Fetopathology and developmental pathology of the embryo and fetus: Tricuspid atresia

+ Introduction

+ Support

+ Atlas of fetal pathology

+ Congenital malformations of individual organ systems

Tricuspid atresia

Macroscopic appearance

Tricupis atresia:

there is a dimple at the usual site of the valve or the valve forms an imperforate membrane
right ventricle is hypoplastic, represented only by the outlet portion of the ventricle,so called the outlet chamber
ventricular septal defect is invariably present

Ebstein malformation of the tricuspid valve: the septal and posterior leaflets adhere to the right ventricular wall. The inlet portion of the ventricle in thus included functionally within the right atrium. The malformed tricuspid valve is insufficient.

Classification

Further classification of tricupis atresia is based on the great vesells position:

tricuspid atresia with normally related great arteries, 70% of case
tricuspid atresia with transposition of great arteries
pulmonary stenosis, pulmonary atresia are common, coarctation of the aorta is seen in tricuspid atresia with transposition of great vessels

Clinical signs

tricuspid atresia is a rare cyanotic defect
the diagnosis of an isolated Ebstein malformation is often late during childhood or early adulthood. Infants with severe regurgitation are severely cyanotic

Case study

Tricuspid atresia

Marta Ježová

History

23-week fetus, tricuspid atresia:

Pictures

Tricuspid atresia, base of the heart: Atresia valvae tricuspidalis, Macro, autopsy (72041)

Tricuspid atresia, view from the right atrium: Atresia valvae tricuspidalis, Macro, autopsy (72042)

Tricuspid atresia, right ventricle hypoplasia: Atresia valvae tricuspidalis, Macro, autopsy (72043)

Tricuspid atresia, ventricular septal defect, view from the left ventricle: Atresia valvae tricuspidalis, Macro, autopsy (72045)

Tricuspid atresia, ventricular septal defect, view from the right ventricle: Atresia valvae tricuspidalis, Macro, autopsy (72044)