Hypoplastic Right Heart Disease (Pulmonary Atresia)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Etiology/Pathophysiology:
This consists of a complete obstruction of the right ventricle outflow tract due to a hypoplastic pulmonary artery. When the ventricular septum is intact a patent foramen ovale is the site of the right to left shunt with a PDA and / or bronchial collateral arteries providing the only source of pulmonary blood flow. There are two types:

• Small right ventricle with a thick wall and a hypoplastic but competent tricuspid valve. This is the most common.
• Normal right ventricle with a patent but incompetent tricuspid valve.

Pathology:
Not applicable

Imaging Findings:
On CXR decreased pulmonary vasculature and cardiomegaly is seen.

DDX:
Cardiomegaly and decreased pulmonary vasculature:

• Pulmonary atresia with an intact VSD
• Ebsteins
• Tricuspid regurgitation secondary to transient myocardial dysfunction
• Persistent fetal circulation

References:
See References Chapter.

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