Nisha A Gilotra1, Aditya Bhonsale2, Cynthia A James2, Anneline S J Te Riele2, Brittney Murray2, Crystal Tichnell2, Abhishek Sawant2, Chin Siang Ong2, Daniel P Judge2, Stuart D Russell2, Hugh Calkins2, Ryan J Tedford2. 1. From the Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, MD (N.A.G., A.B., C.A.J., B.M., C.T., C.S.O., D.P.J., S.D.R., H.C., R.J.T.); Division of Cardiology, University Medical Center Utrecht, The Netherlands (A.S.J.t.R.); and Division of Cardiology, Department of Medicine, University at Buffalo, State University of New York (A.S.). naggarw2@jhmi.edu. 2. From the Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, MD (N.A.G., A.B., C.A.J., B.M., C.T., C.S.O., D.P.J., S.D.R., H.C., R.J.T.); Division of Cardiology, University Medical Center Utrecht, The Netherlands (A.S.J.t.R.); and Division of Cardiology, Department of Medicine, University at Buffalo, State University of New York (A.S.).
Abstract
BACKGROUND: Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D. METHODS AND RESULTS: Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. HF was present in 142 patients (49%): 113 had isolated RV involvement and 29 had evidence of LV dysfunction. Average age of HF onset was 40±14 years. Most commonly reported symptoms were exertional dyspnea (78%) and fatigue (73%). Only 40% (n=57/142) had signs of volume overload. Left-sided HF signs were rare. Patients with clinical HF before ARVC/D diagnosis (n=31) were older (P=0.005) and met fewer Task Force Criteria (P=0.013) than those who developed HF after ARVC/D presentation. Female sex (odds ratio, 2.2; 95% confidence interval, 1.21-4.01; P=0.01) and lateral precordial T-wave inversions (odds ratio, 9.87; 95% confidence interval, 1.07-91.1; P=0.043) were associated with increased odds of HF. Additionally, patients with symptomatic LV dysfunction had higher odds of lateral precordial T-wave inversions (odds ratio, 18.4; 95% confidence interval, 2.92-116.18; P=0.002). Patients with HF were more likely to undergo heart transplantation (15/142 versus 1/147; P<0.001) or die during study follow-up period (7 versus 0; P=0.007). CONCLUSIONS: HF symptoms, especially exertional dyspnea, are common in ARVC/D; yet, classic left-sided signs are typically absent and less than half have evidence of volume overload. Given the unique predominately right-sided phenotype, a large portion of patients with HF may be under-recognized.
BACKGROUND:Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D. METHODS AND RESULTS: Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. HF was present in 142 patients (49%): 113 had isolated RV involvement and 29 had evidence of LV dysfunction. Average age of HF onset was 40±14 years. Most commonly reported symptoms were exertional dyspnea (78%) and fatigue (73%). Only 40% (n=57/142) had signs of volume overload. Left-sided HF signs were rare. Patients with clinical HF before ARVC/D diagnosis (n=31) were older (P=0.005) and met fewer Task Force Criteria (P=0.013) than those who developed HF after ARVC/D presentation. Female sex (odds ratio, 2.2; 95% confidence interval, 1.21-4.01; P=0.01) and lateral precordial T-wave inversions (odds ratio, 9.87; 95% confidence interval, 1.07-91.1; P=0.043) were associated with increased odds of HF. Additionally, patients with symptomatic LV dysfunction had higher odds of lateral precordial T-wave inversions (odds ratio, 18.4; 95% confidence interval, 2.92-116.18; P=0.002). Patients with HF were more likely to undergo heart transplantation (15/142 versus 1/147; P<0.001) or die during study follow-up period (7 versus 0; P=0.007). CONCLUSIONS: HF symptoms, especially exertional dyspnea, are common in ARVC/D; yet, classic left-sided signs are typically absent and less than half have evidence of volume overload. Given the unique predominately right-sided phenotype, a large portion of patients with HF may be under-recognized.
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Authors: Paul J Scheel; Roberta Florido; Steven Hsu; Brittney Murray; Crystal Tichnell; Cynthia A James; Julia Agafonova; Harikrishna Tandri; Daniel P Judge; Stuart D Russell; Ryan J Tedford; Hugh Calkins; Nisha A Gilotra Journal: J Am Heart Assoc Date: 2020-02-03 Impact factor: 5.501