Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.

Introduction: Atrial fibrillation/flutter (Afib/Aflu) in general, and clinical variables predicting Afib/Aflu in particular, are not well defined in patients with arrhythmogenic right ventricular dysplasia (ARVD). Hypothesis: We hypothesized that transthoracic echocardiography (TTE) and ECG could be helpful to predict Afib/Aflu in these patients. Methods and Results: 12-lead ECGs and TTEs of 90 patients from three tertiary-care centers diagnosed with definite or borderline ARVD according to the 2010 Task Force Criteria were analyzed. Data were compared in two patient groups: (1) patients with Afib/Aflu and (2) all other patients. Eighteen (20%) patients experienced Afib/Aflu during a follow-up period of 5.8 years (interquartile range 2.0-10.4 years). Kaplan-Meier analysis (Figure) revealed reduced times to Afib/Aflu among patients with echocardiographic RV fractional area change <27% (p<0.001), left atrial diameter ≥24.4 mm/m2 (p=0.001), and right atrial short axis diameter ≥22.1 mm/m2 (p=0.05). From all ECG variables, P sinistroatriale conferred the highest hazard ratio (3.37, 95% CI 0.92-12.36, p=0.067). Five patients with Afib/Aflu experienced inappropriate ICD shocks compared to four patients without Afib/Aflu (36% vs. 9%, p=0.03). Presence of Afib/Aflu was more prevalent in heart transplanted patients and in those who succumbed to cardiac death compared to the remaining patients (56% vs. 16%, p=0.014). Conclusions: Afib/Aflu are associated with inappropriate ICD shocks, heart transplantation, and cardiac death in patients with ARVD. Echocardiographic evidence of reduced RV function and atrial dilation helps to identify those ARVD patients being at increased risk for Afib/Aflu, which may help to guide individual patient management.

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Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy According to Revised 2010 Task Force Criteria With Inclusion of Non-Desmosomal Phospholamban Mutation Carriers

The American Journal of Cardiology ◽

10.1016/j.amjcard.2013.06.017 ◽

2013 ◽

Vol 112 (8) ◽

pp. 1197-1206 ◽

Cited By ~ 29

Author(s):

Judith A. Groeneweg ◽

Paul A. van der Zwaag ◽

Louise R.A. Olde Nordkamp ◽

Hennie Bikker ◽

Jan D.H. Jongbloed ◽

...

Keyword(s):

Right Ventricular ◽

Task Force ◽

Arrhythmogenic Right Ventricular Dysplasia ◽

Right Ventricular Dysplasia ◽

Mutation Carriers

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Abstract 10906: Heart Failure is Common and Under Recognized in Patients With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Circulation ◽

10.1161/circ.132.suppl_3.10906 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Nisha A Gilotra ◽

Abhishek Sawant ◽

Aditya Bhonsale ◽

Anneline S te Riele ◽

Cynthia A James ◽

...

Keyword(s):

Heart Failure ◽

Heart Disease ◽

Right Ventricular ◽

Structural Changes ◽

Task Force ◽

Structural Heart Disease ◽

Arrhythmogenic Right Ventricular Dysplasia ◽

Small Subset ◽

Study Cohort ◽

Right Ventricular Dysplasia

Introduction: The reported incidence of heart failure (HF) in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) varies depending on study cohort ranging from <10 to 20%. Though risk of fatal arrhythmias is well described, the heterogenous manifestations of HF in this population are less well known. Hypothesis: With more prompt recognition of ARVD/C and subsequent prevention of SCD, HF is becoming more prevalent amongst ARVD/C patients. Methods: Patients from the Johns Hopkins ARVD/C Program Registry meeting the 2010 Revised Task Force Criteria with completed HF symptom survey (n=134) and/or available HF clinical assessment (n=154) were included. HF signs and symptoms were retrospectively adjudicated. Results: Of the 288 patients studied, 236 had evidence of structural heart disease defined as right ventricular (n=199) or biventricular dilatation/dysfunction (n=37). At least one clinical sign or symptom of HF was present in 123 (52%) of those with structural disease (42 had one and 61 had two or more signs/symptoms). The most commonly reported symptoms were dyspnea on exertion (n=87) and fatigue (n=77). Evidence of volume retention (edema or ascites) was found in 46/236 (20%), and left-sided HF symptoms were rare. Of the 105 patients with structural heart disease and known age at HF symptom onset, average time from first ARVD/C presentation to HF symptoms was 6.3 ± 8.0 years, and 40% had at least one episode of sustained ventricular tachycardia or ICD shock prior to HF symptoms. Presence of a genetic mutation for ARVD/C did not correlate with HF symptoms or structural changes. Sixteen patients underwent orthotopic heart transplant. Conclusions: HF symptoms are common in the ARVD/C population with structural heart disease. Though there is a small subset of patients with evident volume overload requiring therapy, there is a larger portion of patients with HF symptoms that may be under recognized.

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