Carbon monoxide diffusion capacity significantly improved the identification of PH in LAM patients
Pulmonary hypertension (PH) is a known complication of LAM but data regarding its occurrence in lymphangioleiomyomatosis (LAM) are scarce.
The aims of this national centre study in Brazil 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 as screening methods for PH in LAM.
105 LAM patients were enrolled in the study and underwent transthoracic echocardiography, pulmonary function tests and 6-min walk test (6MWT). Patients with a suspicion of PH on echocardiography, underwent right heart catheterisation to confirm the diagnosis of PH.
The conclusions of this study showed that 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.
Read the full open access article: Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method published in Orphanet Journal of Rare Diseases; 2017 12:74