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Sponsored by: |
Children's Healthcare of Atlanta |
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Information provided by: | Children's Healthcare of Atlanta |
ClinicalTrials.gov Identifier: | NCT00478296 |
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.
Condition |
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Congenital Disorders |
Study Type: | Observational |
Study Design: | Natural History, Longitudinal, Defined Population, Retrospective Study |
Official Title: | Case Report: Resolution of Pulmonary Hypertension With Sildenafil and Bosentan in Patients With Trisomy 21 and Atrial Septal Defect |
Estimated Enrollment: | 1 |
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.
Ages Eligible for Study: | up to 8 Days |
Genders Eligible for Study: | Both |
Accepts Healthy Volunteers: | No |
Inclusion Criteria:
Exclusion Criteria:
United States, Georgia | |
Children's Healthcare of Atlanta | |
Atlanta, Georgia, United States, 30322 |
Principal Investigator: | heather m phelps, DO | Children's Healthcare of Atlanta |
Study ID Numbers: | 07-021 |
Study First Received: | May 22, 2007 |
Last Updated: | May 23, 2007 |
ClinicalTrials.gov Identifier: | NCT00478296 History of Changes |
Health Authority: | United States: Institutional Review Board |
Pulmonary hypertension Sildenafil Bosentan Trisomy 21 Atrial septal defect |
Heart Septal Defects Aneuploidy Heart Diseases Cardiovascular Abnormalities Vascular Diseases Chromosome Disorders Sildenafil Trisomy Bosentan Heart Septal Defects, Atrial Mental Retardation Respiratory Tract Diseases |
Genetic Diseases, Inborn Hypertension, Pulmonary Lung Diseases Chromosome Aberrations Abnormalities, Multiple Neurologic Manifestations Congenital Heart Septum Defect Down Syndrome Congenital Abnormalities Heart Defects, Congenital Neurobehavioral Manifestations Hypertension |
Heart Septal Defects Aneuploidy Heart Diseases Cardiovascular Abnormalities Nervous System Diseases Vascular Diseases Chromosome Disorders Trisomy Heart Septal Defects, Atrial Mental Retardation Pathologic Processes Respiratory Tract Diseases |
Genetic Diseases, Inborn Hypertension, Pulmonary Lung Diseases Chromosome Aberrations Abnormalities, Multiple Neurologic Manifestations Cardiovascular Diseases Down Syndrome Congenital Abnormalities Heart Defects, Congenital Neurobehavioral Manifestations Hypertension |