Pulmoner arteriyel hipertansiyon: tanı ve tedavisi

Year 2009, Volume: 16 Issue: 1, 39 - 47, 30.03.2009

Önder Öztürk Ünal Şahin

Abstract

Süleyman Demirel Üniversitesi
TIP FAKÜLTESİ DERGİSİ: 2009 Mart; 16 (1)


Pulmoner arteriyel hipertansiyon: tanı ve tedavisi

Önder Öztürk, Ünal Şahin


Özet

Pulmoner arteriyel hipertansiyon (PAH), altta yatan, sebebi bilinmeyen nadir bir hastalıktır. Pulmoner arteriyel hipertansiyon bulguları belirgin olmadığından sessiz seyreder. Ancak eforla artan dispne şikayeti olan olgularda şüphe edildiğinde ve nedeni bilinen PH olgularında tanı konulabilmektedir. PH şüphe edilen olgularda, iki boyutlu Doppler ekokardiyografi en yararlı tanı yöntemlerinden biridir. PH varlığında ise oksijenizasyon, akciğer fonksiyon testleri, yüksek rezolüsyonlu bilgisayarlı tomografi, ventilasyon-perfüzyon sintigrafisi ve kardiyak kateterizasyon gibi ileri tetkiklerin yapılması gerekir. Standart veya kombine tedavilerle egzersiz kapasitesinde, yaşam kalitesinde, hemodinamide ve yaşam süresinde iyileşme saptanır. Yeni tedavi yöntemleri ve ajanlarla PAH tedavisinde yüz güldürücü sonuçlar elde edilmektedir. Akciğer transplantasyonu, tıbbi tedaviye cevap vermeyen pulmoner arteriyel hipertansiyonu olan seçilmiş hastalarda bir umut olarak durmaktadır.

Anahtar kelimeler: pulmoner arteriyel hipertansiyon, semptomlar, tanı, tedavi.

Abstract

Pulmonary arterial hypertension: diagnosis and treatment

Pulmonary arterial hypertension (PAH) is a rare disease of unknown etiology. Signs and symptoms of pulmonary arterial hypertension are often subtle and nonspecific. The diagnosis should be suspected in patients with increasing dyspnea on exertion and a known cause of pulmonary hypertension. Two-dimensional echocardiography with Doppler flow studies is the most useful imaging modality in patients with suspected pulmonary hypertension. If pulmonary hypertension is present, further evaluation may include assessment of oxygenation, pulmonary function testing, high-resolution computed tomography of the chest, ventilation-perfusion lung scanning and cardiac catheterization. Treatment with standart or combination therapy improve exercise capacity, quality of life, hemodynamics and long-term survival in patients with PAH. New agents are also under examination with affirmative results. Lung transplantation remains an option for selected patients with pulmonary arterial hypertension that does not respond to medical management.

Key words: pulmonary arterial hypertension, symptoms, diagnosis, treatment.

