Clinical Profile of Patients With Pulmonary Hypertension Due to Lung Diseases ( Single Center Experience)

Pulmonary Hypertension Clinical Trial

Official title: Clinical Profile of Patients With Pulmonary Hypertension Due to Lung Diseases ( Single Center Experience)

Status Recruiting

Clinical Trial Summary

Development of pulmonary hypertension (PH) in chronic lung diseases has both functional and prognostic implications .

PH in Lung diseases is usually mild to moderate with preserved cardiac output, and evolves slowly alongside the progression of lung disease and hypoxemia .

However, a minority of patients develop severe PH with elevations in pulmonary artery pressure that have been described as ''out of proportion'' to the underlying disease .

The aim of this study is to compare the characteristics and outcomes of consecutive patients with PH-due to lung diseases diagnosed at our specialist referral center over a 1-year period.

Clinical Trial Description

Development of pulmonary hypertension (PH) in chronic lung diseases has both functional and prognostic implications .

PH in Lung diseases is usually mild to moderate with preserved cardiac output, and evolves slowly alongside the progression of lung disease and hypoxemia .

However, a minority of patients develop severe PH with elevations in pulmonary artery pressure that have been described as ''out of proportion'' to the underlying disease .

Hypotheses for the etiology of this phenotype include greater susceptibility to alveolar hypoxia and/or tobacco smoke , destruction of the capillary vascular bed , inflammatory factors initiating remodeling of the pulmonary vascular bed or the coexistence of idiopathic pulmonary arterial hypertension (IPAH) in patients with lung disease .

Severe PH-lung disease has been arbitrarily defined by a resting mean pulmonary artery pressure (PAP) ≥35 mmHg by RHC . The high and increasing prevalence of chronic lung diseases and the substantial consequences of developing severe PH have generated increasing interest in PH-lung diseases.

Pulmonary vascular research unit at Chest department at Kasr-Alani school of Medicine delivers regional, adult PH service to a population with a chronic lung disease.

The aim of this study is to compare the characteristics and outcomes of consecutive patients with PH-due to lung diseases diagnosed at our specialist referral center over a 1-year period.

Patients diagnosed as chronic lung diseases( COPD,ILD,OSA,Sarcoidosis) will be recruited from out-patient's clinics & inpatients wards of Internal medicine & Chest departments, Kasr Al-ainy hospital (from June 2016 to May 2015) A written informed consent matching with Helsinki declaration will be taken from all patients.

2.2 Study methods:

All the enrolled patients will go through the following basically:

1. Detailed standard Demographic & clinical parameters.

2. Assessment of exercise tolerance (6 MWD & WHO functional capacity)

3. Assessment of pulmonary function by PFTs.

4. ECG: (Right ventricular strain,Right axis deviation,Right bundle branch block,& Left side changes 0

5. Echocardiographic evaluation for PAH: ( Right atrial area ,Right ventricular area ,Tricuspid regurgitant jet velocity (TR) ,Tricuspid annular plane systolic excursion)

6. Radiography: including chest X-ray, HRCT chest, V/Q scan& CT angiography if needed

10. RT sided heart catheterization:

• mPAP

• PCWP

• PVR

• CARDIAC INDEX 11. Statistical analysis: ……..

The subjects will be classified according to RHC into either

• Non PAH groups mPAP ≤ 21mmHG

• PAH mPAP≥ 25 mmHG:

• Severe PH-lung diseases mPAP ≥ 35 mmgh ;

Study Design

Observational Model: Cohort, Time Perspective: Prospective

Related Conditions & MeSH terms

• Hypertension
• Hypertension, Pulmonary
• Lung Diseases
• Pulmonary Hypertension

• NCT number: NCT02774928
• Study type: Observational [Patient Registry]
• Source: Cairo University
• Contact: Mostafa I Elshazly, MD
• Phone: 00201001272020
• Email: elshazly66@hotmail.com
• Status: Recruiting
• Phase: N/A
• Start date: June 2016
• Completion date: May 2017