Strategy of surgical management for pulmonary atresia with intact ventricular septum

Abstract

Objective To evaluate the clinical treatment courses, outcomes and follow-up for the pulmonary atresia with intact ventricular septum (PAIVS) in our center, and to delineate strate- gies for the optimal management of PAIVS. Methods From January 1994 to January 2008, 31 con- secutive infants (23 males and 8 females) with PAIVS underwent surgical treatment. Mean age at op- eration was 106.88 days (ranged from 20 hours to 3 years). Mean weight was 4.71kg (ranged from 2.9kg to 12.5kg). Six cases were complicated with severe maldevelopment of right ventricle, 5 with patency of sinusoid, 2 with Ebstcin malformation, 1 with atresia of tricuspid valve(TV), 3 with ste- nosis of TV, and 3 with maldevelopment of TV. Initial surgical treatment included: five Blalock-Taus- sig shunt (BT shunt), 2 closed pulmonary valvotomy (CPV), 2 CPV and BT shunt, 6 right ventricu- lar outlet tract rcconstruction (RVOTR), 12 RVOTR and BT shunt and 4 Bidirectional cavopulmonary connection. The patients were divided into two groups based on the timing of operation: group A (1994 to 2003, 13 cases)and group B (2004 to now, 18 cases). Results Mean duration of mechanical ventilation and intensive care unit stay were 1.98 ± 1.78 days and 6.42 ± 5.98 days,and mean length of stay was 16.12 ± 9.27 days, respectively. There were totally eight patients died during hospitaliza- tion(8/31,25.81%). Mortality of group B was significantly lower than that of group A(2/18 vs 6/13, P<0.01). Multivariable analyses demonstrated that the timing of operation (r=0.40, P=0.03)and mechanical ventilation before surgical treatment(r=0. 56, P=0.001 )could be the risk factors for in- hospital mortality. Twenty-one cases were followed up at the average period of 20 months (ranged from 2 months to 7 years). Conclusions Patients with PAIVS should undergo appropriate operations in time based on individual conditions, such as the size of tricuspid valve, the shape of right ventricle and malformation of coronary artery, which can help achieve excellent short-term outcomes. Postoper- ative survival rate can be achieved by optimizing operations, reinforcing the cardiac function support in perioperative period and prolonging follow-up period. Key words: Pulmonary atresia; Cardiac surgical procedures; Heart diseases, congenital