Sudden arrhythmia death syndrome: importance of the long QT syndrome.

In approximately 5 percent of sudden cardiac deaths, no demonstrable anatomic abnormality is found. Some cases are caused by sudden arrhythmia death syndrome. A prolonged QT interval is a common thread among the various entities associated with sudden arrhythmia death syndrome. A number of drugs are known to cause QT prolongation (e.g., terfenadine), as are hypokalemia, hypomagnesemia, myocarditis, and endocrine and nutritional disorders. Recently, attention has focused on a group of inherited gene mutations in cardiac ion channels that cause long QT syndrome and carry an increased risk for sudden death. Some of the highest rates of inherited long QT syndrome occur in Southeast Asian and Pacific Rim countries. The median age of persons who die of long QT syndrome is 32 years; men are predominately affected. In addition to a prolonged QT interval, which occurs in some but not all persons with long QT syndrome, another characteristic electrocardiographic abnormality is the so-called Brugada sign (an upward deflection of the terminal portion of the QRS complex). Most cardiac events are precipitated by vigorous exercise or emotional stress, but they also can occur during sleep. Torsades de pointes and ventricular fibrillation are the usual fatal arrhythmias. Long QT syndrome should be suspected in patients with recurrent syncope during exertion and those with family histories of sudden, unexpected death. Unfortunately, not all persons with long QT syndrome have premonitory symptoms or identifiable electrocardiographic abnormalities, and they may first present with sudden death. Beta blockers, potassium supplements, and implantable defibrillators have been used for treatment of long QT syndrome. Identifying the specific gene mutation in a given patient with long QT syndrome can help guide prophylactic therapy.

ECHOCARDIOGRAPHIC ABNORMALITIES OF THE MITRAL VALVE ASSOCIATED WITH A LARGE PERICARDIAL EFFUSION.

Features of mitral stenosis and systolic anterior motion of the mitral valve were observed on the echocardiogram of a patient with a large posterior pericardial effusion. The abnormal findings disappeared with resolution of the fluid. Because pericardial effusion may result in the echocardiographic alteration of cardiac structures, repeat study is necessary after the effusion has cleared.

EXERCISE TESTING AND LEFT MAIN CORONARY ARTERY DISEASE: EXPERIENCE WITH 57 PATIENTS.

Results of multistage treadmill tests (TMT) of 57 patients with critical stenosis (>/= 50%) of the left main coronary artery were analyzed. Additional disease was present in the major vessel in three patients (5%), two vessels in 18 patients (32%), and three vessels in 35 patients (61%). The TMT was negative for ischemia in only two patients (4%), positive in 51 (89%), and undetermined in 4 (7%). TMT was strongly positive (>/= 2 mm ST segment depression) in 40 patients (70%), and in 11 (19%) of these, ST depression was >/= 3 mm. Hypotension with exercise was rare and was encountered in only one patient. Arrhythmias were induced with exercise in six patients (10%) and resulted in premature termination of TMT in four. TMT was terminated due to early ST segment depression in 40 patients (70%), in 17 (30%) without chest pain-an unusual finding. Exercise was limited to stage I (Bruce protocol) in 16 (28%), stage II in 26 (46%), stage III in ten (17%), and stage IV in five (9%). Mean exercise tolerance was 298 +/- 22 seconds (SEM). Maximum heart rate (HR) achieved was 76 +/- 2% of their maximal predicted values. Peak double product (systolic BP x HR) was 20490 +/- 830. The data suggest that the TMT is rarely negative in the presence of LM lesions. An early strongly positive response with or without pain should lead one to suspect LM disease. Exercise-induced hypotension is rare. Limited exercise tolerance and/or early ST segment depression in stages I and II of TMT seem to be predictive of the severity of LM lesions.

SILENT MYOCARDIAL INFARCTION WITH POSTERIOR INFERIOR PSEUDOANEURYSM OF THE LEFT VENTRICLE PRESENTING AS A POSTERIOR LUNG MASS.

An asymptomatic 62-year-old man developed a density in his left lower hemithorax in the retrocardiac position on chest roentgenogram, which was discovered during a routine evaluation. This was diagnosed in another hospital as a lung tumor, for which he underwent an exploratory left thoracotomy. This case points out the difficulty in the diagnosis of retrocardiac masses and suggests that echocardiography and cardiac angiography should be accomplished before attempting needle aspiration or an open lung biopsy. The absence of a history of myocardial infarction and classical clinical indications of ventricular aneurysms such as electrocardiographic changes, arrhythmias, and congestive heart failure does not exclude a true aneurysm or pseudoaneurysm of the left ventricle.