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Management evolution of pulmonary atresia and intact ventricular septum
1Department of Paediatrics, Grantham Hospital, University of Hong Kong, Aberdeen.
Insights
Tailoring treatment for pulmonary atresia with intact ventricular septum based on right ventricle morphology significantly improves survival. This approach reduces mortality in infants with this complex congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Morphology
Background:
- Pulmonary atresia with intact ventricular septum presents complex management challenges.
- The role of right ventricular morphology in surgical strategy has been evolving.
- Optimizing outcomes requires careful consideration of individual patient anatomy.
Purpose of the Study:
- To evaluate the impact of incorporating right ventricle morphologic classification into management strategies.
- To compare survival and clinical outcomes between different surgical approaches.
- To assess the long-term clinical course in infants with pulmonary atresia and intact ventricular septum.
Main Methods:
- Retrospective review of 62 consecutive patients (1979-1990).
- Group I (pre-1984): Primary right ventricular outflow reconstruction regardless of morphology.
- Group II (post-1984): Treatment tailored to right ventricle morphology (valvotomy or shunt).
Main Results:
- Group I: 52% total mortality (43% hospital mortality).
- Group II: 26% total mortality (15% hospital mortality), a significant reduction (p < 0.01).
- Long-term survivors in both groups experienced postoperative hemodynamic abnormalities.
Conclusions:
- Tailoring surgical treatment to right ventricular anatomy in pulmonary atresia with intact ventricular septum significantly lowers overall mortality.
- While mortality is reduced, long-term hemodynamic issues persist in survivors.
- Morphologic classification is a crucial factor in optimizing surgical management for this condition.
Abstract:
To examine the impact on survival and clinical course of incorporating the morphologic classification of the right ventricle into the evolving management strategy for babies with pulmonary atresia and intact ventricular septum, the surgical results and follow-up status of the first 62 consecutive patients managed in this hospital between 1979 and 1990 were reviewed. Before 1984, all 23 babies from group I underwent primary right ventricular outflow reconstruction irrespective of right ventricular morphology and size. Since 1984, depending on the morphology and size of the right ventricle, 39 babies from group II had either closed transventricular pulmonary valvotomy (n = 31) or a shunt operation (n = 8). There were 10 hospital (43%) and 2 late deaths (total mortality 52%) in our group I patients. Three of the 11 long-term survivors had cyanosis at rest but none had any residual pressure gradient across the pulmonary outflow. Group II had 6 hospital (15%) and 4 late deaths (total mortality = 26%). Of the 29 long-term survivors, 9 had a second-stage right ventricular outflow reconstruction, 8 had balloon valvuloplasty and 2 had successful Fontan operation. At the latest follow-up, 5 children from this group have cyanosis at rest, 1 has a residual gradient (55 mm Hg) across the infundibulum, and 3 have right ventricular dysfunction. The hospital and total mortality for babies in group II was significantly lower than that in group I (p < 0.01). These data suggest that tailoring the treatment to the right ventricular anatomy results in a lower overall mortality although long term postoperative hemodynamic abnormalities are observed in both groups.