly?

Figure 11.5.

A. Dissection and reimplantation of the right coronary artery

B. Dissection and reimplantation of the left coronary artery

C. Unroofing of the proximal RCA

D. Unroofing of the proximal LCA

E. No intervention, as the risk of repair outweigh the benefits

 45. A 5-year-old child has a sinus venosus ASD with anomalous pulmonary venous drainage. You explain the Warden repair of this defect with the parents. Which of the following maneuvers is incorporated into this procedure?

A. Transection of the anomalous pulmonary veins and reimplantation into the posterior left atrium

B. Transection of the anomalous veins and reimplantation into the posterior right atrium, then baffling of the veins across the ASD

C. Transection of the SVC above the anomalous vein insertion and anastomosis of the SVC to the right atrial appendage

D. Baffling of the anomalous pulmonary veins from their SVC connection to the ASD

E. Patch closure of the defect between the right upper pulmonary vein and the SVC

 46. Which of the following findings in a neonate with d-TGA would be best addressed by a Lecompte maneuver as part of the surgical repair?

A. Anterior–posterior relationship of the semilunar valves

B. Large subarterial VSD

C. Interarterial course of left coronary artery

D. Pulmonary valve stenosis

E. Right aortic arch

 47. Among neonates who undergo the Ross procedure for aortic stenosis, which of the following is the most common indication for re-operation?

A. Arrhythmia

B. Subaortic stenosis

C. Neo-aortic valve failure

D. Coronary artery obstruction

E. Pulmonary homograft failure

 48. Which of the following modifications to repair of Ebstein’s anomaly is most beneficial in patients with a history of supraventricular tachyarrhythmia?

A. MAZE procedure

B. Right reduction atrioplasty

C. Right ventricle plication

D. Tricuspid valve annuloplasty

E. Starnes procedure

Answers

ANSWER: A.  A significant concern during the repair of membranous VSDs, particularly when performed on young infants, is damaging the AV node when suturing the patch in place. The AV node courses along the posterior–inferior rim of membranous VSDs. Damage to the node could result in high-grade AV block immediately or shortly after surgical repair.

ANSWER: B.  Although no longer performed in the USA, many adults with congenital heart disease have had surgical creation of direct aorta to pulmonary artery communication, either via an ascending aorta to right pulmonary artery connection (Waterston shunt) or via a descending aorta to left pulmonary artery connection (Potts shunt). These techniques have since been abandoned, due to difficulty regulating the size of the shunt and a high rate of branch pulmonary stenosis. Inappropriately large surgical shunts carry a high risk of pulmonary hypertension and, ultimately, pulmonary vascular obstructive disease. Central shunts and modified BT shunts utilize synthetic shunts of specific size, allowing for more predictable shunt volume. Classic BT shunts (direct connection of the left or right subclavian artery to the ipsilateral branch pulmonary artery) were frequently complicated by shunt obstruction and are no longer performed. Pulmonary valvectomy (with VSD closure) results in severe pulmonary regurgitation with resultant RV enlargement and dysfunction, but does not increase the risk of pulmonary hypertension.

ANSWER: E.  Modern surgical repair of AVSDs has resulted in tremendous improvement in life expectancy and quality of life for children (and now adults) with Down syndrome. The need for late re-operation after complete AVSD repair is approximately 15% to 20%. While small residual atrial or ventricular shunts may persist after repair and right AV valve (tricuspid) regurgitation may be present, they are uncommon indications for re-operation. Unlike in partial AV septal defects, LVOT obstruction is an infrequent indication for re-operation among patients with complete AV canal defects. Left AV valve (mitral) regurgitation, on the other hand, is the most common reason for late re-operation.

ANSWER: B.  Intuitively, severe pre-operative AV valve regurgitation predicts post-operative AV valve regurgitation. A cleft in the left AV valve is universal in AV canal defects and does not predict post-operative regurgitation. While the use of an annuloplasty ring in repair of the left AV valve may reflect the degree of pre-operative regurgitation, it does not independently predict post-operative regurgitation. Left ventricular size and hemodynamics may influence the degree of mitral regurgitation, but this association is not so strong.

