title: [Pathologic markers for evaluation of reversibility in pulmonary hypertension related to congenital heart disease].
abstract: OBJECTIVE
  To assess the pathologic markers for evaluation of reversibility in pulmonary hypertension (PAH) related to congenital heart disease.
  
  
  METHODS
  Twenty-eight patients with congenital heart disease complicated by PAH were subclassified into reversible pulmonary hypertension (RPAH) and irreversible pulmonary hypertension (IPAH), according to post-operative mean pulmonary artery pressure (MPAP). Pulmonary vascular lesion was analyzed according to Ruan's method. Mean medium thickness percent, mean medium area percent and pulmonary arteriolar density were measured by quantitative morphometry. Immunohistochemical study for transgelin and filamin A was carried out.
  
  
  RESULTS
  Amongst the 28 cases studied, 24 were RPAH and 4 were IPAH. Of the 24 patients with RPAH, 13 (54.2%, 13/24) had pulmonary vascular lesion of grade 0, 9 (37.5%, 9/24) of grade 1 and 2 (8.3%, 2/24) of grade 2. Of the 4 patients with IPAH, 1 had lesion of grade 1, 1 of grade 2 and 2 of grade 3. Both preoperative and postoperative MPAP were higher in IPAH patients than that in RPAH patients[(53.3±23.4) mmHg versus (34.1±12.7) mmHg, P=0.020 and (35.0±8.8) mmHg versus (17.8±3.9) mmHg, P<0.01]. Compared to patients with pulmonary vascular lesion of grades 0 and 1, the preoperative MPAP in patients with grades 2 and 3 showed no significant difference, but the postoperative MPAP was higher (P<0.05 or 0.01). Compared to control group, mean medium thickness percent and mean medium area percent were significantly higher in RPAH and IPAH categories (12.0±3.5, 8.5±2.0 versus 5.7±1.0, P<0.01 and 55.8±11.1, 49.0±9.4 versus 34.0±5.5, P<0.01). Mean medium thickness percent was significantly higher in IPAP group than that in RPAH group (12.0±3.5 versus 8.5±2.0, P=0.001). Correlation analysis demonstrated that mean medium thickness percent and mean medium area percent had positive correlation with preoperative and postoperative MPAP. There was no correlation between grading of pulmonary vascular lesion and reversibility. Transgelin and filamin A had stronger staining in pulmonary vascular smooth muscle cells in IPAH than those in RPAH and controls(P<0.05).
  
  
  CONCLUSIONS
  Pathologic assessment of lung biopsy remains the gold standard for evaluation of the reversibility in PAH related to congenital heart disease. Mean medium thickness percent, mean medium area percent and immunoreactivity for transgelin and filamin A are useful parameters.
authors.affiliation: Departement of Pathology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100037, China.
authors.forename: Li
authors.initials: L
authors.lastname: Li
authors.forename: Li
authors.initials: L
authors.lastname: Huang
authors.forename: Guo
authors.initials: G
authors.lastname: Chen
authors.forename: Shian
authors.initials: S
authors.lastname: Huang
authors.forename: Chao
authors.initials: C
authors.lastname: Liu
authors.forename: Hongyue
authors.initials: H
authors.lastname: Wang
authors.forename: Xuejin
authors.initials: X
authors.lastname: Duan
authors.forename: Qingzhi
authors.initials: Q
authors.lastname: Wang
authors.forename: Ranxu
authors.initials: R
authors.lastname: Zhao
authors.forename: Jianguo
authors.initials: J
authors.lastname: He
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D001706:Biopsy; D064448:Filamins / Q000378:metabolism; D006331:Heart Diseases / Q000150:complications / Q000473:pathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis* / Q000473:pathology*; D008168:Lung / Q000473:pathology; D008840:Microfilament Proteins / Q000378:metabolism; D009124:Muscle Proteins / Q000378:metabolism
chemical_list: D015415:Biomarkers; D064448:Filamins; D008840:Microfilament Proteins; D009124:Muscle Proteins; C051615:transgelin