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.
- Natural history
- Single ventricle
- Surgical outcome
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Cardiology and Cardiovascular Medicine