Associated malformations are frequent, and include pulmonary stenosis or atresia, atrial or ventricular septal defects and, more rarely, atrioventricular septal defect.
The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels.
In patients without pulmonary stenosis, the systolic pulmonary arterial pressure increased concomitantly with the pressure elastic modulus.
The pressure elastic modulus of the pulmonary trunk was abnormally high (>120) in most (13114) patients even relatively early after the operation.
Nevertheless, severe pulmonary hypertension and elevated pulmonary vascular resistance were present.
Pulmonary veins draining wholly to the ductus arteriosus (case 2).
Clues to the aetiology of the disease have been suggested by environmental stimuli associated with the development of severe pulmonary hypertension.
Within one week, the child clinically improved; pulmonary function were normal after 6 weeks and radiographic abnormalities resolved.
