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Course: Adult With Unrepaired Single-Ventricle Defect

CME Credits: 1.00

Released: 2023-09-06

A male adult in his 50s with New York Heart Association class III heart failure was referred for surgical evaluation. On physical examination, he did not appear to have cyanosis. Computed tomography angiography () showed the left atrium and right atrium draining into a double-inlet left ventricle (DILV), a nonrestrictive ventricular septal defect straddling the tricuspid valve, a diminutive right ventricle, levotransposition of the aorta and pulmonary artery (PA), and severe pulmonary stenosis. Cardiac catheterization revealed blood pressures of 125/10 mm Hg in the DILV, 50/20 mm Hg in the PA, and 125/60 mm Hg in the aorta; systemic oxygen saturation of 80%; pulmonary vascular resistance of 4.3 Wood Units · m2, and a systemic flow ratio (Qp:Qs) of 1:1. DILV is typically managed in early childhood according to the Fontan pathway.- If the native circulation is balanced with a protected pulmonary bed, patients with single ventricles can survive long term without intervention.


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