Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary Vascular Disease.
10.4070/kcj.2015.45.5.408
- Author:
Sang Yun LEE
1
;
Soo Jin KIM
;
Jae Sung SON
;
Seong Ho KIM
;
Chang Ha LEE
Author Information
1. Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute, Bucheon, Korea.
- Publication Type:Original Article
- Keywords:
Pulmonary hypertension;
Congenital heart defect;
Vasodilators;
Pulmonary vascular resistance
- MeSH:
Arterial Pressure;
Cardiology;
Follow-Up Studies;
Heart;
Heart Defects, Congenital*;
Hemodynamics;
Humans;
Hypertension;
Hypertension, Pulmonary;
Mortality;
Retrospective Studies;
Thoracic Surgery;
Vascular Diseases*;
Vascular Resistance;
Vasodilator Agents;
Wood
- From:Korean Circulation Journal
2015;45(5):408-415
- CountryRepublic of Korea
- Language:English
-
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 . m2, 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.
