CARDIOVASCULAR-ABNORMALITIES IN CHILDREN WITH AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE

It is known that adults with autosomal dominant polycystic kidney dise ase (ADPKD) have an increased incidence of cardiovascular abnormalitie s, including mitral valve prolapse. The cardiac manifestations of ADPK D in the pediatric population have not been well established. To deter mine the cardiac manifestations of children with ADPKD, echocardiograp hy was performed in 154 children of 66 families in which one parent ha s ADPKD. Eighty-six affected children and 68 unaffected children were evaluated in a prospective, single-blinded manner by echocardiography, Affected children were defined as those with any cysts on a concurren t renal ultrasound or those predicted to be gene carriers by gene link age analysis. A 12% incidence of mitral valve prolapse was found in th e affected children compared with only 3% of the unaffected children ( P < 0.05). ADPKD children, but not their unaffected siblings, demonstr ate a significant correlation between left ventricular mass index and systolic blood pressure. Moreover, hypertensive ADPKD children have si gnificantly larger left ventricular mass index than do normotensive AD PKD children, A 3.5% incidence of congenital heart disease was found i n the affected group, whereas 2.9% of the unaffected children had cong enital heart disease. It was concluded that systemic manifestations of ADPKD, particularly cardiovascular abnormalities, are present even in childhood and these warrant the clinician's attention.