| Abstract: | The authors report 9 cases of atrial septal defect with sever pulmonary hypertension in 7 girls and 2 boys under 10 years of age. These cases represent 3.5% of the 255 cases of atrial septal defect in this age group seen at the Hopital Cardiologique of Lille between 1970 and 1985. Group I comprised 3 children with obstructive pulmonary hypertension from the start; two died, one is still alive after 3 years. Group II was composed of 3 children who presented, at first haemodynamic evaluation, with severe but non-obstructive pulmonary hypertension; the hypertension rapidly became obstructive in 2 of them despite digitalis-diuretic therapy; the third child died after surgical correction. The 3 children in group III had normal or slightly raised pulmonary arterial pressure at first haemodynamic evaluation; one of them initially had a right ventricle-pulmonary artery functional gradient of 45 mmHg, which did not prevent the subsequent development of obstructive pulmonary hypertension; the other 2 patients were operated upon 10 months and 4 years later respectively, as they presented with severe pulmonary hypertension; one of these 2 children died postoperatively, the third one developed obstructive pulmonary hypertension. Contrary to what is generally believed, severe pulmonary hypertension is not exceptional in children with atrial septal defect, and it has a poor prognosis. The mechanism underlying the development of this pulmonary hypertension is unclear, but individual susceptibility to excessive blood flow is probable, as is the role played by bronchopulmonary infections frequently noted in these patients' history. |
