Congenital Heart Defects

Question 1

Allows bypass of hepatic circulation

Answer 1

Ductus Venosus

Question 2

Flow through the foremen ovals allows well oxygenated blood to bypass the high resistance

Answer 2

Pulmonary Circuit

Question 3

Directs relatively lower oxygenated blood to flow to lower extremities

Answer 3

Ductus Arteriosus

Question 4

Following birth, the three shunts that exist during fetal circulation close. These three shunts are the

Answer 4

Ductus venosus, Foramen Ovale, and Ductus arteriosus

Question 5

Normally results in left to right shunt unless Eisenmenger’s is present

-Distinct from patent foramen ovale

Answer 5

Atrial Septal Defect

Question 6

An atrial septal defect can be distinguished by a

Answer 6

Fixed split S2

Question 7

Due to rapid equalization of pressures between right and left heart in presence of shunt

Answer 7

Fixed Split S2

Question 8

There is evidence of right ventricular enlargement on CXR/ECG/Echocardiography with an

Answer 8

Atrial septal defect

Question 9

We see a step-up in oxygen saturation on catheterization with an

Answer 9

Atrial Septal Defect

Question 10

Closure of an ASD is contraindicated once

Answer 10

Eisenmenger’s has developed

Question 11

Most commonly in the membranous or muscular portion of the ventricular septum

Answer 11

Ventricular Septal Defect

Question 12

Although VSD’s cause left to right shunting, initially they lead to

Answer 12

LV not RV volume overload

Question 13

Increased pulmonary blood flow can lead overtime to pulmonary hypertension and

Answer 13

Eisenmenger’s syndrome

Question 14

Characterized by symptoms due to congestive heart failure such as tachyon earth, poor feeding, failure to thrive, etc

Answer 14

VSD

Question 15

On ascultation, a VSD can be heard via a

Answer 15

Harsh holosystolic murmur at the left eternal border in 3rd or 4th intercostal space

Question 16

More than 50% of small and moderate sized VSD’s will

Answer 16

Spontaneously close

Question 17

Results when the ductus arteriosus fails to close after birth

Answer 17

Patent Ductus Arteriosus

Question 18

First trimester rubella infection, premature birth, and high altitude are all risk factors for

Answer 18

PDA

Question 19

The ductus arteriosus normally closes after birth due to an increase in oxygen saturation and decrease in

Answer 19

Prostaglandin levels

Question 20

Results in volume overload and dilation of the left side of the heart

Answer 20

PDA

Question 21

Characterized by a continuous machine-like murmur

Answer 21

Patent Ductus Arteriosus

Question 22

Can usually be seen on Doppler echocardiography

Answer 22

PDA

Question 23

Even the smallest of PDA’s will be closed in order to reduce the risk of

Answer 23

Endocarditis

Question 24

PDA’s can be closed by administration of

Answer 24

Indomethacin

Question 25

A patent ductus arteriosus can be maintained through

Answer 25

Prostaglandin infusion

Question 26

Characterized by a harsh crescendo-decrescendo murmur -More common in males than females

Answer 26

Congenital Aortic Stenosis

Question 27

The harsh crescendo-decrescendo murmur in congenital aortic stenosis is loudest at the

Answer 27

Base

Question 28

We will see evidence of LVH on ECG with

Answer 28

Congenital aortic stenosis

Question 29

In adults, aortic stenosis is commonly caused by

Answer 29

Bicuspid aortic valves

Question 30

Bicuspid aortic valves commonly cause aortic stenosis in adults as a result of progressive

Answer 30

Calcification of the leaflets

Question 31

There is a strong association between congenital aortic stenosis and

Answer 31

Coarctation of the aorta

Question 32

Most patients with coarctation of the aorta will have an abnormal

Answer 32

Aortic Valve

Question 33

In adults, presentation and management of aortic stenosis caused by bicuspid valve calcification is very similar to presentation and management of

Answer 33

Calcification aortic Stenosis

Question 34

Can be seen in valve itself (90%), within RV, or in pulmonary artery

Answer 34

Pulmonic Stenosis

Question 35

Causes increased RV pressures and hypertrophy

Answer 35

Pulmonic Stenosis

Question 36

Characterized by dyspnea on exertion and exercise intolerance

Answer 36

Pulmonic Stenosis

Question 37

On physical exam, shows a prominent jugular a wave and late peaking crescendo-decrescendo murmur at left upper eternal border

Answer 37

Pulmonic Stenosis

Question 38

The only right sided sound that decreases with inspiration

Answer 38

Pulmonic Valve Click

Question 39

Shows an enlarged RV on CXR and ECG

Answer 39

Pulmonic Stenosis

Question 40

Echocardiogram demonstrates increased gradient across pulmonic valve, elevated right sided pressures, and RVH

Answer 40

Pulmonic Stenosis

Question 41

What is the most effective treatment of pulmonic stenosis?

