Treatment Strategies of Pulmonary Arterial Hypertension Associated with Congenital Heart Defects with Shunt: A Group of Important Intensive Care Patient in Cardiology and Cardiovascular Surgery

Öz

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Doğumsal Kardiyak Şantlarla Bağlantılı Pulmoner Arteriyel Hipertansiyonda Tedavi Yaklaşımları: Kardiyoloji ve Kalp-Damar Cerrahisinde Önemli Bir Yoğun Bakım Hasta Grubu

Öz

hipertansiyonda kombinasyon tedavisiyle ilgili yapılan ça- lışmalardan elde edilen veriler çelişkilidir. Bazı çalışmalar- da bosentan tedavisine sildenafi l eklenmesi hemodinamik özellikleri ve egzersiz kapasitesini artırırken; bazı çalışma- larda kombinasyon tedavisi etkisiz bulunmuştur(23). Fakat yine de doğumsal kalp hastalığıyla ilişkili pulmoner arte- riyel hipertansiyon hastalarının, pulmoner arteriyel hiper- tansiyon spesifi k tedaviden fayda gördüğü gösterilmiştir. Ortalama dört yıl takip edilen toplam 229 Eisenmenger sendromlu hastada yapılan retrospektif bir çalışmada pul- moner arteriyel hipertansiyon spesifi k tedavi gören grupta, tedavi almayan gruba göre daha düşük ölüm oranı saptan- mıştır(24)

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Kaynakça

1. D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hyper- tension. Results from a national prospective registry. Ann Intern Med 1991;115:343-9.
2. Korurek M, Yildiz M, Yüksel A, Şahin A. Simulation of Eisenmen- ger syndrome with ventricular septal defect using equivalent elect- ronic system. Cardiol Young 2012;22:301-6.
3. Yıldız M, Yüksel A, Korürek M, Aykan AÇ, Şahin Yıldız B, Şa- hin A, et al. Classifi cation of Aatrial septal defect and ventricular septal defect with documented hemodynamic parameters via vardiac catheterization by genetic algorithms and multi-layered artifi cial neural network. Kosuyolu Kalp Derg 2012;15:45-50.
4. Engelfriet PM, Duffels MGJ, Moller T, Boersma E, Tijssen JGP, Thaulow E, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult conge- nital heart disease. Heart 2007;93:682-7.
5. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, et al. Eisenmenger syndrome. Factors relating to dete- rioration and death. Eur Heart J 1998;19:1845-55.
6. Beghetti M, Galie N. Eisenmenger syndrome: a clinical perspecti- ve in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol 2009;53:733-40.
7. Broberg CS, Ujita M, Prasad S, Li W, Rubens M, Bax BE, et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is as- sociated with biventricular dysfunction and decreased pulmonary fl ow velocity. J Am Coll Cardiol 2007;50:634-42.
8. Hoeper MM, Barst RJ, Bourge RC, Feldman J, Frost AE, Galié N, et al. Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulati- on 2013;127:1128-38.

9. Xiong CM, Lu XL, Shan GL, Wu BX, Zhu XY, Wu GH, et al. Oral sil- denafi l therapy for Chinese patients with pulmonary arterial hyper- tension: a multicenter study. J Clin Pharmacol 2012;52:425-31.
10. Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev 2009;18:154-61.
11. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, La- uer A, et al.; Bosentan Randomized Trial of Endothelin Antago- nist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006;114:48- 54.
12. Cacoub P, Dorent R, Nataf P, Carayon A. Endothelin-1 in pulmo- nary hypertension. N Engl J Med 1993;329:1967-8.
13. Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RM, Lauer A, et al.; BREATHE-5 Investigators. Longer-term bosentan the- rapy improves functional capacity in Eisenmenger syndrome: re- sults of the BREATHE-5 open-label extension study. Int J Cardiol 2008;127:27-32.
14. Zuckerman WA, Leaderer D, Rowan CA, Mituniewicz JD, Rosenz- weig EB. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol 2011;107:1381-5.
15. Prasad S, Wilkinson J, Gatzoulis MA. Sildenafi l in primary pulmo- nary hypertension. N Engl J Med 2000;343:1342.
16. Humpl T, Reyes JT, Holtby H, Stephens D, Adatia I. Benefi cial effect of oral sildenafi l therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-la- bel, pilot study. Circulation 2005;111:3274-80.
17. Chau EM, Fan KY, Chow WH. Effects of chronic sildenafi l in pa- tients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol 2007;120:301-5.
18. Mukhopadhyay S, Sharma M, Ramakrishnan S, Yusuf J, Gupta MD, Bhamri N, et al. Phosphodiesterase- 5 inhibitor in Eisen- menger syndrome: a preliminary observational study. Circulation 2006;114:1807-10.
19. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-65.
20. Thomas IC, Glassner-Kolmin C, Gomberg-Maitland M. Long-term effects of continuous prostacyclin therapy in adults with pulmonary hypertension associated with congenital heart disease. Int J Car- diol 2013;168:4117-21.
21. D’Alto M, Romeo E, Argiento P, Sarubbi B, Santoro G, Grimaldi N, et al. Bosentan-sildenafi l association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisen- menger physiology. Int J Cardiol 2012;155:378-82.
22. Iversen K, Jensen AS, Jensen TV, Vejlstrup NG, Sondergaard L. Combination therapy with bosentan and sildenafi l in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J 2010;31:1124-31.
23. Dimopoulos K, Inuzuka R, Goletto S, Giannakoulas G, Swan L, Wort SJ, et al. Improved survival among patients with Eisenmen- ger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 2010;121:20-5.
24. Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev 2009;18:154-61.