Congenital Heart Disease

Question 1

The most common form of atrial septal defect (ASD) is

A. Sinus venosus defect
B. Ostium primum defect
C. Ostium secundum defect
D. Combined primum and secundum defect

Answer 1

C. Ostium secundum defect

Question 2

Most common age to close asymptomatic
ASDs is

A. In the immediate newborn period
B. After the child reaches 10kg in weight
C. Age 4-5 years
D. During puberty

Answer 2

C. Age 4-5 years

Question 3

Which of the following is NOT an acceptable treatment for aortic valve stenosis with hypoplastic left ventricle

A. Balloon valvotomy
B. Intubation and initiation of prostaglandin
C. Surgical valvotomy
D. Norwood procedure

Answer 3

A. Balloon valvotomy

Question 4

Most common location for a coarctation of the aorta

A. Aortic arch
B. Distal to the left subclavian artery
C. Diaphargm
D. Level of renal arteries

Answer 4

B. Distal to the left subclavian artery

Question 5

Treatment of choice for recurrent COA (after surgical repair) in a preschool-aged child

A. Resection and primary anastomosis
B. Resection with interposition graft
C. Balloon dilatation alone
D. Balloon dilatation with stenting

Answer 5

C. Balloon dilatation alone

Question 6

Which of the ollowing is a true surgical emergency in a newborn?

A. Tetralogy of Fallot
B. Truncus arteriosus
C. Total anomalous pulmonary venous connection (TAPVC)
D. COA

Answer 6

C. Total anomalous pulmonary venous connection (TAPVC)

Question 7

The bidirectional Glenn procedure is used to correct

A. Tricuspid atresia
B. Patent ductus arteriosus
C. Transposition of the great arteries
D. Total anomalous pulmonary venous connection

Answer 7

A. Tricuspid atresia

Question 8

Hypoplastic left heart syndrome is surgically treated with

A. Bilateral pulmonary artery banding and stent placement in the patent ductus arteriosus.
B. Norwood procedure with a Blalock-Taussig (B-T) shunt.
C. Norwood procedure with a right ventricle (RV) to pulmonary artery conduit (Sano shunt).
D. All of the above.

Answer 8

D. All of the above

Question 9

The arterial switch operation for transposition of the great vessels is best performed

A. Within 2 weeks of birth
B. At 1 year of age
C. At 10 kg of weight
D. In adolescence

Answer 9

A. Within 2 weeks of birth

Question 10

Which of the following is NOT one of the components of the tetralogy of Fallot (TOF)?

A. ASD
B. Ventricular septal defect
C. Right ventricular hypertrophy
D. Right ventricular outflow obstruction

Answer 10

A. ASD

Question 11

The most commonly recommended age for correction of a TOF is

A. Neonate younger than 3 months
B. 6 months of age
C. 1 year of age
D. 4–5 years of age

Answer 11

A. Neonate younger than 3 months

Question 12

Which of the following is the most common type of ventricular septal defect (VSD) to require surgical correction?

A. Ostium primum
B. Ostium secundum
C. Muscular
D. Perimembranous

Answer 12

D. Perimembranous

Question 13

What is the best predictor of spontaneous closure of a VSD?

A. Size
B. Age at diagnosis
C. Gestational age
D. Lack of electrocardiogram changes

Answer 13

B. Age at diagnosis

Question 14

Which of the following cardiac abnormalities, all of them well-tolerated during fetal life, becomes a serious problem at birth?

A. Aortic arch
B. Ductus arteriosus
C. Foramen ovale
D. Tricuspid atresia

Answer 14

D. Tricuspid atresia

Question 15

Cor triatriatum is

A. Three atria where the right atrium is divided by a diaphragm with the SVC and inferior vena cava (IVC) drain into separate atria.
B. Three atria where the SVC and IVC drain into the superior right atrium and the inferior drains through the tricuspid valve in the RV.
C. Three atria where the left atrium is divided by a diaphragm and the superior and inferior pulmonary veins drain into separate chambers.
D. Three atria where the left atrium is divided by a diaphragm separating the chamber receiving pulmonary return from the chamber draining through the mitral valve into the LV.

