[Individualized surgical management of pulmonary atresia with intact ventricular septum].
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.


CONCLUSIONS
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
Zhu
Zhong-Qun
ZQ
Department of Pediatric Thoracic & Cardiovascular Surgery, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China. zzqheart@yahoo.com.cn
Liu
Jin-Fen
JF
Su
Zhao-Kang
ZK
Xu
Zhi-Wei
ZW
Cao
Ding-Fang
DF
Ding
Wen-Xiang
WX
Sun
Yan-jun
YJ
D013504:Cardiovascular Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003326:Coronary Circulation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome; D020257:Ventricular Remodeling; D054088:Ventricular Septum