Q: Anticoagulation is indicated in patients in which group of Pulmonary Hypertension (PH)? 

A) Group 2
B) Group 3
C) Group 4
D) Group 5
E) all patients with PH irrespective of cause


Answer: C (Group 4)

Group 1 patients receive anticoagulation on case to case basis per clinical judgement.

The World Health Organization (WHO) classifies patients with PH into five groups based upon etiology - and may carry different management plan due to underlying cause of the PH. 

 Group 1 — Patients with group 1 pulmonary arterial hypertension (PAH). It was called idiopathic pulmonary arterial hypertension (IPAH) or primary pulmonary hypertension. It may be hereditary or due to diseases that localize to small pulmonary arterioles, such as connective tissue diseases, HIV, portal hypertension, congenital heart disease, schistosomiasis, and possible drug use. 

 Group 2 — Patients with group 2 have PH secondary to left heart disease including valvular heart disease. This requires underlying cardiac disease management. 

 Group 3 — Patients with group 3 have PH secondary to diseases causing hypoxemia, such as COPD, ILD, sleep-disorders and others. Supplemental oxygen may be a mainstay of treatment. 

 Group 4 — Patients with group 4 PH have due to thromboembolic occlusion of the proximal or distal pulmonary vasculature. In this group anticoagulation is primary medical therapy, at least in earlier stage. 

 Group 5 — Group 5 PH includes with multi-factorial mechanisms like hematologic disorders (eg, myeloproliferative disorders and chronic hemolytic anemia), systemic disorders (eg, sarcoidosis), metabolic disorders (eg, glycogen storage disease), and others. 


References: 

 1. Olsson KM, Delcroix M, Ghofrani HA, et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014; 129:57. 

 2. Preston IR, Roberts KE, Miller DP, et al. Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL). Circulation 2015; 132:2403.