Quantitative Measures of Right Ventricular Size and Function by Echocardiogram Correlate with Cardiac Catheterization Hemodynamics in Congenital Diaphragmatic Hernia

Objective To evaluate associations between cardiac catheterization (cath) hemodynamics, quantitative measures of right ventricular (RV) function by echocardiogram, and survival in patients with congenital diaphragmatic hernia (CDH). Study design This single-center, retrospective cohort study enrolled CDH patients who underwent index cath from 2003-2022. Tricuspid annular plane systolic excursion Z-score, RV fractional area change (FAC), RV free wall and global longitudinal strain, left ventricular (LV) eccentricity index, RV/LV ratio, and pulmonary artery acceleration time were measured from pre-procedure echocardiograms. Associations between hemodynamic values, echocardiographic measures, and survival were evaluated by Spearman correlation and Wilcoxon rank sum test, respectively. Results Fifty-three patients (68% left-sided, 74% liver herniation, 57% extracorporeal membrane oxygenation, 93% survival) underwent cath (39 during index hospitalization, 14 later) including device closure of a patent ductus arteriosus in 5. Most patients (n=31, 58%) were on pulmonary hypertension (PH) treatment at cath, most commonly sildenafil (n=24, 45%) and/or intravenous treprostinil (n=16, 30%). Overall, hemodynamics were consistent with pre-capillary PH. Pulmonary capillary wedge pressure was > 15 mm Hg in 2 patients (4%). Lower FAC and worse ventricular strain were associated with higher pulmonary artery pressure while higher LV eccentricity index and higher RV/LV ratio were associated with both higher pulmonary artery pressure and higher pulmonary vascular resistance. Hemodynamics did not differ based on survival status. Conclusions Worse RV dilation and dysfunction by echocardiogram correlate with higher pulmonary artery pressure and pulmonary vascular resistance on cath in this CDH cohort. These measures may represent novel, non-invasive clinical trial targets in this population.