Pulmonary Atresia with no Ventricular Septal Defect

In pulmonary atresia with an intact ventriĀ¬cular septum, the pulmonary valve is blocked and blood flow to the lungs depends on whether the pre-birth arterial duct remains open. The right ventricle is of variable size and may be small.

If it is very small, it is unlikely to grow and a shunt procedure alone is necessary to take extra blood into the lungs aiming for a Fontan type operation in due course. If the right ventricle is larger, then the pulmonary valve may be stretched or removed and a shunt procedure is usually undertaken at the same time. Sometimes, by using a catheter technique, it is possible to cross this blocked valve with a special wire and then stretch the valve with a balloon, allowing blood to go from the heart directly towards the lung artery. Additionally, a shunt operation would often need to be performed but this can be done with closed heart surgery.

Further operations are usually necessary to relieve residual obstruction or to overcome leaking in the area of the pulmonary valve.

In some, the right ventricle is of a good size the shunt can be closed and the hole between the filling chambers closed. In others, the upper body vein can be joined to the lung artery reducing some of the work of the right ventricle and in others, a Fontan type operation is the best long term plan.

Long term follow-up and antibiotic prophylaxis is necessary.

Pulmonary Artesia with no VSD

Victoria & Tasmania