What other heritable disorders of connective tissue may result in cardiovascular abnormalities?

Ehlers-Danlos syndrome Osteogenesis imperfecta Homocystinuria

Polyarteritis nodosa causes what cardiac abnormalities?

Polyarteritis nodosa (PAN) is a primary vasculitis syndrome that results in necrotizing vasculitis of medium and small-sized muscular arteries. Though by no means definite, immune complex deposition within vessel walls is likely responsible. In certain patients the antigen has been identified, and in such cases is most commonly hepatitis B surface antigen (HBsAg). The manifestations of the disease result from systemic inflammation (constitutional symptoms such as fatigue, malaise, anorexia, and weight loss that are likely manifestations of circulating cytokines) and focal tissue ischemia and infarction as a consequence of vascular compromise resulting from the vasculitic process. Focal aneurysms of vessels may occur and occasionally rupture.

Involvement of the renal vasculature commonly results in hyperreninemic hypertension,

which may lead to secondary cardiovascular disease. Primary involvement of the heart is also common in PAN, usually due to vasculitis of the medium and small-sized muscular arteries. The vessels involved are usually smaller and more distal than those involved in atherosclerotic heart disease. Occlusion of these vessels due to vasculitis may result in recurrent and usually small myocardial infarctions, leading to patchy myocardial fibrosis on pathologic examination. It may manifest clinically as angina pectoris, acute myocardial infarction, sudden death, dysrhythmias, or congestive heart failure. Fibrinous pericarditis due to the underlying inflammatory disease may occur, but is more commonly due to uremia. Finally, conduction defects may occur as a result of ischemia/infarction or inflammation extending beyond vessels into the SA and AV nodes.

The treatment for PAN includes high-dose glucocorticoids and often cytotoxic therapy, particular the administration of daily, oral cyclophosphamide.

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