Pulmonary hypertension in idiopathic pulmonary fibrosis

Amany Omar Mohammed,Azza Farag Said El-Tooney,Hosny Sayed Abdel Ghani,Nasser Mohammed Taha,Zainab Hassan Saied

doi:10.4103/1687-8426.137348

Amany Omar Mohammed, Azza Farag Said El-Tooney + Show 3 more

Open Access

https://doi.org/10.4103/1687-8426.137348

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Abstract

Pulmonary hypertension (PH) is a common finding in patients with idiopathic pulmonary fibrosis ( IPF) and is associated with increased morbidity and mortality. This study was designed to detect the prevalence of PH in patients with IPF and to determine the correlation between pulmonary function test (PFT) parameters, radiological and echocardiographic findings, and PH among these patients. In addition, the effect of therapy for 1 month (long-term oxygen therapy ± drugs) on PH was evaluated. PFTs, the composite physiologic index, multidetector chest computed tomography, and echocardiography were performed on 60 IPF patients (10 men and 50 women, mean age 51 years). Fifteen healthy age-matched and sex-matched volunteers were also studied as a control group. PH was present in 46 (76.6%) patients of IPF. There was a high negative correlation between parameters of PFTs and composite physiologic index and the mean pulmonary arterial pressure ( mPAP). In contrast, there was a significant positive correlation between mPAP and main pulmonary artery diameter on computed tomography. There was also a positive correlation between echocardiographic findings and mPAP. There was no significant improvement of PH between those patients treated with drugs and oxygen and those on oxygen therapy alone. PH is common in patients with IPF. There is an inverse relationship between lung function measures and PH and a direct one with radiological and echocardiographic findings. The short-term use of oxygen therapy with or without drugs had no effect on PH in patients with IPF. Egypt J Broncho 2014 8:23–31

Highlights

Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonias, is a chronic, progressive, irreversible, and usually lethal lung disease of unknown cause with an estimated survival of 2.5–5 years
Patients and methods pulmonary function test (PFT), the composite physiologic index, multidetector chest computed tomography, and echocardiography were performed on 60 IPF patients (10 men and women, mean age years)
Pulmonary hypertension (PH) is a hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure to at least 25 mmHg at rest as assessed by right heart catheterization (RHC) [5,6]

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonias, is a chronic, progressive, irreversible, and usually lethal lung disease of unknown cause with an estimated survival of 2.5–5 years. Pulmonary hypertension (PH) is a hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure (mPAP) to at least 25 mmHg at rest as assessed by right heart catheterization (RHC) [5,6]. Pulmonary artery pressures (PAPs) and other cardiac hemodynamic parameters can be accurately assessed by RHC, a simple, reliable, and noninvasive method to estimate PAPs and diagnosis of PH in patients with advanced lung disease is preferable. Pulmonary hypertension (PH) is a common finding in patients with idiopathic pulmonary fibrosis (IPF) and is associated with increased morbidity and mortality

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