Trisomy 13 Life Expectancy
The median survival for infants with Trisomy 13 (Patau syndrome) is approximately 4-12.5 days, with 10-year survival rates of approximately 10-13% in population-based studies. 1, 2, 3
Survival Data by Time Period
Early Mortality
- Median survival ranges from 4 to 12.5 days across different population-based studies 1, 3
- The principal mode of death is apnea, occurring in the majority of cases regardless of the presence of cranial abnormalities 3
- In-hospital mortality for those undergoing cardiac interventions is approximately 27.6% 4
One-Year Survival
- Approximately 10-20% of infants survive to one year of age 1, 2
- A Canadian population-based study reported 1-year survival of 19.8% (95% CI, 14.2%-26.1%) 1
- A multi-state U.S. study found 1-year survival rates consistent with these findings 2
Long-Term Survival
- 10-year survival is approximately 12.9% (95% CI, 8.4%-18.5%) in children who survive the neonatal period 1
- Among carefully selected children who undergo cardiac interventions and survive to hospital discharge, median survival extends to 14.8 years (95% CI: 12.3 to 25.6 years) 4
Prognostic Factors Affecting Survival
Strongest Predictors
- Gestational age is the strongest predictor of mortality for both short and long-term outcomes 2
- Female sex is associated with improved survival compared to males 2
- Children of non-Hispanic Black mothers demonstrate lower mortality rates 2
Type of Trisomy 13
- Children with mosaic or partial trisomy 13 have significantly higher life expectancy than those with full trisomy 13 5
- Full trisomy 13 carries the highest mortality risk, with only 5-10% surviving beyond the first year 5
Surgical Intervention
- Among the 23.6% of children with trisomy 13 who undergo surgical interventions, 1-year post-surgical survival is approximately 70.7% (95% CI, 54.3%-82.2%) 1
- However, in-hospital mortality rates for all surgical risk categories remain higher than the general population 4
Clinical Implications
Counseling Considerations
- Early mortality within the first days to weeks of life remains the most common outcome 1, 3
- Survival beyond one year, while uncommon, occurs in approximately 10-20% of cases, challenging older assumptions of uniformly fatal outcomes 1, 2
- The spectrum of survival has improved somewhat compared to historical data, likely reflecting changes in medical management approaches 2
Important Caveats
- Cancer screening and aggressive surgical interventions remain controversial given the overall poor prognosis and high perioperative mortality 5
- Cardiac surgery historically has not been considered justified in trisomy 13, as cardiac lesions typically do not cause the early deaths (apnea is the primary cause) 3
- However, more recent data show that carefully selected patients who undergo cardiac interventions can achieve longer survival than previously reported 4
- The decision to pursue aggressive interventions versus palliative care should be based on individual patient factors, particularly gestational age, type of trisomy 13 (full vs. mosaic/partial), and specific organ involvement 5, 2