Document Detail

Echocardiographic and clinical characteristics of pulmonary hypertension complicating pulmonary Langerhans cell histiocytosis.
MedLine Citation:
PMID:  15473409     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To determine the echocardiographic characteristics and impact on survival of pulmonary hypertension (PH) and correlations between echocardiographic and clinical variables in patients with pulmonary Langerhans cell histiocytosis (PLCH).
PATIENTS AND METHODS: Of 123 adults seen at our institution between January 1976 and December 2002 with histologically proven PLCH, 17 underwent echocardiographic evaluation. Correlations were performed between echocardiographic measures of PH and clinical variables. Cumulative survival probabilities for patients with PH were estimated using the Kaplan-Meier method and were compared to a historical cohort of patients with PLCH using time-dependent proportional hazard regression.
RESULTS: Of the 17 patients, PH (estimated pulmonary artery systolic pressure [PASP] at rest, > 35 mm Hg) was present in 15. Thirteen patients (6 men; median PASP, 67 mm Hg; range, 41.2-90.6 mm Hg) had no other known causes of PH. All patients were smokers. Nine patients had a PASP of more than 50 mm Hg. An inverse correlation was found between the forced vital capacity and PASP (r = -0.61; P = .03); no correlation was found between PASP and other pulmonary function parameters. Seven patients with a PASP greater than 65 mm Hg had an enlarged right ventricle with impaired systolic function. The development of PH in patients with PLCH was associated with increased mortality (hazard ratio, 22.8; 95% confidence interval, 7.6 to > 68.9; P < .001).
CONCLUSION: Severe PH occurs in PLCH, correlates with the forced vital capacity, and has a significant impact on survival. Clinicians should consider echocardiographic screening for PH in all dyspneic patients with PLCH.
Nithima Chaowalit; Patricia A Pellikka; Paul A Decker; Marie-Christine Aubry; Michael J Krowka; Jay H Ryu; Robert Vassallo
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Mayo Clinic proceedings     Volume:  79     ISSN:  0025-6196     ISO Abbreviation:  Mayo Clin. Proc.     Publication Date:  2004 Oct 
Date Detail:
Created Date:  2004-10-11     Completed Date:  2004-11-23     Revised Date:  2013-12-13    
Medline Journal Info:
Nlm Unique ID:  0405543     Medline TA:  Mayo Clin Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1269-75     Citation Subset:  AIM; IM    
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MeSH Terms
Dyspnea / etiology
Echocardiography / methods*
Histiocytosis, Langerhans-Cell / complications,  therapy,  ultrasonography*
Hypertension, Pulmonary / etiology,  therapy,  ultrasonography*
Mass Screening / methods
Middle Aged
Retrospective Studies
Survival Analysis

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

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