Impact of functional class on outlook
Pulmonary Arterial Hypertension (PAH) carries a very poor prognosis if left untreated, with a median survival rate equivalent to some common malignancies: 2.8 years (see Figure 1).1-3 Prognosis is influenced by the status of functional class when treatment is started - patients who start therapy in functional class I or II demonstrate a better prognosis than those whose therapy is started in more severe stages (FC III or IV).4
Figure 1. Prognosis for PAH patients compared with other serious diseases
D'Alonzo et al 1991 Ann Intern Med. 115, Kato et al 2001 Cancer. 92, Bjoraker JA et al 1998 Am J Respir Crit Care Med. 157
References
1. D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115:343-349.
2. Kato I, Severson RK and Schwartz AG. Conditional median survival of patients with advanced carcinoma: surveillance, epidemiology, and end results data. Cancer 2001;92:2211-2219.
3. Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157:199-203.
4. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002;40:780-788.
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