EFFICACY OF MULTIDISCIPLINARY MANAGEMENT OF PULMONARY HYPERTENSION

Aim. To assess the efficacy of regional system of multidisciplinary care in pulmonary hypertension (PH).

Material and  methods. In a  single-center, pro and  retrospective cohort  study,  2001-2015,   the  epidemiological  characteristics  and yearly mortality from PH were assessed in the created  in late 1990s multiple system of PH management in the Udmurt Republic. According to the specific drug therapy tactics, there were three periods of observation: in 2001-2005 PH patients received, by indication calcium channel blockers and supportive therapy; in 2006-2010  sildenafil was added; from 2011 pulmonary arterial hypertension (PAH) patients also received bosentan, ambrisentan, and in chronic thromboembolic PH —inhaled iloprost.

Rеsults. In the  cardiology  and  rheumatology  clinic of the  Udmurt Republic, a multidisciplinary system of PH management was shaped, with three-stage  individualized care   of  patients,   beginning  from newborn  period  (1st    stage   — pre-hospital,   2nd    — hospital,  3rd    — rehabilitation). Within  the  first 5 years  of follow-up, mortality of PH patients was 15-12% per year, from 2006 to 2010 — 10-7,5% per year, in 2011-2015 — 5,6–3,8% (р2015-2001=0,008).

Conclusion. Interprofessional  approach   to  PH patients  care  in thecircumstances of regional center  and  therapy  by PAH-specific drugs significantly increased  survival rate of patients.

1. Galie N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46: 903-75.

2. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Thorax 2008; 63(Suppl 2): ii1-41.

3. Vachiery JL, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev 2012; 21: 313-20.

4. Grunig E, Lichtblau M, Ehlken N, et al. Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 2012; 40: 84-92.

5. Gaisin IR, Valeeva RM, Shilina LV, et al. Effectiveness of specialized management for pregnant women with cardiovascular pathology in the Udmurt Republic. Practical medicine 2015; 2(3): 67-73. Russian (Гайсин И. Р., Валеева Р. М., Шилина Л. В. и др. Эффективность специализированной помощи беременным с кардиова-скулярной патологией в Удмуртской Республике. Практическая медицина 2015; 2(3): 67-73).

6. Gaisin IR, Ezhov AV, Maximov NI, et al. Pulmonary hypertension: work-book. Ed. Gaisin IR. Izhevsk 2015: 52 p. Russian (Гайсин И. Р., Ежов А. В., Максимов Н. И. и др. Легочная гипертензия: учебное пособие. Под ред. И. Р. Гайсина. Ижевск 2015: 52 с).

7. Galie N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Card 2013; 62(25), Suppl.: D60-72.

8. Wang R-C, Jiang F-M, Zheng Q-L, et al. Efficacy and safety of sildenafil treatment in pulmonary arterial hypertension: a systematic review. Respir Med 2014; 108: 531-7.

9. Leuchte HH, Schwaiblmair M, Baumgartner RA, et al. Hemodynamic response to sildenafil, nitric oxide, and iloprost in primary pulmonary hypertension. Chest 2004; 125: 580-6.

10. Guha M. First-in-class guanylate cyclase stimulator approved for PAH. Nature biotechnology 2013; 31(12): 1064.

11. Abman SH, Hansmann G, Archer SL, et al. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation 2015; 132: 1-63.

12. Barst RJ, Ivy DD, Gaitan G, et al. A randomized, double-blind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension. Circulation 2012; 125: 324-34. doi:10.1161/CIRCULATIONAHA.110.016667.

13. Abman SH, Kinsella JP, Rosenzweig EB, et al.; Pediatric Pulmonary Hypertension Network (PPHNet). Implications of the U. S. Food and Drug Administration warning 90 against the use of sildenafil for the treatment of pediatric pulmonary hypertension. Am J Respir Crit Care Med. 2013; 187: 572-5. doi: 10.1164/rccm.201210-1928PP.

14. Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol 2012; 33: 683-8.

15. Jing ZC, Yu ZX, Shen Д et al.; Efficacy and Safety of Vardenafil in the Treatment of Pulmonary Arterial Hypertension (EVALUATION) Study Group. Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med 2011; 183: 1723-9. doi: 10.1164/rccm.201101-0093OC.

16. Stasch J-P, Pacher P, Evgenov OV. Soluble guanylate cyclase as an emerging therapeutic target in cardiopulmonary disease. Circulation 2011; 123(20): 2263-73. doi:10.1161/CIRCULATIONAHA.110.981738.

17. Ghofrani HA, Galie N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369: 330-40.

18. Hoeper MM, Markevych I, Spiekerkoetter E, et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26: 85863. doi:10.1183/09031936.05.00075305.

19. Galie N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J 2010; 31: 2080-6.

20. Galie N, Barbera JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015; 373: 834-44.

21. Beghetti M, Haworth SG, Bonnet D, et al. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: the FUTURE-1 study. Br J Clin Pharmacol 2009; 68: 948-55. doi:10.1111/j.1365-2125.2009.03532.x.

22. Gatfield J, Mueller Grandjean C, Sasse T, et al. Slow receptor dissociation kinetics differentiates macitentan from other endothelin receptor antagonists in pulmonary arterial smooth muscle cells. PLoS One 2012; 7(10): e47662.

23. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369(9): 809-18.

24. Channick RN, Delcroix M, Galie N, et al. The effect of macitentan on long-term outcomes in patients with pulmonary arterial hypertension by WHO functional class: data from the randomized controlled SERAPHIN study. Am J Respir Crit Care Med 2014; 189: A4783.

25. Simonneau G, Torbicki A, Hoeper MM, et al. Selexipag, an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012; 40: 874-80.

26. Sitbon O, Channick R, Chin KM, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 2015; 373(26): 2522-33.