ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

• Published in: The European respiratory journal Vol. 39; no. 4; pp. 945 - 955
• Main Authors: HURDMAN, J, CONDLIFFE, R, BILLINGS, C, LAWRIE, A, SABROE, I, AKIL, M, O'TOOLE, L, KIELY, D. G, ELLIOT, C. A, DAVIES, C, HILL, C, WILD, J. M, CAPENER, D, SEPHTON, P, HAMILTON, N, ARMSTRONG, I. J
• Format: Journal Article
• Language: English
• Published: Leeds Maney 01-04-2012
• Subjects: Adult; Aged; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Diagnosis-Related Groups - classification; Diagnosis-Related Groups - statistics & numerical data; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Endarterectomy - mortality; Female; Follow-Up Studies; Heart; Heart Defects, Congenital - classification; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - mortality; Humans; Hypertension, Pulmonary - classification; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - mortality; Hypertension, Pulmonary - surgery; Male; Medical sciences; Middle Aged; Multivariate Analysis; Pneumology; Prognosis; Pulmonary Disease, Chronic Obstructive - classification; Pulmonary Disease, Chronic Obstructive - diagnosis; Pulmonary Disease, Chronic Obstructive - mortality; Pulmonary Disease, Chronic Obstructive - surgery; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Referral and Consultation - statistics & numerical data; Registries - statistics & numerical data; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Severity of Illness Index; Sleep Wake Disorders - classification; Sleep Wake Disorders - diagnosis; Sleep Wake Disorders - mortality; Survival Analysis; Thromboembolism - classification; Thromboembolism - diagnosis; Thromboembolism - mortality; Immunopathology; Connective tissue disease; registry; Skin disease; Prognosis; Respiratory disease; Autoimmune disease; Cardiovascular disease; Congenital heart disease; Survival; Thrombosis; Embolism; Pulmonary hypertension; Vascular disease; systemic sclerosis; Systemic disease; Congenital cardiopathy; Scleroderma; Thromboembolism; Pneumology
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/09031936.00078411

Pulmonary hypertension (PH) is a heterogeneous condition. To date, no registry data exists reflecting the spectrum of disease across the five diagnostic groups encountered in a specialist referral centre. Data was retrieved for consecutive, treatment-naïve cases diagnosed between 2001 and 2010 using a catheter-based approach. 1,344 patients were enrolled, with a mean follow-up of 2.9 yrs. The 3-yr survival was 68% for pulmonary arterial hypertension (PAH), 73% for PH associated with left heart disease, 44% for PH associated with lung disease (PH-lung), 71% for chronic thromboembolic PH (CTEPH) and 59% for miscellaneous PH. 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 associated with systemic sclerosis (p<0.005). Within PH-lung, 3-yr survival in sleep disorders/alveolar hypoventilation (90%) was superior to PH-lung with chronic obstructive pulmonary disease (41%) and interstitial lung disease (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 differed between and within PH groups. The current system of classification of PH has prognostic value even when adjusted for age and disease severity, emphasising the importance of systematic evaluation and precise classification.