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Analysis of survival in patients with pulmonic valve atresia and ventricular septal defect
M Hofbeck1, J T Sunnegårdh, P E Burrows
1Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Pulmonic valve atresia with ventricular septal defect (VSD) patients have a 69% 10-year survival rate. Systemic collateral arteries impact pulmonary artery development, reducing surgical repair chances.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Thoracic Surgery
Background:
- Pulmonic valve atresia with ventricular septal defect (VSD) is a complex congenital heart defect.
- Pulmonary blood supply significantly influences clinical outcomes in these patients.
- Understanding the pulmonary artery anatomy is crucial for surgical planning.
Purpose of the Study:
- To review the clinical course of patients with pulmonic valve atresia and VSD.
- To investigate the impact of pulmonary blood supply source on patient outcomes.
- To identify factors influencing the feasibility of definitive surgical repair.
Main Methods:
- Retrospective review of 104 patients diagnosed with pulmonic valve atresia and VSD in the first year of life.
- Classification of patients based on pulmonary blood supply: ductus arteriosus (Group I) vs. systemic collateral arteries (Group II).
- Analysis of survival rates, surgical repair rates, and pulmonary artery abnormalities.
Main Results:
- Overall 10-year survival probability was 69%, with no significant difference between groups.
- Definitive surgical repair was performed in 46% of Group I patients versus 16% of Group II patients.
- Abnormalities in pulmonary artery arborization and intrapulmonary stenoses were significantly more common in Group II (p < 0.00001).
Conclusions:
- The source of pulmonary blood supply impacts the likelihood of successful surgical repair in patients with pulmonic valve atresia and VSD.
- Systemic collateral arteries are associated with pulmonary artery anomalies that hinder corrective surgery.
- Further research into managing pulmonary artery issues in these patients is warranted.
Abstract:
This study reviews the clinical course of 104 consecutive patients with pulmonic valve atresia and ventricular septal (VSD) defect who were diagnosed in the first year of life and followed for a mean period of 4.95 years (range 2 days to 13.75 years). Specific attention was paid to the nature of the pulmonary blood supply and to its influence on patient outcome. Confluent pulmonary arteries supplied by a single ductus arteriosus were present in 72 patients (69%, group I), whereas 32 patients (31%, group II) had a pulmonary blood supply that was partially or exclusively dependent on systemic collateral arteries. An estimate of the probability of survival for 10 years was 69% in the entire cohort, with no different between patients in group I and group II. Definitive surgical repair was performed in 33 of 72 group I patients (46%), compared with 5 of 32 group II patients (16%). Arborization and distribution abnormalities of the pulmonary arteries as well as intrapulmonary stenoses that were exclusively present in patients with systemic collateral arteries (p less than 0.00001) accounted for the significantly lower probability of undergoing corrective surgery in group II patients.