1	Sildenafil Improves Exercise Ability and Hemodynamics in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease N Hill¹, G Burgess ², D Badesch³, on behalf of the SUPER-1 Study Group
2	Pulmonary arterial hypertension (PAH) and connective tissue disease (CTD) PAH is a common and serious complication of CTD¹ Pathogenesis may involve autoimmune processes, endothelial dysfunction and interstitial lung disease Left ventricular diastolic dysfunction and thromboembolic disease may also contribute Imparts a poor prognosis in CTD; is progressive and particularly difficult to manage²
3	Sildenafil in PAH Sildenafil citrate inhibits phosphodiesterase type 5¹ Promotes the accumulation of intracellular cGMP Enhances nitric oxide-mediated vasodilatation² Reduces smooth muscle cell proliferation³ Sildenafil improves exercise capacity and hemodynamics in patients with PAH after 12 weeks of treatment⁴
4	Sildenafil in PAH associated with CTD
5	SUPER-1 study: Sildenafil Use in Pulmonary Arterial HypERtension
6	CTD subgroup: Patient disposition
7	CTD subgroup: Baseline characteristics
8	CTD subgroup: 6MWD change from baseline to Wk 12
9	CTD subgroup: WHO FC improvement from baseline to Wk 12
10	CTD subgroup: Hemodynamic changes from baseline to Wk 12
11	CTD subgroup: Tolerability
12	Conclusions (with appropriate caveats) In patients with PAH associated with CTD of different etiologies: 12 weeks of oral sildenafil treatment é exercise capacity (at 20 mg and 40 mg tid) é Functional Class (≥1 category in 29-42% of pts) ê mPAP and PVR (at 20 mg tid) ? Trend toward ê efficacy at highest dose ? Sildenafil was generally well tolerated Long-term prospective studies are warranted
13	"Back-up slides" Back-up slides
14	SUPER Study Group Australia: Prof Anne Keogh and Asst Prof Trevor J Williams; Belgium: Prof Marion Delcroix and Prof Robert Naeije; Brazil: Dr Antonio Augusto Barbosa Lopes; Canada: Dr David Langleben and Dr Sanjay Mehta; Czech Republic: Dr Hikmet Al-hiti; Denmark: Dr Jorn Carlsen; France: Prof Gerald Simonneau; Germany: Dr Ardeschir Ghofrani, Dr Marius Hoeper, Prof Joachim Schauer, and Dr Ingram Schulzeneick; Hong Kong: Dr Cheuk Man Yu; Hungary: Dr Dezso Apro and Dr Kristof Karlocai; Ireland: Dr Sean P Gaine; Israel: Dr Issahar Ben-dov and Prof Mordechai R Kramer; Italy: Dr Nazzareno Galiè and Dr Carlo Marini; The Republic of Korea: Dr Byung-il W Choi; Malaysia: Prof Chim Choy Lang; Mexico: Dr Tomas Pulido; The Netherlands: Dr Anko Boonstra; Norway: Dr Arne K Andreassen; Philippines: Dr Romeo A Divinagracia; Poland: Prof Adam Torbicki and Prof Jan Wodniecki; Singapore: Dr Soo Teik Lim; South Africa: Prof Gillian M Ainslie, Dr Claudia Ickinger, Dr Shirley J Middlemost, and Dr Helmuth Reuter; Spain: Dr Joan A Barbera, Dr Miguel A Gomez, and Dr Antonio Roman; Sweden: Dr Gunnar Martensson; United Kingdom: Dr Paul A Corris, Dr David G Kiely, Dr Andrew J Peacock, Dr Joanna Pepke-zaba, and Ms Teresa Sandinha; United States: Dr David B Badesch, Dr Robyn J Barst, Dr Raymond L Benza, Dr Robert C Bourge, Dr Richard Channick, Dr Teresa S De Marco, Dr Thomas Fleming, Dr Robert P Frantz, Dr Adaani E Frost, Dr Jalal Kamal Ghali, Dr Reda E Girgis, Dr Nicholas S Hill, Dr Vallerie V McLaughlin, Dr Ronald J Oudiz, Dr Stuart Rich, Ivan M Robbins, Dr David J Ross, Dr Melvyn Rubenfire, Dr Lewis J Rubin, Shelley M Shapiro, Dr Victor F Tapson, and Dr Dianne L Zwicke.