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Published on: June 9, 2023
Neonatal Pulmonary Atresia With Intact Ventricular Septum-8-Year Surgical Experience at One Center
Li Guanhua1, Cen Jianzheng2, Xu Gang2
1Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China; Department of Cardiovascular Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, Guangdong, China.
Background:
Surgical treatment of pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is challenging because of the broad variations of right ventricular (RV) malformations. In this retrospective study, we summarized our 8-y experience in surgical management for neonatal PA/IVS patients.
Methods:
Thirty-four neonates with PA/IVS between July 1, 2006 and June 30, 2014, were reviewed. Patients were categorized into three groups: mild, moderate, and severe RV hypoplasia according to RV morphology and development. Patients were on regular follow-up for at least 5 y. Overall survival, complications, reinterventions, risk factors for mortality, and health status were evaluated.
Results:
21 patients (61.8%) were treated with biventricular repair, eight patients (23.5%) with Fontan procedure, and one patient (2.9%) with bidirectional Glenn procedure. There were four postprocedural mortalities and one late death. The 5-y survival rates after final surgical repair for mild, moderate, and severe RV hypoplasia groups were 100%, 100%, and 88.9%, respectively. The reintervention rates were 0% (0/4), 21.4% (3/14), and 55.6% (5/9) for the subgroups, respectively. At the latest follow-up, most patients had a status characterized as New York Heart Association class I (88.9%, 24/27).
Conclusions:
Surgical management for PA/IVS in neonates should be individualized. Favorable early and long-term outcomes can be achieved in neonatal PA/IVS patients by individualized surgical strategies, regardless of the degree of RV hypoplasia. In spite of potential RV catch-up development, the degree of RV hypoplasia is a factor of paramount importance to assess PA/IVS in neonates.
