Mitral valve prolapse.

Mitral valve prolapse (MVP) now is a commonly recognized syndrome with an apparent prevalence of approximately 4-6%. It appears to occur more frequently in females and occasionally it is familial. In most instances, the syndrome is idiopathic, although it occurs in association with many other conditions, particularly Marfan's syndrome, rheumatic heart disease, coronary heart disease, congestive cardiomyopathy, ostium secundum atrial septal defect, Ehlers-Danlos syndrome or abnormalities of the thoracic cage. The majority of patients with the syndrome have minimal, if any, symptoms and have a benign course. When symptoms do occur, more frequently they are palpitations, chest pain, dyspnea on exertion or fatigue. Neuropsychiatric symptoms or even transient ischemic episodes may occur rarely. Very rarely, complications such as severe mitral regurgitation, arrhythmias or infective endocarditis may occur. Characteristically, patients have a midsystolic click, occasionally followed by a systolic murmur. The timing of the click and the onset of the murmur usually is variable, depending on the ventricular volume. The electrocardiogram frequently shows ST-T wave changes. The diagnosis usually can be confirmed by echocardiography or left ventricular angiography. Most patients with MVP require no treatment other than reassurance. If a systolic murmur is present, prophylaxis against infective endocarditis during dental work probably is useful. Patients with palpitations or chest pain usually respond well to treatment with propranolol. Patients with progressive severe mitral regurgitation require mitral valve replacement.

Myocarditis presenting as acute myocardial infarction.

Ten patients with acute myocarditis, who were initially seen with clinical signs of acute myocardial infarction, will be discussed. All had symptoms and seven had laboratory evidence of an acute viral infection. Acute cardiac findings consisted of chest pain in nine patients, compatible ECGs and elevated creatine kinase levels in 10, positive MB fractions in eight, and regional wall motion abnormalities in eight. Acutely, the left ventricular ejection fraction was less than 55% in six patients; ventricular ectopy occurred in five patients, bundle branch block in four, transient junctional escape rhythm in three, and congestive heart failure in three. Among the nine patients followed-up for 1 to 14 months there was one death, five patients had normal results of exercise tests, and three had normal coronary angiograms. Wall motion abnormalities persisted in four patients; ejection fraction improved in five and was less than 55% in three. These findings suggest that focal myocardial damage may occur during acute viral myocarditis and mimic acute myocardial infarction resulting from atherosclerotic coronary artery disease.

Mitral valve prolapse and ophthalmoplegia: a progressive, cardioneurologic syndrome.

Disorders characterized by both neurologic (ataxia, ophthalmoplegia, ptosis, neuromyopathy) and cardiologic (heart block, cardiomyopathy) abnormalities have been previously called the "ophthalmoplegia plus" syndromes. Most are not due to a specific enzyme defect or metabolic abnormality and thus may be similar phenotypic expressions of diverse causes. We studied seven patients with progressive external ophthalmoplegia and variable ataxia, with mitral valve prolapse and mitral regurgitation that progressed in severity as did the neuromuscular manifestations. Abnormal skeletal muscle biopsies showed "ragged-red" fibers or congenital fiber type disproportion; serum alanine levels were elevated; in-vivo and in-vitro tests of pyruvate metabolism gave abnormal results; C4 complement was decreased; and the patients' fibroblasts bound immunoglobulin when incubated with autologous serum. These data suggest a distinct neuromuscular disorder with metabolic and immunologic features associated with mitral valve prolapse and progressive mitral regurgitation.

Serial cardiac function tests in myocarditis.

Sixteen patients (mean age 27 years, range 16 to 39 years) with the diagnosis of myopericarditis established by strict clinical criteria were evaluated following recovery 0.7 to 4.0 years (mean 2.7 years) later. Evidence of an acute viral infection was present in 44%. During the acute illness, the major clinical manifestations consisted of pericarditis in 10 patients, acute myocardial infarction in 5, right ventricular dysfunction in 5, bundle branch or hemiblock in 4, ventricular arrhythmias in 3, congestive heart failure in 3 and cardiogenic shock and inappropriate sinus tachycardia in one patient each. Holter monitoring, echophonocardiography and radionuclide ventriculography results were abnormal in 57, 67 and 64% of patients respectively. At follow-up, these tests were abnormal in 67, 7 and 73%. Focal wall motion abnormalities were present in five. Seventy-five percent of patients had one or more abnormal tests at last follow-up. Although 8 patients had improved by a scoring system, 5 patients had an increase in the number of abnormal tests, including one who died. These findings indicate that persistent abnormalities following recovery from myopericarditis are not rare and support the hypothesis that the syndrome of dilated cardiomyopathy may be a sequel of myopericarditis.