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Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis.
Chest. 2020 Jun 29 [Online ahead of print]Chest

Abstract

BACKGROUND

Pulmonary Langerhans cell histiocytosis (PLCH) determines reduced exercise capacity. The speculated mechanisms of exercise impairment in PLCH are ventilatory and cardiocirculatory limitations, including pulmonary hypertension (PH).

RESEARCH QUESTION

What are the mechanisms of exercise limitation, the exercise capacity, and the prevalence of dynamic hyperinflation (DH) and PH in PLCH?

STUDY DESIGN AND METHODS

In a cross-sectional study, patients with PLCH underwent an incremental treadmill cardiopulmonary exercise test with an evaluation of DH, pulmonary function tests, and transthoracic echocardiography. Those patients with lung diffusing capacity for carbon monoxide (Dlco) < 40% predicted and/or transthoracic echocardiogram with tricuspid regurgitation velocity > 2.5 m/s and/or with indirect PH signs underwent right heart catheterization.

RESULTS

Thirty-five patients were included (68% women; mean age, 47 ± 11 years). Ventilatory and cardiocirculatory limitations, impairment suggestive of PH, and impaired gas exchange occurred in 88%, 67%, 29%, and 88% of patients, respectively. The limitation was multifactorial in 71%, exercise capacity was reduced in 71%, and DH occurred in 68% of patients. FEV1 and Dlco were 64 ± 22% predicted and 56 ± 21% predicted. Reduction in Dlco, an obstructive pattern, and air trapping occurred in 80%, 77%, and 37% of patients. FEV1 and Dlco were good predictors of exercise capacity. The prevalence of PH was 41%, predominantly with a precapillary pattern, and mean pulmonary artery pressure correlated best with FEV1 and tricuspid regurgitation velocity.

INTERPRETATION

PH is frequent and exercise impairment is common and multifactorial in PLCH. The most prevalent mechanisms are ventilatory, cardiocirculatory, and suggestive of PH limitations.

CLINICAL TRIAL REGISTRATION

ClinicalTrials.gov; No.: NCT02665546; URL: www.clinicaltrials.gov.

Authors+Show Affiliations

Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil. Electronic address: bruno.guedes2@terra.com.br.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32615192

Citation

Heiden, Glaucia Itamaro, et al. "Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis." Chest, 2020.
Heiden GI, Sobral JB, Freitas CSG, et al. Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis. Chest. 2020.
Heiden, G. I., Sobral, J. B., Freitas, C. S. G., Pereira de Albuquerque, A. L., Salge, J. M., Kairalla, R. A., Fernandes, C. J. C. D. S., Carvalho, C. R. R., Souza, R., & Baldi, B. G. (2020). Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis. Chest. https://doi.org/10.1016/j.chest.2020.05.609
Heiden GI, et al. Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis. Chest. 2020 Jun 29; PubMed PMID: 32615192.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis. AU - Heiden,Glaucia Itamaro, AU - Sobral,Juliana Barbosa, AU - Freitas,Carolina Salim Gonçalves, AU - Pereira de Albuquerque,André Luis, AU - Salge,João Marcos, AU - Kairalla,Ronaldo Adib, AU - Fernandes,Caio Júlio César Dos Santos, AU - Carvalho,Carlos Roberto Ribeiro, AU - Souza,Rogério, AU - Baldi,Bruno Guedes, Y1 - 2020/06/29/ PY - 2020/02/27/received PY - 2020/05/18/revised PY - 2020/05/29/accepted PY - 2020/7/3/pubmed PY - 2020/7/3/medline PY - 2020/7/3/entrez KW - Langerhans cell histiocytosis KW - exercise KW - lung function testing KW - pulmonary hypertension JF - Chest JO - Chest N2 - BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) determines reduced exercise capacity. The speculated mechanisms of exercise impairment in PLCH are ventilatory and cardiocirculatory limitations, including pulmonary hypertension (PH). RESEARCH QUESTION: What are the mechanisms of exercise limitation, the exercise capacity, and the prevalence of dynamic hyperinflation (DH) and PH in PLCH? STUDY DESIGN AND METHODS: In a cross-sectional study, patients with PLCH underwent an incremental treadmill cardiopulmonary exercise test with an evaluation of DH, pulmonary function tests, and transthoracic echocardiography. Those patients with lung diffusing capacity for carbon monoxide (Dlco) < 40% predicted and/or transthoracic echocardiogram with tricuspid regurgitation velocity > 2.5 m/s and/or with indirect PH signs underwent right heart catheterization. RESULTS: Thirty-five patients were included (68% women; mean age, 47 ± 11 years). Ventilatory and cardiocirculatory limitations, impairment suggestive of PH, and impaired gas exchange occurred in 88%, 67%, 29%, and 88% of patients, respectively. The limitation was multifactorial in 71%, exercise capacity was reduced in 71%, and DH occurred in 68% of patients. FEV1 and Dlco were 64 ± 22% predicted and 56 ± 21% predicted. Reduction in Dlco, an obstructive pattern, and air trapping occurred in 80%, 77%, and 37% of patients. FEV1 and Dlco were good predictors of exercise capacity. The prevalence of PH was 41%, predominantly with a precapillary pattern, and mean pulmonary artery pressure correlated best with FEV1 and tricuspid regurgitation velocity. INTERPRETATION: PH is frequent and exercise impairment is common and multifactorial in PLCH. The most prevalent mechanisms are ventilatory, cardiocirculatory, and suggestive of PH limitations. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT02665546; URL: www.clinicaltrials.gov. SN - 1931-3543 UR - https://www.unboundmedicine.com/medline/citation/32615192/Mechanisms_of_exercise_limitation_and_prevalence_of_pulmonary_hypertension_in_pulmonary_Langerhans_cell_histiocytosis L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-3692(20)31823-7 DB - PRIME DP - Unbound Medicine ER -