Document Detail


Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects.
MedLine Citation:
PMID:  17027100     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: There is increasing evidence for beneficial effects of the type 5 phosphodiesterase inhibitor, sildenafil, in idiopathic pulmonary arterial hypertension (PAH). The effects of sildenafil in severe PAH associated with an atrial septal defect (ASD) have not been clearly delineated. METHODS: This extended case series reviews our experience with sildenafil treatment in three patients with severe PAH and Eisenmenger syndrome associated with an ASD. Case notes were reviewed for baseline and follow-up anatomic, clinical and haemodynamic characteristics. RESULTS: Of three patients identified, median age 44.3 years (range, 28 to 59 years), two had large secundum ASDs and the other a sinus venous defect. All patients had severe PAH and were desaturated at rest and/or during exercise. Sildenafil was started because of progressive and severe effort intolerance. All patients experienced symptomatic improvement, had higher arterial saturations (range 8-19%) and improvement in effort tolerance (88 m and 56 m improvement in 6-min walk test distance (MWTD) in two patients). Right ventricular (RV) function and Doppler derived RV systolic pressure improved in two patients. Pulmonary arterial pressures decreased in two patients who had cardiac catheterization (range 4 to 14 mm Hg). Pulmonary vascular resistance decreased from 7.58 to 3.8 Wood's units in one patient who is now awaiting surgery. Another patient developed significant pulmonary vasoreactivity (8.3 to 6.2 Wood's units with 100% oxygen) after 16 months of sildenafil therapy. CONCLUSIONS: Sildenafil not only relieves symptoms associated with severe PAH in patients with large ASDs, but also improves pulmonary arterial hemodynamics and RV function.
Authors:
Zek S Lim; Anthony P Salmon; Joseph J Vettukattil; Gruschen R Veldtman
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2006-10-05
Journal Detail:
Title:  International journal of cardiology     Volume:  118     ISSN:  1874-1754     ISO Abbreviation:  Int. J. Cardiol.     Publication Date:  2007 May 
Date Detail:
Created Date:  2007-04-23     Completed Date:  2007-08-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8200291     Medline TA:  Int J Cardiol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  178-82     Citation Subset:  IM    
Affiliation:
Wessex Adult Congenital Heart Unit, Wessex Cardiothoracic Unit, Southampton General Hospital, UK.
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MeSH Terms
Descriptor/Qualifier:
Adult
Female
Heart Septal Defects, Atrial / complications*,  diagnosis,  therapy
Humans
Hypertension, Pulmonary / diagnosis,  drug therapy*,  etiology*
Male
Middle Aged
Phosphodiesterase Inhibitors / therapeutic use*
Piperazines / therapeutic use*
Purines / therapeutic use
Retrospective Studies
Sulfones / therapeutic use*
Treatment Outcome
Chemical
Reg. No./Substance:
0/Phosphodiesterase Inhibitors; 0/Piperazines; 0/Purines; 0/Sulfones; 139755-83-2/sildenafil

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


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