Cardiovascular Congenital Disorders

Question 1

Left-to-right shunts (4):

Answer 1

• ASD
• VSD
• PDA
• AV septal defect

Question 2

In ASD the degree of left-to-right shunt is determined by:

Answer 2

The size of the defect and right ventricular compliance

Question 3

ASD leads to (3):

Answer 3

• Right atrial enlargement
• Right ventricular enlargement
• Increased pulmonary blood flow

Question 4

ASD on physical exam (4):

Answer 4

• Prominent RV impulse
• Widely split and fixed second heart sound
• Soft systolic ejection murmur at left upper sternal border
• Diastolic murmur at left lower sternal border with large shunts

Question 5

Most common form of congenital heart disease:

Answer 5

VSD

Question 6

VSD leads to (3):

Answer 6

• Left atrial enlargement
• Left ventricular enlargement
• Increased pulmonary blood flow

Question 7

Degree of shunt in VSD depends on (2):

Answer 7

• Size of the defect

- Pulmonary vascular resistance

Question 8

VSD physical exam findings (4):

Answer 8

• Hyperdynamic precordium
• Holosystolic murmur at the left lower to mid-sternal border
• Mid-diastolic rumble at the apex with large shunts
• Hepatomegaly

Question 9

Degree of shunt in PDA depends on (2):

Answer 9

• Size of the ductus

- Pulmonary vascular resistance

Question 10

PDA leads to:

Answer 10

Left atrial and left ventricular volume overload

Question 11

Clinical features of PDA:

Answer 11

May present with tachypnea, diaphoresis and feeding difficulties

Question 12

PDA on physical exam (3):

Answer 12

• Hyperdynamic precordium
• Continuous harsh “machine-like” murmur
• Tachypnea and hepatomegaly may be present

Question 13

Pathology of a partial AVSD (3):

Answer 13

• Primum atrial septal defect
• Two atrioventricular valve annuli
• Cleft mitral valve

Question 14

Pathology of complete AVSD (3):

Answer 14

• Primum atrial septal defect
• Inlet ventricular septal defect
• Single atrioventricular valve annulus

Question 15

Partial AVSD on physical exam (3):

Answer 15

• RV impulse
• Fixed split S2
• Systolic ejection murmur at the left upper sternal border

Question 16

Complete AVSD on physical exam (3):

Answer 16

• Hyperdynamic precordium
• Loud S2
• Holosystolic murmur at the left lower to mid-sternal border
  
  • Initially there may be no murmur

Question 17

ECG in complete AVSD:

Answer 17

Prolongation of the PR interval and a leftward or superior QRS axis

Question 18

Coarctation of the aorta is associated with:

Answer 18

Bicuspid aortic valve

Question 19

Coarctation of the aorta clinical features (2):

Answer 19

• Infants with severe coarctation present with CHF as the ductus closes
• Older children/adolescents may present with a murmur and HTN

Question 20

Coarctation of the aorta on physical exam (3):

Answer 20

• Respiratory distress with poor perfusion
• Diminished or absent femoral pulses
• Gallop rhythm may be heard

Question 21

Management for coarctation of the aorta includes (3):

Answer 21

• PGE1 to maintain ductal patency
• Correction of metabolic acidosis
• Inotropic support for myocardial dysfunction

Question 22

Hypoplastic left heart syndrome on physical exam (5):

Answer 22

• Mild cyanosis
• Increased work of breath and decreased perfusion as the ductus closes
• Single loud S2
• Nonspecific systolic ejection murmur
• Possible gallop

Question 23

Pathology of tetralogy of Fallot (4):

Answer 23

• Large VSD
• Overriding of the aorta
• RV outflow tract obstruction
• RV hypertrophy

Question 24

Tetralogy of Fallot on physical exam (2):

Answer 24

• Single S2

- Harsh systolic ejection murmur along the left sternal border that radiates into the back

Question 25

Tet spells (2):

Answer 25

- Severe and often prolonged arterial desaturation - Acute and significant increase in R-L shunting secondary to a change in the ratio of pulmonary and systemic vascular resistance

Question 26

Tet spells are characterized by (5):

Answer 26

- Irritability - Hyperpnea - Marked cyanosis - Pallor - Lethargy or unconsciousness

Question 27

Clinical features of tricuspid atresia (4):

Answer 27

- Cyanosis in the neonatal period - LV impulse - Single S2 - Systolic murmur

Question 28

Clinical features of transposition of the great arteries (2):

Answer 28

- Cyanosis in the neonatal period | - Single S2

Question 29

Clinical features of truncus arteriosus (4):

Answer 29

- Symptoms of HF with mild cyanosis - Single S2 - Holosystolic murmur from the VSD - Systolic ejection click and diastolic murmur from truncal valve regurgitation

Question 30

Unobstructed TAPVR presents with (4):

Answer 30

- Feeding problems - Tachypnea - Poor growth - Respiratory infection

Question 31

Unobstructed TAPVR on physical exam (4):

Answer 31

- RV impulse - Widely split S2 - Systolic ejection murmur at left upper sternal border - Diastolic rumble at left lower sternal border

Question 32

Obstructive TAPVR:

Answer 32

Respiratory distress and often severe cyanosis on the first day of life