Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Lymphangioleiomyomatosis (LAM) is included within group 5 of the current PH classification (unclear multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The aims of the study were to describe the prevalence and characteristics of PH in a large cohort of LAM pat...

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Bibliographic Details
Published in:	Orphanet journal of rare diseases Vol. 12; no. 1; p. 74
Main Authors:	Freitas, Carolina S G, Baldi, Bruno G, Jardim, Carlos, Araujo, Mariana S, Sobral, Juliana Barbosa, Heiden, Gláucia I, Kairalla, Ronaldo A, Souza, Rogério, Carvalho, Carlos R R
Format:	Journal Article
Language:	English
Published:	England BioMed Central 20-04-2017 BMC
Subjects:	Adult Blood pressure Carbon monoxide Carbon Monoxide - metabolism Cardiology Classification Diffusion Dyspnea Echocardiography Female Heart Humans Hypertension, Pulmonary - diagnosis Hypertension, Pulmonary - epidemiology Hypertension, Pulmonary - metabolism Hypertension, Pulmonary - pathology Lymphangioleiomyomatosis Lymphangioleiomyomatosis - diagnosis Lymphangioleiomyomatosis - epidemiology Lymphangioleiomyomatosis - metabolism Lymphangioleiomyomatosis - pathology Male Medical diagnosis Medical research Medical screening Middle Aged Normal distribution Patients Prevalence Pulmonary arteries Pulmonary artery Pulmonary fibrosis Pulmonary hypertension Rare diseases Respiration Respiratory function Respiratory Function Tests Right heart catheterisation Transplants & implants Veins & arteries Echocardiography Prevalence Pulmonary hypertension Right heart catheterisation Lymphangioleiomyomatosis
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Summary:	Lymphangioleiomyomatosis (LAM) is included within group 5 of the current PH classification (unclear multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The aims of the study were to describe the prevalence and characteristics of PH in a large cohort of LAM patients with different levels of severity, and to evaluate the role of echocardiography and carbon monoxide diffusion capacity (DL ) as screening methods for PH in LAM. One hundred five LAM patients underwent transthoracic echocardiography, pulmonary function tests (PFTs) and 6-min walk test (6MWT). Patients with a suspicion of PH on echocardiography, defined by the presence of estimated systolic pulmonary artery pressure (PAP) over 35 mmHg or PFT showing DLco below 40% of the predicted value, underwent right heart catheterisation to confirm the diagnosis of PH. Eight patients (7.6%) had PH confirmed on right heart catheterisation, six patients (5.7%) had a pre-capillary pattern and two patients (1.9%) had a post-capillary profile. Only one patient (1%) had mean PAP over 35 mmHg. Patients with PH had lower FEV and DL in PFTs and greater oxygen desaturation and dyspnea intensity during 6MWT compared with those without PH. In 63% of the patients with confirmed PH, the right heart catheterisation was performed based only on DL result. The prevalence of PH is low in LAM patients. Pulmonary hypertension in LAM is typically mild and significantly associated with pulmonary parenchymal involvement. Carbon monoxide diffusion capacity significantly improved the identification of PH in LAM patients.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1750-1172 1750-1172
DOI:	10.1186/s13023-017-0626-0