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Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary
Sang-Yun Lee1, Soo-Jin Kim2, Jae Sung Son2
1Department of Pediatrics, Department of Thoracic and Cardiovascular Surgery, Sejong Cardiovascular Institute, Bucheon, Korea.
Insights
Targeted medical therapy can reduce pulmonary vascular resistance (PVR) in children with congenital heart disease and borderline pulmonary vascular disease (PVD). A stepwise treatment approach improves patient outcomes.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Pulmonary Hypertension
Background:
- Congenital heart disease (CHD) with pulmonary vascular disease (PVD) remains a significant challenge in pediatric cardiology.
- Recent advancements include novel vasodilators showing promise in reducing pulmonary vascular resistance (PVR).
Purpose of the Study:
- To evaluate the effectiveness of targeted medical therapy and a stepwise approach in managing borderline pulmonary vascular disease in pediatric patients with congenital heart disease.
- To assess the impact on pulmonary vascular resistance and patient outcomes.
Main Methods:
- Retrospective review of 22 patients (2000-2011) with congenital septal defects and borderline PVD (PVR 6-16 wood units · m(2) and/or systolic pulmonary arterial pressure > 2/3 systemic pressure).
- Analysis of initial management strategies: targeted medical therapy, complete closure, or partial closure.
- Evaluation of hemodynamic data, vasoreactivity, and clinical outcomes post-intervention.
Main Results:
- Of 22 patients, 11 (50%) received targeted medical therapy, and 21 (95.4%) underwent cardiac surgery (6 complete, 17 partial closure).
- In patients with high PVR and negative vasoreactivity, medical therapy decreased PVR and increased vasoreactivity, with 3 later undergoing surgery.
- Nineteen patients (91%) achieved New York Heart Association functional class I, with two observed deaths.
Conclusions:
- Targeted medical therapy demonstrates efficacy in reducing PVR for pediatric CHD patients with borderline PVD.
- A stepwise management strategy, potentially including medical therapy followed by surgical intervention, can lead to improved clinical outcomes.
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 · 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.
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