Takanari Fujii, Hideshi Tomita, Hiroaki Kise, Kazuto Fujimoto, Kozue Kobayashi, Yoshitaka Watanabe, Takeshi Yamazaki, Takeshi Shimizu, Shunsuke Sakurai, Satoshi Hibino, Junya Iwasaki, Takashi Soga, Shigeru Uemura, Kazuo Itabashi, Shigeo Yamaki
Showa University Northern Yokohama Hospital. Japanese Research Institute of Pulmonary Vasculature.
Japan
Journal of Cardiology Cases
J Cardiol Cases 2013; 9: 3-7
DOI: 10.1016/j.jccase.2013.08.013
Abstract
Primary pulmonary vein stenosis (PVS) is rare within the pediatric population and its pathophysiology remains unclear, especially as to how the histopathology relates to its refractoriness to treatment. We report the case of a 4-month-old girl with primary PVS. The lesion in this patient was characterized by fatal obstruction of intraparenchymal small pulmonary veins, associated with localized stenosis at the four pulmonary veno-atrial junctions. All four localized stenoses underwent transcatheter stent implantation. Although the procedure was technically successful, her clinical status failed to improve, and she died 2 months after stenting. Histopathological examination of lung specimens showed severe luminal obstruction by marked intimal proliferation with fibrosis in the intraparenchymal small pulmonary veins, and these findings were present in every lobe. To the best of our knowledge, the histopathological findings and clinical course in this case, including the response to treatments, are extremely rare. We suggest that the histological findings of the small pulmonary veins are important in deciding the indication and appropriate timing of intervention.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Right Heart Failure Associated with Stenosis or Atresia of Pulmonary Veins
Pulmonary Venous Pathology
Year of Publication: 2013
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes