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NCT00478296 Clinical Trial

Pulmonary Hypertension in Trisomy 21 Patients

Congenital Disorders Clinical Trial

Official title:
Case Report: Resolution of Pulmonary Hypertension With Sildenafil and Bosentan in Patients With Trisomy 21 and Atrial Septal Defect

Clinical Trial Details — Status: Completed

Administrative data
NCT number NCT00478296
Other study ID # 07-021
Secondary ID
Status Completed
Phase N/A
First received May 22, 2007
Last updated March 14, 2012
Start date October 2004
Est. completion date March 2008

Study information
Verified date January 2008
Source Children's Healthcare of Atlanta
Contact n/a
Is FDA regulated No
Health authority United States: Institutional Review Board
Study type Observational

Clinical Trial Summary

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.

Description:

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.

Recruitment information / eligibility
Status Completed
Enrollment 1
Est. completion date March 2008
Est. primary completion date February 2007
Accepts healthy volunteers No
Gender Both
Age group N/A to 8 Days
Eligibility 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

Study Design

Time Perspective: Retrospective

Related Conditions & MeSH terms

Locations
Country Name City State
United States Children's Healthcare of Atlanta Atlanta Georgia

Sponsors (1)
Lead Sponsor Collaborator
Children's Healthcare of Atlanta

Country where clinical trial is conducted

United States, 

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