Document Detail

Chronic thromboembolic pulmonary hypertension as an uncommon presentation of primary antiphospholipid syndrome.
MedLine Citation:
PMID:  18595578     Owner:  NLM     Status:  MEDLINE    
Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema. Electrocardiographic, echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed positive anticardiolipin antibodies and lupus anticoagulant with negative features for lupus with negative primary thrombophilic studies as well. The patient was managed adequately with oral anticoagulation with improvement of his clinical status and referred to a tertiary care center to be screened for pulmonary thromboendarterectomy. For patients meeting surgical selection criteria, pulmonary thromboendarterectomy has demonstrated positive outcomes with respect to survival, functionality and quality of life. We discuss the pathophysiology and treatment as well as novel therapies in nonsurgical candidates with chronic thromboembolic pulmonary hypertension in the setting of primary antiphospholipid syndrome.
Mateo Porres-Aguilar; Miguel Angel Pena-Ruiz; Jose D Burgos; Mateo Porres-Munoz; Harold W Hughes
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the National Medical Association     Volume:  100     ISSN:  0027-9684     ISO Abbreviation:  J Natl Med Assoc     Publication Date:  2008 Jun 
Date Detail:
Created Date:  2008-07-03     Completed Date:  2008-09-04     Revised Date:  2009-08-05    
Medline Journal Info:
Nlm Unique ID:  7503090     Medline TA:  J Natl Med Assoc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  734-6     Citation Subset:  IM    
Department of internal Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA.
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MeSH Terms
Antibodies, Anticardiolipin / blood
Anticoagulants / administration & dosage
Antiphospholipid Syndrome / complications*,  diagnosis,  drug therapy
Biological Markers / blood
Chronic Disease
Diagnostic Imaging
Hypertension, Pulmonary / diagnosis,  etiology*
Lupus Coagulation Inhibitor / blood
Pulmonary Embolism / diagnosis,  etiology*
Risk Factors
Reg. No./Substance:
0/Antibodies, Anticardiolipin; 0/Anticoagulants; 0/Biological Markers; 0/Lupus Coagulation Inhibitor

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