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J Am Coll Cardiol, 2008; 52:2175-2187, doi:10.1016/j.jacc.2008.09.019
© 2008 by the American College of Cardiology Foundation
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CLINICAL RESEARCH: ARRHYTHMOGENIC CARDIOMYOPATHY

Left-Dominant Arrhythmogenic Cardiomyopathy

An Under-Recognized Clinical Entity

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

* Inherited Cardiovascular Disease Group, The Heart Hospital, London, United Kingdom
{dagger} Department of Histopathology, Royal Free and University College Medical School, University College London, London, United Kingdom
{ddagger} 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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