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[Individualized surgical management of pulmonary atresia with intact ventricular septum]
Zhong-Qun Zhu1, Jin-Fen Liu, Zhao-Kang Su
1Department of Pediatric Thoracic & Cardiovascular Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China. zzqheart@yahoo.com.cn
Insights
Individualized surgical strategies for pulmonary atresia with intact ventricular septum (PA/IVS) improve outcomes. Tailoring treatment based on right ventricle-dependent coronary circulation and hypoplasia optimizes surgical results for PA/IVS patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiovascular Physiology
Context:
- Pulmonary atresia with intact ventricular septum (PA/IVS) presents complex surgical challenges.
- Management strategies require careful consideration of right ventricular (RV) function and coronary circulation.
- Previous approaches lacked a standardized, individualized strategy for optimal outcomes.
Purpose:
- To define an individualized surgical management strategy for PA/IVS.
- To evaluate the impact of tailored surgical interventions on patient outcomes.
- To correlate specific patient factors with treatment success in PA/IVS.
Summary:
- A cohort of 52 PA/IVS patients (1997-2005) underwent individualized surgical treatment.
- Patients were stratified based on right ventricle-dependent coronary circulation (RVDCC) and RV hypoplasia severity.
- Surgical approaches included single ventricle repair, RV decompression, and shunts, with outcomes assessed over 1-10 years.
Impact:
- Individualized management improved surgical results for PA/IVS.
- Key factors for success include RVDCC status, RV hypoplasia grading, and hemodynamic response to decompression.
- This approach aids in optimizing surgical decision-making for complex PA/IVS cases.
Objective:
To delineate individualized surgical management strategy for the optimal management of pulmonary atresia with intact ventricular septum (PA/IVS).
Methods:
Between 1997 to 2005, 52 consecutive patients with PA/IVS, 31 male and 21 female, aged 2-9, divided into 2 groups according if right ventricle-dependent coronary circulation (RVDCC) existed, underwent individualized surgical treatment. The patients in Group I (with: RVDCC, n = 6) underwent single ventricle repair. Forty-six patients were in Group II (without RVDCC), 13 of which with severe right ventricular hypoplasia (tricuspid valve Z value < -4)underwent single ventricle repair and 33 of which were with mild to moderate right ventricular hypoplasia (Z value > -4). One critical neonate underwent systemic- pulmonary artery shunt. The other 32 of the 33 patients received right ventricular decompression procedures firstly, and 6 of them presented optimal hemodynamics and oxygenation and achieved biventricular repair. If the patients presented unstable hemodynamics or hypoxemia after right ventricular decompression procedure, an additional shunt was added. 13 young infants (0-2 months) underwent additional systemic-pulmonary artery shunt, and 13 patients (> 3 months-old) underwent additional bidirectional cavopulmonary shunt (1.5 ventricular repair)The patients were followed up for 1-10 years.
Results:
There were 1 early death in Group I and 7 early deaths in Group II. The total early mortality was 15%. A successful definitive repair was achieved in 26 cases (50%). Follow-up reported 3 late deaths.
Conclusion:
Individualized surgical management based on the presence of RVDCC, right ventricular hypoplasia grading, right ventricular growth potential, hemodynamic situation, and oxygen saturation after the decompression procedure is helpful to improve the surgical results of PA/IVS
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