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Outcome of pulmonary atresia and ventricular septal defect during infancy
S Dinarevic1, A Redington, M Rigby
1Paediatric Cardiology Department, Royal Brompton National Heart & Lung Hospital, London, U.K.
Insights
Pulmonary atresia with ventricular septal defect outcomes were similar regardless of blood supply source. Systemic collateral arteries were linked to abnormal pulmonary artery branching, reducing corrective surgery rates.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Disease
Background:
- Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex congenital heart defect.
- The source of pulmonary blood supply significantly impacts surgical management and outcomes.
- Understanding these variations is crucial for effective treatment strategies.
Purpose of the Study:
- To evaluate the influence of pulmonary blood supply on outcomes in patients with PA/VSD.
- To compare survival and surgical intervention rates based on blood supply source.
- To identify factors associated with surgical outcomes in this patient population.
Main Methods:
- Retrospective analysis of 54 patients with PA/VSD diagnosed in their first year of life (1972-1992).
- Classification of patients into two groups based on pulmonary blood supply: ductal (Group I) versus systemic collateral arteries (Group II).
- Comparison of actuarial survival, rates of corrective surgery, and incidence of pulmonary artery abnormalities between groups.
Main Results:
- No significant difference in actuarial survival between Group I (ductal supply) and Group II (systemic collateral supply).
- Corrective surgery was performed more frequently in Group I (26.7%) than in Group II (16.7%).
- Abnormalities in pulmonary artery arborization were significantly more common in Group II (p < 0.03).
- Mortality rates for corrective surgery decreased significantly from the first decade (42%) to the second decade (26%).
Conclusions:
- The source of pulmonary blood supply in PA/VSD does not significantly affect long-term survival.
- Systemic collateral artery dependence is associated with pulmonary artery abnormalities, limiting surgical correction options.
- Advancements in surgical techniques and preoperative assessment have improved outcomes over time.
Abstract:
We evaluated 54 patients with pulmonary atresia and ventricular septal defect who were referred during the first year of life between 1972 and 1992. Particular emphasis was given to the nature of the pulmonary blood supply and its influence on outcome. Ductal supply of confluent pulmonary arteries was present in 30 patients (55.6%, group I), whereas 24 patients (44.4%, group II) had a pulmonary blood supply that was entirely (31.4%) or predominantly (13.0%) dependent on systemic collateral arteries. Over the 20 years there was no significant difference in actuarial survival between the two groups. Corrective surgery was performed in 8 of 30 patients in group I (26.7%)-significantly more than in group II (4 of 24, 16.7%). Arborization abnormalities of the pulmonary arteries (stenosis of unbranched and intrapulmonary arteries) were almost exclusively present in patients with systemic collateral arteries (p < 0.03), accounting for the lower probability of undergoing corrective surgery in group II patients. During the first decade of this study (1973-1983) corrective surgery was attempted in 9.6% of patients, with 42% mortality; and during the second decade (1983-1993) surgery was performed in 39.1% of patients, with 26% mortality, a significantly lower figure. Improving surgical results, complete preoperative demarcation of the pulmonary blood supply, and a more aggressive approach with early unifocalization of the pulmonary blood supply may invalidate comparison with retrospective data on the advisability of attempting to correct this anomaly. The present paper provides data against which treatment of infants with pulmonary atresia and ventricular septal defect presenting during the next decade can be compared.