부산대학교 도서관

Key Words Shone's syndrome; congenital heart disease; mitral valve intervention Abstract Objective Shone's syndrome (SS) has a varied anatomic spectrum without consensus on need and timing for mitral valve intervention (MVI). We sought to (1) characterize the anatomic spectrum and treatment pathways; (2) describe long-term outcomes and their determinants; and (3) define the impact of MVI timing on survival. Methods In total, 121 patients with SS who underwent operation at Cleveland Clinic between 1956 and 2021 were reviewed. Multivariable parametric hazard analyses including time-varying covariables, and modulated renewal to account for repeated events, were performed. End points included time-related survival and reintervention. Results Median follow-up was 9.9 years. Mitral stenosis (MS) (98%), coarctation (80%), and aortic stenosis (70%) predominated. The most common combination was MS + aortic stenosis + coarctation (26%). Median initial mean mitral and aortic gradients were 3.6 (15th/85th percentiles: 2.0/6.8) and 9.0 (2.1/46) mm Hg, respectively. Median initial surgery age was 0.041 (0.011/3.2) years. Initial surgeries included coarctation repair (43%), arch repair (18%), and staged biventricular repair (18%). Overall survival was 92% at 20 years. Freedom from reoperation was 66% and 24% at 1 and 20 years. Patients with no MVI or initial MVI (N = 7) tended to be associated with better early survival compared with those with MVI at subsequent operation (N = 29) (P = .06). Risk factors for early reintervention included initial Norwood operation, with younger age and arch hypoplasia increasing later reintervention. Conclusions Despite excellent long-term survival, reoperation in SS is frequent and occurs most commonly on left ventricular outflow tract and mitral valve. Although MS is present in most, few require MVI. Delaying MVI may compromise early survival. Abbreviations and Acronyms AS, aortic stenosis; AV, aortic valve; CCF, Cleveland Clinic Foundation; LV, left ventricle; MS, mitral stenosis; MV, mitral valve; MVI, mitral valve intervention; SS, Shone's syndrome Author Affiliation: (a) Department of Thoracic and Cardiothoracic Surgery, Cleveland Clinic, Cleveland, Ohio (c) Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland, Ohio (d) Division of Pediatric Cardiac Surgery and the Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio (a) Department of Thoracic and Cardiothoracic Surgery, Cleveland Clinic, Cleveland, Ohio (b) Case Western Reserve University School of Medicine, Cleveland, Ohio (c) Department of Quantitative Health Sciences, Lerner Research Institute, Cleveland, Ohio (d) Division of Pediatric Cardiac Surgery and the Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio * Address for reprints: Tara Karamlou, MD, MSc, Division of Pediatric Cardiac Surgery, Cleveland Clinic Children's, M41-022A, 9500 Euclid Ave, Cleveland, OH 44195. Article History: Received 16 November 2021; Revised 3 May 2022; Accepted 6 May 2022 (footnote)* Working Group Members: Moses Anabila, MSc, Yezan Salam, MBBSa, Munir Ahmad, MDd, Robert Stewart, MDd, Gosta Pettersson, MD, PhDd, Hani K. Najm, MD, MScd Byline: Leah J. Lee, BA (a,b), Dominique L. Tucker, BS (a,b), Sohini Gupta, BA (a,b), Naseeb Shaheen, MD (a), Jeevanantham Rajeswaran, PhD (c), Tara Karamlou, MD, MSc [karamlt@ccf.org] (d,*), Moses Anabila, MS (c), Yezan Salam, MBBS (a), Munir Ahmad, MD (d), Robert Stewart, MD (d), Gosta Pettersson, MD, PhD (d), Hani K. Najm, MD, MSc (d)