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.

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

Eligibility Criteria:
Inclusion Criteria:

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

Exclusion Criteria:

* Bronchogenic carcinoma