Cachexia is associated with a high risk of death in heart failure (HF) patients [1,2]. Right ventricular dysfunction (RVD) reportedly often coexists with cachexia and is associated with accelerated weight loss, abnormal body composition, and a worsened prognosis in advanced HF [3]. Multiple mechanisms are thought to be involved in the development of cardiac cachexia. In particular, this condition is related to hemodynamics of HF including pulmonary hypertension [4] as well as increased neurohumoral and cytokine responses [5,6], impaired gastrointestinal function [7], and an increased metabolic rate [8].However, only a few studies have investigated pulmonary arterial hypertension (PAH) and nutritional status [9]. In the present study, we sought to examine the relationships among nutritional status, markers of congestion, and echocardiographic parameters in patients with stable PAH. Findings from the present study would provide insights on the mechanism underlying the effect of PAH alone on nutritional status.We enrolled 8 patients with stable (N6 months) pulmonary hypertension from our out-patient clinic prospectively, and written informed consent was obtained. The study conformed to the ethical guidelines of the 1975 Declaration of Helsinki and was approved by the ethical committee of Kyoto University Hospital. Patients underwent the scored Patient-Generated Subjective Global Assessment (PG-SGA) for nutritional status, blood tests, and comprehensive echocardiography. After morning fasting, patients underwent blood testing for complete blood counts, chemistry, zinc, pre-albumin, transferrin, retinol-binding proteins, and brain natriuretic peptide (BNP) levels. Glomerular filtration rate (GFR) was estimated with the CKD-EPI Creatinine Equation.Spearman's rank correlation tests were conducted to assess relationships between nonparametric variables, and multiple regression analyses were performed to exclude multicollinearity. Data were analyzed with JMP 12 software (SAS Institute, Inc., Cary, NC).Patient characteristics are summarized in Table 1. The estimated systolic pulmonary artery pressure (ePAP) and BNP levels were 82.7 ± 15.7 (mean ± SD) mm Hg and 176 ± 146 ng/L, respectively. LV diastolic diameter (LVDd) was negatively correlated with ePAP (rs = − 0.76, p = 0.04), which implied a dilated RV and compressed LV. Body mass index (BMI) was positively correlated with white blood cell count (WBC, rs = 0.84, p = 0.03) and fasting insulin levels (rs = − 0.57, p = 0.03) and negatively correlated with aspartate aminotransferase concentration (AST, rs = −0.47, p = 0.02), serum sodium concentration (rs = − 0.60, p = 0.03), and inferior vena cava (IVC) diameter (rs = −0.76, p = 0.04). Blood urea nitrogen, a marker of protein catabolism, was positively correlated with SGA scores (rs = 0.81, p b 0.01) and BNP (rs = 0.68, p = 0.04) and negatively correlated with LV diameter (rs = − 0.87, p b 0.01). Serum pre-albumin -a rapid-turnover hepatic protein -was positively correlated with ePAP (rs = 0.70, p = 0.01) and negatively cor...