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Effects of PAH Specific Therapy on Pulmonary Hemodynamics and Six-Minute Walk Distance in Pulmonary Hypertension Secondary to Interstitial Lung Disease: A Meta-analysis FREE TO VIEW

Bashar Mourad; Abubakr Bajwa; Adil Shujaat
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University of Florida, Jacksonville, FL

Chest. 2014;146(4_MeetingAbstracts):367A. doi:10.1378/chest.1995016
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SESSION TITLE: Interstitial Lung Disease Posters I

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: Pulmonary arterial hypertension (PAH) specific therapy has been shown to result in significant improvement in pulmonary hemodynamics and exercise capacity in patients with WHO group I pulmonary hypertension (PH). However, little is known about the effectiveness of PAH specific therapy in patients with PH secondary to interstitial lung disease (ILD). We sought to perform a literature search for studies of such therapy in PH secondary to ILD to determine the effects of such therapy on pulmonary hemodynamics and six minute walk distance (6MWD).

METHODS: We performed a PUBMED search using the terms “pulmonary hypertension” AND “interstitial lung disease”. We included studies that met the following criteria: ≥ 5 patients, AND PH confirmed by right heart catheterization (RHC), AND follow up RHC data, AND/OR baseline and follow up 6MWD available. We used Comprehensive Meta-analysis and random effects model to perform the meta-analysis.

RESULTS: Five studies met our inclusion criteria. Three were prospective in design and 2 retrospective. All were single arm studies. None were a RCT. There were a total of 126 patients. The majority of patients were treated with an endothelin receptor antagonist or phosphodiesterase 5 inhibitor. The mean ± SD age was 62.7 ± 6.4 years and 60% were females. The cause of ILD in the majority was idiopathic pulmonary fibrosis or connective tissue disease associated ILD. The baseline mean ± SD FVC was 64.6 ± 6.9 % predicted and DLCO 36.9 ± 5.1% predicted. The baseline mean ± SD mean pulmonary artery pressure (mPAP) was 42.4 ± 9.2 mm Hg, cardiac index 2.4 ± 0.1 L/min/m2, and pulmonary vascular resistance 7.7± 0.1 Wood units. The baseline 6MWD was 227.8± 117.9 meters. The use of PAH specific therapy did not result in any significant improvement in mPAP (n = 75) or 6MWD (n = 116). The mPAP changed by -3.9 mm Hg (95%CI -12.6 to 4.7; I2 53%, Q 2.1) and 6MWD by 17.6 meters (95%CI -17.9 to 53.2; I2 77.6%, Q 17.9).

CONCLUSIONS: The use of PAH specific therapy in PH secondary to ILD does not result in any significant improvement in pulmonary hemodynamics or 6MWD.

CLINICAL IMPLICATIONS: The result of this meta-analysis allows physicians to make a more informed decision when treating PH secondary to ILD. While PAH specific therapy may be appopriate for other forms of PH there is currently limited evidence to suggest that available options help improve patient symptoms in those who suffer from ILD-induced PH. Continued investigations will be needed to determine what, if any, intervention can help .

DISCLOSURE: The following authors have nothing to disclose: Bashar Mourad, Abubakr Bajwa, Adil Shujaat

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