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CLINICAL RESEARCH: ARRHYTHMOGENIC CARDIOMYOPATHY

Left-Dominant Arrhythmogenic Cardiomyopathy

An Under-Recognized Clinical Entity

Srijita Sen-Chowdhry, MBBS, MD (Cantab), MRCP^_*,,_*, Petros Syrris, PhD^_*, Sanjay K. Prasad, MD, MRCP, Siân E. Hughes, MBBS, PhD, MRCPath, Robert Merrifield, PhD, Deirdre Ward, MBBS, MRCPI^_*, Dudley J. Pennell, MD, FACC and William J. McKenna, MD, DSc, FACC^_*

^_* Inherited Cardiovascular Disease Group, The Heart Hospital, London, United Kingdom
Department of Histopathology, Royal Free and University College Medical School, University College London, London, United Kingdom
Cardiovascular Magnetic Resonance Unit, National Heart & Lung Institute, London, United Kingdom
Wolfson Foundation Medical Image Computing Laboratory, Imperial College, London, United Kingdom

Manuscript received July 23, 2008; accepted September 4, 2008.

^_* Reprint requests and correspondence: Dr. Srijita Sen-Chowdhry or Prof. William J. McKenna, Inherited Cardiovascular Disease Group, The Heart Hospital, 16–18 Westmoreland Street, London W1G 8PH, United Kingdom (Email: srijita{at}aol.com).

Objectives: We sought to investigate the clinical-genetic profile of left-dominant arrhythmogenic cardiomyopathy (LDAC).

Background: In the absence of coronary disease and left ventricular (LV) systolic dysfunction, lateral T-wave inversion and arrhythmia of LV origin are often considered benign. Similarly, chest pain with enzyme release might be attributed to viral myocarditis. We hypothesized that these abnormalities might be manifestations of the "left-dominant" subtype of arrhythmogenic right ventricular cardiomyopathy.

Methods: The 42-patient cohort was established through clinical evaluation of individuals with unexplained (infero)lateral T-wave inversion, arrhythmia of LV origin, and/or proven LDAC/idiopathic myocardial fibrosis in the family.

Results: Patients presented from adolescence to age >80 years with arrhythmia or chest pain but not heart failure. Desmosomal mutations were identified in 8 of 24 families (15 of 33 patients). Magnetic resonance findings included LV late-enhancement in a subepicardial/midwall distribution, corresponding to fibrofatty replacement and fibrosis on histopathology. Fifty percent had previously been misdiagnosed with viral myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, or idiopathic ventricular tachycardia. Arrhythmic events included presentation with ventricular fibrillatory arrest in 1 patient and 2 instances of sudden cardiac death during follow-up.

Conclusions: Arrhythmogenic cardiomyopathy is distinguished from DCM by a propensity towards arrhythmia exceeding the degree of ventricular dysfunction. The left-dominant subtype is under-recognized owing to misattribution to other disorders and lack of specific diagnostic criteria. Clinicians are alerted to the possibility of LDAC in patients of any age with unexplained arrhythmia of LV origin, (infero)lateral T-wave inversion, apparent DCM (with arrhythmic presentation), or myocarditis (chest pain and enzyme rise with unobstructed coronary arteries).

Key Words: arrhythmia • cardiomyopathy • electrocardiography • genetics • magnetic resonance imaging • sudden death

Abbreviations and Acronyms   ARVC = arrhythmogenic right ventricular cardiomyopathy   CI = confidence interval   CMR = cardiovascular magnetic resonance   DCM = dilated cardiomyopathy   ECG = electrocardiogram   HCM = hypertrophic cardiomyopathy   IMF = idiopathic myocardial fibrosis   LBBB = left bundle branch block   LDAC = left-dominant arrhythmogenic cardiomyopathy   LGE = late gadolinium enhancement   LV = left ventricle/ventricular   LVEDV = left ventricular end-diastolic volume   LVEF = left ventricular ejection fraction   LVNC = left ventricular noncompaction   PVC = premature ventricular complex   RBBB = right bundle branch block   RV = right ventricle/ventricular   SCD = sudden cardiac death   VT = ventricular tachycardia   WMA = wall motion abnormality

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