Abstract
Pulmonary hypertension (PH) is a heterogeneous condition. To date no registry data exists reflecting the spectrum of disease across the 5 diagnostic groups encountered in a specialist referral centre.
Data was retrieved for consecutive, treatment-naïve cases diagnosed during 2001-10 using a catheter-based approach. 1344 patients were enrolled, mean follow-up 2.9years. Three-year survival was 68% for pulmonary arterial hypertension (PAH), 73% for PH-Left heart disease, 44% for PH-Lung disease, 71% for chronic thromboembolic PH (CTEPH) and 59% for PH-Miscellaneous. Compared with PAH, survival was inferior in PH-Lung and superior in CTEPH (p<0.05). Multivariate analysis demonstrated that diagnostic group independently predicted survival. Within PAH, Eisenmenger's survival was superior to idiopathic PAH which was superior to PAH-systemic sclerosis (p<0.005). Within PH-Lung, 3-year survival in sleep disorders/alveolar hypoventilation (90%) was superior to PH-Lung with COPD (41%) and ILD (16%)(p<0.05). In CTEPH, long-term survival was best in patients with surgically accessible disease undergoing pulmonary endarterectomy.
In this large registry of consecutive, treatment-naïve patients identified at a specialist PH centre outcomes and characteristics differ between and within PH groups. The current system of classification of PH has prognostic value even when adjusted for age and disease severity emphasizing the importance of systematic evaluation and precise classification.
- ERS