J Pediatr

. 2023 Jun 15;113564.

 doi: 10.1016/j.jpeds.2023.113564. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37329980/

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

Catherine M Avitabile ¹, Sabrina Flohr ², Leny Mathew ², Yan Wang ³, Devon Ash ³, David B Frank ⁴, Jennifer E Tingo ⁴, Natalie E Rintoul ⁵, Holly L Hedrick ²

Affiliations expand

• PMID: 37329980
• DOI: 10.1016/j.jpeds.2023.113564

Abstract

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.

Keywords: neonatal; pulmonary hypertension; survival.

Copyright © 2023. Published by Elsevier Inc.