Elevated plasma Pim-1 and its clinical significance in patients with pulmonary arterial hypertension.

This study was aimed to determine plasma Pim-1 levels in patients with pulmonary arterial hypertension (PAH) and to estimate the clinical value of Pim-1 as a biomarker of PAH. This was a single-centre retrospective study in 111 patients with congenital heart disease (CHD) and idiopathic PAH (IPAH). Those CHD patients were divided into two groups: PAH associated with CHD (PAH-CHD) and CHD without PAH (nPAH-CHD). Plasma Pim-1 levels were measured by enzyme-linked immunosorbent assay. (a) Plasma Pim-1 levels were significantly increased in patients with PAH-CHD and IPAH compared with the healthy control group (27.81 ± 11.34 ng/mL vs 13.02 ± 5.30 ng/mL; 32.81 ± 12.28 ng/mL vs 13.02 ± 5.30 ng/mL, P < 0.05) and nPAH-CHD (27.81 ± 11.34 ng/mL vs 17.33 ± 7.99 ng/mL; 32.81 ± 12.28 ng/mL vs 17.33 ± 7.99 ng/mL, P < 0.05). Pim-1 levels were substantially increased in patients with severe PAH-CHD compared with mild-to-moderate PAH-CHD (19.12 ± 6.70 ng/mL vs 8.54 ± 3.71 ng/mL, P < 0.05). (b) Pim-1 levels were correlated positively with the mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) (r = 0.582, 0.516; P < 0.001, respectively), while negatively with tricuspid annular plane systolic excursion (TAPSE), tricuspid annular plane systolic velocity (S') and right ventricular fractional area changes (RVFAC) (r = -0.375, -0.354, -0.507; P < 0.05, respectively). (c) PAH-CHD and severe PAH-CHD was identified by plasma Pim-1 with a cutoff value of 16.8 ng/mL (P < 0.001) with a sensitivity of 87.3% and a specificity of 65%, and a cutoff value of 20.53 ng/mL (P < 0.001) with a sensitivity of 87.3% and a specificity of 52%, respectively. Plasma Pim-1 levels were significantly higher in patients with PAH-CHD and IPAH. Plasma Pim-1 may represent an effectively biomarker in patients with PAH.

[Pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis: A case report and literature review].

Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension. The diagnosis of PVOD/PCH can be established by the combination of clinical features, physical examination, radiological findings, lung function, bronchoscopy and other resources. There is no established medical therapy for PVOD/PCH, and the only curative therapy for PVOD/PCH is lung transplantation. A girl with PVOD/PCH was diagnosed in the Second Xiangya Hospital. Combining the characteristics for this case with the relevant literature, we summarized the epidemiology, etiology, diagnosis and treatment for the disease to raise doctors' awareness for this rare disease.