A 15 year old youth, who presented with out-of-hospital cardiac arrest due to documented ventricular fibrillation, was found to have nonobstructive hypertrophic cardiomyopathy. Electrophysiologic study demonstrated inducible sustained atrial fibrillation with a rapid ventricular response. This rhythm, associated with hypotension and evidence of myocardial ischemia, spontaneously degenerated into ventricular fibrillation. No ventricular arrhythmias were inducible by programmed ventricular stimulation. Therapy with metoprolol and verapamil slowed the ventricular rate during atrial fibrillation and maintained hemodynamic stability, both during follow-up electrophysiologic study and during a subsequent spontaneous episode.

Our findings, and similar observations by others, suggest involvement of the sinus node and the distal conduction system in this form of the LQTS. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed. Development of an internal cardiac defibrillator for this young age group is necessary.

An 18-year-old man presented with a history of oral sores and presence of high fever, scrotal ulcerations and haemoptysis. Multiple mural cardiac masses were present in the right atrium, right ventricle and left ventricle. Furthermore, pulmonary vasculitis with aneurysm formation and venous thrombosis involving the superior sagittal sinus and right transverse sinus were found, and the diagnosis was made of (incomplete) Behçet's disease. While receiving anticoagulation and later, treatment with prednisone and cyclophosphamide, the cardiac thrombi gradually disappeared. We stress the importance of early echocardiography to evaluate cardiac abnormalities in Behçet's disease.

We analyzed long-term follow-up data accumulated during an 8 year study of survivors of prehospital cardiac arrest. All patients included in this study were DURING THE PAST 15 years. the success of community-based systems for intervention in prehospital cardiac arrest has established a new population of patients -the survivors of prehospital cardiac arrest who require highly specialized medical attention because of the risk of recurrent cardiac arrest.`Survival after cardiac arrest can be measured in three distinct time frames: (1) from the onset of cardiac arrest to hospitalization, (2) from the period of initial in-hospital care to discharge, and (3) as long-term outcome in patients who have been discharged from the hospital. The earli- Presented in part at the 56th Scientific Sessions of The American Heart Association, Anaheim, CA. November, 1983. est available data were discouraging with respect to outcome in each of these periods: a 60% to 70% prehospital mortality rate, a 50% to 60% mortality rate in hospitalized patients (i.e., 10% to 15% of the total prehospital arrest population survived both prehospital and in-hospital care), and a 45% 24 month rate of recurrence of cardiac arrest among posthospital survivors.

The relation between time to first shock and clinical outcome was studied in 60 patients who received an automatic implantable cardioverter-defibrillator (AICD) from August 1983 through May 1988. The mean (+/- SD) patient age was 64 +/- 10 years, 82% were men and the mean ejection fraction was 33 +/- 13%. During follow-up, 38 patients (63%) had one or more shocks; there were no differences in age, gender distribution or ejection fraction at entry between the shock and no shock groups. Among 51 patients with coronary artery disease, 31 (61%) had one or more shocks, whereas all seven patients with cardiomyopathy had one or more shocks (p less than 0.05). Neither of the two patients with idiopathic ventricular fibrillation had shocks. Of the 13 deaths, 12 occurred during post-hospital follow-up and 1 during the index hospitalization. Of the four sudden post-hospital deaths, only one was due to tachyarrhythmia in the absence of acute myocardial infarction. All four sudden deaths and five of eight post-hospital nonsudden deaths occurred in patients who had had one or more appropriate shocks during follow-up. Eight of the nine first appropriate shocks among patients who subsequently died occurred within the first 3 months of follow-up, but the actual deaths were delayed to a mean of 14.1 +/- 13.9 months (p less than 0.05). The mean time to all deaths was 14.8 +/- 13.1 months. The ejection fraction was significantly lower among patients who died than among patients who survived (25 +/- 7% versus 35 +/- 14%, p less than 0.02), but it did not distinguish risk of first shocks.(ABSTRACT TRUNCATED AT 250 WORDS)

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