Prognosis

Primary pulmonary hypertension is a progressive and usually fatal disorder.

The estimated median survival in primary arterial pulmonary hypertension without specific treatment is 2.8 years from diagnosis. 1-year, 3-year, and 5-year survival rates are 68%, 48%, and 34%, respectively (1).

The terminal event is often a fatal arrhythmia in a patient with a low cardiac output.

Poor prognosis is associated with: (2,3)

• syncope
• male sex
• older age
• renal insufficiency
• New York Heart Association (NYHA) functional class IV
• echocardiography showing pericardial effusion and/or tricuspid annular plane systolic excursion <1.5 cm
• elevated B-type natriuretic peptide (BNP) levels (>180 nanograms/L or >180 picograms/mL)
• invasive haemodynamics showing right atrial pressure >15 mmHg and/or cardiac index ≤2 L/minute/m²

References:

1. D'Alonzo GE et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991 Sep 1;115(5):343-9.
2. Benza RL et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010 Jul 13;122(2):164-72.
3. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010 Jul 13;122(2):156-63.