Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease

D. Dunbar Ivy, Elizabeth M. Shaffer, Ann M. Johnson, William J. Kimberling, Asya Dobin, Patricia A. Gabow

Research output: Contribution to journalArticlepeer-review

92 Scopus citations


It is known that adults with autosomal dominant polycystic kidney disease (ADPKD) have an increased incidence of cardiovascular abnormalities, including mitral valve prolapse. The cardiac manifestations of ADPKD in the pediatric population have not been well established. To determine the cardiac manifestations of children with ADPKD, echocardiography was performed in 154 children of 66 families in which one parent has 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 concurrent renal ultrasound or those predicted to be gene carriers by gene linkage analysis. A 12% incidence of mitral valve prolapse was found in the affected children compared with only 3% of the unaffected children (P < 0.05). ADPKD children, but not their unaffected siblings, demonstrate a significant correlation between left ventricular mass index and systolic blood pressure. Moreover, hypertensive ADPKD children have significantly larger left ventricular mass index than do normotensive ADPKD children. A 3.5% incidence of congenital heart disease was found in the affected group, whereas 2.9% of the unaffected children had congenital 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,

Original languageEnglish (US)
Pages (from-to)2032-2036
Number of pages5
JournalJournal of the American Society of Nephrology
Issue number12
StatePublished - Jun 1995
Externally publishedYes


  • Autosomal dominant polycystic kidney disease
  • Congenital heart disease
  • Echocardiography
  • Left ventricular mass index
  • Mitral valve prolapse

ASJC Scopus subject areas

  • General Medicine


Dive into the research topics of 'Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease'. Together they form a unique fingerprint.

Cite this