Korean Circulation Journal

The Korean Society of Cardiology (대한심장학회)
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Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary Vascular Disease

  • Lee, Sang-Yun (Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute) ;
  • Kim, Soo-Jin (Department of Pediatrics, Konkuk University School of Medicine) ;
  • Son, Jae Sung (Department of Pediatrics, Konkuk University School of Medicine) ;
  • Kim, Seong-Ho (Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute) ;
  • Lee, Chang-Ha (Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute)
  • Received : 2014.09.26
  • Accepted : 2015.04.28
  • Published : 2015.09.30
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Abstract

Background and Objectives: 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). Subjects and 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 $units{\cdot}m^2$, 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. Conclusion: 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.

Keywords

References

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