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 . 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