Fact-sheet: Tricuspid atresia
Updated on 04/21/2016 at 11:48 PM
View all RADEOS cases associated with this fact-sheet
Definition
Absence of the tricuspid valve, replaced by muscular tissue or a membrane.
Very rare and severe anomaly, accounting for approximately 1% of congenital heart disease, with a prevalence of approximately 1/25,000 births.
Clinical features
Constant, early, and severe cyanosis.
No signs of heart failure.
Then, depending on complications: hepatomegaly due to cardiac cirrhosis...
Radiography
Cardiomegaly.
Dilatation of the pulmonary arteries and their branches.
Ultrasound
Echocardiography: establishes the diagnosis but requires complementary cardiac catheterization
CT
Anomalies secondary to atresia:
- Cardiomegaly with right atrial dilatation
- Right ventricular hypoplasia
- Non-visualization of a communication between the right atrium and right ventricle.
Anomalies related to reparative surgery:
- Patent (reopened) ductus arteriosus
- Atrial septal defect
- Ventricular septal defect
- Anastomosis between the superior vena cava and the pulmonary trunk
Management
Neonatal surgery
Classification
The most widely used classification is that of Edwards and Burchell:
Type 1: normally positioned great vessels (70%)
-A: pulmonary atresia with intact septum
-B: pulmonary stenosis, often restrictive VSD
-C: no stenosis of the right ventricular outflow tract
Type 2: D-transposition of the great vessels (30%)
-A: pulmonary stenosis
-B: no pulmonary stenosis, pulmonary overcirculation due to a non-restrictive VSD.
The most common form is type 1B ++++