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A group of 14 patients with mitral valve prolapse syndrome was referred for coronary angiographic study. The group was selected on the basis that all members had recurrent chest pain as their chief complaint and all had interpreted their chest pain as serious enough to warrant at least two previous emergency visits to medical facilities in the six months preceding the study. All were found to have normal left ventricular function, and only one had a significant fixed obstructive coronary lesion. Seven of 14 patients (50 percent) showed evidence of coronary artery spasm during the catheterization study, five of whom had histories highly suggestive of coronary vasospasm. During file catheterization, spasm occurred spontaneously in three patients, was ergonovine-induced in two, and was catheter-tip-induced in two. Ergonovine was administered to nine of the 14 patients. The drug induced vasospasm in two patients but failed in seven (two of whom had previously demonstrated catheter tip spasm). Of those seven patients who showed evidence of spasm, four had typical chest pain in association with reversible ST segment elevation and manifested a variant anginal syndrome.

Three patients had hypertrophic obstructive cardiomyopathy and coronary artery spasm. The clinical diagnosis of hypertrophic obstructive cardiomyopathy, in all patients, was confirmed by echocardiography and angiography. Significant spasm of the right coronary artery was demonstrated in each patient by selective coronary arteriography. One patient had atherosclerotic obstructive three vessel disease, while the other two showed no evidence of any fixed organic narrowing of the coronary arteries. ST segment elevation in the inferior ECG leads was documented in two of the patients in association with coronary spasm.

Nineteen patients with congestive heart failure were given a continuous infusion of glucagon 3 to 5 mg/hour. Those with acute processes improved, while only 1 of 12 with chronic CHF benefited. Urine output increased 495 ml/24 hours, BUN rose 4.9 mg percent, serum glucose increased 22.1 mg percent, and serum potassium decreased 0.35 mEq/liter. No induced arrhythmias were noted, even in the presence of digitalis toxicity. Evidence of increased “ischemia” (angina, depressed ST segments and inverted T waves) during the infusion were seen in four patients. In chronic congestive failure associated with coexisting manifest chronic obstructive pulmonary disease or pulmonary heart disease, the response to glucagon was uniformly unfavorable. Glucagon should be reserved for acute CHF and then only after other modes of therapy, especially digitalization, are unsuccessful.

A patient with delayed metal fragment embolism to the heart is presented. The foreign body was embedded near the right ventricular side of the septum within the myocardium, an impression supported by ultrasonic echo studies. Implantation of the foreign body was causally related to a pattern of papillary muscle fibrosis on the electrocardiogram. The complications and indications for surgical removal of intracardiac foreign bodies are reviewed.

Clinical and pathologic investigations of a family whose members suffered from a heritable syndrome of syncopal spells, sudden death, and QT interval prolongation on the electrocardiogram are reported. All members had normal hearing. The mode of inheritance appeared to be autosomal dominance. The syndrome is very similar to one first reported in 1957 by Jervell and Lange-Nielsen except in the latter disorder, nerve-deafness is present and the mode of inheritance is autosomal recessive. In the syndromes reported, attacks of syncope and sudden death are usually due to ventricular fibrillation and this is frequently triggered by physical or acute emotional stress. Of importance and as illustrated by the propositus of this study, the electrocardiogram may be normal at rest only to show the characteristic bizarre QT prolongation after exercise. Pathologic study of the heart of one of the family members who died suddenly revealed diffuse and extensive fibrosis of the conducting system along with abnormal changes in the small arterial vessels in this area. The part these lesions play in the pathogenesis of the clinical syndrome is not entirely clear. The syndrome is probably more unrecognized than rare and may explain a number of instances of “atypical seizures.” Any individual with such seizures should have an electrocardiogram, preferably after exercise, as an integral part of the diagnostic evaluation.