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Surgical outcome of aortopulmonary window repair in early infancy
Chun-An Chen1, Shuenn-Nan Chiu, En-Ting Wu
1Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei 100, Taiwan.
Insights
Surgical repair of aortopulmonary window (APW) in infants leads to good long-term outcomes. However, associated interrupted aortic arch (IAA) or severe coarctation of the aorta (CoA) may increase hospital stay and reintervention risk.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Neonatal Medicine
Background:
- Aortopulmonary window (APW) is a rare congenital heart defect requiring early surgical intervention.
- Few studies specifically address surgical outcomes in infants with APW.
Purpose of the Study:
- To define clinical features and outcomes of surgical repair for APW in early infancy.
- To evaluate the impact of associated cardiovascular anomalies on infant APW repair outcomes.
Main Methods:
- Retrospective review of 14 patients with APW (1983-2004), focusing on 10 infants repaired before 4 months of age.
- Analysis of APW types, concomitant anomalies, surgical techniques, and clinical outcomes including mortality, follow-up, and reintervention.
Main Results:
- All 10 infants had concomitant cardiovascular anomalies, predominantly aortic arch anomalies (80%), including interrupted aortic arch (IAA) and severe coarctation of the aorta (CoA).
- One early postoperative death occurred; survivors had a median follow-up of 34 months with no late deaths.
- Patients with IAA or severe CoA experienced longer hospital stays and required reintervention more frequently (60%), with significant residuals noted in type III APW cases.
Conclusions:
- Surgical correction of APW in early infancy generally yields favorable long-term results.
- Associated IAA or severe CoA are risk factors for prolonged hospitalization and increased need for early reintervention.
- Type III APW with associated IAA/CoA may present with persistent hemodynamic residuals even after reintervention.
Background/Purpose:
Aortopulmonary window (APW) is a rare congenital heart disease and surgical correction is advised as early as possible. However, few studies have focused on infants. This study sought to define the clinical features and outcomes in patients who underwent surgical repair of APW in early infancy.
Methods:
Between 1983 and 2004, there were 14 patients (0.15%) with APW out of 9414 patients with congenital heart disease. Ten underwent surgical repair of APW when they were younger than 4 months of age and constituted the study population.
Results:
There were four patients with type I APW, three with type II and three with type III. Concomitant cardiovascular anomalies were present in all patients, mainly aortic arch anomalies (80%, including right aortic arch, interrupted aortic arch [IAA] and severe coarctation of the aorta [CoA]). Patients were further grouped according to the presence (n = 5) or absence (n = 5) of IAA or severe CoA. All patients underwent surgical repair of APW with various techniques, including direct ligation, division and patch or flap closure. There was one early postoperative death. None of the survivors died during a median follow-up of 34 months (range, 8-116 months). Patients with IAA or severe CoA had longer intubation time, intensive care unit stay and hospital stay. Early reintervention was required only in patients with IAA or severe CoA (n = 3, 60%). Among them, two had significant residuals and both had type III APW.
Conclusion:
Long-term outcomes are generally good in patients with APW who have undergone surgical correction in early infancy. However, associated IAA or severe CoA may predict a prolonged hospital course and an increased risk of early reintervention. In patients with type III APW requiring early reintervention, significant hemodynamic residuals may be common even after reintervention.
