Thomas Gilljam1, Kristina H Haugaa2, Henrik K Jensen3, Anneli Svensson4, Henning Bundgaard5, Jim Hansen6, Göran Dellgren7, Finn Gustafsson8, Hans Eiskjær3, Arne K Andreassen9, Johan Sjögren10, Thor Edvardsen2, Anders G Holst8, Jesper Hastrup Svendsen8, Pyotr G Platonov11. 1. Department of Cardiology, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. 2. Department of Cardiology, Centre for Cardiological Innovation, Institute for Surgical Research, Oslo University Hospital, Rikshospitalet, Oslo, Norway; University of Oslo, Oslo, Norway. 3. Department of Cardiology, Aarhus University Hospital, and Department of Clinical Medicine, Health, Aarhus University, Aarhus, Denmark. 4. Department of Cardiology and Department of Medical and Health Sciences, Linköping University, Linköping, Sweden. 5. Unit for Inherited Cardiac Diseases, The Heart Center, The National University Hospital, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 6. Department of Cardiology, Gentofte Hospital, University of Copenhagen, Hellerup, Denmark. 7. Transplant Centre, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. 8. Department of Cardiology, The Heart Centre, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 9. Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. 10. Department of Cardiothoracic Surgery, Skåne University Hospital, Lund University, Lund, Sweden. 11. Dept. of Cardiology, Lund University and Arrhythmia Clinic, Skåne University Hospital, Lund, Sweden. Electronic address: pyotr.platonov@med.lu.se.
Abstract
OBJECTIVE: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants. METHODS: Data from 31 ARVC/HTx patients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46years (14-65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry. RESULTS: The HTx patients were younger at presentation, median 31 vs. 38years (p=0.001). There was no difference in arrhythmia-related events. The indication for HTx was heart failure in 28 patients (90%) and ventricular arrhythmias in 3 patients (10%). During median follow-up of 4.9years (0.04-28), there was one early death and two late deaths. Survival was 91% at 5years after HTx. Age at first symptoms under 35years independently predicted HTx in our cohort (OR=7.59, 95% CI 2.69-21.39, p<0.001). CONCLUSION: HTx in patients with ARVC is performed predominantly due to heart failure. This suggests that current 2016 International Society for Heart and Lung Transplantation heart transplant listing recommendations for other cardiomyopathies could be applicable in many cases when taking into account the haemodynamic consequences of right ventricular failure in conjunction with ventricular arrhythmia.
OBJECTIVE: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants. METHODS: Data from 31 ARVC/HTxpatients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46years (14-65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry. RESULTS: The HTxpatients were younger at presentation, median 31 vs. 38years (p=0.001). There was no difference in arrhythmia-related events. The indication for HTx was heart failure in 28 patients (90%) and ventricular arrhythmias in 3 patients (10%). During median follow-up of 4.9years (0.04-28), there was one early death and two late deaths. Survival was 91% at 5years after HTx. Age at first symptoms under 35years independently predicted HTx in our cohort (OR=7.59, 95% CI 2.69-21.39, p<0.001). CONCLUSION:HTx in patients with ARVC is performed predominantly due to heart failure. This suggests that current 2016 International Society for Heart and Lung Transplantation heart transplant listing recommendations for other cardiomyopathies could be applicable in many cases when taking into account the haemodynamic consequences of right ventricular failure in conjunction with ventricular arrhythmia.
Authors: Pasquale Vergara; Wendy S Tzou; Roderick Tung; Chiara Brombin; Alessandro Nonis; Marmar Vaseghi; David S Frankel; Luigi Di Biase; Usha Tedrow; Nilesh Mathuria; Shiro Nakahara; Venkat Tholakanahalli; T Jared Bunch; J Peter Weiss; Timm Dickfeld; Dhanunjaya Lakireddy; J David Burkhardt; Pasquale Santangeli; David Callans; Andrea Natale; Francis Marchlinski; William G Stevenson; Kalyanam Shivkumar; William H Sauer; Paolo Della Bella Journal: Circ Arrhythm Electrophysiol Date: 2018-12
Authors: Katherine Giuliano; Paul Scheel; Eric Etchill; Charles D Fraser; Alejandro Suarez-Pierre; Steven Hsu; Ilan S Wittstein; Edward K Kasper; Roberta Florido; Harikrishna Tandri; Hugh Calkins; Chun W Choi; Kavita Sharma; Ahmet Kilic; Nisha A Gilotra Journal: ESC Heart Fail Date: 2022-02-08
Authors: Hanne M Boen; Bart L Loeys; Maaike Alaerts; Johan B Saenen; Inge Goovaerts; Lut Van Laer; Anne Vorlat; Tom Vermeulen; Constantijn Franssen; Patrick Pauwels; Inez Rodrigus; Hein Heidbuchel; Emeline M Van Craenenbroeck Journal: J Heart Lung Transplant Date: 2022-04-09 Impact factor: 13.569