[MCQ] Congenital Heart Disease in the Adult- Part 2

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Congenital Heart Disease in the Adult- Part 2

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Question 1

Coronary “steal” syndrome best relates to ?

A	Congenital aneurysm of aortic sinus of Valsalva with fistula
B	Coronary arteriovenous fistula
C	Anomalous origin of left coronary artery from pulmonary trunk
D	All of the above

Question 1 Explanation:

In coronary arteriovenous fistula, shunt is usually of small magnitude and myocardial blood flow is not usually compromised. But if the shunt is large, there may be a coronary “steal” syndrome with myocardial ischemia and possible angina or ventricular arrhythmias.

Question 2

In which of the following condition, coronary arteries are subjected to elevated systolic pressures from left ventricle ?

A	Congenital Aortic Stenosis
B	Subaortic Stenosis
C	Supravalvular Aortic Stenosis
D	All of the above

Question 2 Explanation:

Coronary arteries are subjected to elevated systolic pressures from the left ventricle in supravalvular aortic stenosis.

Question 3

Which of the following is not a feature of Williams-Beuren syndrome ?

A	“Elfin” facies
B	Mental retardation with retained language skills
C	Supravalvular aortic stenosis
D	Transient hyperkalemia

Question 3 Explanation:

Supravalvular aortic stenosis is associated cardiac defect in Williams-Beuren syndrome, typically comprising of “elfin” facies, low nasal bridge, cheerful demeanor, mental retardation with retained language skills and love of music, and transient hypercalcemia

Question 4

Coarctation of the aorta occurs in what percentage of patients with congenital heart disease ?

A	2 %
B	3 %
C	5 %
D	7 %

Question 4 Explanation:

Narrowing or constriction of the lumen of the aorta may occur anywhere along its length but is most common distal to the origin of the left subclavian artery near the insertion of the ligamentum arteriosum. Coarctation of the aorta occurs in 7% of patients with congenital heart disease and is more common in males than females.

Question 5

Which of the following is more common in coarctation of the aorta ?

A	Turner syndrome
B	Bicuspid aortic valve
C	Circle of Willis aneurysms
D	All of the above

Question 5 Explanation:

Coarctation of the aorta is frequent in patients with gonadal dysgenesis (Turner syndrome) and is associated with bicuspid aortic valve (75%) and Circle of Willis aneurysms.

Question 6

In coarctation of the aorta, enlarged and pulsatile collateral vessels may be palpated in ?

A	Intercostal spaces
B	Axillae
C	Interscapular area
D	All of the above

Question 6 Explanation:

In coarctation of the aorta, enlarged and pulsatile collateral vessels may be palpated in the intercostal spaces anteriorly, in the axillae, or posteriorly in the interscapular area

Question 7

In Chest x-ray, “3” sign relates to which of the following ?

A	Patent ductus arteriosus
B	Supravalvular Aortic Stenosis
C	Coarctation of the aorta
D	Tetralogy of Fallot

Question 7 Explanation:

Chest x-ray in Coarctation of the aorta shows a dilated left subclavian artery high on the left mediastinal border and a dilated ascending aorta. Indentation of the aorta at the site of coarctation with pre- and poststenotic dilatation (“3” sign) along left paramediastinal shadow are pathognomonic

Question 8

In coarctation of the aorta, radiographic notching of which of the following ribs is seen ?

A	3rd to 5th ribs
B	3rd to 7th ribs
C	3rd to 9th ribs
D	3rd to 12th ribs

Question 8 Explanation:

n coarctation of the aorta, notching of the third to ninth ribs is an important radiographic sign. This is due to inferior rib erosion by dilated collateral vessels.

Question 9

Which of the following is the hazard of coarctation of aorta ?

A	Cerebral aneurysms and hemorrhage
B	Aortic dissection and rupture
C	Premature coronary arteriosclerosis
D	All of the above

Question 9 Explanation:

In coarctation of the aorta, the chief hazards of proximal aortic severe hypertension include cerebral aneurysms and hemorrhage, aortic dissection and rupture, premature coronary arteriosclerosis, and LV failure.

Question 10

In Tetralogy of Fallot, severity of which of the following determines the clinical presentation ?

