Pulmonary Hypertension in Trisomy 21 Patients
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|ClinicalTrials.gov Identifier: NCT00478296|
Recruitment Status : Completed
First Posted : May 24, 2007
Last Update Posted : March 16, 2012
|Condition or disease|
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.
|Study Type :||Observational|
|Actual Enrollment :||1 participants|
|Official Title:||Case Report: Resolution of Pulmonary Hypertension With Sildenafil and Bosentan in Patients With Trisomy 21 and Atrial Septal Defect|
|Study Start Date :||October 2004|
|Actual Primary Completion Date :||February 2007|
|Actual Study Completion Date :||March 2008|
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Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00478296
|United States, Georgia|
|Children's Healthcare of Atlanta|
|Atlanta, Georgia, United States, 30322|
|Principal Investigator:||Heather M Phelps, DO||Children's Healthcare of Atlanta|