Answer 15

D. Three atria where the left atrium is divided by a diaphragm separating the chamber receiving pulmonary return from the chamber draining through the mitral valve into the LV.

Question 16

Flow across a VSD is dependent upon

A. Size of defect
B. Left and right ventricular pressure and size of the defect
C. Pulmonary and systemic vascular resistance and defect size
D. Pulmonary and systemic vascular resistance

Answer 16

C. Pulmonary and systemic vascular resistance and defect size

Question 17

A child with a large VSD and no other cardiac lesion can be expected to develop all of the following EXCEPT

A. Cyanosis
B. Failure to thrive
C. Left ventricular hypertrophy greater than left ventricular dilation
D. Increased susceptibility to lower respiratory tract infection

Answer 17

C. Left ventricular hypertrophy greater than left ventricular dilation

Question 18

Beyond early childhood, high pulmonary blood flow is most apt to produce

A. Cyanosis on exercise
B. Diminished exercise tolerance
C. Periodic episodes of hemoptysis
D. Right ventricular hypertrophy

Answer 18

B. Diminished exercise tolerance

Question 19

The most important diagnostic assessment modality for evaluating infants and children with congenital heart disease is

A. Cardiac catheterization
B. Chest X-ray
C. Transesophageal echocardiogram
D. Transthoracic echocardiogram

Answer 19

D. Transthoracic echochardiogram

Question 20

The major determinant of operability in patients who have a VSD is

A. The size of the defect.
B. The location of the defect.
C. The pulmonary vascular resistance. D. The age of the patient

Answer 20

C. The pulmonary vascular resistance

Question 21

Transplant-free survival after Norwood with B-T shunt (system to pulmonary) versus Sano shunt (RV to pulmonary shunt) in patients with hypoplastic left heart syndrome is

A. Equal at 12 months, though worse or B-T shunt with longer term follow-up B. Worse for B-T shunt at 12 months and at longer term follow-up
C. Better at 12 months for B-T shunt, but equal at longer term follow-up
D. Worse at 12 months for B-T shunt, but equal at longer term follow-up

Answer 21

D. Worse at 12 months for B-T shunt, but equal at longer term follow-up

Question 22

A premature infant is discovered at birth to have a patent ductus arteriosus with moderate respiratory distress. The infant does not improve after 48 hours of medical management with fluid restriction, diuretics, and respiratory support. The next step in management is

A. Acetylsalicylic acid
B. Indomethacin
C. Surgical correction of the ductus
D. Transvenous occlusion of the ductus

Answer 22

B. Indomethacin

Question 23

Which of the following is NOT a type of VSD?

A. Perimembranous
B. Atrioventricular canal
C. Supracristal
D. Sinus venosal

Answer 23

D. Sinus venosal

Question 24

During left thoracotomy for repair of patent ductus arteriosus the blood pressure is 70/22. Immediately after placement of a clip across the duct the blood pressure is

A. 70/22
B. 70/40
C. 90/22
D. 90/40

Answer 24

B. 70/40

Question 25

All of the following are true about truncus arteriosus EXCEPT

A. Truncal valves most commonly have three leaflets.
B. Patients usually present with mild to moderate cyanosis and congestive heart failure in the neonatal period.
C. Patients should undergo repair at 6 months of age.
D. There is a continuous left to right shunt

Answer 25

C. Patients should undergo repair at 6 months of age

Question 26

All are true regarding closure of ASDs in adults EXCEPT

A. Atrial arrhythmias are common postoperatively.
B. Postoperative mortality is significantly higher with increasing age.
C. Closure of ASDs in patients older than 60 decreases risk of paradoxical embolism, but has little effect on functional status.
D. Secundum ASD closure is more commonly performed using transcatheter approach compared with surgical approaches

Answer 26

C. Closure of ASDs in patients older than 60 decreases risk of paradoxical embolism, but has little effect on functional status.