ANSWER: D.  The Nakata index is commonly used to predict operability in patients with pulmonary atresia-VSD. Angiographic measurements of the central pulmonary arteries just proximal to the first lobar branches (and any MAPCAs that perfuse an entire pulmonary segment and can be unifocalized) are taken, and the cross-sectional area of each branch (π × radius2) is calculated. The sum of these areas is divided by body surface area:

The calculation for this patient is as follows:

Patients with a Nakata index >200 are generally considered good candidates for complete repair (including unifocalization, if necessary). Patients with an index <200 may be candidates as well, but are at higher risk of pulmonary hypertension and right heart failure. They may be better off without surgical intervention or with limited surgical palliation. For the patient in the scenario, RPA patch angioplasty or stent placement would be indicated as part of the repair.

ANSWER: B.  Post-operative heart block, ST segment changes, or ventricular dysfunction with regional wall motion abnormalities should raise concern of compromised coronary artery perfusion. This may result from mechanical compression or obstruction by a prosthetic valve, transection of a coronary artery, or tension and kinking with coronary artery reimplantation. The child in this vignette presents a history typical of coronary artery obstruction after a procedure (aortic valve replacement) that presents risk of the same. While definitive identification of the compromised artery requires angiography, the AV node is supplied by a branch of the right coronary artery in 90% of humans, making disruption of the RCA the most likely cause of AV node dysfunction in this patient.

ANSWER: C.  The image demonstrates a connection between the superior vena cava and the right pulmonary artery with continuity between the RPA and the LPA—a modified bidirectional cavopulmonary (Glenn) anastomosis. A (modified) BT shunt consists of synthetic graft connecting the right or the left subclavian artery with the ipsilateral pulmonary artery. A classic Glenn anastomosis consists of transection of the SVC and RPA and end-to-end anastomosis, excluding the LPA from receiving SVC venous return. An extracardiac Fontan consists of a modified Glenn and a graft directing IVC venous return to the pulmonary arteries. A unifocalization procedure is most often performed in patients with MAPCAs and involves incorporation of these MAPCAs into a central confluence, to be supplied by a systemic–pulmonary (e.g., BT) shunt or RV–PA conduit.

ANSWER: A.  The sinus node is located in the posterior right atrium along the superior-lateral aspect of the superior vena cava. As a result, surgical disruption of this area may result in damage to the sinus node. Of the choices available, only the atrial switch procedure affects the posterior aspect of the right atrium. In the Mustard/Senning atrial switch operations, systemic venous return is directed across the atrial septum to the left-sided, subpulmonary ventricle. This is done by suturing a patch baffle along the posterior (sinus venosus) wall of the right atrium, in close proximity to the sinus node.

ANSWER: D.  The hybrid Norwood procedure has been developed recently as an alternative to the traditional stage 1 Norwood procedure and reduces early-stage mortality in high-risk neonates. The goal of the hybrid procedure is the same as the traditional stage 1 procedure—to provide systemic blood flow from the right ventricle while maintaining adequate, but not excessive, pulmonary blood flow. During the hybrid Norwood procedure, the cardiac surgeon opens the sternum, exposing the heart and pulmonary arteries. The surgeon then bands the right and left pulmonary artery branches, to limit the amount of pulmonary blood flow. The role of the interventional cardiologist is to place a stent within the ductus arteriosus to maintain patency. This allows for the child to discontinue prostaglandin therapy in anticipation of dismissal from the hospital. The hybrid procedure takes place without the use of cardiopulmonary bypass and circulatory arrest, thus minimizing the associate risks.

10 ANSWER: E.  The role of the cardiologist in the operating room includes providing accurate echocardiographic description of cardiac anatomy, particularly the presence of shunts that may complicate cardiopulmonary bypass. In this scenario, the patient develops left heart distention after being placed on bypass, which suggests ongoing pulmonary venous return to the left atrium. This results from persistent pulmonary blood flow that is not accounted for in the bypass circuit. The most likely cause in this situation is a systemic to pulmonary shunt, such as aortopulmonary collateral arteries. If the aorta is not cross-clamped, significant aortic valve regurgitation may result in left ventricular distension, but this is less likely to be the case for this patient. The other options are important findings to note prior to bypass, but would not cause left heart distension. A persistent left SVC typically drains to the coronary sinus and would result blood return to the right atrium.

