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

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 · 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: Congenital heart defect; Pulmonary hypertension; Pulmonary vascular resistance; Vasodilators.

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.

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.

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.

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.