Pathoma - Cardiac congenital defects

Question 1

Describe Eisenmenger Syndrome

Answer 1

Reversal of a shunt from L–>R to R–>L

L to R shunt causes increased flow through pulmonary circulation which then leads to hypertrophy of the pulmonary vessels and pulmonary

HTN Increased pulmonary resistance results in reversal of the shunt and late cyanosis

Question 2

What is the most common congenital heart defect

Answer 2

Ventricular septal defect

Question 3

Describe the presentation of ventricular septal defect

Answer 3

L to R shunt

Small shunts often asymptomatic

Large defects can lead to Eisenmenger syndrome

Question 4

Common association of VSD

Answer 4

Fetal alcohol syndrome

Question 5

Describe the presentation of atrial septal defect

Answer 5

L to R shunt with split S2 on auscultation (increased blood in R heart delays closure of pulmonary valve)

Question 6

Complication of ASD

Answer 6

Paradoxical emboli (venous emboli that travel from RA to LA to arterial system)

Question 7

Disease associate with patent ductus arteriosus

Answer 7

Congenital rubella

Question 8

Presentation of PDA

Answer 8

L to R shunt between aorta and pulmonary artery

May lead to Eisenmenger syndrome

Question 9

Describe the pattern of cyanosis in PDA

Answer 9

Lower extremity cyanosis (ductus arteriosus occurs after the upper extremities already branch off)

Question 10

Treatment of PDA

Answer 10

Indomethacin (decreases PGE, which is what keeps the PDA open)

Question 11

What are the 4 defects in tetralogy of fallot?

Answer 11

(1) Pulmonic artery stenosis
(2) RV hypertrophy
(3) VSD
(4) Displacement of aorta over the VSD

Question 12

Presentation of Tetralogy of fallot

Answer 12

R to L shunt (due to pulmonic stenosis) causes early cyanosis

Patients squat in response to cyanotic spell

‘Boot-shaped’ heart on X-ray

Question 13

Describe the defect in Transposition of the great vessels

Answer 13

Pulmonary artery arises from the LV and aorta arises from the RV

Results in two independent circuits that do not mix

Question 14

Disease associated with transposition of the great vessels

Answer 14

Maternal diabetes

Question 15

Treatment of Transposition of the great vessels

Answer 15

Creation of a shunt is necessary for survival

PGE can be given to maintain PDA until definitive surgical repair is performed

Question 16

Describe the defect in Truncus Arteriosus

Answer 16

Single large vessel arising from both ventricles

Question 17

Presentation of Truncus arteriosis

Answer 17

Early cyansosis because deoxygenated blood from RV mixes with oxygenated blood from LV before pulmonary and aortic circulations separate

Question 18

How is the RV affected by tricuspid atresia

Answer 18

RV is hypoplastic

Question 19

What other defect is associated with tricuspid atresia?

Answer 19

ASD, resulting in R to L shunt

Presents with early cyanosis

Question 20

Where is the location of infantile aortic coarctation and describe the presentation

Answer 20

Coarctation lies after (distal to) the aortic arch, but proximal to the PDA

Associated with PDA

There will be a R to L shunt at the PDA (after the coarct), leading to lower extremity cyanosis

Question 21

What disease is associated with infantile aortic coarctation

Answer 21

Turner syndrome

Question 22

Where is the location of adult aortic coarctation and describe the presentation

Answer 22

Coarctation lies distal to the aortic arch with NO patent ductus arteriosis

This lead to shunting of the blood to the vessels of the upper extremities (HTN of UE and hypotension and weak pulses of LE)

Question 23

Describe X-ray findings of adult aortic coarctation

Answer 23

Collateral vessels will form proximal to the coarct

Collaterals will develop across intercostal arteries, and engorged arteries will cause ‘notching’ of the ribs on x-ray

Question 24

What other defect is associated with adult aortic coarctation

Answer 24

Bicuspid aortic valve