A	Size of VSD
B	Aortic override of the VSD
C	RV outflow obstruction
D	RV hypertrophy

Question 10 Explanation:

The four components of the tetralogy of Fallot are malaligned VSD, obstruction to RV outflow, aortic override of the VSD, and RV hypertrophy due to the RV’s response to aortic pressure via the large VSD. The severity of RV outflow obstruction determines the clinical presentation

Question 11

Which of the following may also be present in TOF ?

A	Pulmonary valve stenosis
B	Unilateral absence of a pulmonary artery
C	Right-sided aortic arch & descending thoracic aorta
D	All of the above

Question 11 Explanation:

In tetralogy of Fallot, severity of hypoplasia of the RV outflow tract varies from mild to complete (pulmonary atresia). Pulmonary valve stenosis and supravalvular and peripheral pulmonary arterial obstruction may coexist. Rarely, there is unilateral absence of a pulmonary artery (usually left). A right-sided aortic arch and descending thoracic aorta occur in 25%.

Question 12

Which of the following about Chest x-ray findings in Tetralogy of Fallot is false ?

A	Enlarged heart
B	Boot-shaped heart (coeur en sabot)
C	Oligemic lung fields
D	Concavity in the region of pulmonary conus

Question 12 Explanation:

In Tetralogy of Fallot, chest x-ray shows a normal-sized, boot-shaped heart (coeur en sabot) with a prominent right ventricle and a concavity in the region of pulmonary conus. Pulmonary vascular markings are typically diminished, and the aortic arch and knob may be on the right side.

Question 13

Which of the following about treatment in TOF is false ?

A	Reoperation in adults is mostly for severe PR
B	Ventricular and atrial arrhythmias common
C	Aortic regurgitation common
D	Endocarditis risk eliminated after surgical repair

Question 13 Explanation:

Most adults with tetralogy of Fallot have had some form of previous surgical intervention. Reoperation in adults is most commonly for severe pulmonary regurgitation. Ventricular & atrial arrhythmias require treatment. Aortic root has a medial tissue defect & it is commonly enlarged & associated with aortic regurgitation. Endocarditis remains a risk despite surgical repair.

Question 14

Which is the most common communication in complete transposition of the great arteries ?

A	ASD
B	VSD
C	PDA
D	All of the above

Question 14 Explanation:

Most patients of complete transposition of the great arteries have an interatrial communication, two-thirds have a patent ductus arteriosus, and about one-third have an associated VSD.

Question 15

Most common finding in Ebstein’s Anomaly is ?

A	MS
B	MR
C	TS
D	TR

Question 15 Explanation:

Ebstein’s Anomaly is characterized by a downward displacement of tricuspid valve into right ventricle, due to anomalous attachment of tricuspid leaflets, tissue of which is dysplastic. This results in tricuspid regurgitation

Question 16

During pregnancy, congenital cardiac malformations that are “well” tolerated (NYHA class I) include all except ?

A	Aortic or mitral regurgitation (mild-moderate)
B	Aortic or mitral stenosis (moderate)
C	Pulmonary or tricuspid regurgitation
D	Pulmonary stenosis (mild-moderate

Question 17

Heritable syndromes with associated ASD include all except ?

A	TAR (thrombocytopenia-absent radius)
B	Noonan
C	Holt-Oram
D	Ellis-van Creveld

Question 18

Heritable syndromes with associated VSD include all except ?

A	Holt-Oram
B	Apert
C	Shprintzen
D	Conradi-Hunermann

Question 19

Heritable syndromes with associated PDA include all except ?

A	Conradi-Hunermann
B	Incontinentia pigmenti
C	Noonan
D	Crouzon

Question 20

Heritable syndromes with associated Tetralogy of Fallot include all except ?

A	Catch-22 (DiGeorge)
B	TAR (thrombocytopenia-absent radius)
C	Multiple lentigines (LEOPARD) syndrome
D	Shprintzen (velocardiofacial)

Question 21

Heritable syndrome with associated supravalvular aortic stenosis is ?

A	Kartagener
B	Laurence-Moon-Biedl-Bardet
C	Williams
D	Crouzon

Question 22

Heritable syndromes with associated aortic regurgitation include ?

A	Morquio
B	Scheie
C	Maroteaux-Lamy
D	All of the above

Question 23

Chromosomal abnormalities with associated VSD include ?

A	Cri du chat
B	Trisomy 13 (D)
C	Trisomy 21 (Down syndrome)
D	All of the above

Question 24

Ebstein’s anomaly occurs with which of the following teratogenic drugs ?

