Pulmonary Hypertension in Trisomy 21 Patients
Information source: Children's Healthcare of Atlanta
ClinicalTrials.gov processed this data on August 23, 2015 Link to the current ClinicalTrials.gov record.
Condition(s) targeted: Congenital Disorders
Phase: N/A
Status: Completed
Sponsored by: Children's Healthcare of Atlanta Official(s) and/or principal investigator(s): Heather M Phelps, DO, Principal Investigator, Affiliation: Children's Healthcare of Atlanta
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.
Clinical Details
Official title: Case Report: Resolution of Pulmonary Hypertension With Sildenafil and Bosentan in Patients With Trisomy 21 and Atrial Septal Defect
Study design: Time Perspective: Retrospective
Detailed 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.
Eligibility
Minimum age: N/A.
Maximum age: 8 Days.
Gender(s): Both.
Criteria:
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
Locations and Contacts
Children's Healthcare of Atlanta, Atlanta, Georgia 30322, United States
Additional Information
Starting date: October 2004
Last updated: March 14, 2012
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