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 (Figure) 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.1- 3 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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Published Online: September 6, 2023. doi:10.1001/jamacardio.2023.2748
Corresponding Author: Taufiek K. Rajab, MD, Section of Pediatric Cardiothoracic Surgery, Medical University of South Carolina, 10 McClennan Banks Dr, SJCH 2190/MSC 918, Charleston, SC 29425 (firstname.lastname@example.org).
Conflict of Interest Disclosures: Dr Rajab reported receiving grants from the American Association for Thoracic Surgery, the South Carolina Clinical and Translational Research Institute, the National Institutes of Health/National Center for Advancing Translational Sciences, the Brett Boyer Foundation, the Saving Tiny Hearts Society, the Emerson Rose Heart Foundation, and philanthropy by Senator Paul Campbell. No other disclosures were reported.
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