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

Cairo University (CU)

Status: Unknown

Conditions: Pulmonary Hypertension

Study type: Observational

Funder types: Other

Identifiers

NCT02774928

Cairo2001

Details and patient eligibility

About

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.

Full 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

7. 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

Enrollment: 150 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

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

Exclusion criteria

• Bronchogenic carcinoma

Trial design

150 participants in 3 patient groups

Chronic Obstructive Pulmonary Disease

Description:

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

Description:

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)

Description:

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.