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Survival and probability of cure without and with operation in complete atrioventricular canal
Insights
Surgical repair of complete atrioventricular (A-V) canal defects offers improved survival rates. Optimal surgical timing at 14 months maximizes long-term "surgical cure" for infants with this congenital heart condition.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
Background:
- Complete atrioventricular (A-V) canal defect is a serious congenital heart condition.
- Nonsurgical management results in poor long-term survival rates, with only 4% surviving to 5 years of age.
Purpose of the Study:
- To evaluate the feasibility and outcomes of primary surgical repair in infants with complete A-V canal defect.
- To determine the optimal age for surgical intervention to maximize survival and functional outcomes.
Main Methods:
- Actuarial analysis of postmortem data for nonsurgically treated patients.
- Review of surgical outcomes in 39 patients undergoing primary repair since 1975.
- Analysis of five-year survival rates and "surgical cure" based on age at operation.
Main Results:
- Surgical repair is feasible in small infants, with hospital death risk decreasing to 17% by 12 months of age.
- Five-year survival rate after hospital discharge was 91% for patients operated on between 1967 and 1976.
- Maximal "surgical cure" rate of 73% was achieved when repair was performed around 14 months of age.
Conclusions:
- Primary surgical repair significantly improves survival for infants with complete A-V canal defect.
- Optimal surgical timing around 14 months of age yields the best long-term functional outcomes.
- Early repair is often necessitated by the disease's natural progression, despite risks in younger infants.
Abstract:
Actuarial analysis based on postmortem examination of patients who had been treated nonsurgically for complete atrioventricular (A-V) canal defect shows that only 54% survive to 6 months of age, 35% to 12 months, 15% to 24 months, and 4% to 5 years of age. Our surgical experience since 1975 in 39 patients confirms the idea that primary repair is feasible in small infants. The highest risk of hospital death is when the operation is done in the early months of life; it falls to 17% by age 12 months. Between 1967 and October, 1976, the five-year survival rate among patients leaving the hospital alive after repair was 91%. The age-specific probability of "surgical cure" of patients operated upon for complete A-V canal (alive five years later with mean pulmonary artery pressure less than 25 mm Hg) is maximal at 73% when the operation is done at about 14 months of age. Urgent earlier repair is frequently necessitated by the life history of the disease.