Samuel G Wittekind1,2,3, Thomas D Ryan4,5, Zhiqian Gao4, Farhan Zafar4, Richard J Czosek4,5, Clifford W Chin4, John L Jefferies4,5. 1. Cincinnati Children's Hospital Medical Center, Heart Institute, Cincinnati, OH, USA. Samuel.Wittekind@cchmc.org. 2. Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA. Samuel.Wittekind@cchmc.org. 3. Cincinnati Children's Hospital Medical Center, Heart Institute, Heart Failure/Transplant Program, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, 45229-3026, USA. Samuel.Wittekind@cchmc.org. 4. Cincinnati Children's Hospital Medical Center, Heart Institute, Cincinnati, OH, USA. 5. Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Abstract
BACKGROUND: Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx. METHODS: All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated. Outcomes of those listed for HTx (N = 18) were compared to a benchmark of contemporaneous pediatric RCM patients in the UNOS database (N = 377). Proportional hazards models were used to determine predictors of adverse outcomes. RESULTS: The mean age was 11 ± 9 years and 49% were male. 14 of 18 patients listed received HTx. Overall mortality (12%) was identical between the phenotypes; however, RCM patients were more likely to be listed (P = 0.001) and receive HTx (P = 0.02) compared to RCM/HCM. Prior to HTx, 60% had documented arrhythmia, 16% had cardiac arrest, and 7% required mechanical circulatory support. 4 of 17 patients with an ICD/PM received device therapies (four of five shocks appropriate for VT/VF, and two effective anti-tachycardia pacing interventions). Outcomes of those listed for HTx at our center were similar to the UNOS benchmark. In multivariate analysis, markers of congestive heart failure were associated with adverse outcomes. CONCLUSION: Heart failure and arrhythmia treatments can delay or possibly prevent the need for HTx in some cases of pediatric RCM. Survival post-HTx is not compromised using this approach.
BACKGROUND: Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx. METHODS: All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated. Outcomes of those listed for HTx (N = 18) were compared to a benchmark of contemporaneous pediatric RCMpatients in the UNOS database (N = 377). Proportional hazards models were used to determine predictors of adverse outcomes. RESULTS: The mean age was 11 ± 9 years and 49% were male. 14 of 18 patients listed received HTx. Overall mortality (12%) was identical between the phenotypes; however, RCMpatients were more likely to be listed (P = 0.001) and receive HTx (P = 0.02) compared to RCM/HCM. Prior to HTx, 60% had documented arrhythmia, 16% had cardiac arrest, and 7% required mechanical circulatory support. 4 of 17 patients with an ICD/PM received device therapies (four of five shocks appropriate for VT/VF, and two effective anti-tachycardia pacing interventions). Outcomes of those listed for HTx at our center were similar to the UNOS benchmark. In multivariate analysis, markers of congestive heart failure were associated with adverse outcomes. CONCLUSION:Heart failure and arrhythmia treatments can delay or possibly prevent the need for HTx in some cases of pediatric RCM. Survival post-HTx is not compromised using this approach.
Authors: Joshua M Friedland-Little; Anna Joong; Svetlana B Shugh; Matthew J O'Connor; Neha Bansal; Ryan R Davies; Michelle S Ploutz Journal: Pediatr Cardiol Date: 2022-03-24 Impact factor: 1.838
Authors: Tyler R Reinoso; Maicon Landim-Vieira; Yun Shi; Jamie R Johnston; P Bryant Chase; Michelle S Parvatiyar; Andrew P Landstrom; Jose R Pinto; Hanna J Tadros Journal: J Muscle Res Cell Motil Date: 2020-11-11 Impact factor: 3.352
Authors: Maully J Shah; Michael J Silka; Jennifer N Avari Silva; Seshadri Balaji; Cheyenne M Beach; Monica N Benjamin; Charles I Berul; Bryan Cannon; Frank Cecchin; Mitchell I Cohen; Aarti S Dalal; Brynn E Dechert; Anne Foster; Roman Gebauer; M Cecilia Gonzalez Corcia; Prince J Kannankeril; Peter P Karpawich; Jeffery J Kim; Mani Ram Krishna; Peter Kubuš; Martin J LaPage; Douglas Y Mah; Lindsey Malloy-Walton; Aya Miyazaki; Kara S Motonaga; Mary C Niu; Melissa Olen; Thomas Paul; Eric Rosenthal; Elizabeth V Saarel; Massimo Stefano Silvetti; Elizabeth A Stephenson; Reina B Tan; John Triedman; Nicholas H Von Bergen; Philip L Wackel Journal: Indian Pacing Electrophysiol J Date: 2021-07-29