11 ANSWER: C.  While the most common cause of re-operation in patients with partial AV canal defects is mitral valve regurgitation, LVOT obstruction is a common cause and much more common than in the complete form of AVSD. Shortness of breath, cardiomegaly, and increased pulmonary vascularity may be caused by mitral valve regurgitation or LVOT obstruction. However, the ejection-type systolic murmur that diminishes with Valsalva presented in this patient clearly suggests outflow tract obstruction as the underlying problem. Primary pulmonary hypertension is very rare in children, and one would expect to find diminished pulmonary vascularity on chest x-ray. Mitral stenosis may cause this patient’s symptoms, but it is a less common late finding after partial AV canal defect repair and not suggested by the other findings in the vignette. A residual ASD could cause this patient’s symptoms if it was large enough, but, again, LVOT obstruction is a more common post-operative complication.

12 ANSWER: D.  VSD is the most common congenital heart defect. Children often present with a murmur in the first couple of months of life or sometimes with congestive heart failure, in the case of large (nonrestrictive) VSDs. While surgical repair of VSDs is often dictated by the presence of symptoms, such as failure to thrive, some children may continue to grow in the presence of a moderate VSD. Closure of the defect in those cases is necessary to prevent irreversible pulmonary vascular obstructive disease (Eisenmenger syndrome). While surgical repair is often performed at 1 year of age, the risk of Eisenmenger syndrome does not increase significantly until after 2 years of age.

13 ANSWER: A.  ALCAPA typically presents in the second or third month of life after pulmonary vascular resistance falls and the anomalous coronary artery loses perfusion pressure. Infants typically present with a dilated, poorly functioning left ventricle caused by myocardial ischemia. These children are at risk of ischemia and infarction of the mitral valve papillary muscles and resultant mitral valve regurgitation. The tricuspid valve papillary muscles are usually perfused by branches of the right coronary artery.

14 ANSWER: C.  Scimitar syndrome is the eponym for anomalous right pulmonary veins connecting to the inferior vena cava. Surgical repair typically depends on the proximity of the anomalous venous connection to the right atrium and the presence of an ASD. Typically the anomalous connection is transected and the right veins are reimplanted, either into the right or into the left atrium. If implanted into the right atrium, the right pulmonary venous return is then directed across an ASD into the left atrium by a patch baffle. The most common complication of this type of repair is obstruction of pulmonary venous return.

15 ANSWER: B.  The development of pulmonary arteriovenous fistulae has been identified as a risk of the classic cavopulmonary anastomosis (Glenn). Subsequently, it has been found to relate to the absence of hepatic effluent blood from the pulmonary circulation. While this patient had bilateral modified (bidirectional) Glenn anastomoses, the absence of hepatic venous return within the pulmonary circulation (due to IVC interruption) results in a similar lack of the hepatic factor that would otherwise prevent the development of the pulmonary AV fistulae. When surgically feasible, incorporation of hepatic venous return into the pulmonary circulation often results in diminution of the fistulae.

16 ANSWER: E.  The questions being posed by this scenario are whether this patient is a candidate for a Fontan-type palliation and how should the operation be performed. This patient’s cardiac hemodynamics are favorable for single-ventricle palliation, specifically the PA pressure is low (mean 11 mm Hg) with a low transpulmonary gradient (5 mm Hg). This is not surprising in the context of pulmonary stenosis, which protects the pulmonary vascular bed from systemic pressure. While infants presenting with single-ventricle physiology typically are repaired in a staged fashion, consisting of a BT shunt shortly after birth, Glenn anastomosis by 6 to 9 months and Fontan completion in young childhood. As this child is 3 years old, there is no need to perform a Glenn anastomosis prior to Fontan completion. The modified Fontan, using an extracardiac conduit or a lateral tunnel approach is favored over a direct RA to PA anastomosis (classic Fontan). The question of when to fenestrate a Fontan connection is still debated, but it is clearly not indicated in children with optimal hemodynamics, such as in this patient.

