Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary Vascular Disease
Korean Circulation Journal
; : 408-415, 2015.
Article
in En
| WPRIM
| ID: wpr-225165
Responsible library:
WPRO
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Thoracic Surgery
/
Vascular Diseases
/
Vascular Resistance
/
Vasodilator Agents
/
Wood
/
Cardiology
/
Retrospective Studies
/
Follow-Up Studies
/
Mortality
/
Arterial Pressure
Type of study:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
Korean Circulation Journal
Year:
2015
Type:
Article