J Korean Pediatr Cardiol Soc.  2006 Jun;10(2):171-182.

Ventricular Septal Defect and Pulmonary Atresia with Major Aorto-Pulmonary Collateral Artery: Diagnosis, Management and Review

• Choi JY
• Yu JJ
• Zhu L
• Bae EJ
• Noh CI
• Yun YS
• Kim WH
• Lee JR
• Kim YJ

Abstract

PURPOSE: Based on our previous studies on ventricular septal defect (VSD), pulmonary atresia (PA) and major aortopulmonary collateral arteries (MAPCA), a management strategy formulated. To evaluate this strategy, it applied to a selected group of patients and outcome measured.
METHODS
A management strategy was to promote growth of pulmonary arterial confluence (PAC), initially by right ventricle-pulmonary artery (RV-PA) conduit operation, later by balloon and or surgical angioplasty. Unifocalization performed only after PAC became big enough. If PAC absent, management plan discussed with surgeons after complete work-up. This strategy applied to four consecutive patients, initially diagnosed to have VSD+PA with MAPCA from January 2000 to December 2000.
RESULTS
The age at diagnosis ranged from 8 days-2.5 months. PAC present in 3 children, all of whom underwent initial RV-PA conduit operation at the age range of 15 days-5 months. The remaining one patient not have PAC, underwent bilateral unifocalization at the age of 4 months. All had multiple operations (2.5 operations in average) and multiple interventions (4.5 interventional catheterizations in average) before reached to corrective or semi-corrective operation. All of them currently alive and have had either corrective operation (2 patients) or semi-corrective operation (fenestration in VSD patch in 2 patients). All but one asymptomatic and on no medication.
CONCLUSION
Our management strategy for VSD+PA with MAPCA seems to work well. The cases with increased pulmonary blood flow needs meticulous plan to prevent both pulmonary vascular obstructive disease and naturally occurring pulmonary arterial stenosis and obstruction. However further study with more patients necessary.

Keyword

Pulmonary atresia; Ventricular septal defect; Major aortopulmonary collateral artery

MeSH Terms

Angioplasty
Arteries*
Catheterization
Catheters
Child
Constriction, Pathologic
Diagnosis*
Heart Septal Defects, Ventricular*
Humans
Pulmonary Atresia*