Pulmonary Atresia/Intact Ventricular Septum

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This defect consists of an underdeveloped and small right ventricle and tricuspid valve, with atresia of the pulmonary valve (total absence or blockage of the valve between the right ventricle and pulmonary artery).  These children typically are also born with a communication between the upper filling chambers or atria (atrial septal defect).  Because of this and the small right ventricle, the children are typically blue or cyanotic at birth and require their patent ductus arteriosus (PDA) to remain open prior to surgery to keep blood flowing to the lungs. The infant is maintained on a medication, prostaglandin (PGE1), intravenously from birth to maintain blood flow to the body and lungs until surgery or a cardiac catheterization can be done. Treatment of these children can be done by cardiac catheterization where the blocked valve is opened using a balloon catheter.

Surgical correction:  Is performed if this isn’t possible and consists of opening of the blocked valve using an incision to open the valve with a surgical knife and a shunt with a tube made of synthetic material (Goretex) either between the aorta and pulmonary artery (central shunt) or between the artery to the right arm and artery to the right lung (Blalock-Taussig Shunt).

Later surgery may include reopening or even replacement of the abnormal pulmonary valve and closure of the atrial septal defect (the opening between the upper two chambers of the heart).  In some circumstances, the right ventricle does not grow large enough and a bidirectional Glenn or Fontan procedure is needed.