17 ANSWER: D.  The development of pulmonary arteriovenous fistulae has been identified as a risk of the classic cavopulmonary anastomosis (Glenn), which consists of a direct, end-to-end anastomosis of the SVC to the right pulmonary artery. Subsequently, it has been found to relate to the absence of hepatic effluent blood from the pulmonary circulation, which contains a hepatic factor that prevents the development of these fistulae. Incorporation of hepatic venous return into the pulmonary circulation often results in diminution of the fistulae and improvement in systemic saturation as a result.

18 ANSWER: D.  The vignette above describes a neonate with single ventricle and inadequate pulmonary blood flow, as demonstrated by the child’s oxygen saturation and paucity of pulmonary vascularity. This may result from valvular stenosis or obstruction at the VSD (bulboventricular foramen). The initial palliation, therefore, should be directed at increasing pulmonary blood flow. This is best accomplished by placing a modified BT shunt from the subclavian artery to the branch pulmonary artery. Balloon atrial septostomy may be necessary if the ASD is restrictive, but this is not the case with this patient. Pulmonary artery banding would decrease pulmonary blood flow. A DKS anastomosis consists of a direct ascending aorta to MPA (end-to-side) anastomosis. This is used in single-ventricle situations where there is left ventricular outflow obstruction (subaortic stenosis, valvular stenosis/atresia, coarctation) and allows for retrograde perfusion of the coronary arteries via a reconstructed “neo-aortic” arch.

19 ANSWER: D.  A DKS anastomosis consists of a direct ascending aorta to MPA (end-to-side) anastomosis. This is used in single-ventricle situations where there is systemic outflow obstruction (subaortic stenosis, valvular stenosis/atresia, coarctation) and allows for retrograde perfusion of the coronary arteries via a reconstructed “neo-aortic” arch. After a DKS anastomosis, the native pulmonary valve functions as the “neo-aortic” valve. The neonate in the vignette has tricuspid atresia with d-TGA—the aorta arises from the diminutive right ventricle. Since the tricuspid valve is atretic, systemic outflow is dependent on the size of the VSD (bulboventricular foramen). This patient presents with adequate pulmonary blood flow, as evidenced by a systemic saturation of 85%, but with signs of poor systemic perfusion and a harsh ejection-type murmur. This may result from a restrictive VSD, subvalvular aortic stenosis, or aortic valve stenosis. In this situation, a DKS anastomosis allows for adequate systemic output and coronary artery perfusion. A BT shunt placed at the same time provides a stable source of pulmonary blood flow once the MPA is separated from the PA branches. Surgical enlargement of the VSD risks damage to the cardiac conduction system causing rhythm disturbances and ventricular dysfunction. A BT shunt alone does not address the problem of systemic outflow obstruction. PA banding would not be indicated as the primary problem is inadequate systemic output, not excessive pulmonary blood flow.

20 ANSWER: E.  Pulmonary valve stenosis can be managed initially with percutaneous pulmonary balloon valvuloplasty or surgical valvotomy. Percutaneous valvuloplasty tends to produce better relief of stenosis, but patients are often left with a greater degree of regurgitation than the surgical approach. A good initial surgical outcome, as described in this patient, carries a low risk of need for future operation, probably not more than 5% over the next 10 years.

