Systemic lupus erythematosus with coronary vasculitis and massive myocardial infarction. A case report.

A 32-year-old white woman presented with angina pectoris and an acute myocardial infarction (MI) complicated by congestive cardiac failure. Other symptoms and results of immunological investigation were highly suggestive of systemic lupus erythematosus (SLE). Thallium-201 scintigraphy confirmed an extensive MI, as initially suspected from an ECG. Cardiac catheterization delineated a poorly contracting left ventricle secondary to MI. Selective coronary angiography showed features suspicious of coronary arteritis involving the left anterior descending and left circumflex coronary arteries. Right ventricular endomyocardial biopsy failed to show any 'small-vessel disease', vasculitis or myocarditis. We suggest that the acute MI was caused by coronary arteritis due to SLE. Overview of the literature indicates that coronary arteritis is not as rare a complication of SLE as previously believed; however, acute MI is most unusual.

Hypertrophic obstructive cardiomyopathy with pseudo-myocardial infarction pattern. A case report.

A 60-year-old woman with mild hypertension and presumed ischaemic heart disease was followed up over a very long period on account of angina pectoris. Acute myocardial infarction (MI) was suspected on the basis of the history, ECG findings and serum enzyme values, but disproved by radioisotope investigation. Echocardiography demonstrated features of hypertrophic obstructive cardiomyopathy (HOCM), a diagnosis supported by cardiac catheterization and endomyocardial biopsy (EMB). Histological features of HOCM were absent from left ventricular EMB specimens despite a significant intraventricular gradient, but the right ventricular EMB demonstrated extensive changes of HOCM despite a small intraventricular gradient. Cardiac catheterization excluded previous MI and coronary artery disease. A further interesting feature was the development of congestive cardiac failure, which necessitated modification of her drug therapy).

Cardiac involvement in mixed connective tissue disease. A fatal case of scleroderma combined with systemic lupus erythematosus.

A 27-year-old black woman with cardiac failure, angina pectoris and Raynaud's syndrome is presented. Skin biopsy and barium studies established the diagnosis of scleroderma (progressive systemic sclerosis (PSS)). Systemic lupus erythematosus (SLE) was strongly suggested by the results of immunological studies and increasing severity of renal failure. Because of the possibility of a cardiomyopathy, cardiac catheterization, selective coronary angiography and right ventricular endomyocardial biopsy were carried out but failed to show any histological features of either SLE or PSS. The patient went into progressive renal failure despite immunosuppressive therapy and plasmapheresis and died; consent for autopsy was refused. A final diagnosis of mixed connective tissue disease (MCTD) was made. The salient features of cardiac involvement in SLE, PSS and MCTD are outlined.

Hypertrophic cardiomyopathy complicated by complete heart block. Case report and review of the literature.

A 48-year-old man with symptoms of presyncope and congestive cardiac failure had hypertrophic cardiomyopathy (HCM) without obstruction. Complete heart block (CHB), a rare complication of this disease, was preceded by complete left bundle-branch block. Right ventricular (RV) heart failure was a dominant clinical feature but improved dramatically after temporary transvenous RV pacing prompting the insertion of a permanent RV inhibited pacemaker. Repeated ventricular fibrillation was successfully controlled by amiodarone. This is the seventh case of HCM complicated by CHB reported in the literature, and the first in which RV endomyocardial biopsies were undertaken. Two other patients reported in the literature had RV inhibited permanent pacemakers implanted, and a further 2 had atrioventricular sequential pacemakers.

Familial dilated (congestive) cardiomyopathy. Occurrence in two brothers and an overview of the literature.

Two young White brothers had dilated (congestive) cardiomyopathy. The elder came to autopsy after a chronic course of congestive cardiac failure, the younger underwent repeated cardiac catheterization and transvenous right ventricular endomyocardial biopsy specimens demonstrated histopathological features in keeping with a diagnosis of idiopathic dilated cardiomyopathy. These brothers may have the familial form of the disease, although post-viral myocarditis cardiomyopathy cannot be entirely excluded. The literature relating to familial dilated (congestive) cardiomyopathy is reviewed.

Mitral valve prolapse complicated by acute cerebral embolism, arrhythmias and painless myocardial infarction. A case presentation and overview.

A case of 'primary' mitral valve prolapse is documented. The patient was admitted with right-sided hemiplegia of sudden onset, probably caused by a cerebral embolus from the mitral valve. He also had a painless transmural inferior myocardial infarction (MI) of indeterminate age which was diagnosed electrocardiographically and on left ventricular cine angiography. Since selective coronary arteriography delineated the absence of fixed obstructive atherosclerotic disease, and since coronary vasospasm could not be provoked with the ergonovine (ergometrine) maleate test, it is further postulated that a coronary embolus from the abnormal mitral valve apparatus was responsible for the painless MI. A percutaneous right ventricular endomyocardial biopsy specimen displayed findings not indicative of a 'cardiomyopathy'.

A study of a family with inherited disease of cardiac and skeletal muscle. Part I. Clinical, electrocardiographic, echocardiographic, haemodynamic, electrophysiological and electron microscopic studies.

A family consisting of parents and their 6 sons were investigated to elucidate the relationship between a hypertrophic cardiomyopathy, musculoskeletal abnormalities and mental subnormality. The proband was diagnosed as having definite hypertrophic obstructive cardiomyopathy and the remaining family members were shown to have a spectrum of hypertropic non-obstructive cardiomyopathy. Mild muscle weakness was present in 3 sons. All the subjects except for 1 son showed definite signs of electromyographic abnormality, whereas sensory and motor conduction velocities were normal. All the EEGs except for that of the proband were normal. Testicular hypoplasia was present in 3 sons. The inheritance pattern appears to be polygenic autosomal recessive in type. Definite evidence of linkage between hypertrophic cardiomyopathy and HLA awaits further data.

A study of a family with inherited disease of cardiac and skeletal muscle. Part III. Genealogical considerations and associations with low intelligence.

A family with inherited cardiac and skeletal muscle disease was also found to have members with low intelligence. The effects of social and environmental conditions upon the behaviour of family members are described, with particular attention to the sociomedical problems created by the combination of a hereditary disease and low intelligence.

A study of a family with inherited disease of cardiac and skeletal muscle. Part II. Skeletal muscle morphology and mitochondrial oxidative phosphorylation.

Skeletal muscle morphology and mitochondrial oxidative phosphorylation capacity were examined in a family whose members showed very combinations of mental subnormality, cardiomyopathy and muscle weakness. Light and electron microscopic findings suggested a neuropathic process, while tests of mitochondrial function indicated a state of tight coupling of oxidative phosphorylation, a feature in marked contrast to those in biochemical studies so far reported.

Pseudoxanthoma elasticum with cardiac involvement. A case report and review of the literature.

A young Black man with many features of pseudoxanthoma elasticum (PXE), confirmed by skin biopsy, complained of classic angina pectoris, decreasing effort tolerance, and palpitations. Clinically he was in severe congestive cardiac failure which was confirmed by echocardiography and cardiac catheterization, investigations which indicated the presence of a 'congestive' cardiomyopathy. Selective coronary arteriography showed normal epicardial vessels. Antemortem endomyocardial biopsy in this condition is described for the first time in the literature. This showed abnormal light microscopic and electron microscopic features. It is postulated that the predominant cause of congestive cardiac failure and angina pectoris in this disease is a diffuse arteriopathy secondary to elastic fibre dysgenesis, involving the small intramural coronary vessels ('small-vessel disease'). Hitherto it has been accepted that the endocardial changes have been most important in the pathophysiology. A review of the literature as it applies to cardiac involvement in PXE is undertaken.