Abstract

Purpose Adult studies have shown utility of ECGs in disease stratification and monitoring in pulmonary hypertension (PH). We sought to identify the ECG findings that are present in pediatric PH and correlate with disease severity and change over time. Methods and Materials We retrospectively identified all patients ≤21 years old with PH (Dana Point 1.1 and 3.7) confirmed by catheterization (cath) and had an ECG within 60 days of cath. ECG and cath findings included: right axis deviation (RAD); right atrial enlargement (RAE); right ventricular hypertrophy (RVH) and strain; pulmonary artery pressure (PAP) and capillary wedge pressure; right atrial pressure; cardiac index; and indexed pulmonary vascular resistance (PVR). A change in PVR of ≥20% was deemed significant between caths. Results We identified 48 patients (46% group 1.1), yielding 107 cath-ECG pairs. Median age at cath was 49 months (IQR: 4-202). Median PVR was 7.2 WU/m2 (2.2-27.9). Of the 107 pairs, 94% had ECG abnormalities: 54% with RAD; 80% with RVH; 57% with a rSR’/qR in V1/V4R; 19% with RAE; and 3% with bundle branch block. Individual ECG parameters weakly correlated with PVR and the transpulmonary gradient (TPG). Combination of RAD and RVH significantly correlated with PVR (β=3.9), PAP (β=14.8), and TPG (β=15.1), (p Conclusions ECG abnormalities are common in pediatric PH. RAD and RVH on ECG was associated with worse hemodynamics, while their absence predicted a lack of disease progression.

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