Chinese expert consensus on surgical treatment of congenital heart disease (3): pulmonary atresia with ventricular septal defect / 中国胸心血管外科临床杂志

ABSTRACT

@#Pulmonary atresia with ventricular septal defect (PA/VSD) is a complicated congenital heart defect. The consensus of Chinese experts is developed based on the evidence-based data and expert opinions provided by the literature. Tchervenkov classification (A, B and C, three types) is adopted. Echocardiography is preferred to assess the abnormalities of the heart, physiological function and pulmonary artery development, but it is not absolutely accurate for the evaluation of collateral vessels. Furthermore, multi-row CTA can confirm the development of the native pulmonary artery, the number, origin and morphological characteristics of collateral vessels. Additionally, angiography provides more details of the number, origin and distribution of collateral vessels. Genetic testing is important to understand the genetic etiology and to determine the prognosis. After definite diagnosis, treatment plan is made according to the classification and clinical manifestations. Pulmonary artery flow is arterial duct dependent for Type A and surgical treatment is usually performed within 6 months. But patients with severe hypoxia were treated during neonatal period. The strategy for type B/C is more complex. Severe hypoxemia may occur in infants with type B PA/VSD and decreased pulmonary flow, and systemic to pulmonary artery shunt should be performed during neonatal or early infant period, complete repair following after good pulmonary arterial development. On the other hand, for patients with over circulating pulmonary flow, primary repair is perform with 3~6 months for congestive heart failure. For patients with balanced pulmonary flow and repairable condition, one-stage complete repair can be performed in infancy stage. Otherwise, palliative systemic to pulmonary artery shunt or right ventricle to pulmonary artery shunt should be first, and second-staged complete repair or pulmonary arterial development promotion is adopted according to following assessment. The surgical option for type C PA/VSD should be discreet. Pulmonary arterial flow study is demanded after unifocalization to decide to close the VSD completely or not. Close follow-up after operation is imperative and cardiac catheterization is perform to reveal the pulmonary artery and collateral vessels if necessary and dilates the site of stenosis.