Aneurysms of coronary arteries in a patient with adult polycystic kidney disease: arteriosclerosis or involvement by the primary disease?

Abstract

Adult polycystic kidney disease is frequently associated with gastrointestinal and cardiovascular abnormalities. These include hypertension, mitral valve prolapse, mild dilation of the aortic root, abdominal aneurysms, and predisposition to aortic, mitral, and tricuspidal valve regurgitation reminiscent of Marfan's syndrome. Although the exact molecular mechanisms of adult polycystic kidney disease are not well established, a generalized defect of collagen structure is hypothesized. The most severe vascular problems, however, are typical intracranial aneurysms with a high incidence of subarachnoid hemorrhage and a high mortality rate. We report a case of dilated coronary arteries found incidentally in a patient with adult polycystic kidney disease and stress-induced angina pectoris. The typical angina pectoris of the patient is explained by left ventricular hypertrophy and coronary heart disease. Multiple liver cysts, mitral valve prolapse, and the coronary aneurysms in this patient with adult polycystic kidney disease appear to reflect the manifestation of a generalized connective tissue disorder in this syndrome.

A large coronary aneurysm and its probable precursor lesions in a patient with autosomal dominant polycystic kidney disease: An implication for the process of aneurysmogenesis

“…Aneurysms are divided into saccular and fusiform types by their morphology. About half of the reported cases of coronary aneurysms associated with ADPKD are saccular, and the rest is fusiform 2–6 . The aneurysmogenic process of these two types may be different, considering that formation of saccular aneurysms require focal structural problems of the vascular walls but fusiform ones reflect more diffuse abnormality of entire circumference of the vessel walls.…”

“…In contrast, coronary aneurysms are rare complications of ADPKD although the prevalence appears to be higher in ADPKD patients than in the general population 2 . Eleven cases of coronary aneurysms associated with ADPKD have been reported previously, 2–6 but all of them were observed only by angiography, most being small (<1 cm) in diameter, and pathologic analysis was not performed. Moreover, although the pathogenesis of extracranial aneurysms in ADPKD has been vaguely ascribed to polycystin abnormality in vascular walls, little is known about the real process of aneurysmogenesis in ADPKD.…”

Coronary Artery Disease in Patients With Renal Failure

“…Churg‐Strauss syndrome 90 and systemic lupus erythematosus 91 are rare, but known. Aneurysms of the coronary arteries have been seen in classical polyarteritis nodosa, 92 Kawasaki disease of children, 93 Churg‐Strauss syndrome, 94 fibromuscular dysplasia 95 and autosomal polycystic kidney disease 96–98 …”

“…Aneurysms of the coronary arteries have been seen in classical polyarteritis nodosa,9 2 Kawasaki disease of children, 93 Churg-Strauss syndrome, 94 fibromuscular dysplasia 95 and autosomal polycystic kidney disease. [96][97][98] The incidence and pathology of small vessel/myocardial arteriolar disease in uraemic patients is not known. However, like diabetes, uraemic arterial changes occur in both large and small vessels.…”

Aneurismas das artérias coronárias: uma rara manifestação da doença renal policística autossômica dominante

“…2 Aneurismas coronários associados à doença renal policística autossômica dominante têm sido raramente reportados na literatura. [3][4][5][6] Neste artigo descrevemos o RESUMO A doença renal policística autossômica dominante (DRPAD), uma das anormalidades genéticas mais comuns, é responsável por 10% dos casos de insuficiência renal crônica terminal. O acometimento extrarrenal da DRPAD manifesta-se por cistos no fígado e pâncreas, aneurismas intra e extracranianos, e anormalidades cardíacas valvares, comprometendo a raiz da aorta e as valvas aórtica e mitral.…”