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Hong Gu, MD. PhD Department of Pediatric Cardiology Beijing Anzhen Hospital Beijing, China

Outcome of B osentan treatment in patients with pulmonary arterial hypertension associate with congenital heart disease. Hong Gu, MD. PhD Department of Pediatric Cardiology Beijing Anzhen Hospital Beijing, China koko_gu@yahoo.com. Background.

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Hong Gu, MD. PhD Department of Pediatric Cardiology Beijing Anzhen Hospital Beijing, China

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  1. Outcome of Bosentan treatment in patients with pulmonary arterial hypertension associate with congenital heart disease Hong Gu, MD. PhD Department of Pediatric Cardiology Beijing Anzhen Hospital Beijing, China koko_gu@yahoo.com

  2. Background 1. Bosentan is known as a safe and effective vasodilators in patients with idiopathic pulmonary hypertension (IPAH). 2. Evidences of Bosentan used in patients withPAH associated with unrepaired congenital heartdisease are limited.

  3. Purpose Describe the safety and effectivity of Bosentan used in patients with PAH-CHD.

  4. Methods 1. A monocentre, open-label, uncontrolled, observational study was conducted. 2. 32 patients with PAH associated with unrepairedCHD weretreatedwith bosentan asmonotherapy (n=26) or as an add-on to pre-existing oral Sildenafil (n=6), between Janurory 2008and May 2011. 3. The diagnosis was conformed by echocardiogram, and cardiaccatheterizition.

  5. Methods 4. NYHA functional class, 6-minute walkdistance (6MWD) and SPO2 wereassessed beforestarting bosentan treatment and during follow-up. 5. Hemodynamicparameters (mPAP,Qp/Qs, PVRI, and Rp/Rs)wereobtained by cardiac catheterization in all the patients before Bosentan treatment and the follow up cath in some of the patients.

  6. Patient Data Male/Female9/23 Age (years) 13.5±8.2 Adult/Pediatric 9/23 Mean duration of therapy(months)13.3±9.9 （range 2.1-35.9） （range 3-51）

  7. Diagnosis VSD 12 ASD 3 PDA 5 VSD+ASD 4 VSD+PDA 3 ASD+PDA 1 VSD+ASD+PDA 3 TECD+PDA 1

  8. Clinical Response to therapy N Baseline After treatment P SpO2% 32 89±5 91±5 0.002 ﹡ 6MWT(m) 19 452±86 482±71 0.004 ﹡ (age older than 7 years) NYHA-FC I 0 1 0.011 ﹡ II 25 30 III 5 1 IV 2 0

  9. Demographics of patients with twice cardiac catheterization Male/Female 4/10 Age (years) 13.2±9.2（2.1-36.2） Mean duration of therapy(months) 9.3±4.2（5.3-20.5） N=14

  10. Changes of hemodynamic parameters N=14 Before After P mPAP (mmHg) 77±6 77±16 0.832 PVRI (WU·M2) 22.9±10.520.7±11.50.257 Qp/Qs 0.98±0.3 1.3±0.6 0.011﹡ Rp/Rs 0.98±0.29 0.86±0.37 0.198 TPR 28.8±17.4 23.2±12.7 0.09

  11. Side effects 1. No abnormal liver function 2. No systemic hypotension 3. Two patients had hematochezia (fresh blood on the surface).

  12. Side effects No1 (10 years old boy, VSD+PDA, treated with Bosentan and Sidenafil), was diagnosed as ulcerative colitis by colonoscope. No2 (25 years old young lady, huge VSD, treated only with Bosentan) Both of them recovered by reducing the dose of Bosentan, they are all still on Bosentan treatment now.

  13. Conclusion 1. All patients alived during treatment of Bosentan. 2. Bosentan was well tolerated by all patients with PAH related to unrepaired CHD, even in chidren. 2. Bosentan caused significant improvements in 6MWD、 NYHA-FC 、 SpO2. 3. Bosentan significantly increased Qp/Qs, reduced the PVRI, TPR and Rp/Rs. 4. There is a potential for side effect likebleeding.

  14. Thank you very much!

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