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23 AN UNUSUAL ASSOCIATION BETWEEN ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA AND ANOMALOUS CORONARY ARTERY.
  1. P. M. Huang,
  2. W. N. O'Connor,
  3. C. M. Cottrill
  1. University of Kentucky, Department of Pediatrics, Lexington, KY

Abstract

The etiology of arrhythmogenic right ventricular dysplasia (ARVD) is not well understood. There appears to be a genetic component, with an autosomal dominant pattern, although a much rarer autosomal recessive inheritance pattern has also been seen. In most cases, ARVD is believed to be caused by a nonischemic injury that leads to thinning and fatty infiltration of the right ventricular muscle cells, with sparing of the left ventricle. The fatty infiltrate is the cause of the arrhythmias. Individuals with ARVD usually have a normal right coronary artery. Two unusual cases of this disease are associated with anomalous origin of the right coronary artery (ARC) from the left coronary cusp with an anomalous course between the aorta and pulmonary artery. One is a 17-year-old male who had multifocal PVCs. After ECHO suggested ARC, an MRI was diagnostic for both ARVD and ARC. The other is a 33-year-old airline pilot who developed ventricular tachycardia (VT). EP study documented two foci of inducible VT, which were ablated. When VT continued and cardiac catheterization showed ARC, he underwent bypass surgery. The arrhythmias continued to occur and further workup yielded the diagnosis of ARVD. Neither of these patients have a family history of ARVD. The potential relationship between coronary flow and developmental myocardial abnormalities will be discussed.

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