Pulmonary Artery Pressure as a Treatment Target to Improve the Prognosis of Idiopathic Pulmonary Arterial Hypertension ― Insight From a Cohort From Two Japanese Pulmonary Hypertension Centers ―

Background: The prognosis of pulmonary arterial hypertension (PAH) has been improving since the introduction of epoprostenol (EPO). The 3-year survival of naïve idiopathic PAH (IPAH) and hereditary PAH (HPAH) was 96% in a recent prospective Japanese registry. This increase in survival in Japan may have been due to the reduction of pulmonary artery pressure (PAP) by a rapid and sufficient dosage of EPO. The aim of this retrospective study was therefore to analyze whether decreasing the PAP contributes to improving PAH prognosis. Methods and Results: Sixty-four patients with IPAH/HPAH followed up at Keio and Kyorin University Hospitals between 1999 and 2011 were enrolled and divided into 2 groups: surviving or non-surviving. Of 14 variables, EPO use, most improved mean PAP (mPAP), brain natriuretic peptide level, cardiac output, 6-min walk distance, and sex were significantly different between the 2 groups. The former 3 variables were significantly related to death on multiple regression analysis. mPAP had the highest odds ratio of 1.44 and the largest area under the receiver operating characteristic curve. The value of mPAP with the optimal combination of sensitivity and specificity was 42 mmHg. Conclusions: The best treatment target for the prognosis of IPAH/HPAH may be the reduction of mPAP; a similar large-scale study is anticipated.

[1]  M. Nishimura,et al.  Guidelines for the Treatment of Pulmonary Hypertension (JCS 2017/JPCPHS 2017). , 2019, Circulation journal : official journal of the Japanese Circulation Society.

[2]  Z. Jing,et al.  Risk stratification and medical therapy of pulmonary arterial hypertension , 2019, European Respiratory Journal.

[3]  S. Miyata,et al.  Sex differences in hemodynamic responses and long-term survival to optimal medical therapy in patients with pulmonary arterial hypertension , 2018, Heart and Vessels.

[4]  M. Humbert,et al.  Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension , 2017, European Respiratory Journal.

[5]  K. Fukuda,et al.  Survival of Japanese Patients With Idiopathic/Heritable Pulmonary Arterial Hypertension. , 2017, The American journal of cardiology.

[6]  A. Ogawa,et al.  Rapid and high-dose titration of epoprostenol improves pulmonary hemodynamics and clinical outcomes in patients with idiopathic and heritable pulmonary arterial hypertension. , 2016, Journal of cardiology.

[7]  Simon Gibbs,et al.  2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. , 2016, Revista espanola de cardiologia.

[8]  A. Ogawa,et al.  Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan. , 2014, Life sciences.

[9]  R. Benza,et al.  An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. , 2012, Chest.

[10]  A. Ogawa,et al.  Marked hemodynamic improvements by high-dose epoprostenol therapy in patients with idiopathic pulmonary arterial hypertension. , 2010, Circulation journal : official journal of the Japanese Circulation Society.

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

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

[13]  J. Hanley,et al.  A method of comparing the areas under receiver operating characteristic curves derived from the same cases. , 1983, Radiology.

[14]  H. Miyata,et al.  Effectiveness and Outcome of Pulmonary Arterial Hypertension-Specific Therapy in Japanese Patients With Pulmonary Arterial Hypertension. , 2017, Circulation journal : official journal of the Japanese Circulation Society.

[15]  Keith McNeil,et al.  International guidelines for the selection of lung transplant candidates. The American Society for Transplant Physicians (ASTP)/American Thoracic Society(ATS)/European Respiratory Society(ERS)/International Society for Heart and Lung Transplantation(ISHLT). , 1998, American journal of respiratory and critical care medicine.