Keywords

pulmoner arteriyel hipertansiyon, semptomlar, taný, tedavi

References

• Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43 (12 Suppl.):40-7
• Karabýyýkoðlu G. Pulmoner arteriyel hipertansiyon. In: Karabýyýkoðlu G. ed. Pulmoner Hipertansiyon. Ankara, Antýp Basýmevi; 2007;112-30
• Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. Am J Med. 1951;11(6):686–705
• Rubin LJ. American College of Chest Physicians. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guýdelines. Chest 2004;126 (1 Suppl):7-10
• Stewart S. Pulmonary arterial hypertension. Oxon–England, Taylor and Francis Group,. 2005; 4- 45.
• Tanabe N, Kimura A, Amano S, Okada O, Kasahara Y, Tatsumi K, et al. Association of clinical features with HLA in chronic pulmonary tromboembolism. Eur Respir J. 2005;25(1):131-38.
• Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 Suppl):5-12.
• Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, et al. Appetite suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 335(9):609–16.
• D Langleben. The International Primary Pulmonary Hypertension Study Group (IPPHS). Familial primary pulmonary hypertension. Chest 1994;105(2 Suppl):13- 16
• Appelbaum L, Yigla M, Bendayan D, Reichart N, Fink G, Priel I, et al. Primary pulmonary hypertension in Israel: a national survey. Chest 2001;119(6):1801–06
• Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006;173(9):1023–30
• Nichols WC, Koller DL, Slovis B, Foroud T, Terry VH, Arnold ND, et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-q32. Nat Genet. 1997;15(3):277–80
• Morse JH, Jones AC, Barst RJ, Hodge SE, Wilhelmsen KC, Nygaard TG. Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31-q32. Circulation. 1997; 95(12):2603–6
• Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000; 67(3):737–44
• Nauser TD, Stites SW. Diagnosis and Treatment of Pulmonary Hypertension. Am Fam Physician. 2001;63(9):1789-98
• Lee SH. Rubin LJ. Current treatmnet strategies for pulmonary arterial hypertension. J Int Med. 2005;258:199-215
• Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107(2):216–23
• Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL, et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984;70(4):580-7
• Brown JW, Heath D, Whitaker W. Idiopathic pulmonary hypertension. Br Heart J. 1957; 19(1):83-92
• D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. .Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991; 115(5):343-9
• Trow TK, McArdle JR. Diagnosis of pulmonary arterial hypertension. Clin Chest Med. 2007; 28(1): 59–73
• Bull, TM. Physical Examination in Pulmonary Arterial Hypertension. Adv in Pulm Htn. 2005; 4(3) 6-10
• Galiè N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. 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(24):2243-78
• Hoeper MM, Rubin LJ. Update in pulmonary hypertension 2005. Am J Respir Crit Care Med. 2006;173(5): 499-505
• Rubin LJ. Pulmonary arterial hypertension. Proc Am Thorac Soc. 2006;3(1):111-15
• Leuchte HH, Holzapfel M, Baumgartner RA, Neurohr C, Vogeser M, Jürgen Behr J. Characterization of brain natriuretic peptide in long-term follow-up of pulmonary arterial hypertension. Chest. 2005;128(4):2368-74
• Akkoca YÖ. Pulmoner hipertansiyonda solunum fonksiyon testleri, arter kan gazlarý ve egzersiz testleri. In: Karabýyýkoðlu G. ed. Pulmoner Hipertansiyon. Ankara, Antýp Basýmevi, 2007;112-30
• Patrat JF, Jondeau G, Dubourg O, Lacombe P, Rigaud M, Bourdarias JP, et al. Left main coronary artery compression during primary pulmonary hypertension. Chest. 1997;112(3):842–43.
• McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126(1 Suppl):14–34
• Denton CP, Cailes JB, Phillips GD, Wells AU, Black CM, Bois RM. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol. 1997;36(2): 239–43.
• Penning S, Robinson KD, Major CA, Garite TJ. A comparison of echocardiography and pulmonary artery catheterization for evaluation of pulmonary artery pressures in pregnant patients with suspected pulmonary hypertension. Am J Obstet Gynecol. 2001;184(7):1568–70
• Gorcsan J, Edwards TD, Ziady GM, Katz WE, Griffith BP. Transesophageal echocardiography to evaluate patients with severe pulmonary hypertension for lung transplantation. Ann Thorac Surg. 1995;59(3):717–22.
