Korean Circ J.  2015 Sep;45(5):408-415. 10.4070/kcj.2015.45.5.408.

Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary Vascular Disease

• Lee SY
• Kim SJ
• Son JS
• Kim SH
• Lee CH

• KMID: 2223805
• DOI: http://doi.org/10.4070/kcj.2015.45.5.408

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.

Keyword

Pulmonary hypertension; Congenital heart defect; Vasodilators; Pulmonary vascular resistance

MeSH Terms

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
Vasodilator Agents

Figure

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  Fig. 1 Management summary according to the treatment strategy for PAH in the borderline group. PAH: pulmonary arterial hypertension, PA: pulmonary artery, FU: follow up, ED: early death, LD: late death, VSD: ventricular septal defect.

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  Fig. 2 Comparison of pulmonary vascular resistance between the three groups. (A) Pulmonary vascular resistance measured in room air. (B) Pulmonary vascular resistance after 100 % oxygen inhalation. (C) Amount of left-to-right shunt (Qp/Qs). Qp/Qs: systemic flow amount/pulmonary flow amount, Rp: pulmonary resistance, WU: wood unit.

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  Fig. 3 Change in pulmonary vascular resistance after partial closure and subsequent targeted medical therapy in group 2. Only 5 patients in group 2 underwent the follow up cardiac catheterization. PVR: pulmonary vascular resistance, ASD: atrial septal defect, VSD: ventricular septal defect.

• 

  Fig. 4 Changes after targeted medical therapy in group 3. (A) Change in PVR during follow-up. (B) Change in PVR during vasoreactivity test. (C) Change in Qp/Qs during vasoreactivity test. PVR decreased markedly up to the acceptable level to allow closure of the septal defect. PVR: pulmonary vascular resistance, WU: wood unit, Qp/Qs: systemic flow amount/pulmonary flow amount.

• 

  Fig. 5 Serial changes in PVR over time at each stage of the stepwise combined targeted medical therapy and surgical/interventional closure in two patients of group 3 who underwent complete septal defect closure. PVR: pulmonary vascular resistance, WU: wood unit, AVSD: atrioventricular septal defect, PDA: patent ductus arterriosus.

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  Fig. 6 Suggested management algorithm for the patients with congenital heart disease and severe pulmonary hypertension. PVR: pulmonary vascular resistance, WU: wood unit, p(RV/Ao): ratio of peak right ventricular pressure/peak aortic pressure.

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