Prognosis of Pulmonary Hypertension

The prognosis of PPH is variable but generally poor. Patients often are diagnosed with a mean of 2 years delay from the onset of symptoms and present with already severe PH and right ventricular dysfunction / decompensation.

Survival correlates with the severity of pulmonary hypertension and right ventricular dysfunction, NYHA classification, exercise tolerance, hemodynamic parameters and on the response to vasodilator therapy.

Series from the United Kingdom, Japan and France reported a median survival of approximately 3 years from diagnosis (Oakley CW, 1994; Brenot F, 1994; Okada O et al., 1999).

Patients with moderate elevations in pulmonary artery pressure (mean pressure less than 55 mmHg) and preserved right heart function (normal right atrial pressure and cardiac index) had a median survival in  slight excess of 2,5 years in the National Institutes of Health (NIH) Registry (D'Alonzo GE et al., 1991).

Patients with severe pulmonary hypertension or right heart failure tend to die within one year.
Assessing functional status using the New York Heart Association classification can also be useful in determining prognosis and making decisions regarding therapy. Patients who fall into functional classes I and II tend to live far longer than those who fall into classes III and IV.

Since survival in patients who fall into NYHA classes III or IV is poor (9-18 months), patients with severe symptoms and evidence of impaired cardiac function, which is refractory to conventional therapy or epoprostenol infusion should be considered for transplantation.

Alternatively, those patients with mild exertional symptoms, preserved exercise tolerance, and stable cardiac function (NYHA classes I and II) appear to have a 5 year survival more than 50 percent with medical therapy alone.

Pericardial effusion, which corellates with elevated right atrial pressure, is a prognostically unfavorable sign (Hinderliter AL et al.,1999).

Oral anticoagulation and vasodilator therapy have improved the prognosis of PPH. Long-term infusion of epoprostenol may also be beneficial in treating severe secondary pulmonary hypertension due to congenital heart disease, collagen vascular disease, sarcoidosis, distal thromboembolic disease, or portopulmonary hypertension (McLaughlin VV et al., 2000).

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Hinderliter AL et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Am J Cardiol 1999 Aug 15;84(4):481-4, A10

Kunieda T et al. Prognoses of primary pulmonary hypertension and chronic majorvessel thromboembolic pulmonary hypertension determined from cumulative survival curves. Intern Med 1999 Jul;38(7):543-6

McLaughlin VV et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med. 2000 Jan 18;132(2):165

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Okada O et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990. Intern Med 1999 Jan;38(1):12-6