21 ANSWER: E.  The type of surgical intervention indicated depends on a patient’s age, hemodynamics, and underlying cardiac anatomy. The vignette describes a child with pulmonary atresia and intact ventricular septum. These patients may qualify for a two-ventricle–type repair with placement of a conduit from the right ventricle to the pulmonary arteries (which may be confluent or surgically unifocalized). If the right ventricle is not usable as a functional pumping chamber, either because of hypoplasia or because of coronary to RV fistulae, then the patient is not a candidate for complete repair and requires some form of palliation. This patient has a very diminutive RV, demonstrated by a tricuspid valve Z-score of −4. It has been shown that when the tricuspid annulus Z-score is less than −3, the outcome is very poor after attempted complete repair. As a result, this patient should undergo palliation. Her low pulmonary vascular resistance suggests that she is a good candidate for a Fontan operation. At 4 years of age, a complete Fontan can be performed as a single operation, rather than first performing a staged bidirectional cavopulmonary anastomosis.

22 ANSWER: C.  Patients with pulmonary atresia and intact ventricular septum may qualify for a two-ventricle–type repair with placement of a conduit from the right ventricle to the pulmonary arteries (which may be confluent or surgically unifocalized). If the right ventricle is not usable as a functional pumping chamber, either because of hypoplasia or because of coronary to RV fistulae, then the patient is not a candidate for complete repair and requires some form of palliation. It has been shown that when the tricuspid annulus Z-score is less than −3, the outcome is very poor after attempted complete repair. Infants with a Z-score of −2 to −2.5 and a functional tricuspid valve may undergo placement of an RV to PA conduit. This patient has a small but usable RV, demonstrated by a tricuspid valve Z-score of −2 and the absence of coronary-cameral fistulae (RV-dependent coronary circulation). Low oxygen saturation suggests that he is outgrowing his BT shunt and should have the next stage operation, in his case a two-ventricle complete repair.

23 ANSWER: B.  The angiogram demonstrates a very small RV chamber with multiple RV–coronary fistulae. These fistulae are common in pulmonary atresia with intact ventricular septum, where the RV is severely hypoplastic. Decompression of the RV by surgical creation of an RV outflow tract (either by pulmonary valvotomy or placement of a conduit) would decrease coronary perfusion pressure and cause diffuse myocardial ischemia. This patient should continue down a single-ventricle palliation pathway, with placement of a bidirectional cavopulmonary anastomosis as a first stage. Given his age, he does not need a BT shunt prior to placement of the Glenn anastomosis.

24 ANSWER: B.  Successful outcome after complete repair of pulmonary atresia and VSD has been shown to correlate with the post-operative right ventricular systolic pressure. When RVSP is above 70% of systemic pressure, outcome is very poor. Under these circumstances, the surgeon should re-open the VSD to decrease RV workload. If pulmonary resistance cannot be improved by medical means, these patients should have surgical palliation rather than complete repair. The patient described is at high risk of pulmonary hypertension given the history of hypoplastic pulmonary arteries. The first step in the operating room should be reopening the VSD, given the near-systemic RV pressure.

25 ANSWER: C.  Complete repair of truncus arteriosus has become the favorable approach in US congenital cardiac surgery centers. This patient will need intervention early in life to prevent irreversible pulmonary vascular obstructive disease. Historically, these children underwent pulmonary artery banding procedures within the first month of life. Now, complete repair within the first month of life – with VSD closure, repair of the truncal (neo-aortic) valve, and placement of a conduit from the RV to the pulmonary arteries – is the operation of choice in most centers. This child has nonrestrictive pulmonary blood flow and does not need a BT shunt or Glenn anastomosis to improve pulmonary blood flow.

26 ANSWER: C.  All of the choices listed are potential indications for re-operation after repair of truncus arteriosus. Homograft or synthetic conduits tend to last 10 to 15 years but almost universally become stenotic and/or regurgitant. Neo-aortic valve regurgitation is very common, but often can be managed without surgical intervention until it becomes moderate or severe. Stenosis of the neo-aortic valve is less common than regurgitation. Residual VSDs and branch PA stenosis are infrequently reasons for re-operation.

27 ANSWER: A.  In the absence of progression of symptoms, significant, discrete coarctation of the aorta should be repaired prior to 3 years of age. Operation in young childhood decreases the risk persistent post-repair rebound hypertension and myocardial dysfunction due to LV hypertrophy. A systolic gradient >40 mm Hg and progressive LV hypertrophy are indications for coarctation repair, but surgery may still be indicated in the absence of these findings.

