Final answer:
Epstein anomaly is a congenital heart defect characterized by displacement of the tricuspid valve into the right ventricle, abnormalities in the chordae tendineae, arterialization of the right ventricle, enlarged right atrium, potential atrial septal defects, and possible inferior vena cava anomalies.
Step-by-step explanation:
Features of Epstein anomaly, which is a rare congenital heart defect primarily affecting the tricuspid valve, include:
- Displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle.
- Attachment of the tricuspid leaflets to the underlying myocardium by abnormally formed chordae tendineae, which may be redundant, fenestrated, short, or even absent.
- Atrialization of a portion of the right ventricle, which is the incorporation of the ventricular tissue into the right atrium due to the downward displacement of the valve.
- Enlargement of the right atrium due to tricuspid regurgitation or failure of the heart chambers to function properly.
- Potential association with a patent foramen ovale or an atrial septal defect, which is a hole in the fossa ovalis region of the interatrial septum, leading to the mixing of oxygenated and deoxygenated blood.
- Abnormalities of the Inferior vena cava, which brings deoxygenated blood from the lower half of the body to the heart, may also be present.
These features lead to a variety of clinical manifestations ranging from asymptomatic to serious heart failure or cyanosis, necessitating timely diagnosis and management.