Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery

Abstract

Palliative surgical treatment of CHDs associated with UAPA can be performed with a relatively low risk. Systemic-to-pulmonary artery shunt and transluminal balloon pulmonary valvuloplasty are methods of choice in patients with non-severe hypoplasia of the single pulmonary artery. The intravascular intervention is indicated more in patients with a prevailing valvular component of the pulmonary stenosis. Palliative reconstruction of the right ventricular outflow tract is a more favourable procedure for patients with a severe hypoplasia of the single pulmonary artery.

Management of Tetralogy of Fallot With Unilateral Absence of Pulmonary Artery

“…According to Bockeria and associates, 12,18 primary complete repair is indicated in patients with a normal size of the contralateral PA (Nakata index Z score more than À2 and Nakata index 336 þ 63 mm 2 /m 2 ) or its mild hypoplasia (Nakata index Z score equal to or less than À2 but more than À4 and Nakata index 240 þ 21 mm 2 /m 2 ). Complete repair in patients with a moderate hypoplasia of the contralateral PA (Nakata index more than À6 but less than or equal to À4 and Nakata index 180 þ 7 mm 2 /m 2 ) is associated with a higher risk.…”

“…Palliative procedures relieve cyanosis and polycythemia, prepare the contralateral lung for repair, and promote PA growth LV growth. 18,20 Bockeria and associates 18 have proposed the following classification system for palliative procedures in TOF with UAPA: Group 2 interventions are indicated in patients whose PA anatomy is inadequate for one-stage, double-lung repair. They help in promoting PA growth so that they can endure the complete cardiac output at the time of complete repair.…”

Long‐term outcomes of percutaneous closure of patent ductus arteriosus associated with unilateral absence of a pulmonary artery

“…The choice of treatment is based on the symptoms of individual patients, PA anatomy and associated aortopulmonary collaterals, associated cardiovascular anomalies, and pulmonary hypertension 2 . An “ideal” surgical treatment for UAPA would achieve the restoration of antegrade blood flow in the ipsilateral lung and the repair of any congenital heart defects 9,10 . However, very few studies have reported on the treatment of UAPA, and selective pulmonary vein angiogram is not performed routinely to evaluate PA development 11 .…”

“…Unfortunately, selective pulmonary vein angiography is not performed routinely to evaluate PA development in our center. When the hilar artery in the route of the ipsilateral lung cannot be identified and the inclusion of this lung into pulmonary circulation is not possible, the surgical treatment is directed at the repair of the concomitant congenital heart defect(s) 9 …”

A case series of three patients with unilateral disconnected pulmonary artery supplied by an ipsilateral patent ductus arteriosus: neonatal ductal stenting as palliation to preserve pulmonary arterial patency

“… 6 Association with a number of other lesions further complicates estimates of incidence. 7 , 8 Surgical re-anastomosis is well-established. 1 , 3 , 7 …”