RIGHT BUNDLE-BRANCH BLOCK AND ST-SEGMENT ELEVATION IN LEADS V-1 THROUGH V-3 - A MARKER FOR SUDDEN-DEATH IN PATIENTS WITHOUT DEMONSTRABLE STRUCTURAL HEART-DISEASE

Background-Five years ago, we described a specific ECG pattern of righ t bundle-branch block and ST-segment elevation in leads V-1 through V- 3 associated with sudden death in patients without demonstrable struct ural heart disease. Information on long-term outcome has become availa ble due to pooled data on a large cohort of patients with this syndrom e who are followed at 33 centers worldwide. Methods and Results-Data o n 63 patients (57 men; mean age, 38 +/- 17 years) with the described E CG pattern were analyzed in terms of arrhythmic events and sudden deat h. Events were analyzed for patients with at least one episode of abor ted sudden death or syncope of unknown origin before recognition of th e syndrome (symptomatic patients, n=41) and for patients in whom the E CG pattern was recognized by chance or because of screening related to sudden death of a relative (asymptomatic patients, n=22). During a me an follow-up of 34 +/- 32, months, an arrhythmic event occurred in 14 symptomatic patients (34%) and 6 asymptomatic patients (27%). An autom atic defibrillator was implanted in 35 patients, 15 received pharmacol ogical therapy with beta-blockers and/or amiodarone, and 13 did not re ceive treatment The incidence of arrhythmic events was similar in all therapy groups (log-rank 0.86); however, total mortality was 0% in the implantable defibrillator group, 26% in the pharmacological group, an d 31% in the no therapy group (log-rank 0.0005). All mortality was due to sudden death. Conclusions-Patients without demonstrable structural heart disease and an ECG pattern of right bundle-branch block and ST- segment elevation in leads V-1 through V-3 are at risk for sudden deat h. Amiodarone and/or beta-blockers do not protect them against sudden death, and an implantable defibrillator seems to be the present treatm ent of choice.