• Wax D, Garofano R, Barst RJ. Effects of long-term infusion of prostacyclin on exercise performance in patients with primary pulmonary hypertension. Chest. 1999;116(4):914–20.
• Karabýyýkoðlu G. Kronik tromboembolik pulmoner hipertansiyon: giriþ ve hemodinami. In Metintaþ M (ed). Pulmoner tromboemboli. Eskiþehir, ASD Yayýnlarý, 2001;253-64
• Kuriyama K, Gamsu G, Stern RG, Cann CE, Hefkens RJ, Brundage BH. CT-determined pulmonary artery diameters in predicting pulmonary hypertension. Invest Radiol. 1984;19(1):16-22
• Remy-Jardin M, Remy J. Spiral CT angiography of the pulmonary circulation 1999;212(3): 615-36.
• Krüger S, Haage P, Hoffmann R, Breuer C, Bücker A, Hanrath P, Günther RW. Diagnosis of pulmonary arterial hypertension and pulmonary embolism with magnetic resonance angiography. 2001;120(5):1556-61
• Oudiz RJ, Langleben D. Cardiac catheterization in pulmonary arterial hypertension: an updated guide to proper use. Adv in Pulm Htn. 2005;4(3):15–25
• Nootens M, Wolfkiel CJ, Chomka EV, Rich S. Understanding right and left ventricular systolic function and interactions at rest and exercise in primary pulmonary hypertension. Am J Cardiol. 1995;75(5): 374–77
• Shapiro BP, Nishimura RA, McGoon MD, Redfield MM. Diagnostic dilemmas: diastolic heart failure causing pulmonary hypertension and pulmonary hypertension causing diastolic dysfunction. Adv in Pulm Htn 2006;5(1):13–20.
• Bressollette E, Dupuis J, Bonan R, Doucet S, Cernacek P, Tardif JC. Intravascular ultrasound assessment of pulmonary vascular disease in patients with pulmonary hypertension. 2001;120(3):809-15
• Bush A, Gray H, Denison DM. Diagnosis of pulmonary hypertension from radiographic estimates of pulmonary arterial size. 1988;43(2):127-31
• Naeije R, Vachiery JL. Medical therapy of pulmonary hypertension: conventional therapies. Clin Chest Med. 2001;22(3):517–27.
• Weissmann N, Tadic A, Hänze J, Rose F, Winterhalder S, Nollen M, et al. Hypoxic vasoconstriction in intact lungs: a role for NADPH oxidase-derived H(2)O(2)? Am J Physiol Lung Cell Mol Physiol. 2000;279(4):683–90
• Alam S, Palevsky HI. Standard therapies for pulmonary arterial hypertension. Clin Chest Med. 2007;28(1):91–115
• Yu L, Quinn DA, Garg HG, Hales CA. Gene expression of cyclin-dependent kinase inhibitors and effect of heparin on their expression in mice with hypoxia- induced pulmonary hypertension. Biochem Biophys Res Commun. 2006;345(4):1565–72
• Hassoun PM, Thompson BT, Steigman D, Hales CA. Effect of heparin and warfarin on chronic hypoxic pulmonary hypertension and vascular remodeling in the guinea pig. Am Rev Respir Dis. 1989; 139(3):763–68
• Garg HG, Hales CA, Yu L, Butler M, Islam T, Xie J, Linhardt RJ, et al. Increase in the growth inhibition of bovine pulmonary artery smooth muscle cells by an O-hexanoyl low-molecular-weight heparin derivative. Carbohydr Res. 2006;341(15):2607–12
• Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996;334(5):296–302
• Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000;132(6):425–34
• Olschewski H, Simonneau G, Galiè N, Higenbottam T, Naeije R, Rubin LJ, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347(5):322–29
• Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo controlled trial. Am J Respir Crit Care Med. 2002; 165(6):800–4.
• Gomberg-Maitland M, Tapson VF, Benza RL, McLaughlin VV, Krichman A, Widlitz AC, et al. Transition from intravenous epoprostenol to intravenous treprostinil in pulmonary hypertension. Am J Respir Crit Care Med.2005;172(12):1586–9
• O’Callaghan D, Gaine SP. Bosentan: a novel agent for the treatment of pulmonary arterial hypertension. Int J Clin Pract. 2004;58(1):69–73.
• Galié N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2005;46(3):529–35.
• Barst RJ, Langleben D, Badesch D, Frost A, Lawrence EC, Shapiro S, et al. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol. 2006;47(10):2049–56
• McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galiè N, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005; 25(2):244–49
• Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;169(4):441–7
• Wharton J, Strange JW, Mİller GM, Growcott EJ, Ren X, Franklyn AP, et al. Antiproliferative effects of phosphodiesterase type 5 inhibition in human pulmonary artery cells. Am J Respir Crit Care Med. 2005;172(1):105–13
• Galiè N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353(20):2148–57