Document Detail

Fenestrated ASD closure in a child with idiopathic pulmonary hypertension and exercise desaturation.
MedLine Citation:
PMID:  21426528     Owner:  NLM     Status:  MEDLINE    
Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right-to-left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½-year-old was diagnosed with pulmonary hypertension, a large atrial septal defect and right-to-left shunting. Medical therapy over an 18-month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left-to-right atrial flow.
Russel Hirsch; Michelle C Bagby; Matthew E Zussman
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Congenital heart disease     Volume:  6     ISSN:  1747-0803     ISO Abbreviation:  Congenit Heart Dis     Publication Date:    2011 Mar-Apr
Date Detail:
Created Date:  2011-03-23     Completed Date:  2011-07-18     Revised Date:  2013-08-23    
Medline Journal Info:
Nlm Unique ID:  101256510     Medline TA:  Congenit Heart Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  162-6     Citation Subset:  IM    
Copyright Information:
© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.
Cincinnati Children's Hospital Medical Center-The Heart Institute, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.
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MeSH Terms
Antihypertensive Agents / therapeutic use
Blood Pressure
Cardiac Surgical Procedures*
Child, Preschool
Echocardiography, Doppler, Color
Exercise Tolerance*
Heart Septal Defects, Atrial / complications,  diagnosis,  physiopathology,  surgery*
Hemodynamics* / drug effects
Hypertension, Pulmonary / complications,  diagnosis,  drug therapy,  physiopathology*
Treatment Outcome
Vascular Resistance
Vasodilator Agents / therapeutic use
Grant Support
Reg. No./Substance:
0/Antihypertensive Agents; 0/Vasodilator Agents

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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