Pulmonary Hypertension in Trisomy 21 Patients
Pulmonary Hypertension in Trisomy 21 Patients
Evidence has shown poor outcome for adult patients with pre-operative pulmonary hypertension
 following closure of an atrial septal defect. Life-threatening pulmonary hypertensive crises
 may occur in these patients when they no longer have an atrial communication to decompress
 high right heart pressures. This concern has led some to advocate fenestrated patch closure
 of ASDs in patients with pulmonary hypertension with the prospect of a repeated procedure in
 order to close the fenestrations at a later date.
Pre-operative resolution of pulmonary hypertension would clearly provide the best long-term
 outcome for these patients, allowing a definitive surgical procedure. There are no reported
 cases of resolution of pulmonary hypertension with the use of pulmonary vasodilators allowing
 successful ASD closure.

 We report the case of a patient with trisomy 21 and a primum atrial septum defect with
 associated cleft mitral valve who was diagnosed with pulmonary hypertension at 8 days of
 life. Elevated pulmonary pressures were documented by cardiac catheterization during which
 the patient was found to have minimally responsive pulmonary vasculature to the
 administration of oxygen. He was started on pulmonary vasodilators which were continued for
 the following 5 months which resulted in gradual reduction in estimated PA pressure allowing
 successful closure of the primum ASD and repair of the cleft mitral valve. Following surgery,
 he was not restarted on pulmonary vasodilators and has continued to do well. The ability to
 reverse his pulmonary hypertension through the use of pulmonary vasodilators made him a
 candidate for his successful, definitive surgical repair. He currently has no residual
 cardiac problems and requires no cardiac medications. There will be no patient follow-up.
Inclusion Criteria:

 -  case report of a patient with trisomy 21 and primum atrial septum defect with
 associated cleft mitral valve who was diagnosed with pulmonary hypertension at 8 days
 of life

 Exclusion Criteria:

 -  those who do not fit into inclusion criteria