A	Alcohol
B	Lithium
C	Dilantin
D	Thalidomide

Question 25

Which of the following heritable syndromes is associated with ASD ?

A	Ellis-van Creveld syndrome
B	TAR (Thrombocytopenia-absent radius) syndrome
C	Holt-Oram syndrome
D	All of the above

Question 26

Which of the following heritable syndromes is associated with PDA ?

A	Crouzon syndrome
B	Incontinentia pigmenti
C	Conradi-Hunermann syndrome
D	All of the above

Question 27

Which of the following connective tissue disorders is associated with peripheral coronary arterial disease ?

A	Marfan’s
B	Osteogenesis imperfecta
C	Pseudoxanthoma elasticum
D	Ehlers-Danlos syndrome

Question 28

Which of the following connective tissue disorders is associated with peripheral pulmonic stenosis ?

A	Marfan’s
B	Osteogenesis imperfecta
C	Pseudoxanthoma elasticum
D	Cutis laxa

Question 29

Which of the following chromosomal disorders is associated with Tetralogy of Fallot ?

A	Turner syndrome
B	Down syndrome
C	Trisomy 8
D	Cri du chat syndrome

Question 30

Which of the following chromosomal disorders is associated with coarctation of aorta ?

A	Turner syndrome
B	Down syndrome
C	Trisomy 18
D	Cri du chat syndrome

Question 31

Which of the following heritable syndromes is associated with Tetralogy of Fallot?

A	Turner syndrome
B	Catch-22 syndrome
C	Shprintzen syndrome
D	All of the above

Question 32

Which of the following heritable syndromes is associated with supravalvular aortic stenosis ?

A	Turner syndrome
B	Catch-22 syndrome
C	Shprintzen syndrome
D	Williams syndrome

Question 33

Which congenital malformation is typical of rubella embryopathy ?

A	Multiple sites of narrowing of peripheral pulmonary arteries
B	Coarctation of aorta
C	Tricuspid atresia
D	VSD

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Congenital Heart Disease in the Adult- Part 1| Congenital Heart Disease in the Adult- Part 2

1. Coronary “steal” syndrome best relates to ?
A. Congenital aneurysm of aortic sinus of Valsalva with fistula
B. Coronary arteriovenous fistula
C. Anomalous origin of left coronary artery from pulmonary trunk
D. All of the above

2. In which of the following condition, coronary arteries are subjected to elevated systolic pressures from left ventricle ?
A. Congenital Aortic Stenosis
B. Subaortic Stenosis
C. Supravalvular Aortic Stenosis
D. All of the above

3. Which of the following is not a feature of Williams-Beuren syndrome ?
A. “Elfin” facies

B. Mental retardation with retained language skills
C. Supravalvular aortic stenosis
D. Transient hyperkalemia

4. Coarctation of the aorta occurs in what percentage of patients with congenital heart disease ?
A. 2 %
B. 3 %
C. 5 %
D. 7 %

5. Which of the following is more common in coarctation of the aorta ?
A. Turner syndrome
B. Bicuspid aortic valve
C. Circle of Willis aneurysms
D. All of the above

6. In coarctation of the aorta, enlarged and pulsatile collateral vessels may be palpated in ?
A. Intercostal spaces
B. Axillae
C. Interscapular area
D. All of the above

7. In Chest x-ray, “3” sign relates to which of the following ?
A. Patent ductus arteriosus
B. Supravalvular Aortic Stenosis
C. Coarctation of the aorta
D. Tetralogy of Fallot

8. In coarctation of the aorta, radiographic notching of which of the following ribs is seen ?
A. 3rd to 5th ribs
B. 3rd to 7th ribs
C. 3rd to 9th ribs
D. 3rd to 12th ribs

9. Which of the following is the hazard of coarctation of aorta ?
A. Cerebral aneurysms and hemorrhage
B. Aortic dissection and rupture
C. Premature coronary arteriosclerosis
D. All of the above

10. In Tetralogy of Fallot, severity of which of the following determines the clinical presentation ?
A. Size of VSD
B. Aortic override of the VSD
C. RV outflow obstruction
D. RV hypertrophy

11. Which of the following may also be present in TOF ?
A. Pulmonary valve stenosis
B. Unilateral absence of a pulmonary artery
C. Right-sided aortic arch & descending thoracic aorta
D. All of the above

