Mitral valve orifice area predicts outcome after biventricular repair in patients with hypoplastic left ventricles

• Published in: Journal of cardiovascular magnetic resonance Vol. 26; no. 1; p. 101029
• Main Authors: Liddle, David, Gearhart, Addison, Sleeper, Lynn A., Lu, Minmin, Feins, Eric, Schidlow, David N., Ghelani, Sunil, Powell, Andrew J., Emani, Sitaram, Beroukhim, Rebecca S.
• Format: Journal Article
• Language: English
• Published: England Elsevier Inc 2024; Elsevier
• Subjects: Biventricular; Cardiac Surgical Procedures - adverse effects; Child; Child, Preschool; Congenital heart disease; Female; Humans; Hypoplastic left heart syndrome; Hypoplastic Left Heart Syndrome - diagnostic imaging; Hypoplastic Left Heart Syndrome - physiopathology; Hypoplastic Left Heart Syndrome - surgery; Infant; Male; Mitral valve; Mitral Valve - abnormalities; Mitral Valve - diagnostic imaging; Mitral Valve - physiopathology; Mitral Valve - surgery; Mitral Valve Insufficiency - diagnostic imaging; Mitral Valve Insufficiency - physiopathology; Mitral Valve Insufficiency - surgery; Original Research; Predictive Value of Tests; Retrospective Studies; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome; Ventricular Function, Left; AVV; TV; BTT; Hypoplastic left heart syndrome; RV; EFE; Svi; LV; Congenital heart disease; BiV; BSA; EDVi; HLV; ECMO; ESVi; EF; Mitral valve; VSD; ICC; CMR; MV; IQR; Biventricular; AVC; PA; AV
• ISSN: 1097-6647; 1532-429X
• DOI: 10.1016/j.jocmr.2024.101029

Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV). Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m2) and no endocardial fibroelastosis who underwent BiV repair from 2005–2022. CMR measurements included MV orifice area in diastole. The primary composite outcome included time to death, transplant, BiV takedown, heart failure admission, left atrial decompression, or unexpected reoperation; and the secondary outcome included more than or equal to moderate mitral stenosis and/or regurgitation. Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p < 0.01). Independent predictors of primary outcome for AVC patients included MV orifice area z-score <−2 and transitional AVC; for non-AVC patients, predictors included MV orifice area z-score <−2, abnormal MV anatomy, and conal-septal ventricular septal defect. Independent predictors of secondary outcome for AVC patients included older age at surgery, transitional AVC, and transposition of the great arteries. In children with HLV, low MV orifice area and pre-existing MV pathology are risk factors for adverse outcome after BiV repair. [Display omitted]