ABSTRACT
Background Management of pediatric pulmonary hypertension (PH) may require manipulation of multiple receptor sites to maximize response to medical therapy. Assessment of response typically occurs through imaging, labs, physical exam and recurrent cardiac catheterization, with anesthetic exposure to assess pulmonary artery pressures (PAP) and vascular resistance (PVR). We aimed to assess feasibility, safety and utility of remote PAP monitoring in pediatric PH patients. Methods We reviewed 4 pediatric patients with significant PH, each of whom underwent cardiac catheterization with pulmonary vasoreactivity testing and placement of a CardioMEMS remote PAP monitoring device. Results Four patients (P1-4: ages 5, 6, 8 and 10 years old) underwent CardioMEMS insertion without procedural complication. P1, P2 and P3 presented with unrepaired VSD;ASD with partial anomalous pulmonary venous return;and ASD and PDA, respectively, while P4 had prior repair of atrioventricular canal. Three patients had Down syndrome. All had elevated PAP and PVR. Mean left lower PA branch size was 7 mm. Mean PAP prior to therapy was 70 mm Hg for P1, 82 for P2, 93 for P3 and 30 for P4. All 4 patients required initiation of triple therapy for treatment of PH, with improvement or normalization of PAP by CardioMEMS, which also included surgical or catheter based intervention for 3 patients. Post-repair of P2, he was unable to be separated from cardiopulmonary bypass and was placed on ECMO. Right ventricular cardiac output improved over 2 weeks, with improvement of PAP determined through serial CardioMEMS. He was successfully decannulated, utilizing CardioMEMS in the OR. Two patients also developed COVID respiratory infections at home with CardioMEMS assessments allowing for oxygen and medication titration. Conclusion Remote PAP monitoring is feasible and appears safe in pediatric patients with adequate PA size. It allows for manipulation of medical therapy with real time knowledge of impact on PAP and can augment management during weaning of mechanical cardiac support. It may also augment decision-making in management of PH patients with developmental disabilities in whom traditional assessments may be more challenging.Copyright © 2023 American College of Cardiology Foundation