Clinical: Congenital Disease

Question 1

What is important to remember regarding the atria in utero?

Answer 1

There is always a communication between atria so blood can flow from the RA to the LA.

Question 2

What results from a failure of the ventricular outflow tracts to form from a normal spiral pattern?

Answer 2

Transposition of the great vessels.

Question 3

What are the two normal physiologic Right to Left shunts that occur in fetal circulation?

Answer 3

1. RA to LA: oval foramen

2. Pulmonary Artery to Descending Aorta: Ductus Arteriosus

Question 4

What is the most common ASD and how can it be treated?

Answer 4

Patent Ostium Secundum

-only ASD that can be treated percutaneous (as of now). PCI button seals the defect.

Question 5

Major complication with Ostium Primum ASD.

Answer 5

Because the defect is so low in the atria, sometimes the ventricles are also involved as well as both AV valves (more commonly mitral valve). Is asymptomatic in early life but eventually presents as HF symptoms.

Question 6

Condition associated with early symptoms of Ostium Primum ASD.

Answer 6

Down Syndrome

Question 7

Describe Eisenmenger Syndrome and how it’s treated.

Answer 7

Congenital (VSD) or surgical left to right shunt that results in high pulmonary pressure. Eventually the pulmonary pressure rises high enough to switch the direction of the shunt resulting in a Right to Left shunt and cyanosis.

By the time symptoms arise the only treatment is heart and/or lung transplant because correction of the shunt results in RV failure and death.

Question 8

What medications can close a Patent Ductus Arteriosus?

Answer 8

NSAIDs

Question 9

What meds can keep a PDA open and why would this be done?

Answer 9

Prostaglandins can keep a PDA open. In these individuals often the lungs were poorly perfused in utero and continue to be poorly perfused so keeping the ductus arteriosus open allows better perfusion of the lungs.

Question 10

If a patient presents with pulmonary stenosis, what two other conditions should always be ruled out?

Answer 10

Tetralogy of Fallot

ASD

Question 11

Which conditions are associated with coarctation of the aorta?

Answer 11

Bicuspid aortic valve (75-85%)
Turner’s syndrome
Berry aneurysms (cranial vessels)
Ascending aortic aneurysms
Aortic dissection
Subacute bacterial endocarditis (SBE)
PDA
VSD (Coarctation Syndrome)

Question 12

Name the 4 characteristics of Tetralogy of Fallot (TOF).

Answer 12

1. VSD
2. Overriding Aorta
3. Pulmonary Stenosis
4. RVH

Question 13

In TOF not much blood can reach the lungs from the RV due to the pulmonary stenosis. How does blood reach the lungs after birth?

Answer 13

PDA

-the fetal circulatory path stays open longer

Question 14

How do children present with TOF?

Answer 14

Cyanosis and squatting with exertion. The squatting increases TPR which increases venous return to the RA allowing blood to go from the right side of the heart into the lungs to get oxygenated.

Question 15

What is a Blalock-Taussig (BT) Shunt?

Answer 15

Original treatment for TOF. Involves anastomosing the Rt. subclavian artery to the right pulmonary arteries to increase blood flow to the lungs.

Question 16

What is the problem with the Blalock-Taussig Shunt?

Answer 16

Results in pulmonary hypertension. The lungs are normally designed to handle low pressure. With this shunt they experience systemic pressure.

Question 17

What is the current treatment for TOF?

Answer 17

Fix the VSD and remove the RV outflow tract obstruction.

Question 18

What is the surgical treatment for Tricuspid Valve Atresia?

Answer 18

Tx depends on the severity of the pulmonary valve stenosis.

1. BT shunt if severe pulmonary stenosis
2. If moderate pulmonary stenosis

• Classic Glenn (unidirectional): attach SVC to the right pulmonary artery (prevents pulmonary HTN)
• Bidirectional Glenn: attach SVC to the pulmonary vessels near the pulmonary trunk
• Fonton Procedure

Question 19

Describe the two stages of the Fonton Procedure.

Answer 19

Stage 1
-bidirectional Glenn procedure
-remove BT shunt
Stage 2
-attach the IVC to the pulmonary arteries effectively bypassing the right heart

Question 20

With transposition of the great arteries, why was an atrial septation and baffle not an effective long term treatment?

Answer 20

It only served to redirect the flow from the atria to the ventricles. The RV had to deal with the high systemic pressure and would eventually fail.

Question 21

Describe a Total (or partial) Anomalous Pulmonary Venous Return (TAPVR).

Answer 21

The pulmonary veins do not flow into the LA. Sometimes they may flow into the SVC or RA resulting in cyanosis.