Assessment of Prognostic Factors and Natural History of Idiopathic Pulmonary Arterial Hypertension in Eastern India

• Published in: Journal of the Association of Physicians of India Vol. 67; no. 10; pp. 57 - 61
• Main Authors: Vinayak, Manish, Sharma, Anupam, Sinha, Dhurjati Prasad
• Format: Journal Article
• Language: English
• Published: India 01-10-2019
• Subjects: Familial Primary Pulmonary Hypertension - diagnosis; Humans; Hypertension, Pulmonary; India; Prognosis; Ventricular Function, Right; India
• ISSN: 0004-5772

Idiopathic pulmonary arterial hypertension (IPAH) is rare disorder of unknown aetiology associated with poor survival. Disease severity assessment by various prognostic factors play important role in management of these patients. The aim of our study was to assess various factors and their natural history and course of disease in Indian population.
We followed 27 patients of IPAH after complete work up of exclusion of other causes of pulmonary hypertension and analysed various demographic, echocardiographic and haemodynamic parameters and their correlation with mortality.
A total of 27 patients (14 new and 13 previously diagnosed) were followed for mean duration of 18 months. At time of data analysis, 11 patients were alive and 16 patients died with overall mortality rate of 59.25%. Among various factors, presence of pericardial effusion (p=0.005), pulmonary artery acceleration time (PAAT) (p = 0.005), tricuspid Annular Plane Systolic Excursion (TAPSE) (p = 0.0004), heart rate (p=0.031), mean blood pressure (p =0.017), right atrial pressure (p=0.045), mean pulmonary artery pressure (PAP) (p=0.039) and six minute walk distance (p= 0.0002) were significantly associated with mortality. On multivariate cox proportional hazard analysis, PAAT (p =0.034), TAPSE (p=0.003) and six minute walk distance (p=0.002) remained significant predictors of mortality.
Idiopathic pulmonary arterial hypertension is associated with poor prognosis and survival despite advancements of disease specific therapies. Higher mortality in our study is due to delayed presentation and diagnosis. Also lack of availability of prostacyclins and lung transplantation in advanced stages of disease contribute to higher mortality in Indian setup. Non-invasive echocardiographic factors and six minute walk distance are important prognostic factors that help in disease severity stratification to identify patients in need of intensive medical management.