Tricuspid Atresia Flashcards
1
Q
What is Tricuspid Atresia?
A
- The absence of the tricuspid valve with hypoplasia of the right ventricle
2
Q
What is the pathophysiology of Tricuspid Atresia?
A
- The tricuspid valve is absent which means the right ventricle is hypoplastic due to the absence of inflow of blood to the right ventricle.
- There also tends to be a ventricular septal defect
- There also must be an inter atrial communication - foramen ovale
3
Q
What are the clinical features of Tricuspid Atresia?
A
- History: Poor feeding
- Examination:
General - Progressive Cyanosis, Palpation - Systolic thrill associated with pulmonary stenosis
Auscultation - Single S2 with pan- systolic murmur = VSD, Patent Ductus Arteriosus = mechanical murmur
4
Q
What Investigations would you do for Tricuspid Atresia?
A
- Chest X-ray: Reduced/ Increased Pulmonary markings depending in relation of the great vessels. Heart size may be normal/ increased with enlargement of the RA and LV.
- ECHO
5
Q
What Management would you do for Tricuspid Atresia?
A
- IV PGE1 infusion - to prevent the closure of the PDA
- Balloon Atrial Septostomy - balloon septostomy is required if the inter-atrial communication is inadequate which shows a restrictive flow pattern
- Surgical - Fontan Procedure - allows blood from the systemic venous return to avoid the heart and go straight to the lungs. This means only a single ventricle is required to pump blood to the body.
