Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Abstract

Objectives: Identification of variables influencing surgical outcome in patients treated for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Methods: A total of 90 consecutive patients (median age, 12 months; range, 20 days to 35 years), who had primarily undergone either 1-stage unifocalization (n ¼ 69) or palliation to promote native pulmonary arterial development (n ¼ 21), were studied. Chromosome 22q11 deletion had occurred in 37% of the cases. Ventricular septal defect closure was accomplished in 70 patients (78%), with a mean postoperative right/left ventricular pressure ratio of 0.48 AE 0.14. Results: The rate of 14-year survival, freedom from conduit reintervention, and freedom from percutaneous intervention on the pulmonary arteries was 75%, 46%, and 52%, respectively. At a median interval of 95 months (range, 1.5-164 months), the right/left ventricular pressure ratio did not differ significantly from early postoperatively. Univariate analysis showed that an absence of confluent intrapericardial pulmonary arteries favorably affected the postoperative right/left ventricular pressure ratio after ventricular septal defect closure (P ¼ .04). Kaplan-Meier estimates showed age of 30 days or younger (P ¼ .0004) and weight of 3 kg or less (P ¼ .0004) at unifocalization and chromosome 22q11 deletion (P ¼ .001) significantly affected survival. Chromosome 22q11 deletion was significantly associated with mortality, even in the Cox regression model (hazard ratio, 8.26; P ¼ .003). Finally, ventricular septal defect closure during single-stage and single/multiple-stage procedures significantly correlated with both early (P ¼ .0013 and P < .00001, respectively) and overall (P ¼ .013 and P ¼ .0007, respectively) survival. Conclusions: The results of surgery were satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions. The outcomes were negatively affected by neonatal age and low body weight and positively affected by simultaneous or staged ventricular septal defect closure. Finally, chromosome 22q11 deletion remained an independent variable affecting survival.

Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries

“…One aspect of this diversity is the variable anatomy of the central branch pulmonary arteries [2][3][4][5]. Approximately 80% of patients with PA/VSD/MAPCAs have central branch pulmonary arteries, whereas 20% of patients have a complete absence of intrapericardial pulmonary arteries [6][7][8][9][10][11]. The developmental factors resulting in the presence or absence of central pulmonary arteries in PA/VSD/MAPCAs are currently not well defined.…”

“…Several groups continue to advocate the use of either a central shunt or right ventricle to pulmonary artery conduit as the principal method to achieve "pulmonary artery rehabilitation" [12][13][14][15][16]. The central pulmonary arteries can also be utilized in conjunction with unifocalization of MAPCAs for repair of PA/VSD/MAPCAs [6][7][8][9][10][11][17][18][19]. This strategy provides a versatile approach to the management of PA/VSD/MAPCAs and has yielded excellent hemodynamic results [20].…”

Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience

“…The major reasons for the excellent detection of pulmonary arterial morphology with CT in this study might be (1) sufficient distribution of contrast medium in the patent part of pulmonary arteries distal to the atretic segment from several cardiac cycles in CT, (2) volumetric data acquisition in CT that prevent an overlapping shadow in projection [20,24,26], and (3) atretic stump of the pulmonary trunk is sometimes enhanced only by retrograde flow or inadequately filled in ductal angiography or descending aortogram, which has only a limited contrast medium-enhanced vascular shadow [7,27]. The adequate demonstration of pulmonary arterial aborization should be beneficial to repair TOF-PA [3,17,28,29]. For example, Carotti et al [3] demonstrated that the absence of confluent intrapericardial pulmonary arteries affected the postoperative right/left ventricular pressure ratio after ventricular septal defect closure, and the right/ left ventricular pressure ratio has been shown the most powerful factor related to survival in patients with TOF-PA [19].…”

“…The adequate demonstration of pulmonary arterial aborization should be beneficial to repair TOF-PA [3,17,28,29]. For example, Carotti et al [3] demonstrated that the absence of confluent intrapericardial pulmonary arteries affected the postoperative right/left ventricular pressure ratio after ventricular septal defect closure, and the right/ left ventricular pressure ratio has been shown the most powerful factor related to survival in patients with TOF-PA [19]. Selective angiograms with the injection of contrast medium in the ductus arteriosus are usually suggested to identify the confluence of pulmonary arteries [29].…”

“…This progress has been related to the better characterization of native pulmonary arteries together with improvements in the surgical care of small infants. Complete delineation of the anatomy, size, and morphology of the pulmonary circulation is essential and necessary to plan the appropriate surgical management [3,28,29]. Ishibashi et al [17] showed that survival in TOF-PA patients with central pulmonary arteries was significantly higher than the survival of those without.…”