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

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.

Study Overview

• Status: Unknown
• Conditions: Pulmonary Hypertension

Detailed 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 Type: Observational
• Enrollment (Anticipated): 150

Contacts and Locations

Study Locations

• Egypt
  • Cairo, Egypt, 12411
    • Recruiting
    • Kasr Alaini
    • Sub-Investigator: Hebatallah H Assel, MD
    • Sub-Investigator: Amira I Alameldin, MD
    • Sub-Investigator: Eman Kamal, MD
    • Sub-Investigator: Naglaa Bakry, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

patients with chronic lung diseases COPD ILD OSA sarcodosis

Description

Inclusion Criteria:

1. COPD diagnosed according to Gold 2015
2. Interstial lung diseases
3. Obstructive Sleep Apnea
4. sarcoidosis

Exclusion Criteria:

• Bronchogenic carcinoma

Study Plan

How is the study designed?

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort
Chronic Obstructive Pulmonary Disease; Chronic Obstructive Pulmonary Disease (COPD) is a major cause of morbidity and mortality throughout the world.COPD, a common preventable and treatable disease, is characterized by persistent airflow limitation that is usually progressive and associated with an enhanced chronic inflammatory response in the airways and the lung to noxious particles or gases. Exacerbations and comorbidities contribute to the overall severity in individual patients.Spirometry is required to make a clinical diagnosis of COPD; the presence of a post-bronchodilator FEV1/FVC < 0.70 confirms the presence of persistent airflow limitation and thus of COPD .
Interstitial lung diseases; Interstitial lung disease is a general category that includes many different lung conditions. All interstitial lung diseases affect the interstitium, a part of the lungs' anatomic structure.
Obstructive sleep apnea (OSA); Obstructive sleep apnea (OSA) is a sleep disorder that involves cessation or significant decrease in airflow in the presence of breathing effort. It is the most common type of sleep-disordered breathing and is characterized by recurrent episodes of upper airway collapse during sleep. These episodes are associated with recurrent oxyhemoglobin desaturations and arousals from sleep. OSA that is associated with excessive daytime sleepiness is commonly called obstructive sleep apnea syndrome-also referred to as obstructive sleep apnea-hypopnea syndrome.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
prevalence of PH	1 year

Collaborators and Investigators

• Sponsor: Cairo University
• Investigators: Principal Investigator: Mostafa I Elshazly, MD, Kasr Alaini School of Medicine; Study Director: Yusif Amin, MD, Kasr Alaini School of Medicine

Study record dates

Study Major Dates

• Study Start: June 1, 2016
• Primary Completion (ANTICIPATED): May 1, 2017
• Study Completion (ANTICIPATED): May 1, 2017

Study Registration Dates

• First Submitted: May 13, 2016
• First Submitted That Met QC Criteria: May 16, 2016
• First Posted (ESTIMATE): May 17, 2016

Study Record Updates

• Last Update Posted (ESTIMATE): July 12, 2016
• Last Update Submitted That Met QC Criteria: July 11, 2016
• Last Verified: July 1, 2016

More Information

Terms related to this study

• Keywords: COPD; OSA; Pulmonary Hypertension; ILD
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Hypertension; Lung Diseases; Hypertension, Pulmonary
• Other Study ID Numbers: Cairo2001

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: publication at international journal