title: Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary Vascular Disease.
abstract: OBJECTIVE
  Despite remarkable advances in pediatric cardiology, pulmonary arterial hypertension associated with congenital heart disease remains a major problem. In the past decade new vasodilators have been introduced and appear to be effective in reducing pulmonary vascular resistance (PVR).
  
  
  METHODS
  From 2000 to 2011, we retrospectively reviewed the records of 22 patients who had congenital septal defects and borderline pulmonary vascular disease (PVD). The PVR in these patients was from 6 to 16 wood units · m(2), and/or the systolic pulmonary arterial pressure was more than 2/3 of the systemic arterial pressure.
  
  
  RESULTS
  The median age was 16 years (range, 9 months-46 years). The median duration of follow-up was 7.4 years (range, 1.4-11.7 years). According to hemodynamic data and clinical symptoms, the initial management comprised targeted medical therapy in four (18%), complete closure in four (18%), and partial closure in 14 patients (63.6%). In the four patients who had a high PVR and negative vasoreactivity, the PVR decreased and vasoreactivity increased after targeted medical therapy; three of these patients underwent cardiac surgery later. Finally, 11 (50%) received targeted medical therapy and 21 patients (95.4%) underwent cardiac surgery. Complete closure resulted in six patients and partial closure in 17 patients. Mortality was observed in two patients. The other 19 patients (91%) had New York Heart Association functional class I.
  
  
  CONCLUSIONS
  Targeted medical therapy may be effective in reducing PVR in patients with congenital heart disease and borderline PVD. A stepwise approach may help to achieve improved outcomes in these patients.
authors.affiliation: Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute, Bucheon, Korea.
authors.forename: Sang-Yun
authors.initials: SY
authors.lastname: Lee
authors.affiliation: Department of Pediatrics, Konkuk University School of Medicine, Seoul, Korea.
authors.forename: Soo-Jin
authors.initials: SJ
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, Konkuk University School of Medicine, Seoul, Korea.
authors.forename: Jae Sung
authors.initials: JS
authors.lastname: Son
authors.affiliation: Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute, Bucheon, Korea.
authors.forename: Seong-Ho
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute, Bucheon, Korea.
authors.forename: Chang-Ha
authors.initials: CH
authors.lastname: Lee
keywords: Congenital heart defect; Pulmonary hypertension; Pulmonary vascular resistance; Vasodilators