Pulmonic Atresia with Ventricular Septal Defect-natural History in Unoperated Cases

Pulmonic Atresia with Ventricular Septal Defect [PA –VSD] and absent or minute central pulmonary arteries is a complex cyanotic heart defect in which pulmonary blood flow [pbf] is usually dependant on aortic bronchial vessels ["mapcas"] PBF is either decreased, balanced or increased . In the modern era infants are usually treated surgically by unifocalization. In the 70`s and 80"s most patients had palliative shunts or were followed without surgery.

We present 10 unoperated adults who represent the natural history of PA-VSD. Ages ranged from 28 to 52 years. Four females and six males. P.B.F was decreased in 4 cases, increased in 2 cases and balanced in 3 cases. Two patients had 3 normal pregnancies and deliveries. One patient had a miscarriage. One patient died aged 34 of pneumonia. One died after complications of catheterization Two patients suffered from S.B.E. Aortic insufficiency was present in 4 cases.

Two patients developed Eisenmenger Syndrome. Uniquely one of the two has balanced flow in one lung and severe vascular disease in the other. One patient has congestive heart failure Three patients developed stenosis of mapcas and underwent dilatation and stent implantation.

The follow up of these patients will be described and the importance of ct and catheterization emphasized. These patients represent a control group for comparison to patients who underwent palliative surgery and later to those who underwent unifocalization.