12/17/2023 0 Comments Iftikhar kullo mayo clinic![]() The diagnosis was made in two patients as a result of screening after right DISCUSSION At the time of initial assessment, five patients had right-sided congestive heart failure. In 11 patients, arrhythmia was the initial manifestation of these patients, 6 had sustained ventricular tachycardia, and 1 had an out-of hospital cardiac arrest. Ages at the time of diagnosis ranged from 3 to 60 years (mean age, 30). ![]() General Features.-Of the 20 patients in the study group, 12 (60%) were female (Table 1). Patients with conditions associated with right ventricular dilatation, such as RESULTS Study Subjects.-Study patients were identified by two criteria: (1) presence of abnormal amounts of adipose tissue in either endomyocardial biopsy specimens or explanted hearts from patients with ventricular arrhythmias or heart failure and (2) echocardiographic appearance of dilated cardiomyopathy confined to the right ventricle in the setting of a clinical picture consistent with right ventricular dysplasia. Of the 16 living patients, 8 are doing well, 3 have an implanted cardiac defibrillator, 3 are receiving antiarrhythmic agents, and 2 have undergone cardiac transplantation because of progressive biventricular failure. During the follow up period, four patients died: two died suddenly, one died of congestive heart failure, and one died of respiratory failure after a coronary artery bypass operation. Variable left ventricular involvement was noted in 50% of the cases. ![]() Inordinate right ventricular enlargement was present in 60% of the patients at first echocardiographic assessment and in two other patients on follow - up assessment. Characteristic fatty infiltration of the myocardium was present in 7 of 13 right ventricular biopsy specimens. On Holter monitoring, all but 2 of 15 patients studied had frequent ventricular ectopic activity (Lown grade 2 or more). Ventricular tachycardia with morphologic features of left bundle branch block was inducible in seven of nine patients. First-order relatives were affected in 30% of the patients. In the 12 female and 8 male patients (mean age, 30 years range, 3 to 60), the initial manifestations of right ventricular dysplasia included ventricular arrhythmia (45%), congestive heart failure (25%), heart murmur (10%), asymptomatic (10%), complete heart block (5%), and sudden death (5%).
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