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[Aortopulmonary window: clinical assessment and surgical results]
Antonio Moruno Tirado1, José Santos De Soto, Josefina Grueso Montero
1Secciones de Cardiología y Hemodinámica, Hospital Infantil Virgen del Rocío, Sevilla. aamoruno@supercable.es
Insights
Early surgical repair of aortopulmonary septal defect (APSD) is crucial for preventing irreversible pulmonary hypertension. Prompt intervention in infants with this rare congenital heart defect leads to favorable long-term outcomes.
Area of Science:
- Congenital Cardiac Surgery
- Pediatric Cardiology
- Vascular Biology
Context:
- Aortopulmonary septal defect (APSD) is a rare congenital heart anomaly, with approximately 300 cases reported.
- Associated complex cardiac anomalies are common, impacting surgical complexity and outcomes.
Purpose:
- To present surgical experience with aortopulmonary septal defect (APSD).
- To emphasize the importance of early correction to prevent irreversible pulmonary hypertension.
- To highlight the role of echocardiography and cardiac catheterization in diagnosis.
Summary:
- Seven patients with APSD underwent surgical repair between 1979 and 2000.
- Four patients had associated complex cardiac anomalies, including interrupted aortic arch and transposition of great arteries.
- No intraoperative deaths occurred; one patient died postoperatively due to severe pulmonary hypertension. Mean follow-up for survivors was 69 months, with all remaining asymptomatic.
Impact:
- Early surgical repair of APSD before 6 months of age can prevent irreversible pulmonary vascular damage.
- Timely diagnosis and intervention are essential for managing patients with complex congenital heart disease presenting with early cardiac failure and pulmonary hypertension.
Introduction And Objectives:
Aortopulmonary septal defect is an uncommon congenital cardiac anomaly. To date, approximately 300 cases have been reported. We present our experience, emphasizing the importance of early correction to avoid irreversible pulmonary hypertension.
Patients And Method:
Between 1979 and 2000, seven patients underwent surgical repair of this heart defect in our hospital. Two had type I (proximal), 4 had type II (distal) and 1 had type III (complete). Complex associated cardiac anomalies were present in 4 cases: type A interruption of the aortic arch in 2 cases, hypoplastic aortic arch in 1 and transposition of great arteries with ventricular septal defect in 1. Four cases (57%) were diagnosed by echocardiography. In all patients diagnoses were confirmed by cardiac catheterization. Patient records were reviewed retrospectively, with special attention to clinical, echocardiographic and hemodynamic data as well as surgical characteristics.
Results:
No intraoperative deaths occurred. The patient with associated transposition of great arteries died 22 days after surgery as a result of severe pulmonary hypertension. The remaining patients are asymptomatic without treatment after a mean follow-up period of 69 months.
Conclusions:
Even though aortopulmonary septal defect is a rare anomaly, it should be considered whenever the course of complex congenital heart disease includes early cardiac failure and pulmonary hypertension. Repair before 6 months will prevent irreversible damage of pulmonary vessels.