Document Detail

Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.
MedLine Citation:
PMID:  18006164     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension. METHODS: A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics. RESULTS: Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil. CONCLUSIONS: Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.
Zek S Lim; Joseph J Vettukattill; Anthony P Salmon; Gruschen R Veldtman
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article     Date:  2007-11-14
Journal Detail:
Title:  International journal of cardiology     Volume:  129     ISSN:  1874-1754     ISO Abbreviation:  Int. J. Cardiol.     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2010-02-23     Completed Date:  2010-09-23     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8200291     Medline TA:  Int J Cardiol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  339-43     Citation Subset:  IM    
Congenital Cardiac Centre, Southampton University Hospital, United Kingdom.
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MeSH Terms
Aortopulmonary Septal Defect / complications,  drug therapy,  physiopathology
Cross-Sectional Studies
Hypertension, Pulmonary / complications,  drug therapy*,  physiopathology
Middle Aged
Piperazines / therapeutic use*
Pulmonary Atresia / complications,  drug therapy*,  physiopathology
Purines / therapeutic use
Retrospective Studies
Sulfones / therapeutic use*
Tetralogy of Fallot / complications,  drug therapy,  physiopathology
Young Adult
Reg. No./Substance:
0/Piperazines; 0/Purines; 0/Sulfones; 139755-83-2/sildenafil

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