Congenital Heart Disease

Question 1

A bounding and bisferiens pulse occurs in…

Answer 1

HOCM.

Question 2

HCM syncope can be treated with … or … and if repetitive with…

Answer 2

betablockers, calcium blockers or disopiramide AND dual chamber PPM AND myotomy/myectomy.

Question 3

Ebstein anomaly is more frequent in this gender. …

Answer 3

equal

Question 4

Complete AV canal is most common in … syndrome.

Answer 4

Down

Question 5

Pulmonic valve stenosis and MVP are present in patients with … syndrome.

Answer 5

Costello

Question 6

Pulmonic valve stenosis and HOCM are present in patients with … syndrome.

Answer 6

Noonan

Question 7

Dilation of the coronary sinus can occur with this anomaly, …

Answer 7

persistent left SVC.

Question 8

…(pressor) is contraindicated in the setting of idiopathic hypertrophic subaortic stenosis.

Answer 8

Dobutamine

Question 9

The most common anomaly leading to sudden cardiac death is the…

Answer 9

anomalous origin of the LM from the right sinus of Valsalva. The subsequent course between the aorta and pulmonary artery to the left ventricle may result in compression of the vessel, myocardial ischemia, and sudden death in both adults and teenagers dur

Question 10

A commonly encountered anomaly of the atrial septum is… in which there is benign infiltration by lipomatous tissue of the primum and superior atrial septum; the valve of the foramen ovale is spared, resulting in a “dumbbell” configuration of the atrial

Answer 10

lipomatous atrial hypertrophy

Question 11

A substantial percentage of individuals have a multilobed…, which can result in a confusing appearance because the septation between the two lobes may be confused for thrombus.

Answer 11

left atrial appendage

Question 12

In a true… , there will be only two sinuses and variable location of the coronary ostia.

Answer 12

two-leaflet bicuspid aortic valve

Question 13

… is the single most common (NOT NECESSARELY INHERITED) congenital cardiac defect and occurs in approximately 2 percent of the population.

Answer 13

The bicuspid aortic valve

Question 14

There is a strong association between coarctation of the aorta and…

Answer 14

bicuspid aortic valve.

Question 15

The most common congenital abnormality associated with the pre-excitation syndrome is…

Answer 15

Ebstein’s anomaly (abnormalities of the tricuspid valve and right ventricle).

Question 16

In patients with pre-excitation syndrome and right parietal accessory pathway (the delta wave has a similar R-R progression with transition in V2-4),… (test) is needed.

Answer 16

routine echo - to look for associated anomalies (like Ebstein’s).

Question 17

The … procedure involves the removal of the patient’s own pulmonary valve and adjacent main pulmonary artery, which are used to replace the diseased aortic valve, usually with the neighboring aorta, and reimplantation of the coronary arteries into the g

Answer 17

Ross

Question 18

Tetralogy of Fallot represents a constellation of lesions, including a… , obstruction to right ventricular outflow tract at either the infundibular or valvular level, right ventricular hypertrophy and right-sided aortic arch.

Answer 18

ventricular septal defect with overriding aorta

Question 19

Tetralogy of Fallot represents a constellation of lesions, including a ventricular septal defect with overriding aorta, obstruction to right ventricular outflow tract at either the infundibular or valvular level, right ventricular hypertrophy and …

Answer 19

right-sided aortic arch.

Question 20

… represents the most common congenital intracardiac shunt encountered in infants and children.

Answer 20

Ventricular septal defect

Question 21

The threshold for recommending closure of an atrial septal defect has diminished to a Qp/Qs (pulmonary/systemic flow ratio) of >…

Answer 21

1.5.

Question 22

… is an isolated malposition of the great vessels due to failure of the conotruncos to appropriately coil (pulmonary artery

Answer 22

D-Transposition

Question 23

Tetralogy of Fallot represents a constellation of lesions, including a ventricular septal defect with overriding aorta, obstruction to right ventricular outflow tract at either the infundibular or valvular level, … and right-sided aortic arch.

Answer 23

right ventricular hypertrophy

Question 24

The most common location of an atrial septal defect is … (70% of the cases).

Answer 24

secundum

Question 25

... is a common association with Ebstein anomaly and should be considered in all instances.

Answer 25

Atrial septal defect

Question 26

Narrowing or coarctation of the aorta occurs ... (location).

Answer 26

after the takeoff of the left subclavian artery

Question 27

... of the great vessels results in "corrected" transposition, in which physiological blood flow is maintained.

Answer 27

L-Transposition

Question 28

... represents the most common cyanotic congenital lesion to be encountered that is likely to result in survival to adulthood and, furthermore, the most common complex lesion to be encountered in the adult population after repair.

Answer 28

Tetralogy of Fallot

Question 29

The most common location for a ventricular septal defect is a ...

Answer 29

small perimembranous septal defect.

Question 30

Tetralogy of Fallot represents a constellation of lesions, including a ventricular septal defect with overriding aorta, ... , right ventricular hypertrophy and right-sided aortic arch.

Answer 30

obstruction to right ventricular outflow tract at either the infundibular or valvular level

Question 31

This congenital abnormality of the MITRAL valve, likely to be encountered in adult populations, is... , seen in association with a primum atrial septal defect.

Answer 31

the cleft mitral valve

Question 32

... of the great vessels results in two parallel circulations and is obviously not compatible with life in the absence of an intracardiac shunt such as a persistent ductus or large ventricular or atrial septal defect.

Answer 32

D-Transposition

Question 33

... occurs when there is both inversion of the ventricles and transposition of the great arteries (anatomical right atrium > anatomical mitral valve > anatomical left ventricle > pulmonary artery; left atrium > anatomical tricuspid valve > anatomical righ

Answer 33

L-Transposition

Question 34

The outflow tract obstruction results in a characteristic ... pattern on the aortic valve in hypertrophic cardiomyopathy (dz), and is best visualized with M-mode echocardiography.

Answer 34

systolic notching

Question 35

Women with... cardiac congenital defects tolerate pregnancy well.

Answer 35

asymptomatic acyanotic

Question 36

Medical therapy with... is first-line management of hypertrophic cardiomyopathy; verapamil is an alternative.

Answer 36

beta-blockers

Question 37

A PDA should be closed even with a small shunt to reduce the risk of...

Answer 37

endarteritis.

Question 38

Coarctation of the aorta should be corrected when the patient has proximal hypertension and a gradient of... mm Hg or more.

Answer 38

20

Question 39

Women with Eisenmenger’s syndrome should be advised against...

Answer 39

pregnancy.

Question 40

In pregnant patients with SLE, an anti-Ro antibody titer should be obtained, positive results increasing the likelihood that the baby will have...

Answer 40

neonatal heart block.