28 ANSWER: B.  The classic Norwood procedure for hypoplastic left heart syndrome consists of three stages. Stage 1 consists of excision of any remaining atrial septal tissue, separation of the pulmonary arteries from the MPA trunk, placement of a BT shunt to the branch PAs, and construction of a neo-aortic arch using the MPA tissue. The right ventricle then serves as the systemic ventricle and the pulmonary valve functions as the systemic (neo-aortic) valve. This stage is usually performed within the first week of life in term neonates without comorbidities. Stage 2 consists of takedown of the BT shunt and placement of an SVC to PA anastomosis (bidirectional cavopulmonary anastomosis). This typically occurs around 6 to 9 months of age, depending on the patient growth and degree of cyanosis. The Norwood is completed in stage 3 with anastomosis of the IVC to the PAs via an extracardiac conduit or tunnel along the lateral wall of the right atrium (modified Fontan). This final stage may be performed as early as 2 years of age. As one would expect, the highest mortality occurs early during the Norwood stages. Specifically, it has been shown to be highest after stage 1 and prior to stage 2.

29 ANSWER: D.  This child is showing signs of poor perfusion and cyanosis. The presence of tachypnea and increased pulmonary vascularity suggest pulmonary congestion from pulmonary venous obstruction. In children with hypoplastic left heart syndrome, the only way for pulmonary venous return to reach the systemic circulation is through an ASD. A small, restrictive ASD would best explain the findings in this child.

30 ANSWER: D.  Neonates with hypoplastic left heart syndrome have no left ventricular output, so systemic blood flow is derived from RV/pulmonary artery output via a patent ductus arteriosus. This circulation is adequate in utero, but is not a stable situation post-natally. As a result the goal of the first stage of the Norwood palliation is to provide a stable source of systemic blood flow. Surgically, this is done by separating the branch pulmonary arteries from the MPA trunk, placing a BT shunt to the branch PAs, and creating a neo-aortic arch by enlarging the native aortic arch using tissue from the MPA trunk. After this procedure, neonates do not require ongoing prostaglandin-E1 infusion and can be dismissed from the hospital.

31 ANSWER: A.  The arterial switch procedure has replaced the atrial switch (Mustard/Senning) as the repair of choice for d-TGA. It provides a more anatomic repair, with the left ventricle serving as the systemic ventricle and the right ventricle as the pulmonary ventricle. Complications of the arterial switch are primarily related to the mobilization and relocation of the great arteries and coronary arteries. In d-TGA the aorta is located anterior to the pulmonary artery. Relocation of the pulmonary artery anteriorly introduces the risk of supravalvar stenosis as the artery is stretched behind the ascending aorta. For this reason, the Lecompte maneuver is sometimes used. This is performed by transecting the main pulmonary artery and relocating the entire PA trunk and bifurcation anterior to the aorta. Distortion of the coronary arteries carries a higher risk of mortality than supravalvular PS, but is less common overall.

32 ANSWER: D.  In addition to verifying the status of the surgical repair, the role of the pediatric cardiologist performing the TEE in the operating room is to assess ventricular function. Markedly diminished ventricular function after bypass should be investigated and explained. The patient in this scenario demonstrates poor function with regional dysfunction, concerning for focal myocardial ischemia. In addition, any procedures involving manipulation of the coronary arteries portend a risk of occlusion of the coronary arteries and myocardial ischemia. This patient clearly has evidence of inadequate coronary artery perfusion, likely related to mechanical obstruction.

33 ANSWER: E.  This patient’s anatomy consists of a subpulmonary left ventricle with obstruction. This manifests clinically as severe hypoxemia due to inadequate pulmonary blood flow. The best intervention in this neonate to increase pulmonary blood flow is placement of a modified BT shunt. The arterial switch procedure may be part of this child’s future treatment, but not in the context of LV outflow tract obstruction. The Mustard (atrial switch) operation is now usually performed only in patients with congenitally corrected TGA. Manipulation of the LVOT with a balloon is unlikely to relieve obstruction. PDA stenting as an alternative to BT shunt placement is advocated in some centers, but is not a widely accepted option.

