Usefulness of Total 12-Lead QRS Voltage for Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy in Patients With Heart Failure Severe Enough to Warrant Orthotopic Heart Transplantation and Morphologic Illustration of Its Cardiac Diversity

Although several electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) (also called dysplasia) have been described, total 12-lead QRS voltage is not one of them. This report describes total 12-lead QRS voltage in 11 patients with ARVC who underwent orthotopic heart transplantation (OHT) because of progressively severe heart failure. Additionally, it illustrates the varied morphologic features of ARVC. The total 12-lead nonpaced QRS voltages before OHT ranged from 28 to 118 mm (mean 74 ± 32), and those in the paced tracings, from 33 to 129 mm (62 ± 32). The voltages are the lowest we have encountered among 12 previously reported cardiovascular conditions. The heart weights among the 11 ARVC patients ranged from 285 to 670 g (mean 448 ± 125). Very low 12-lead QRS voltage is characteristic of patients with ARVC with heart failure severe enough to warrant OHT, and thus may serve as a clue to its diagnosis.