Aamir Hameed, Nasir Rehman, Fahim H Jafary.
Arrhythmogenic right Ventricular Dysplasia - case report from Pakistan and review of literature.
Pak Heart J Jan ;35(1-4):20-5.
A 55-year-old retired, soldier from Gilgit in northern Pakistan was referred to our hospital with a three-year history of intermittent retrosternal chest discomfort and a few months history of progressively increasing dyspnea. At presentation, he was limited to walking a few steps. A few weeks prior to presentation he suffered a syncopal episode and presented to a local hospital. At that time he was found to be in sustained ventricular tachycardia, requiring cardioversion and treatment with antiarrhythmic drugs. However, no specific etiology of this patient`s arrhythmia was identified. At presentation to our hospital, the patient had overt signs of right heart failure. The ECG showed an incomplete RBBB with nonspecific T wave changes in the anterior leads. An echocardiogram showed normal left ventricular function, a massively dilated and poorly contracting right ventricle. In addition, there was hypertrophy of the moderator band and akinesis and loss of muscle at the right ventricular apex. A coronary angiogram was negative for any obstructive disease. Angiography of the right ventricle revealed massive dilatation, trabecular thickening and localized bulging. A tentative diagnosis of ARVD was made in view of the clinical and echocardiographic features. The patient improved somewhat with diuretics. Cost constraints did not permit the implantation of an implantable defibrillator and the patient was discharged on oral amiodarone.
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