Platelet level as a new prognostic factor for idiopathic pulmonary arterial hypertension in the era of combination therapy.

BACKGROUND The recent development of various effective drugs, such as epoprostenol, sildenafil, and bosentan, has improved the prognosis for patients with idiopathic pulmonary arterial hypertension (IPAH). This study sought to determine survival rates and to identify predictive prognostic factors in patients with IPAH in the current era of combination therapy with new and more effective vasodilators. METHODS AND RESULTS In 65 consecutive IPAH patients treated from 2004 to 2009, hemodynamic parameters were significantly improved and brain natriuretic peptide was significantly decreased by combination therapy (observation period: 35±18 months). The Kaplan-Meier survival curves were determined, and 22 prognostic variables, including 9 hemodynamic variables and 6 biomarkers, were evaluated to obtain the best variables. The 1-year and 3-year survival rates were 98% and 86%, respectively. Only the platelet level was correlated with death (P<0.05), and the platelet level was significantly correlated with mean pulmonary arterial pressure (P<0.01). Patients with a lower platelet level (<20×10(4)/µl (median value)) before treatment had a higher mortality rate compared to the other patients (78% vs. 95% for 3-year survival, P<0.01). CONCLUSIONS Combination therapy contributed to an improvement in the prognosis of IPAH patients. Platelet level is a significant prognostic predictor in this new treatment era.

[1]  M. Recht,et al.  Thrombocytopenia caused by immunologic platelet destruction , 2013 .

[2]  G. Rodgers,et al.  Miscellaneous causes of thrombocytopenia , 2013 .

[3]  G. Rodgers Thrombocytopenia: Pathophysiology and classification , 2013 .

[4]  Yoshihiro Fukumoto,et al.  Recent progress in the management of pulmonary hypertension. , 2011, Circulation journal : official journal of the Japanese Circulation Society.

[5]  Christopher S Coffey,et al.  Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) , 2010, Circulation.

[6]  H. Shimokawa,et al.  Identification of new prognostic factors of pulmonary hypertension. , 2010, Circulation journal : official journal of the Japanese Circulation Society.

[7]  M. Humbert,et al.  Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension , 2010, European Respiratory Journal.

[8]  M. Humbert,et al.  Survival in Patients With Idiopathic, Familial, and Anorexigen-Associated Pulmonary Arterial Hypertension in the Modern Management Era , 2010, Circulation.

[9]  Sanjiv J. Shah,et al.  Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation , 2009, European Respiratory Journal.

[10]  H. Ghofrani,et al.  Updated evidence-based treatment algorithm in pulmonary arterial hypertension. , 2009, Journal of the American College of Cardiology.

[11]  J. D. de Lemos,et al.  Hemodynamics and epoprostenol use are associated with thrombocytopenia in pulmonary arterial hypertension. , 2009, Chest.

[12]  G. Simonneau,et al.  Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial , 2008, The Lancet.

[13]  M. Humbert,et al.  Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. , 2006, European heart journal.

[14]  M. Pillinger,et al.  Sildenafil Citrate Therapy for Pulmonary Arterial Hypertension , 2006 .

[15]  R. Barst,et al.  New predictors of outcome in idiopathic pulmonary arterial hypertension. , 2005, The American journal of cardiology.

[16]  小川 愛子 Risk of alveolar hemorrhage in patients with primary pulmonary hypertension : anticoagulation and epoprostenol therapy , 2005 .

[17]  W. Seeger,et al.  Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. , 2004, Journal of the American College of Cardiology.

[18]  A. Shillington,et al.  Survival in Primary Pulmonary Hypertension: The Impact of Epoprostenol Therapy , 2002, Circulation.

[19]  Gilles Garcia,et al.  Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. , 2002, Journal of the American College of Cardiology.

[20]  K. Kangawa,et al.  Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. , 2000, Circulation.

[21]  A. Lopes,et al.  Endothelial cell dysfunction correlates differentially with survival in primary and secondary pulmonary hypertension. , 2000, American heart journal.

[22]  M. Uematsu,et al.  Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. , 1999, American journal of respiratory and critical care medicine.

[23]  Y. Okano,et al.  Prognoses of primary pulmonary hypertension and chronic majorvessel thromboembolic pulmonary hypertension determined from cumulative survival curves. , 1999, Internal medicine.

[24]  A J Tajik,et al.  Doppler echocardiographic index for assessment of global right ventricular function. , 1996, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[25]  B. Groves,et al.  A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. , 1996, The New England journal of medicine.

[26]  R. Barst,et al.  Survival in Primary Pulmonary Hypertension with Long-Term Continuous Intravenous Prostacyclin , 1994, Annals of Internal Medicine.

[27]  J. Sandoval,et al.  Survival in primary pulmonary hypertension. Validation of a prognostic equation. , 1994, Circulation.

[28]  E H Bergofsky,et al.  Survival in Patients with Primary Pulmonary Hypertension: Results from a National Prospective Registry , 1991 .

[29]  N. Reichek,et al.  Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension. , 1989, Circulation.

[30]  N. Browse,et al.  Lymphoedema: pathophysiology and classification. , 1985, The Journal of cardiovascular surgery.

[31]  W. E. Davis Enlargement of Lymph Nodes and Spleen , 1985 .