Clinical course of arrhythmogenic right ventricular cardiomyopathy with end-stage heart failure and outcome after heart transplantation - 05/02/23
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Highlights |
• | Extended disease at diagnosis is frequent in patients with ARVC who need HT. |
• | Patients have LV dysfunction, diffuse repolarization abnormalities & DSG2 mutations. |
• | The main indication for HT is end-stage biventricular dysfunction. |
• | Usually, it takes a long time to progress to end-stage heart failure needing HT. |
• | The median time between ARVC diagnosis and HT was 9 years. |
• | Primary graft dysfunction needing ECMO was frequent after HT. |
• | Primary graft dysfunction occurred despite the absence of PH and low PVR. |
• | Survival after HT improved over the years, with better outcomes from the 2010s. |
Abstract |
Background |
Few data exist on the characteristics and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy and advanced heart failure who undergo heart transplantation.
Aim |
To explore the pretransplant course and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy after heart transplantation.
Methods |
This observational retrospective monocentric study included all consecutive patients with arrhythmogenic right ventricular cardiomyopathy who underwent heart transplantation during a 13-year period (2006–2019) at Pitié-Salpêtrière University Hospital (Paris).
Results |
A total of 23 patients with arrhythmogenic right ventricular cardiomyopathy underwent heart transplantation between 2006 and 2019. The median time from diagnosis to heart transplantation was 9 years, and the median age at transplantation was 50 years. At diagnosis, half of the patients had left ventricular dysfunction, 59% had extensive T-wave inversion and 43% had a history of sustained ventricular tachycardia. Only five patients were involved in intensive sport activity. Indications for heart transplantation were end-stage biventricular dysfunction in 13 patients, end-stage right ventricular heart failure in seven and electrical storm in three. Only three patients had pulmonary hypertension, and half of the patients had atrial arrhythmias. The survival rate 1 year after heart transplantation was 74% (95% confidence interval 53–88%). Eight patients experienced primary graft dysfunction needing extracorporeal membrane oxygenation.
Conclusions |
Patients with arrhythmogenic right ventricular cardiomyopathy who eventually needed heart transplantation mostly exhibited extended disease with biventricular dysfunction at diagnosis. Intensive sport activity did not seem to be a major determinant. Advanced heart failure usually occurred late in the course of the disease. Primary graft dysfunction after heart transplantation was frequent, and should be anticipated. Additional data are needed to identify the optimal timing for heart transplantation and predictors of end-stage heart failure in patients with arrhythmogenic right ventricular cardiomyopathy.
Le texte complet de cet article est disponible en PDF.Keywords : Arrhythmogenic right ventricular cardiomyopathy, Arrhythmogenic right ventricular dysplasia, ARVC, Heart transplantation, Heart failure
Abbreviations : anti-HLA, ARVC, ECMO, HF, HT, ICD, IMPACT, IQR, LV, LVEF, PGD, RV, RVEF, TTE, VT
Plan
Vol 116 - N° 1
P. 9-17 - janvier 2023 Retour au numéro
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