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. 2018 Jan 29;13(1):e0191869.
doi: 10.1371/journal.pone.0191869. eCollection 2018.

Hypoxemia in patients with idiopathic or heritable pulmonary arterial hypertension

Ghaleb Khirfan  1 , Tawfeq Naal  2 , Batool Abuhalimeh  2 , Jennie Newman  2 , Gustavo A Heresi  3 , Raed A Dweik  3 , Adriano R Tonelli  3
Affiliations

Hypoxemia in patients with idiopathic or heritable pulmonary arterial hypertension

Ghaleb Khirfan et al. PLoS One. .

Abstract

Background: The prevalence and prognostic implications of hypoxemia either at rest or during six-minute walk test (6MWT) in patients with idiopathic or heritable pulmonary arterial hypertension (IPAH or HPAH) have not been systemically studied.

Objectives: We sought to determine the prevalence, phenotypic and prognostic implications of hypoxemia in patients with IPAH and HPAH.

Methods: Patients with IPAH or HPAH were identified from the Cleveland Clinic Pulmonary Hypertension Registry. Pulse oximetry (SpO2) at rest and during 6MWT was used to define hypoxemia at rest or during activities when measurements were lower than 90%, respectively.

Results: A total of 292 patients (age 50.6 ± 18.0 years, 73% females) with IPAH (88%) and HPAH (12%) were included. Of them, 143 (49%) had SpO2 >90% at rest and during 6MWT, 89 (31%) subjects had hypoxemia during 6MWT and 60 (20%) had hypoxemia at rest. Patients with hypoxemia had older age, greater body mass index, higher prevalence of cardiovascular risk factors, worse functional capacity and pulmonary function tests but less severe pre-capillary pulmonary hypertension. Individuals with hypoxemia either at rest or during the initial 6MWT had worse long-term survival when compared to subjects without hypoxemia, even when adjusting for a great number of potential confounders. (HR: 2.5 (95% CI: 1.54-3.98)).

Conclusions: Hypoxemia in patients with IPAH and HPAH is associated with more comorbidities, less severe pre-capillary pulmonary hypertension and worse survival.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist except for Gustavo A. Heresi, who received personal fees for being a member in Bayer Healthcare – Advisory Board and Speaking. This does not alter our adherence to PLOS ONE policies on sharing data and materials. There are no patents, products in development or marketed products to declare.

Figures

Fig 1
Fig 1. Selection of study participants.
Definition of abbreviations: CHD: congenital heart disease, CTD: connective tissue disease, HIV: human immunodeficiency virus, HPAH: heritable pulmonary arterial hypertension, IPAH: idiopathic pulmonary arterial hypertension, PAH: pulmonary arterial hypertension, PCH: pulmonary capillary hemangiomatosis, PFTs: pulmonary function tests, PH: pulmonary hypertension, PVOD: pulmonary veno-occlusive disease.
Fig 2
Fig 2. Kaplan-Meier and Cox survival analysis for the entire cohort.
Fig 3
Fig 3. Kaplan-Meier and Cox survival analysis for cases not on PAH-specific therapies.
See this image and copyright information in PMC

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