34 ANSWER: D.  The patient in the vignette has complicated anatomy consisting of congenitally corrected TGA, pulmonary stenosis, and a large VSD. He has a bidirectional cavopulmonary anastomosis providing (presumably) the bulk of his pulmonary blood flow. His AV valve and the presence of two well-formed ventricles, however, are favorable for a two-ventricle, physiologic repair. This would be accomplished by directing the remainder of his systemic venous return across the atrial septum to the left-sided AV valve (tricuspid valve), baffle closure of the VSD to direct right ventricle output across the aortic valve, and placement of a pulmonary conduit from the morphologic left ventricle to the pulmonary arteries.

35 ANSWER: D.  The straddling of the mitral valve chordae precludes physiologic repair of this neonate’s defect. As a result, the best option of the choices listed is a Norwood palliation with Sano (RV–PA shunt). While the severe coarctation does not itself necessitate a single-ventricle repair, a Norwood-style aortic arch reconstruction is appropriate in this situation as the left AV valve morphology has already dictated a Fontan palliation. The Sano shunt is sometimes used as an alternative to a BT shunt to provide pulmonary blood flow from the right ventricle. Some advocate this approach rather than a BT shunt, as the pulsatile flow from a Sano may promote better growth of the branch pulmonary arteries over time. Like the BT shunt, the Sano is temporary and would be removed at the time of bidirectional cavopulmonary anastomosis. In the presence of a functionally normal left AV valve, this patient would likely be a candidate for repair of the VSD and coarctation and resection of the subaortic stenosis (choice E).

36 ANSWER: B.  Taussig–Bing anomaly is a form of double-outlet right ventricle where the VSD is located below the pulmonary valve. The physiology of this anomaly resembles that of complete transposition of the great arteries. The pulmonary artery receives primarily saturated blood from the left ventricle through the subpulmonic VSD. The aorta, which is remote from the VSD, receives desaturated systemic venous blood from the right ventricle. Given the arrangement of the great arteries relative to the VSD, the left ventricle cannot be baffled to the distant aortic valve. Instead, the great arteries are surgically switched, as they are for d-TGA, and the VSD is closed to the pulmonic (neo-aortic) valve.

37 ANSWER: E.  This child’s heart defect does not allow for complete (two-ventricle) repair. The aorta arises from a hypoplastic right ventricle which itself has not AV valve inflow. Thus, systemic outflow is dependent on the size of the VSD and the patency of the LVOT. With a restrictive VSD (bulboventricular foramen) and severe subaortic stenosis, systemic outflow is severely compromised and should be addressed surgically. The remainder of the aortic arch is presumably normal, so this child can have a direct anastomosis of the main pulmonary artery to the ascending aorta (DKS). Pulmonary blood flow is then accomplished by placement of a BT shunt. Later palliation would consist of a modified Glenn, and, ultimately, a modified Fontan. Enlargement of the VSD may improve LVOT obstruction, but does not address the issue of ductal-dependent pulmonary blood flow. PA banding by itself does not address the issue of LVOT obstruction. Placement of a bidirectional Glenn is not indicated in this patient as pulmonary vascular resistance has not yet fallen. The hybrid Norwood procedure is reserved for neonates with hypoplastic left heart syndrome and not used in children with DILV and subaortic stenosis.

38 ANSWER: D.  Most centrally located secundum ASDs can be closed safely and effectively in the cardiac catheterization laboratory. Indications for surgical repair of a secundum ASD includes deficiency of the posterior-inferior septal rim and the presence of coexisting abnormalities that would benefit from surgical repair. Specific recommendations for surgical repair include moderate or severe tricuspid valve regurgitation, which would benefit from annuloplasty or repair of the valve.

39 ANSWER: B.  Completion of the Fontan in this patient would result in persistent connection of the anomalous pulmonary vein to the systemic venous (Fontan) circuit. While initially this may seem to cause persistent left-to-right shunt, in this patient, there is no driving pressure across the right lower pulmonary artery (RLPA pressure = Fontan pressure = RLPV pressure). As a result this lung segment would inevitably become ischemic and infarct.

