35. Congenital heart disease with right to left shunt.

Question 1

RIGHT-LEFT-SHUNTS

Answer 1

Tetralogy of Fallot

Transpositon of the Great Arteries

Tricuspid Atresia

Question 2

Tetralogy of Fallot

the Tetralogy

Answer 2

1. VSD
2. Pulmonary Stenosis
3. Overriding Aorta
4. Right Ventricular Hypertrophy

Question 3

Tetralogy of Fallot

CLINIC

IMAGING

TREATMENT

Answer 3

• CLINIC:
  
  • might initially be acyanotic
  • pulmonary stenosis murmur
  • single S2 & right ventricular impulse
  • hypoxic spells
  • usually: restlesness, agitated crying
  • squatting
• IMAGING:
  
  • ECG - usually right axis deviation
  • x-ray: right ventricular hypertrophy, boot-shaped heart
  • visible in echo
• TREATMENT:
  
  • hypoxic spells: Oxygen admin. knee-chest position, morphine, alpha-agonist (phenyleprine)
  • COMPLETE SURGICAL REPAIR

Question 4

Transposition of the Great Arteries

Abstract

CLINICAL MANIFESTATIONS

Answer 4

• • aorta arises from the RV; PA arises from the LV
    
    • desaturated blood returns to the right side of the heart and oxygenated blood flows back to the lungs
• CLINICAL MANIFESTATIONS:
  
  • history of cyanosis is always present
  • quiet tachypnea & single S2
  • if VSD is also present - improved mixing & less cyanosis

Question 5

Transposition of the Great Arteries

Answer 5

• IMAGING:
  
  • ECG: right axis deviation, right ventricular hypertrophy
  • x-ray:
    
    • increased pulmonary vascularity
    • cardiac shadow: egg on a string
• TREATMENT:
  
  • prostaglandin E1 to maintain ductal patency
  • if persists: balloon atrial septostomy
    
    • improved mixing
  • COMPLETE SURGICAL REPAIR

Question 6

Tricuspid Atresia

Abstract

CLINIC

Answer 6

• abscence of the tricuspid valve
• hypoplastic right ventricle
• PDA/VSD is neccesary for survival
• CLINIC:
  
  • severe cyanosis
  • single S2
  • IF VSD murmur

Question 7

Tricuspid Atresia

IMAGING

TREATMENT

Answer 7

• IMAGING:
  
  • left ventricular hypertrophy & superior QRS axis
  • x-ray: normal/mildly enlarged cardiac silhouette
  • echo: anatomy, assoc. lesions are source of pulm blood flow
• TREATMENT:
  
  • management depends on the presence of VSD
  • prostaglandin E1 maintains pulmonary blood flow until surgery