scholarly journals Heart transplantation strategies in arrhythmogenic right ventricular cardiomyopathy: a tertiary ARVC centre experience

2021 ◽  
Author(s):  
Paul J. Scheel ◽  
Katherine Giuliano ◽  
Crystal Tichnell ◽  
Cynthia James ◽  
Brittney Murray ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

Abstract 13646: Clinical Profile and Ablation Outcomes in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy and Biventricular Involvement

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Lishui Shen ◽  
Lihui Zheng ◽  
Yan Yao
Keyword(s):  
Catheter Ablation ◽  
Heart Transplantation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Clinical Profile ◽  
Ventricular Cardiomyopathy ◽  
Free Survival ◽  
Younger Age ◽  
Procedural Success

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive disease which can involve both ventricles. This study aimed to elucidate the clinical profile and ablation efficacy in Chinese ARVC patients with biventricular (BiV) involvement. Methods and Results: Based on cardiovascular magnetic resonance findings, 255 ARVC patients were divided into two groups: the BiV involved group (n=137) and the isolated right ventricular (RV) involved group (n=118). Patients with BiV involvement were more likely to suffer from heart failure (28.5% vs. 8.5%; P <0.001) and recurrent syncope (32.8% vs. 21.2%; P =0.038), and presented with lower heart transplantation-free survival and composed endpoint (death or heart transplantation)-free survival (all P <0.05). Of the 255 subjects, 52 BiV involved patients and 77 isolated RV involved patients underwent catheter ablation for ventricular tachycardia (VT). After 5 years’ follow-up, the outcomes including VT recurrence and all-cause mortality in 2 groups showed no difference (all P >0.05). The age, amount of induced VT during electrophysiology study and non-acute procedural success (partial success, defined as inducing the same VT with cycle length 30% longer after ablation; substrate modification only) were independent predictors of VT recurrence in BiV involved patients (all P <0.05). Conclusion: BiV involvement in ARVC indicated worse symptoms and poor prognosis. Nonetheless, catheter ablation remained effective for VT control in this population. The younger age, more induced VTs and non-acute procedural success were associated with VT recurrence after ablation.

scholarly journals Heart transplantation in arrhythmogenic right ventricular cardiomyopathy — Experience from the Nordic ARVC Registry

2018 ◽  
Vol 250 ◽  
pp. 201-206 ◽  
Author(s):  
Thomas Gilljam ◽  
Kristina H. Haugaa ◽  
Henrik K. Jensen ◽  
Anneli Svensson ◽  
Henning Bundgaard ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

Survival After Heart Transplantation in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

2017 ◽  
Vol 23 (2) ◽  
pp. 107-112 ◽  
Author(s):  
Eugene C. DePasquale ◽  
Richard K. Cheng ◽  
Mario C. Deng ◽  
Ali Nsair ◽  
William J. McKenna ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

Three Cases of Orthotopic Heart Transplantation for Arrhythmogenic Right Ventricular Cardiomyopathy

2005 ◽  
Vol 80 (6) ◽  
pp. 2358-2360 ◽  
Author(s):  
Masataka Yoda ◽  
Kazutomo Minami ◽  
Dirk Fritzsche ◽  
Gero Tendrich ◽  
Sebastian Schulte-Eistrup ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Orthotopic Heart Transplantation ◽  
Ventricular Cardiomyopathy

Abstract 2935: Clinical-Instrumental Characteristics and Prognosis in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy: 30 Years of Experience in a Referral Center

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Andrea Di Lenarda ◽  
Bruno Pinamonti ◽  
Marco Merlo ◽  
Alberto Pivetta ◽  
Stylianos Pyxaras ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Stress Test ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Ventricular Cardiomyopathy

There are few studies regarding the clinical and instrumental presentation of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC); all available data derive from few specialized centers. We sought to describe clinical characteristics, instrumental findings and prognosis in a large cohort of patients (pts) with an ARVC diagnosis. From 1976 to 2006 all pts matching ARVC diagnostic criteria were enrolled in the Heart Muscle Disease Registry of Trieste and underwent a structured diagnostic protocol and follow-up. 104 pts were studied, 66% males, mean age 33±13 years; 84% were symptomatic at enrolment (mean duration 44±67 months); 75% had symptomatic arrhythmias (43% with previous sustained ventricular tachycardia or ventricular fibrillation (SVT/VF)), 36% syncope, 20% heart failure (12% in NYHA class III-IV). Familial ARVC were present in 46% of pts. ECG was abnormal in 72% (right bundle branch block 25%; epsilon potentials 15%; anterior negative T waves 51%; QRS dispersion >40ms 11%). At signal averaged ECG late potentials were present in 58% of pts. At 2D echo, mean right ventricle (RV) end-diastolic area was 30± 8 cm2 and RV fractional area contraction (FAC) 30±13%; RV aneurysms were present in 67%. RV systolic dysfunction (defined as FAC<40%) was present in 78%, characterized severe (FAC <30%) in 48%. Left ventricular systolic dysfunction was present in 27% of pts. Stress test was interrupted in 11% of cases for ventricular arrhythmias (55% SVT/VF). RV fibrofatty tissue was present in 52% of Nuclear Magnetic Resonance and in 59% of RV endomyocardial biopsy (performed respectively in 24 and 27% of pts). During a mean follow-up of 132±89 months, 3 pts were lost; 20 out of the remaining 101 pts (20%) experienced death/heart transplantation, 6 (3%) died for refractory HF; 4 pts (2%) underwent heart transplantation (HT); 6 pts (3%) died suddenly; one (0.5%) for extracardiac disease and 3 (1.6%) for unknown reason. Ten-year cumulative survival free from HT was 57%. In our population ARVC presentation was characterized by symptomatic, frequently sustained ventricular arrhythmias. Notably, LV involvement was present in approximately one third of pts at enrolment. In the long term, the overall prognosis was severe.

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