Congenital heart diseases

Question 1

Ventricular septal defect (VSD) murmur

Answer 1

Holosystolic at LLSB. Continuous throughout systole. Smaller shunt the louder the murmur.

Question 2

VSD Management

Answer 2

Diuretic, ACE inhibitor +/- digoxin.

Question 3

VSD surgical indications

Answer 3

unmanageable heart failure, failure of medical management, a shut >1.5-2.

Question 4

Atrial septal defect

Answer 4

Usually secundum which is a failure of the septum primum and secundum to overlap. Patent foramen ovale is where they don’t fuse (not as serious). 2:1 shunt.

Question 5

ASD murmur

Answer 5

low pressure to low pressure so no murmur through the shunt. The murmur is a pulmonary flow murmur due to increased pulmonary flow. Often mistaken as an innocent murmur.

Question 6

ASD surgical indications

Answer 6

Elective closure by 1-5 years old.

Question 7

Untreated ASD complications

Answer 7

Right heart enlargement, Eisenmenger’s syndrome (pulmonary resistance increases so much the direction of flow changes leading to acute cyanosis and death), paradoxical emboli (usually filtered by the lung but can enter systemic circulation).

Question 8

Atrioventricular septal defect (AVSD)

Answer 8

endocardial cushions are absent so there is one common mitral/tricuspid valve and an AV canal. Very common in kids with down syndrome. Leads to pulmonary vascular obstructive disease.

Question 9

Patent ductus arteriosus murmur

Answer 9

During diastole and systole. Continuous machinery murmur usually heard best from the back.

Question 10

Patent ductus arteriosus symptoms

Answer 10

hyperdynamic precordium with a wide pulse pressure due to diastolic run off.

Question 11

Patent ductus arteriosus management

Answer 11

Indomethacin or ibuprofen that inhibit prostaglandins. Can need surgery.

Question 12

Still’s murmur

Answer 12

Innocent murmur. Musical/vibratory systolic at the LSB (not in back). Decreases with expiration and standing.

Question 13

Physiologic peripheral pulmonic stenosis (PPPS)

Answer 13

Innocent murmur due to fetal anatomy. soft/harsh systolic ejection murmur best heard in the axilla (bilaterally). Usually disappears by 12 months.

Question 14

Right-to-left shunt 5Ts

Answer 14

Truncus arteriosus, transposition, tricuspid atresia, tetralogy of fallot, total anomalous pulmonary venous return (TAPVR).

Question 15

Cyanosis

Answer 15

need 5g/dl of deoxygenated Hb. Often hidden with anemia or exaggerated in babies due to the higher affinity of fetal Hb. Need to check sats in a LE and UE (differential cyanosis).

Question 16

Tetralogy of Fallot

Answer 16

1. ) Right ventricular output tract obstruction (due partly to an over-riding aota and supravalvular stenosis secondary to decreased blood flow)
2. ) VSD
3. ) Over-riding aorta (due to abnormal rotation)
4. ) right ventricular hypertrophy

Question 17

Tet spell causes

Answer 17

dehydration, anesthesia, crying.

Question 18

Tet spell management

Answer 18

Increase systemic pressure by squatting, volume resuscitation or a vasoconstrictor.

Question 19

Truncus arteriosus

Answer 19

One common aorta and pulmonary artery with a large VSD. huge increase in pulmonary blood flow. Commonly associated with digeorge syndrome. Surgically repair within the first two weeks.

Question 20

Transpostion of the great vessels

Answer 20

improper rotation. completely separates the two circulations. Have to have an ASD and/or patent ductus.

Question 21

Transpostion of the great vessels treatment

Answer 21

1. ) PGE (prostaglandins) keep the ductus arteriosus open. “blue baby think prostaglandins!”
2. ) Balloon atrial septostomy to open the ASD and allow more mixing.
3. ) arterial switch operation within the first two weeks.

Question 22

Transpostion of the great vessels XRAY

Answer 22

narrow mediastinum with an enlarged heart. “egg on a string”

Question 23

Total Anamolous Pulmonary Venous Return (TAPVR)

Answer 23

The pulmonary veins migrate towards the heart during development but can get lost and form a confluence that can often get obstructed. Also has an ASD (need some mixing).

Question 24

Supracardiac TAPVR

Answer 24

Joins a vertical vein up to the brachiocephalic vein. 25-50% become obstructed.

Question 25

Cardiac TAPVR

Answer 25

Drain into the coronary sinus. Acts a lot like an ASD. Reparied within the first six months.

Question 26

Infracardiac TAPVR

Answer 26

drains through a descending vein to the liver then through the ductus venosus into the IVC. Extremely sick often obstructive. EMERGENCY! Rule out with an echo before assuming a respiratory problem!

Question 27

Coarctation of the Aorta

Answer 27

Narrowing of the aorta usually “juxtaductal” because when the ductus arteriosus closes at birth it can close part of the aorta. Associated with turner’s syndrome.

Question 28

Coarctation of the Aorta in neonates

Answer 28

Extremis usually before 2 days old. LV begins to fail. absent/decreased femoral pulses. Treat with prostaglandins!

Question 29

Coarctation of the Aorta in infants

Answer 29

irritable, tachypneic, CHF, absent/decreases femoral pulses

Question 30

Coarctation of the Aorta in teens

Answer 30

Can be well tolerated due to blood flow through the internal thoracics back into the descending aorta. Diastolic runoff. Rib notching. HA, nose bleeds, decreased femoral pulses. Unexplained HTN (differential pressures).

Question 31

Coarctation of the Aorta murmur in teens

Answer 31

systolic at LUSB or left interscapular (aorta is in the back) can be continuous murmur.

Question 32

Coarctation of the Aorta diagnostics

Answer 32

CT-angio is gold standard.

Question 33

Acyanotic Defects

Answer 33

left-to-right. VSD, ASD, PDA, A-V canal, Coartation of the aorta