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Congenital Defects

Adult With Unrepaired Single-Ventricle Defect

To identify the key insights or developments described in this article

1 Credit CME

JAMA Cardiology

Published Online: September 6, 2023

Cardiovascular Images

Article Information and Disclosures

David Gregg IV, MD¹;

Andrew D. Vogel, MS²;

Taufiek K. Rajab, MD²

Author Affiliations

¹Section of Adult Congenital Cardiology, Medical University of South Carolina, Charleston
²Section of Pediatric Cardiothoracic Surgery, Medical University of South Carolina, Charleston

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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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Article Information

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 (rajabt@musc.edu).

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.

References

Rao PS . Double-inlet left ventricle. Children (Basel). 2022;9(9):1274.PubMed Google Scholar

Rao PS . Management of congenital heart disease: state of the art—part ii—cyanotic heart defects. Children (Basel). 2019;6(4):54. doi:10.3390/children6040054 PubMed Google Scholar Crossref

Muller WH Jr , Danimann JF Jr . The treatment of certain congenital malformations of the heart by the creation of pulmonic stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow; a preliminary report. Surg Gynecol Obstet. 1952;95(2):213-219.PubMed Google Scholar

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.