Functional Class and Targeted Therapy Are Related to the Survival in Patients with Pulmonary Arterial Hypertension
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Purpose: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. Materials and Methods: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9±14.5 years, 80% of female) were enrolled. Results: Causes were congenital heart diseasein 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6±45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, mediansurvival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). Conclusion: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore,early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.
Abstract
Purpose: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. Materials and Methods: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9±14.5 years, 80% of female) were enrolled. Results: Causes were congenital heart diseasein 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6±45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, mediansurvival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). Conclusion: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore,early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.
참고문헌 (30)
- Farber HW, "Pulmonary arterial hypertension", N Engl J Med, 351, pp.1655~1665 (2004)
- Badesch DB, "Diagnosis and assessment of pulmonary arterial hypertension", J Am Coll Cardiol, 54(1 Suppl), pp.S55~S66 (2009)
- Chin KM, "Pulmonary arterial hypertension", J Am Coll Cardiol, 51, pp.1527~1538 (2008)
- Weir EK, "The acute administration of vasodilators in primary pulmonary hypertension. Experience from the National Institutes of Health Registry on Primary Pulmonary Hypertension", Am Rev Respir Dis, 140, pp.1623~1630 (1989)
- Barst RJ, "A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension", N Engl J Med, 334, pp.296~301 (1996)
- Simonneau G, "Updated clinical classification of pulmonary hypertension", J Am Coll Cardiol, 54(1 Suppl), pp.S43~S54 (2009)
- Sahn DJ, "Recommendations regarding quantitation in M-mode echocardiography: results of a survey of echocardiographic measurements", Circulation, 58, pp.1072~1083 (1978)
- Kircher BJ, "Noninvasive estimation of right atrial pressure from the inspiratory collapse of the inferior vena cava", Am J Cardiol, 66, pp.493~496 (1990)
- Dabestani A, "Evaluation of pulmonary artery pressure and resistance by pulsed Doppler echocardiography", Am J Cardiol, 59, pp.662~668 (1987)
- Opitz CF, "Pulmonary hypertension: hemodynamic evaluation: hemodynamic evaluation - recommendations of the Cologne Consensus Conference 2010", Dtsch Med Wochenschr, 135(Suppl 3), pp.S78~S86 (2010)
- Jing ZC, "Iloprost for pulmonary vasodilator testing in idiopathic pulmonary arterial hypertension", Eur Respir J, 33, pp.1354~1360 (2009)
- D’Alonzo GE, "Survival in patients with primary pulmonary hypertension. Results from a national prospective registry", Ann Intern Med, 115, pp.343~349 (1991)
- Olivari MT, "Primary pulmonary hypertension", Am J Med Sci, 302, pp.185~198 (1991)
- Sitbon O, "Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival", J Am Coll Cardiol, 40, pp.780~788 (2002)
- McLaughlin VV, "Survival in primary pulmonary hypertension: the impact of epoprostenol therapy", Circulation, 106, pp.1477~1482 (2002)
- Humbert M, "Treatment of pulmonary arterial hypertension", N Engl J Med, 351, pp.1425~1436 (2004)
- Barst RJ, "Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin", Ann Intern Med, 121, pp.409~415 (1994)
- Simonneau G, "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, 165, pp.800~804 (2002)
- Olschewski H, "Inhaled iloprost for severe pulmonary hypertension", N Engl J Med, 347, pp.322~329 (2002)
- McLaughlin VV, "Pulmonary arterial hypertension", Circulation, 114, pp.1417~1431 (2006)
- Galiè N, "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, 25, pp.2243~2278 (2004)
- 손대원, "Beneficial and Adverse Effects of Bosentan Treatment in Korean Patients With Pulmonary Artery Hypertension", Korean Circulation Journal, 39(3), pp.105~110 (2009), 대한심장학회
- 정욱진, "폐고혈압의 치료", 대한내과학회지, 78(1), pp.28~35 (2010), 대한내과학회
- Galiè N, "Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT)", Eur Heart J, 30, pp.2493~2537 (2009)
- Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC), "Guidelines for the diagnosis and treatment of pulmonary hypertension", Eur Respir J, 34, pp.1219~1263 (2009)
- Kemp K, "Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: an observational study", J Heart Lung Transplant, 31, pp.150~158 (2012)
- Fuster V, "Primary pulmonary hypertension: natural history and the importance of thrombosis", Circulation, 70, pp.580~587 (1984)
- 이우석, "중증 폐동맥 고혈압 환자의 임상적 특징과 예후 예측 인자", Korean Circulation Journal, 37(6), pp.265~270 (2007), 대한심장학회
- Rich S, "Primary pulmonary hypertension. A national prospective study", Ann Intern Med, 107, pp.216~223 (1987)
- 이원동, "일차성 폐동맥 고혈압에 대한 임상적 고찰", Korean Circulation Journal, 33(6), pp.507~512 (2003), 대한심장학회