Document Detail

Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.
MedLine Citation:
PMID:  21801314     Owner:  NLM     Status:  MEDLINE    
Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.
Utku Arman Orun; Osman Yilmaz; Meki Bilici; Selmin Karademir; Cigdem Uner; Filiz Senocak; Vehbi Dogan
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2011-07-31
Journal Detail:
Title:  Congenital heart disease     Volume:  7     ISSN:  1747-0803     ISO Abbreviation:  Congenit Heart Dis     Publication Date:    2012 May-Jun
Date Detail:
Created Date:  2012-05-28     Completed Date:  2012-09-24     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  101256510     Medline TA:  Congenit Heart Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E6-9     Citation Subset:  IM    
Copyright Information:
© 2011 Wiley Periodicals, Inc.
Department of Pediatric Cardiology, Dr. Sami Ulus Pediatric Research and Training Hospital, Ankara, Turkey.
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MeSH Terms
Abnormalities, Multiple*
Antihypertensive Agents / therapeutic use
Cardiac Catheterization
Dyspnea / etiology
Heart Septal Defects, Atrial / complications*,  diagnosis
Hypertension, Pulmonary / diagnosis,  drug therapy,  etiology*
Pulmonary Artery / abnormalities*,  radiography
Sulfonamides / therapeutic use
Tomography, X-Ray Computed
Treatment Outcome
Reg. No./Substance:
0/Antihypertensive Agents; 0/Sulfonamides; Q326023R30/bosentan

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