Satoshi Ideno, Shinichi Yamamoto, Fujiko Oda, Rie Wakamiya, Kana Ozawa, Haruka Kaneko, Taku Matsuoka, Atsushi Shinto, Hiromi Mikasa, Noriko Miyazawa
Tokyo Metropolitan Children’s Medical Center.
Japan
Masui
Masui 2015; 64: 168-173
DOI: Not Available
Abstract
Congenital pulmonary vein stenosis (CPVS) is a rare fetal congenital heart disease with a prevalence of 1.7 per 100,000 children younger than two years of age. Because of the difficulty of maintaining the pulmonary blood flow, CPVS is associated with a 50% survival rate within five years of diagnosis. We describe a successful management of pulmonary blood flow for a 4-month-old-girl with CPVS, combined with atrial septal defect and ventricular septal defect, undergoing pulmonary vein obstruction release (PVOR). In this case, CPVS was the only cause for pulmonary hypertension because there was no significant pressure gradient between each pulmonary capillary wedge pressure and the paired pulmonary vein pressure, indicating the normal pulmonary vascular structure prior to pulmonary vein stenosis. As pulmonary blood flow was estimated to be high after PVOR, pulmonary artery banding was also performed. Management of pulmonary blood flow is the most important issue for anesthesia of this surgery, especially in postcardiopulmonary bypass period, when the pulmonary vasoconstriction is induced by endothelial dysfuncion.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2015
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: No, Article in Japanese