Eriş Banu GÜLBAY, Zeynep Pınar ÖNEN, Gülseren KARABIYIKOĞLU, Akkoca Öznur YILDIZ

Inhaled iloprost as a long-term additional therapy to oral sildenafil in severe idiopathic pulmonary arterial hypertension

İdiyopatik pulmoner arteryel hipertansiyon (İPAH) oldukça nadir görülen ve tedavi edilmediğinde hızlı bir seyirle sağ kalp yetmezliği ve ölüme kadar giden morbidite ve mortalitesi yüksek bir hastalıktır. Son yıllarda tedavi alanındaki gelişmelerle hastalığın seyri değiştirilebilmektedir. Ancak alternatif tedavi protokollerinin uzun dönem sonuçları ve hastaların bu tedavilere olan yanıtları ile ilgili yeterli veri bulunmamaktadır. Daha önce geçici olarak konvansiyonel tedavi ve oral kalsiyum blokerine yanıt veren İPAH olgusunda sildenafil monoterapisinin uzun dönem klinik ve fizyolojik etkilerini, inhale sildenafil tedavisi eklenmesi sonrasındaki değişikliklerle karşılaştırdık. İPAH olgularında vazodilatör tedavilere kısa süreli ve geçici yanıtlar verilebilmektedir. Ancak uzun dönem sonuçları ile ilgili yeterli veri olmayıp, mutlaka takip edilmelidirler ve bu hastalarda kombinasyon tedavileri daha etkili olabilmektedir.

Ağır idiyopatik pulmoner arteryel hipertansiyonda oral sildenafil tedavisine eklenen inhale iloprost tedavisinin uzun dönem sonuçları

Idiopathic pulmonary arterial hypertension (IPAH) is an uncommon and devastating disease which, if untreated, progresses rapidly and leads to right heart failure and death. The course of the disease has been altered by advances in medical therapies. However, the effects of long-term alternative therapies and responses to each treatment protocols are not definite. We want to define an IPAH case, which had long-term temporary responses to the conventional therapy plus calcium channel blockers treatment and moreover compared the long-term clinical and physiologic effects of oral sildenafil mono therapy and additional inhaled iloprost therapy. Patients with IPAH may have response to a short-term vasodilatation therapy but they have to follow for the long-term results and may be of benefit from combination treatments.

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1.Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107: 216-23.
2.Simonneau G, Nazzareno G, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43 (Suppl S): 5-12.
3.McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension. Chest 2004; 126: 78-92.
4.Fuster V, Steele PM, Edwards WD, et al. Primary pulmonary hypertension: Natural history and the importance of thrombosis. Circulation 1984; 70: 580-7.
5.Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blocker on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 76-81.
6. Rich S, Seidlitz M, Dodin E, et al. The short term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998; 114: 787-92.
7. Wilkens H, Guth A, König J, et al. Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation 2001; 104: 1218-22.
8. Beavo JA. Cyclic nucleotide phosphodiesterases: Functional implications of multiple isoforms. Physiol Rev 1995; 75: 725-48.
9. Ahn HS, Foster M, Cable M, et al. Ca/CaM-stimulated and cGMP-specific phosphodiesterases in vascular and nonvascular tissues. Adv Exp Med Biol 1991; 308: 191-7.
10. Bogdan M, Humbert M, Francoul J, et al. Urinary cGMP concentrations in severe primary pulmonary hypertension. Thorax 1998; 53: 1059-62.
11. Olschewski H, Ghofrani HA, Schemel T, et al. Inhaled iloprost to treat severe pulmonary hypertension. Ann Intern Med 2000; 132: 435-43.
12. Sulica R, Poon M. Current medical treatment of pulmonary arterial hypertension. Mt Sinai J Med 2004;71:103-14.
13. Ogata M, Ohe M, Shirato K, et al. Effects of a combination therapy of anticoagulant and vasodilator on the longterm prognosis of primary pulmonary hypertension. Jpn Circ J 1993; 57: 63-9.
14. Bharani A, Mathew V, Sahu A, et al. The efficacy and tolerability of sildenafil in patients with moderate-to-severe pulmonary hypertension. Indian Heart J 2003; 55: 55-9.
15. Ghofrani HA, Rose F, Schermuly RT, et al. Oral sildenafil as a long-term adjunct therapy to inhaled iloprost in severe pulmonary arterial hypertension. J Am Coll Cardiol 2003; 42: 158-64.
16. Ghofrani HA, Wiedemann R, Rose F, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002; 136:515-22.
17.Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000; 161: 487-92.
18. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assesment of pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43 (Suppl S): 40-47.