The spectrum of cardiovascular abnormalities in autosomal dominant polycystic kidney disease: a 10-year follow-up in a five-generation kindred.

Clinical, electrocardiographic and echocardiographic data were collected in a group of 228 patients with autosomal dominant polycystic kidney disease (PKD) and in another group of 146 unaffected members (NPKD) both comprised in a five-generation kindred followed for 10 years, in order to determine the profile and prevalence of cardiovascular derangement of the genetic disease. A family of 181 members was used as a control. The prevalence of left ventricular hypertrophy in the three groups was 24, 14 and 6% respectively (p less than 0.01); after 10 years it increased up to 35, 26 and 13% respectively (p less than 0.05). The evidence of mitral-valve prolapse was more frequent in PKD and in NPKD group (25 and 20% respectively) than in control subjects (2%) (p less than 0.0001). Mitral incompetence was found in 30, 18 and 8% of those groups respectively (p less than 0.002). The large difference in mitral involvement did not change over time. Tricuspid valve prolapse was detected in 5, 4 and 1% of the three groups, respectively (p less than 0.05). A small increase in frequency was found after 10 years only in polycystic kidney disease patients. Regurgitant aortic lesions were present in higher prevalence in PKD (19%) and NPKD (17%) members than in controls (5%) (p less than 0.001). After 10 years they were 23, 20 and 8%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)