Pulmonary Atresia with Ventricular Septal Defect

There is complete blockage between the heart and the lung arteries, and a hole between the two pumping chambers. The right ventricle is of good size in this condition. Blood supply to the lungs may be from the duct or from collateral vessels. Detailed angiographic studies are required to look at the collateral vessels and lung circulation in order to plan the optimum management for each child. Many will require shunt procedures to increase the blood flow to the lungs. These shunts may be performed through the side of the chest or through the breast bone.

Often the lung arteries are very small and a series of shunts may be necessary joining up the central lung arteries to a number of the extra collateral vessels (unifocalisation). The hope is to produce an adequate sized source of blood supply to each lung which can in due course be connected to the right pump chamber.

In some patients, the collateral vessels themselves can be stretched with balloons and stented via a catheter technique to improve the blood flow towards the lung. In other patients, no operation or procedure is required as the condition is reasonably well balanced. Chromosome tests are often performed. Long term follow-up and antibiotic prophylaxis is required.

Pulmonary Atresia with VSD

Victoria & Tasmania