Pulmoner arteriyel hipertansiyon hastalarının tedavisinde vazoaktif ajanların etkinliği
Pulmoner arteriyel hipertansiyon (PAH) nadir gözlenen vasküler bir hastalıktır ve pulmoner vasküler rezistans ve pulmoner arter basıncındaki (PAB) ilerleyici artıs neticesinde sağ kalp yetmezliği ve ölüme neden olmaktadır. İdiyopatik PAH, konnektif doku hastalıkları, soldan sağa santlı doğumsal kalp hastalıkları PAH nedenidir PAH tanısı hemodinamik olarak, ortalama PAB’ nın istirahatte 25 mmHg, egzersiz ile 30 mmHg üzerinde olması seklinde tanımlanmaktadır. Ciddi dispnesi olup altta ya- tan akciğer ve kalp hastalığı bulunmayan kisilerde mutlaka PAH akla getirilmelidir Transtorasik ekokardiyograf ve sağ kalp kateterizasyonu tanısal olarak büyük önem tasımaktadır. PAH için güncel tedavi yaklasımları prostanoid analogları, endothelin antagonistleri ve PDE-inhibitörlerini içermektedir. Bu çalısmada farklı etiyolojik nedenlere bağlı PAH gelisen hastalarda vazoaktif ajanlarla tedavinin etkinliğinin değerlendirilmesi amaçlandı.
The effect of vasoactive agents in the treatment of pa-tients with pulmonary arterial hypertension
Pulmonary arterial hypertension (PAH) is a rare vascular disease and is associatedwith progressive increase in pulmonary vascular resistance and pulmonary arterialpressure, which leads to right ventricular failure and death. Idiopathic PAH, connective tissue disease, and congenital heart disease with left to right shunts causePAH. The diagnosis of PAH is established hemodynamically with a mean pulmonaryartery pressure > 25 mmHg at rest and > 30 mmHg in exercise. PAH must be takeninto consideration for patients with complaints of progressive dyspnea even if theyhave no underlying pulmonary or heart disease. Transthoracic echocardiography andright heart catheterization are important tools for diagnosis. The current treatmentapproaches are prostanoid analogues, endothelin antagonists, and PDE-inhibitors.In our report we aimed to evaluate the effect of vasoactive agents in the treatmentof patients with PAH.
___
- 1. Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Hihenbottam T, Olscewski H, Peacock A, Pietra G, Rubin LJ, Simonneau G, Priori SG, Garcia MA, Blanc JJ, Budaj A, Cowie M, Dean V, Deckers J, Burgos EF, Lekakis J, Lindahl B, Mazzotta G, McGregor K, Morais J, Oto A, Smiseth OA, Barbera JA, Gibbs S, Hoeper M, Humbert M, Naeije R, Pepke-Zaba J. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004; 25: 2243-78
- 2. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343-9.
- 3. Friedman R, Mears JG, Barst RJ. Continuous infusion of prosta- cyclin normalizes plasma markers of endothelial cell injury and platelet aggregation in primary pulmonary hypertension. Circulation 1997; 96: 2782-4.
- 4. Lane KB, Machado RD, Pauciulo MW, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genetr 2000; 67: 737-44.
- 5. Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spec- trum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132: 425-34.
- 6. Hoeper MM, Schwarze M, Ehlerding S, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000; 342: 1866-70.
- 7. Budak B, Tütün U, Katırcıoğlu SF. Pulmoner hipertansiyon ve prostasiklin analoglarının yeri. Medical Network 2006; 13: 61-78.
- 8. McLaughlin W, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled Iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006; 174: 1257-1263.
- 9. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903.
- 10. Humbert M, Segal ES, Kiely DG, Carlsen J, Schwierin B, Hoeper MM. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J 2007; 30: 338-344.
- 11. Hanson KA, Burns F, Rybalkin SD, et al. Developmental changes in lung c-GMP phosphodiesterase-5 activity, protein, and message. Am J Respis Crit Care Med 1998; 158: 279-88.
- 12. Sayın T, Özenci M. Sustained long-term beneft of sildenafl in primary pulmonary hypertension. Ankara Üniversitesi Tıp Fakültesi Mecmuası 2006; 59: 23-25.
- 13. Bhatia S, Frantz RP, Severson CJ, et al. Immediate and long-term hemodynamic and clinical effects of sildenafl in patients with pulmonary arterial hypertension receiving vasodilator therapy. Mayo Clin Proc 2003; 78: 1207-13.
