title: Elevated serum HMGB1 in pulmonary arterial hypertension secondary to congenital heart disease.
abstract: This study investigated the potential value of serum high mobility group box-1 (HMGB1) level in the diagnosis, staging and treatment response of patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD).
  
  
  
  This was a single-center prospective study in 106 CHD patients. Serum HMGB1 levels were measured by enzymelinked immunosorbent assay. HMGB1 levels were significantly increased in patients with PAH compared to patients without PAH (P<0.01) and healthy controls (P<0.001). HMGB1 levels significantly correlated with pulmonary arterial pressure (P<0.001) and pulmonary vascular resistance (PVR) (P<0.001). In patients with severe PAH, HMGB1 levels were significantly higher in patients with Eisenmenger syndrome (ES) than in patients exhibiting low PVR (P<0.001). Severe PAH and ES was identified by serum HMGB1 with a cutoff value of 13.62ng/mL (P<0.001) with a specificity of 82.8% and a sensitivity of 90%, and a cutoff value of 21.62ng/mL (P=0.001) with a specificity of 85.2% and a sensitivity of 64.3%, respectively. HMGB1 levels were significantly decreased after sildenafil therapy for 6months (P<0.01).
  
  
  
  Our study suggests that serum HMGB1 level may be used as a biomarker to identify PAH in CHD patients, assess pulmonary vascular remodeling, and evaluate the treatment response to sildenafil.
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China.
authors: Yi-Yuan
authors: YY
authors: Huang
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China.
authors: Wei
authors: W
authors: Su
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China.
authors: Zhao-Wei
authors: ZW
authors: Zhu
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China.
authors: Liang
authors: L
authors: Tang
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China.
authors: Xin-Qun
authors: XQ
authors: Hu
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China.
authors: Sheng-Hua
authors: SH
authors: Zhou
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China. Electronic address: zhenfeifang@sina.com.
authors: Zhen-Fei
authors: ZF
authors: Fang
authors: Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, PR China. Electronic address: lijiangcs@163.com.
authors: Jiang
authors: J
authors: Li
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D004541:Eisenmenger Complex / Q000097:blood / Q000150:complications*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D024243:HMGB1 Protein / Q000097:blood*; D006330:Heart Defects, Congenital / Q000097:blood / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000008:administration & dosage / Q000494:pharmacology; D011446:Prospective Studies; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate / Q000008:administration & dosage / Q000494:pharmacology; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000502:physiology; D055815:Young Adult
chemical_list: D015415:Biomarkers; D024243:HMGB1 Protein; C585491:HMGB1 protein, human; D058986:Phosphodiesterase 5 Inhibitors; D000068677:Sildenafil Citrate
keywords: Biomarkers; Congenital malformations; HMGB1; Pulmonary hypertension