Myocarditis and endomyocardial biopsy in unexplained heart failure: a diagnosis in search of a disease.
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Biomedical subjects
Publications and source records attributed to J T Lie.
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Persons older than 85 years of age will constitute the fastest growing segment of the US population in the next decade, and despite the recent decline in mortality from heart disease, cardiovascular disease remains the single greatest cause of death in these elderly persons. We studied the pathologic changes in the hearts of 237 patients (93 men and 144 women) who had lived to the age of 90 years or older. The degree and extent of coronary atherosclerosis in these senescent hearts were similar to findings reported in younger patients who died of coronary heart disease, but calcification of the coronary arteries, mitral annulus, and aortic valve was more prevalent, as was cardiac amyloidosis. Multiple cardiac disorders were common in these very elderly hearts, but they seemed to play a lesser role in precipitating heart failure or shortening the life-span of the patients. Important factors in attaining longevity seem to be protection from the development of severe coronary artery disease by an unexplained mechanism and an innate resistance to cardiac dysfunction from a multitude of structural changes that occur with advancing age.
Thromboangiitis obliterans (Buerger's disease) is a nonatherosclerotic, inflammatory, occlusive vascular disease occurring almost exclusively in young male smokers. It involves principally medium sized and small arteries and veins of the lower and upper extremities, and only rarely the visceral and cerebral blood vessels. Buerger's disease of the temporal arteries, unassociated with the involvement of blood vessels of either the upper or lower extremities has not been previously reported. Three such cases, clinically mimicking the classic (giant cell) temporal arteritis of the elderly, are described. This unusual arterial lesion also bears some resemblance to subcutaneous angiolymphoid hyperplasia with eosinophilia (Kimura's disease).
Klippel-Trenaunay syndrome is a congenital angiodysplasia consisting of the triad of angiomas, osteohypertrophy and venous varicosities. Visceral involvement is not uncommon and may cause life-threatening complications. Presented here is a detailed study of the pathology of angiodysplasia in Klippel-Trenaunay syndrome. Venous fibromuscular dysplasia is the most prominent and consistent vascular lesion other than the cavernous hemangioma which is not unique to this syndrome. Dysplastic veins may also have deformed, insufficient, or absent valves. No agenesis or atresia of the deep veins is found in this series of patients.
Defiency of protein C has been reported to be associated with recurrent thrombosis and pulmonary embolism, disseminated intravascular coagulation, and coumarin-induced skin necrosis and peripheral gangrene. That all of these serious and eventually lethal complications of protein C deficiency, including embolic myocardial infarction, may occur in the same person is the subject of this case report and description of pathological findings.
Behçet syndrome is a systemic disease rather than the initial more narrowly characterized triple symptom-complex of recurrent oral and genital ulcers and chronic iridocyclitis. Major cardiac and pulmonary manifestations of the disease are relatively uncommon and their pathologic features have seldom been documented. Described herein is a fatal case of Behçet syndrome with co-existing myocarditis, pulmonary thromboangiitis, and pulmonary artery aneurysms.
An arterial aneurysm of any kind is rare in infancy, and one that occurs in the brachial artery is probably even more rare; when such an aneurysm is asymptomatic and congenital, it must be exceedingly rare. One such unique case is described herein, together with a brief review of the literature.
A polyarteritis nodosalike systemic vasculitis in hairy-cell leukemia was first reported in 1979, and since then at least 30 additional cases of this unusual association have been reported in the literature. Reported herein is, to my knowledge, the first known example of hairy-cell leukemia with necrotizing vasculitis limited to the testis only.
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Inflammatory aneurysm of the aorta is not merely a variant of atherosclerosis, but more probably it is a manifestation of a peculiar systemic disease related to idiopathic retroperitoneal and mediastinal fibrosis. Rarely, aortitis and coronary arteritis may coexist with the inflammatory aneurysm; one of each of such unusual cases are described. Because inflammatory aneurysms of the aorta tend to occur in elderly persons, aortitis and coronary arteritis, when present, cannot be distinguished from the concomitant age-related atherosclerosis without a careful histologic examination.
For the clinician, the recognition and treatment of different types of the vasculitic syndromes are hampered by the absence of a satisfactory classification of vasculitis and the lack of diagnostic laboratory tests. Different approaches to formulating a classification of vasculitis include the practical, pathological and pathogenetic; and at different times all these approaches have been adopted, abandoned and revived. A combined clinicopathological classification of vasculitis with emphasis on the predominant size of vessels affected (Table 1) is recommended. Several immunodiagnostic tests of vasculitis have been described recently. These novel laboratory tools may shed new light on the pathogenesis of certain types of vasculitis, but as diagnostic tests they remain an unfulfilled expectation. The search continues.
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The association of the Ehlers-Danlos syndrome with many cardiovascular abnormalities is well known. To our knowledge, however, renovascular hypertension due to renal arterial aneurysms has not previously been reported in patients with this disease. We describe a patient with type IV Ehlers-Danlos syndrome who had multiple systemic and bilateral renal arterial aneurysms and hypertension. The hypertension could have been related to compression of renal tissue or arteries (or both) by the large aneurysms, associated stenoses, or small peripheral renal infarctions. A review of all patients in whom the Ehlers-Danlos syndrome had been diagnosed at our institution between 1967 and 1985 revealed that 4 of the 200 patients had hypertension. Three of these patients, and probably all four, had secondary causes of hypertension: Cushing's syndrome, renal insufficiency, or renovascular hypertension (in the present case). We conclude that hypertension is rare in patients with the Ehlers-Danlos syndrome and that, if present, it most likely is secondary hypertension. A thorough search for secondary causes of hypertension should be undertaken in these patients.
Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily the renal and internal carotid arteries and less often the vertebral, iliac, subclavian, and visceral arteries. Although its pathogenesis is not completely understood, humoral, mechanical, and genetic factors as well as mural ischemia may play a role. The natural history is relatively benign, with progression occurring in only a minority of the patients. Typical clinical manifestations are renovascular hypertension, stroke, subarachnoid hemorrhage, abdominal angina, or claudication of the legs or arms. In patients with symptoms, percutaneous transluminal angioplasty has emerged as the treatment of choice in most involved vascular beds.