Improved 10-year survival of SSc-PAH in the contemporary treatment - PowerPoint PPT Presentation

Improved 10-year survival of SSc-PAH in the contemporary treatment era: A single centre retrospective study of 451 patients Hossam Fayed Clinical Research Fellow, Royal Free London Honorary Clinical Lecturer, UCL, UK

Background • SSc-PAH has amongst the worst prognosis in Gp 1 PAH. • New treatment strategies have been introduced – Goal-directed therapy in 2006 – Early combination therapy in 2015.

Methods • 451 patients with SSc-PAH diagnosed and treated in the RFH 1997 to 2017 were included. • Divided to 1997-2005 and 2006-2017 groups. • Death confirmed by NHS Spine and Survival confirmed by most recent clinical encounter. • Survival analysis over a 10-year period was performed using Kaplan-Meier analysis.

Results Median (interquartiles) 1997-2005 (n=137) 2006-2017 (n=314) P value Females n. (%) 111 (81%) 273 (86.9%) Age (years) 66 (60-74) 68 (59-74) 0.303 Right atrial pressure(mmHg) 7 (5-10) 7 (5-10) 0.304 Pulmonary wedge pressure mmHg) 10 (8-11) 10 (8-12) 0.003 Mean pulmonary artery pressure 36 (30-48) 40 (31-50) 0.07 (mmHg) Cardiac output (mmHg) 4.3 (3.56-5) 4.5 (3.7-5.4) 0.149 Pulmonary vascular resistance 503.27 (328-800) 509.09 (322-782) 0.942 (dyn·sec·cm −5 )

Results Pre 2006 2006- 17 1 year survival 82% 89% 3 year survival 58% 61% 5 year survival 39% 45%

Conclusion • SSc-PAH survival has significantly improved in the contemporary treatment era. • Improved screening would unlikely explain this as the baseline profile of the more recent patient population was at least as severe as the historical cohort.

Danke! Dr Gerry Coghlan Prof Chris Denton Dr Voon Ong Dr Clive Handler Dr Svetlana Dr Benjamin Schreiber Nihtyanova Dr Phil Marino Dr Dan Knight Tony Piworaski Sally Redcliffe Adele Dawson Jenniffer Halliwell