Which of the following is the MOST LIKELY diagnosis for a newborn with cyanosis, diminished pulmonary vascularity, and an enlarged cardiac silhouette?
Cardiac Imaging
No
U
B
Patients with pulmonary atresia (or critical pulmonary stenosis) with an intact ventricular septum present with diminished pulmonary blood flow because of the outflow obstruction. The right atrium is always enlarged and right ventricular enlargement is also present when the tricuspid valve is regurgitant. Therefore, the cardiac silhouette is typically enlarged. Affected patients are dependent on a patent ductus.
WRONG ANSWERS:
Patients with Tetralogy of Fallot do not have an enlarged cardiac silhouette.
Patients with a complete atrioventricular canal defect are typically acyanotic and present with overcirculation once pulmonary vascular resistance decreases in the first few weeks of life.
Patients with complete transposition of the great vessels can present with either increased or decreased pulmonary blood flow, but the cardiac silhouette is typically not enlarged.
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