Echocardiographic and Clinical Characteristics of Pulmonary Hypertension Complicating Pulmonary Langerhans Cell Histiocytosis

doi:10.4065/79.10.1269

Mayo Clinic Proceedings

Volume 79, Issue 10, October 2004, Pages 1269-1275

https://doi.org/10.4065/79.10.1269 Get rights and content

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.

Section snippets

PATIENTS AND METHODS

We identified 123 adult patients with histopathologically confirmed PLCH seen at our institution between January 1976 and December 2002. Clinical outcomes of 102 patients from this series were reported previously.³ The diagnosis was established by light microscopy in all patients. Of these 123 patients, 17 underwent comprehensive echocardiographic evaluation, with an indication of suspected PH in 8, shortness of breath in 6, cor pulmonale in 2, and palpitation in 1. The medical records were

Clinical Characteristics of PLCH Patients with PH

Of the 17 patients who underwent echocardiographic evaluation, evidence of PH was detected in 15 (estimated PASP at rest, >35 mm Hg), and only 2 had normal echocardiographic results (PASP, 26 and 33 mm Hg, respectively). Two of the 15 patients whose echocardiogram showed evidence of PH were excluded from further analysis because of the presence of an identifiable cause of PH; 1 patient had moderate mitral stenosis, and 1 had prior thromboembolism. The clinical characteristics of the 13 patients

DISCUSSION

The current study reports echocardiographic characteristics and survival of patients with PH associated with PLCH. Pulmonary hypertension is a complication of advanced pulmonary disease and seems to be particularly severe in patients with advanced PLCH.⁷ The current study suggests that severe PH is not limited to patients with end-stage pulmonary disease due to PLCH; PH was found in most patients in whom echocardiography was performed for the evaluation of dyspnea. Our study suggests that

CONCLUSION

Our data confirm prior observations that severe PH may complicate the course of PLCH and is associated with a significantly diminished survival. The degree of PH does not correlate with pulmonary function parameters, except inversely with FVC. Echocardiographic screening for PH should be considered in dyspneic patients with PLCH.

REFERENCES (17)

S Harari et al.
Advanced pulmonary histiocytosis X is associated with severe pulmonary hypertension [letter]
Chest
(1997)
RA Nishimura et al.
Doppler echocardiography: theory, instrumentation, technique, and application
Mayo Clin Proc
(1985)
PJ Currie et al.
Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients
J Am Coll Cardiol
(1985)
BJ Kircher et al.
Noninvasive estimation of right atrial pressure from the inspiratory collapse of the inferior vena cava
Am J Cardiol
(1990)
C Tei et al.
Noninvasive Doppler-derived myocardial performance index: correlation with simultaneous measurements of cardiac catheterization measurements
J Am Soc Echocardiogr
(1997)
RS Crausman et al.
Pulmonary vascular involvement in pulmonary histiocytosis X [letter]
Chest
(1997)
TV Colby et al.
Histiocytosis X in the lung
Hum Pathol
(1983)
A Tazi et al.
Adult pulmonary Langerhans' cell histiocytosis
Thorax
(2000)

There are more references available in the full text version of this article.

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Citation Excerpt :
PH is a common complication of PLCH and especially of advanced PLCH [174]. Interestingly, literature shows that PH is more frequent and more severe in PLCH than in other chronic lung diseases including IPF, possibly due to the development of an intrinsic pulmonary vasculopathy leading to vascular remodeling and luminal obstruction [175,176]. In particular, RHC unveiled PH in 92% of 39 subjects with PLCH that were referred for lung transplantation [177].
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¹: Dr Chaowalit was supported by Siriraj Hospital, Mahidol University, Bangkok, Thailand.

²: Dr Vassallo was supported by a Robert N. Brewer Family Foundation grant.

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Original ArticleEchocardiographic and Clinical Characteristics of Pulmonary Hypertension Complicating Pulmonary Langerhans Cell Histiocytosis

OBJECTIVE

PATIENTS AND METHODS

RESULTS

CONCLUSION

Section snippets

PATIENTS AND METHODS

Clinical Characteristics of PLCH Patients with PH

DISCUSSION

CONCLUSION

Chest

Mayo Clin Proc

J Am Coll Cardiol

Am J Cardiol

J Am Soc Echocardiogr

Chest

Hum Pathol

Adult pulmonary Langerhans' cell histiocytosis

Thorax

Original Article
Echocardiographic and Clinical Characteristics of Pulmonary Hypertension Complicating Pulmonary Langerhans Cell Histiocytosis