222 - Hemodynamic Characterization of Neonates with Congenital Diaphragmatic Hernia-Associated Pulmonary Hypertension by Cardiac Catheterization

Poster Number: 222
Publication Number: 222.221

Paula Dias Maia, University of Colorado School of Medicine, Denver, CO, United States; Jason Gien, University of Colorado School of Medicine, Englewood, CO, United States; John P. Kinsella, University of Colorado School of Medicine, Highlands Ranch, CO, United States; Jenny E. Zablah, University of Colorado School of Medicine, Aurora, CO, United States; Gareth Morgan, University of Colorado School of Medicine, Denver, CO, United States; Steven Abman, University of Colorado School of Medicine, denver, CO, United States; Benjamin S. Frank, University of Colorado School of Medicine, Denver, CO, United States

Background: Patients with congenital diaphragmatic hernia (CDH) are at high risk for pulmonary hypertension
(PH). Abnormal left ventricular structure and function have increasingly been recognized as
important contributors to the cardiopulmonary pathobiology in CDH patients. Early left
ventricular dysfunction can contribute to PH in infants with CDH and is strongly associated with
increased mortality and the use of extracorporeal membrane oxygenation therapy. However, the
incidence and impact of late left ventricular diastolic dysfunction (LVDD) in CDH infants with
sustained PH are uncertain. Cardiac catheterization is the gold standard for PH diagnosis and
evaluation of ventricular filling pressures in other groups of children with suspected PH, but its
role for CDH-PH patients is not well defined.

Objective: To examine the role of cardiac catheterization in the characterization of late PH and the potential
contribution of LVDD to the pathophysiology and course of infants with CDH.

Design/Methods: This single-center retrospective study included 17 neonates with CDH and suspected PH by
echocardiogram who underwent cardiac catheterization during their initial admission to the
NICU. Clinical, echocardiographic, and catheterization data were evaluated. LVDD was defined as
pulmonary wedge capillary pressure (PCWP) ≥10 mmHg at catheterization. Clinical courses were
compared between infants with LVDD and infants with no evidence of LVDD.

Results: The median mean pulmonary artery (PA) pressure was 45 mmHg (IQR, 36-51), the systolic PA to
systemic arterial pressure ratio was 85% (median; IQR 66-99), the median pulmonary vascular
resistance index was 6.8 Woods units*m2 (IQR, 3.7-8.8), and the median pulmonary wedge
pressure was 10 mmHg (IQR 8-11). Fifty-three percent of patients had a PCWP of ≥10 mmHg.
There were no differences in mPAP or PVRi between infants with or without LVDD. Infants with
LVDD and PH spent more days on invasive ventilation (161 vs 100, p=0.044), and trended towards
longer hospitalization stays. Cardiac catheterization resulted in a change in management in 88%
of the patients, which included initiation, discontinuation, or change in dose of PH-target
therapies, diuretics, and LV afterload reduction medications, or ductus arteriosus occlusion.Conclusion(s): In patients with CDH-PH selected for catheterization, the burden and severity of PH were high.
The presence of LVDD by cardiac catheterization may identify infants at risk for greater morbidity.
Cardiac catheterization led to identifiable changes in clinical management for most patients.
Table 1- Demographic and clinical characteristics
Table 2- Cardiac catheterization and clinical course data