40 ANSWER: D.  Of the interventions listed, synthetic patch repair of coarctation is known to carry a significant risk of pseudoaneurysm at the site of repair. In this patient, this is clearly seen as a large mass in the area of the previous coarctation repair.

41 ANSWER: B.  The ECG shown demonstrates sinus rhythm with right bundle branch block. In the post-operative patient, this is most often due to incision of the right ventricular wall. Patch enlargement of the RVOT requires incision into the infundibular myocardium, which produces a right bundle branch block. The remaining choices are all accomplished without damage to the RV myocardium.

42 ANSWER: A.  Atrial switch operation (Mustard/Senning) is accomplished by baffling SVC and IVC flow across the ASD to the left-sided AV valve and into the subpulmonic ventricle. Obstruction of either baffle can occur and produces symptoms of central venous obstruction. SVC obstruction is more common and results in jugular venous distension, facial edema, and may cause increased head size in infants with an open fontanelle. IVC obstruction, which is less common, may cause hepatomegaly, ascites, and lower extremity edema. SVC and IVC baffle obstruction may be amenable to stent placement in the interventional catheterization laboratory, but may require surgical revision.

43 ANSWER: D.  Neonatal repair of truncus arteriosus is now advocated in most congenital heart centers. Studies have shown that early intervention (before 6 months of age) decreases the risk of pulmonary vascular disease in children with truncus and unobstructed pulmonary blood flow. The patient in this scenario has unobstructed pulmonary arterial flow from the truncus, as demonstrated by high arterial oxygen saturation. Initial pulmonary artery banding with later complete repair was the intervention of choice in the past, but this approach is associated with higher long-term mortality than complete repair in the neonatal period.

44 ANSWER: C.  The echocardiographic image demonstrates anomalous origin of the right coronary artery from the left sinus of Valsalva. The treatment of this lesion is unroofing the intramural segment of the RCA to enlarge the effective orifice. Some question whether any intervention is necessary when this anomaly is found incidentally in asymptomatic individuals. As this patient is going to have a VSD repair, though, unroofing the coronary artery does not add significant additional risk to the operation.

45 ANSWER: C.  The Warden repair for sinus venosus ASD with anomalous right upper pulmonary veins involves transection of the SVC above the insertion of the pulmonary veins, baffling of the anomalous pulmonary vein/SVC stump to the ASD, and reimplantation of the upper SVC to the right atrial appendage.

46 ANSWER: A.  The Lecompte maneuver is performed as part of the arterial switch operation when mobilization of the pulmonary artery is complicated by the arrangement of the great arteries. This is particularly problematic in cases where the pulmonary artery is directly posterior to the aorta, where simple “switch” of the great arteries would result in stenosis of the MPA or RPA. The Lecompte maneuver is performed by transecting the main pulmonary artery and relocating the entire PA trunk and bifurcation anterior to the aorta.

47 ANSWER: E.  The Ross procedure involves autograft replacement of the stenotic native aortic valve with the native pulmonary valve. A homograft is then placed in the pulmonary position. While all of the choices are risks associated with the Ross procedure, the most common indication for re-operation is failure of the pulmonary homograft.

48 ANSWER: A.  The MAZE ablative procedure involves intraoperative cryoablation or radiofrequency ablation across several segments of the right atrium. This procedure is very effective in treating supraventricular tachyarrhythmias, including atrial fibrillation and atrial flutter. The remaining choices may be performed during repair of Ebstein’s anomaly but has not been shown to significantly reduce the risk of atrial arrhythmias. The Starnes procedure is typically performed in cases of neonatal Ebstein’s anomaly with severe right ventricle hypoplasia and significant tricuspid valve regurgitation. It involves patch occlusion of the tricuspid valve at the annulus, removal of the atrial septum, and placement of a systemic to pulmonary artery shunt (e.g., BT shunt). This procedure is performed in anticipation of a single-ventricle (Fontan) palliation.