Prevalence, clinical presentation and natural history of patients with single ventricle

Patrick W. O'Leary

Research output: Contribution to journalArticle

33 Scopus citations

Abstract

Single ventricle is a rare, but complex cardiac disorder. It represents 7.7% of congenital heart disease diagnosed in childhood and has a birth incidence of approximately 4-8 per 10000. Most patients will present with neonatal cyanosis, but an increasing number are being recognized by prenatal ultrasound. The true natural history for these patients is quite poor. In fact, even with palliative surgery, the 'best' historical 3-year survival rate was 75%. The most 'definitive' conventional surgical procedure available for these patients is the Fontan operation. While early reports showed improved long-term survival rates, recent modifications have produced even better results. Multiple centers have reported greater than 80% survival rates at more than 5 years after Fontan. One recent series has even achieved a 10-year survival of 87%. Evolving surgical techniques, improved selection criteria and the ability to perform the operation at younger ages with low early risk have all contributed to improved outcomes. While the functional and cognitive status of these patients may not be 'statistically' normal, most patients lead productive lives, attend school and report minimal limitations. Hopefully, although they face an ongoing risk for morbidity and mortality, the promise of these improving results will be sustained for these patients.

Original languageEnglish (US)
Pages (from-to)31-38
Number of pages8
JournalProgress in Pediatric cardiology
Volume16
Issue number1
DOIs
StatePublished - Oct 2002

Keywords

  • Incidence
  • Natural history
  • Single ventricle
  • Surgical outcome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Fingerprint Dive into the research topics of 'Prevalence, clinical presentation and natural history of patients with single ventricle'. Together they form a unique fingerprint.

  • Cite this