Answer 41

Ballon valvuloplasty

Question 42

What are the two cyanotic congenital heart defects

Answer 42

Tetralogy of fallout and transposition of the great arteries

Question 43

Has the basic defect of abnormal anterior and cephalad displacement of infundibular portion of intraventricular septum

Answer 43

Tetralogy of Fallot

Question 44

Tetralogy of Fallot results in which 4 abnormalities

Answer 44

VSD, subvalvular pulmonic stenosis, overriding aorta, and RVH

Question 45

Results in a net right to left shunt through the VSD, resulting in cyanosis

Answer 45

Tetralogy of Fallot

Question 46

Depends on severity of pulmonic stenosis and systemic and pulmonary vascular resistance

Answer 46

Degree of shunting in TOF

Question 47

With TOF, children may have spells of cyanosis following activity when systemic vasodilation results in increased

Answer 47

Right to Left shunting

Question 48

Children learn to relieve their TOF symptoms by

Answer 48

Squatting

Question 49

A common finding on physical exam with TOF is

Answer 49

Clubbing

Question 50

With TOF, CXR demonstrates a

Answer 50

“Boot shaped” Heart

Question 51

Surgical closure of VSD and enlargement of subpulmonary infundibulum is the treatment for

Answer 51

TOF

Question 52

Results in the aligning of the aorta superior to the right ventricle -Aorta and pulmonary artery are in opposite positions than usual

Answer 52

Transposition of the great arteries

Question 53

Places systemic and pulmonary circuits in parallel rather than in series

Answer 53

Transposition of the great arteries

Question 54

The initial treatment for transposition of the great arteries is? -Allows mixing of blood which is necessary to keep baby alive

Answer 54

Maintenance of Ductus Arteriosus through prostaglandin

Question 55

When a congenital defect with left to right shunting overwhelms the pulmonary circuit and results in the direction of the shunt being switched -Once this develops, correction of original defect is contraindicated

Answer 55

Eisenmenger’s Syndrome

Question 56

Presentation is usually before age one, with frequent respiratory infections, poor weight gain, and heart failure

Answer 56

Atrioventricular Septal (Endocardial Cushion) Defect

Question 57

Characterized by a drastically enlarged RA and small RV which causes small LA and LV -Associated w/ Lithium exposure in utero

Answer 57

Ebstein’s anomaly

Question 58

Tricuspid valve never opens and there is an ASD with

Answer 58

Tricuspid Atresia

Question 59

Characterized by absence of tricuspid orifice, ASD, hypoplastic RV, and VSD

Answer 59

Tricuspid Atresia

Question 60

Thought to be caused by unequal division of AV canal

Answer 60

Tricuspid Atresia

Question 61

Immediately managed by maintaining a PDA, placement of Blalock-Taussig shunt

Answer 61

Tricuspid Atresia

Question 62

3-6 months after tricuspid atresia -The second stage of treatment

Answer 62

Bidirectional Glenn Procedure

Question 63

Then, at 3-5 years of age, we perform a -Third stage of tricuspid atresia treatment

Answer 63

Fontan Procedure

Question 64

Infants present with cyanosis and signs/symptoms of heart failure -Corrective surgery should be performed as soon as possible

Answer 64

Total Anomalous Return

Question 65

Linked to cardiac neural crest cells -Always associated with VSD due to underdevelopment on distal pulmonary infundibulum

Answer 65

Truncus Arteriosus

Question 66

30% of truncus arteriosus cases involve right sided

Answer 66

Aortic arch

Question 67

Truncus Arteriosus often presents as part of

Answer 67

DiGeorge Syndrome

Question 68

During the first few days of life, mild to moderate cyanosis may be the only sign. However, as pulmonary vascular resistance falls, symptoms of HF and pulmonary congestion develop

Answer 68

Truncus Arteriosus

Question 69

Treatment is surgical and involves closing the VSD and attaching pulmonary arteries through a conduit to the right ventricle

Answer 69

Truncus Arteriosus