Until recently there was believed that PPH and CTEPH have the same unfavorable prognosis. Newer data show a better outcome for CTEPH. One prospective study from Japan for the period prior to the introduction of prostacyclin treatment investigated the survival of 32 patients with PPH and 48 CTEPH patients. Both groups had equally elevated pulmonary arterial pressures. Mean survival was calculated to be 3.6 years for PPH and 6.8 years for CTEPH. Patients with CTEPH had a longer survival than those with PPH, despite the fact that the age at diagnosis of PPH was younger by 17 years on average than that of CTEPH (1).
Pulmonary thrombendarterectomy ameliorates the prognosis of proximal CTEPH, when performed in specialized centers.
Altman RD; Medsger TA Jr; Bloch DA; Michel BA. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 1991 Apr;34(4):403-13
Brenot F. Primary pulmonary hypertension. Case series from France.
Chest 1994 Feb;105(2 Suppl):29S-32S
Chest 1994 Feb;105(2 Suppl):33S-36S
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
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
Oakley CW. Primary pulmonary hypertension. Case series from the United Kingdom.
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