12. Which of the following about Chest x-ray findings in Tetralogy of Fallot is false ?
A. Enlarged heart
B. Boot-shaped heart (coeur en sabot)
C. Oligemic lung fields
D. Concavity in the region of pulmonary conus

13. Which of the following about treatment in TOF is false ?
A. Reoperation in adults is mostly for severe PR
B. Ventricular and atrial arrhythmias common
C. Aortic regurgitation common
D. Endocarditis risk eliminated after surgical repair

14. Which is the most common communication in complete transposition of the great arteries ?
A. ASD
B. VSD

C. PDA
D. All of the above

15. Most common finding in Ebstein’s Anomaly is ?
A. MS
B. MR
C. TS
D. TR

16. During pregnancy, congenital cardiac malformations that are “well” tolerated (NYHA class I) include all except ?
A. Aortic or mitral regurgitation (mild-moderate)
B. Aortic or mitral stenosis (moderate)
C. Pulmonary or tricuspid regurgitation
D. Pulmonary stenosis (mild-moderate)
17. Heritable syndromes with associated ASD include all except ?
A. TAR (thrombocytopenia-absent radius)
B. Noonan
C. Holt-Oram
D. Ellis-van Creveld
18. Heritable syndromes with associated VSD include all except ?
A. Holt-Oram
B. Apert
C. Shprintzen
D. Conradi-Hunermann
19. Heritable syndromes with associated PDA include all except ?
A. Conradi-Hunermann
B. Incontinentia pigmenti
C. Noonan
D. Crouzon
20. Heritable syndromes with associated Tetralogy of Fallot include all except ?
A. Catch-22 (DiGeorge)
B. TAR (thrombocytopenia-absent radius)
C. Multiple lentigines (LEOPARD) syndrome
D. Shprintzen (velocardiofacial)
21. Heritable syndrome with associated supravalvular aortic stenosis is ?
A. Kartagener
B. Laurence-Moon-Biedl-Bardet

C. Williams
D. Crouzon
22. Heritable syndromes with associated aortic regurgitation include ?
A. Morquio
B. Scheie
C. Maroteaux-Lamy
D. All of the above
23. Chromosomal abnormalities with associated VSD include ?
A. Cri du chat
B. Trisomy 13 (D)
C. Trisomy 21 (Down syndrome)
D. All of the above
24. Ebstein’s anomaly occurs with which of the following teratogenic drugs ?
A. Alcohol
B. Lithium
C. Dilantin
D. Thalidomide
25. Which of the following heritable syndromes is associated with ASD ?
A. Ellis-van Creveld syndrome
B. TAR (Thrombocytopenia-absent radius) syndrome
C. Holt-Oram syndrome
D. All of the above
26. Which of the following heritable syndromes is associated with PDA ?
A. Crouzon syndrome
B. Incontinentia pigmenti
C. Conradi-Hunermann syndrome
D. All of the above
27. Which of the following connective tissue disorders is associated with peripheral coronary arterial disease ?
A. Marfan’s
B. Osteogenesis imperfecta
C. Pseudoxanthoma elasticum
D. Ehlers-Danlos syndrome
28. Which of the following connective tissue disorders is associated with peripheral pulmonic stenosis ?
A. Marfan’s
B. Osteogenesis imperfecta
C. Pseudoxanthoma elasticum
D. Cutis laxa

29. Which of the following chromosomal disorders is associated with Tetralogy of Fallot ?
A. Turner syndrome
B. Down syndrome
C. Trisomy 8
D. Cri du chat syndrome
30. Which of the following chromosomal disorders is associated with coarctation of aorta ?
A. Turner syndrome
B. Down syndrome
C. Trisomy 18
D. Cri du chat syndrome
31. Which of the following heritable syndromes is associated with Tetralogy of Fallot?
A. Turner syndrome
B. Catch-22 syndrome
C. Shprintzen syndrome
D. All of the above
32. Which of the following heritable syndromes is associated with supravalvular aortic stenosis ?
A. Turner syndrome
B. Catch-22 syndrome
C. Shprintzen syndrome
D. Williams syndrome
33. Which congenital malformation is typical of rubella embryopathy ?
A. Multiple sites of narrowing of peripheral pulmonary arteries
B. Coarctation of aorta
C. Tricuspid atresia
D. VSD

Congenital Heart Disease in the Adult

[MCQ] Congenital Heart Disease in the Adult- Part 2

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Congenital Heart Disease in the Adult- Part 2

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