- 14. Keleş T, Aydoğdu S, Polat K, Durmaz T, Gürsel K, Şahin D, Can- bay A, Diker E. Primer pulmoner hipertansiyonda kısa süreli oral sildenafl tedavisinin etkinliği. Türk Kardiyoloji Derneği Arşivi 2003; 31: 82-87.
- 15. Kılıç H, Tokgözoğlu L. Beş yıl prostasiklin infüzyonu alan primer pulmoner hipertansiyonlu hastanın klinik izlemi ve primer pulmoner hipertansiyon tedavisinde gelişmeler. Türk Kardiyoloji Derneği Arşivi 2003; 31: 105-112.
- 16. Kayıkçıoğlu M, Can LH, Payzin S, Kültürsay H, Soydan İ. Primer pulmoner hipertansiyonlu bir olguda kombine sildenafl ve epoprostenol kullanımı. Anadolu Kardiyoloji Dergisi, 2002; 2: 262-264.
- 17. McLaughlin VV, Oudiz RJ, Frost A, Tapson VF, Murali S, Channick RN, Badesch DB, Barst RJ, Hsu HH, Rubin LJ. Randomized study of adding inhaled Iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006; 174: 1257-1263.
- 18. Steiner MK, Preston IR, Klinger JR, Criner GJ, Waxman ab, Farber HW, Hill NS. Conversion to Bosentan from Prostacyclin Infusion Therapy in Pulmonary Arterial Hypertension: A Pilot Study. Chest 2006; 130: 1471-1480.
- 19. Mathai SC, Girgis RE, Fisher MR, Champion HC, Housten-Harris T, Zaiman A, Hassoun PM. Addition of sildenafl to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 2007; 29: 469-475.
- 20. Önen ZP, Yıldız Ö, Akkoca G, Eriş B, Karabıyıkoğlu G. Inhaled iloprost as a long-term additional therapy to oral sildenafl in severe idiopathic pulmonary arterial hypertension. Tüberküloz ve Toraks 2006; 54: 177-181.
- 21. Diller GP, Dimopoulos K, Kaya MG, Harnies C, Uebing A, Li W, Koltside E, Gibbs SR, Gatzoulis MA. Long-term safety, tolerability and effcacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart 2007; 93: 974-6.
- 22. D’Alto M, Vizza CD, Romeo E, Badagliacca R, Santoro G, Poscia R, Sarubbi B, Mancone M, Argiento P, Ferrante F, Russo MG, Fedele F, Calabrio R. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisen-
- menger physiology): safety, tolerability, clinical, and haemodynamic effect. Heart 2007; 93: 621-625.
- 23. Apostolopoulou SC, Manginas A, Cokkinos DV, Rammos S. Long-term oral bosentan treatment in patients with pulmonary arterial hy- pertension related to congenital heart disease: a 2-year study. Heart 2007; 93: 350-354.
- 24. Hoeper MM, Seyfarth HJ, Hoeffken G, Wirtz H, Spiekerkoetter E, Pletz MW, Welte T, Halank M. Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 2007; 30: 1096-102.
- 25. Özdemir S. Kronik karaciğer hastalığı ve pulmoner komplikasyon- lar. İstanbul Üniversitesi İstanbul Tıp Fakültesi Dergisi 2006; 69: 126-130.
- 26. Kadakal F, Silahtaroğlu P, Soysal F, Aras G, Çetinkaya E, Bayram NG, Yılmaz V. Konnektif doku hastalıklarında pulmoner tutulum (3 olgu nedeni ile). İstanbul Üniversitesi İstanbul Tıp Fakültesi Mecmuası 1998; 61: 248-251.
- 27. Galie N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, placebocontrolled study. Circulation 2006; 114: 48-54.
- 28. Asherson RA, Higenbottam TW, Dinh Xuan AT, Khamasta MA, Hughes GR. Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. J Rheumatol 1990; 17: 1292-8.
- 29. Chung SM, Lee CK, Lee EY, Yoo B, Lee SDL, Moon HB. Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol 2006; 25: 866-872.
- 30. Heresi GA, Minai OA. Lupus-associated pulmonary hypertension: Long-term response to vasoactive therapy. Respiratory Medicine. 2007; 101: 2099-107.
- 31. Mok MY, Tsang PL, Lam YM, Lo Y, Wong WS, Lau CS. Bosentan use in systemic lupus erythematosus patients with pulmonary arterial hypertension. Lupus 2007; 16: 279-285.