- Emergency
- Prenatal information.
- Congenital heart defects.
- Atrial septal defect (ASD).
- Atrioventricular septal defect (AVSD).
- Ventricular septal defect (VSD).
- Aortopulmonary window.
- Aortic arch anomalies.
- Coarctation of the aorta.
- Interrupted aortic arch.
- Persistent ductus arteriosus (PDA).
- Cor triatriatrum.
- Ebstein’s anomaly.
- Pulmonary stenosis.
- Fallot’s tetralogy.
- Coronary artery anomalies.
- D-transposition of the great arteries (d-TGA).
- Aortic stenosis (AS).
- Total anomalous pulmonary venous connection (TAPVC).
- Pulmonary atresia with intact ventricular septum (PA + IVS).
- Double Outlet Right Ventricle (DORV).
- Truncus Arteriosus (TA).
- Hypoplastic left heart syndrome (HLHS).
- Univentricular heart (UVH).
- Treatment principles.
- Heart transplantation in infancy and childhood.
- Children's ward.
Pulmonary atresia with intact ventricular septum (PA + IVS)
The condition of pulmonary atresia with intact ventricular septum ranges broadly in severity, from fusion of the pulmonary valve leaflets alone to severe forms in which there is a considerable reduction of the right ventricular cavity in association with fistulas from the coronary artery to the right ventricle. About 25% of the patients also have severe hypoplasia of the tricuspid valve. In contrast to pulmonary atresia with VSD, the central pulmonary arteries are usually well developed and there are hardly ever any arterio-pulmonary collaterals.
The range of treatments is varied because of the varied pathological anatomy of the defect. The operative procedure is determined especially by the extent of the fistulas from the coronary artery to the right ventricle, and by the size of the right ventricular cavity and tricuspid valve.
If the coronary circulation depends completely on the coronary fistulas, which is observed in about 9% of cases, definitive correction is impossible. In these children, lung perfusion can be improved only by creating an arterio-pulmonary shunt.
In severe forms without coronary fistulas, the first operation usually consists of creating an arterio-pulmonary shunt, generally a modified Blalock-Taussig shunt. The disadvantage of this procedure is that the chance of later two-chamber correction is reduced as the size of the tricuspid valve ring often diminishes further after this operation.
Pulmonary valvotomy is theoretically the ideal procedure. It allows antegrade flow from the right ventricle and avoids the volume load of an arterio-pulmonary shunt. However, this procedure is limited to forms with a sufficiently large tricuspid valve ring. If the diameter is inadequate, the pulmonary valve must be extended by a transannular patch. Patients without adequate lung perfusion require creation of a modified Blalock-Taussig shunt in addition, which is removed after satisfactory growth of the right ventricle.