Glossary of Abbreviations:BHLH (borderline hypoplastic left heart), BiVC (biventricular conversion), BSA (body surface area), CMR (cardiac magnetic resonance), EDVi (end diastolic volume/body surface area), EF (ejection fraction), EFE (endocardial fibroelastosis), HR (hazard ratio), LVEDP (left ventricular end diastolic pressure), LV (left ventricular), PVR (pulmonary vascular resistance), SVi (stroke volume/body surface area)
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Dr. Beattie’s current affiliation is Lucile Packard Children’s Hospital, Stanford University School of Medicine, Palo Alto, CA.
Dr. Kapoor’s current affiliation is State University of New York Downstate Medical Center, New York, NY.
Dr. Oladunjoye’s current affiliation is Reading Hospital, Reading, PA.
Dr. Banka’s current affiliation is Merck & Co, Inc, Rahway, NJ.
Disclosures: The authors have indicated they have no potential conflicts of interest.
Funding: Funded in part by the Higgins Family Noninvasive Research Fund.
The study design was approved by the Institutional Review Board and Committee on Clinical Investigation at Boston Children’s Hospital with a waiver of informed consent (Protocol IRB-P00027334, approved 2/18/17).
Central picture: Estimated freedom from the primary outcome based on presence of endocardial fibroelastosis.
Endocardial fibroelastosis and smaller indexed left ventricular stroke volume are associated with mortality, transplant, and adverse hemodynamic outcome after biventricular conversion.
Patients with borderline hypoplastic left hearts may be considered for left ventricular recruitment and biventricular conversion after initial single ventricle palliation, but optimal patient selection remains challenging. This study identifies risk factors associated with long-term adverse outcomes including diastolic dysfunction, mortality, transplant, and takedown to single ventricle circulation.