Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method
Abstract Background 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 lar...
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| Format: | Article |
| Language: | English |
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BMC
2017-04-01
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| Series: | Orphanet Journal of Rare Diseases |
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| Online Access: | http://link.springer.com/article/10.1186/s13023-017-0626-0 |
| _version_ | 1818893549221969920 |
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| author | Carolina S. G. Freitas Bruno G. Baldi Carlos Jardim Mariana S. Araujo Juliana Barbosa Sobral Gláucia I. Heiden Ronaldo A. Kairalla Rogério Souza Carlos R. R. Carvalho |
| author_facet | Carolina S. G. Freitas Bruno G. Baldi Carlos Jardim Mariana S. Araujo Juliana Barbosa Sobral Gláucia I. Heiden Ronaldo A. Kairalla Rogério Souza Carlos R. R. Carvalho |
| author_sort | Carolina S. G. Freitas |
| collection | DOAJ |
| description | Abstract Background 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 (DLCO) as screening methods for PH in LAM. Methods 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. Results 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 FEV1 and DLCO 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 DLCO result. Conclusions 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. |
| first_indexed | 2024-12-19T18:14:22Z |
| format | Article |
| id | doaj.art-632f554d5f3f4fe1ad778172e5af6f52 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-19T18:14:22Z |
| publishDate | 2017-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-632f554d5f3f4fe1ad778172e5af6f522022-12-21T20:11:09ZengBMCOrphanet Journal of Rare Diseases1750-11722017-04-011211710.1186/s13023-017-0626-0Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening methodCarolina S. G. Freitas0Bruno G. Baldi1Carlos Jardim2Mariana S. Araujo3Juliana Barbosa Sobral4Gláucia I. Heiden5Ronaldo A. Kairalla6Rogério Souza7Carlos R. R. Carvalho8Pulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolPulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolPulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolPulmonology Department, Federal University of ParanáEchocardiography Laboratory, Radiology Institute (InRad), University of São Paulo Medical SchoolPulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolPulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolPulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolPulmonary Division, Heart Institute (InCor), University of São Paulo Medical SchoolAbstract Background 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 (DLCO) as screening methods for PH in LAM. Methods 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. Results 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 FEV1 and DLCO 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 DLCO result. Conclusions 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.http://link.springer.com/article/10.1186/s13023-017-0626-0EchocardiographyLymphangioleiomyomatosisPrevalencePulmonary hypertensionRight heart catheterisation |
| spellingShingle | Carolina S. G. Freitas Bruno G. Baldi Carlos Jardim Mariana S. Araujo Juliana Barbosa Sobral Gláucia I. Heiden Ronaldo A. Kairalla Rogério Souza Carlos R. R. Carvalho Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method Orphanet Journal of Rare Diseases Echocardiography Lymphangioleiomyomatosis Prevalence Pulmonary hypertension Right heart catheterisation |
| title | Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method |
| title_full | Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method |
| title_fullStr | Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method |
| title_full_unstemmed | Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method |
| title_short | Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method |
| title_sort | pulmonary hypertension in lymphangioleiomyomatosis prevalence severity and the role of carbon monoxide diffusion capacity as a screening method |
| topic | Echocardiography Lymphangioleiomyomatosis Prevalence Pulmonary hypertension Right heart catheterisation |
| url | http://link.springer.com/article/10.1186/s13023-017-0626-0 |
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