Jill E. O’Hara, Terry L. Buchmiller, Lori J. Bechard, Alireza Akhondi-Asl, Gary Visner, Catherine Sheils, Ronald Becker, Mollie Studley, Lindsay Lemire, Mary P. Mullen, Sally Vitali, Nilesh M. Mehta, Belinda Dickie, Jill M. Zalieckas, Ben D. Albert
Boston Children’s Hospital and Harvard Medical School.
United States
Pediatric Critical Care Medicine
Pediatr Crit Care Med 2023;
DOI: 10.1097/PCC.0000000000003249
Abstract
Objectives: Congenital diaphragmatic hernia (CDH) is a birth defect associated with long-term morbidity. Our objective was to examine longitudinal change in Functional Status Scale (FSS) after hospital discharge in CDH survivors.
Design: Single-center retrospective cohort study.
Setting: Center for comprehensive CDH management at a quaternary, free-standing children’s hospital.
Patients: Infants with Bochdalek CDH were admitted to the ICU between January 2009 and December 2019 and survived until hospital discharge.
Interventions: None.
Measurements and main results: One hundred forty-two infants (58% male, mean birth weight 3.08 kg, 80% left-sided defects) met inclusion criteria. Relevant clinical data were extracted from the medical record to calculate FSS (primary outcome) at hospital discharge and three subsequent outpatient follow-up time points. The median (interquartile range [IQR]) FSS score at hospital discharge was 8.0 (7.0-9.0); 39 patients (27.5%) had at least moderate impairment (FSS ≥ 9). Median (IQR) FSS at 0- to 6-month (n = 141), 6- to 12-month (n = 141), and over 12-month (n = 140) follow-up visits were 7.0 (7.0-8.0), 7.0 (6.0-8.0), and 6.0 (6.0-7.0), respectively. Twenty-one patients (15%) had at least moderate impairment at over 12-month follow-up; median composite FSS scores in the over 12-month time point decreased by 2.0 points from hospital discharge. Median feeding domain scores improved by 1.0 (1.0-2.0), whereas other domain scores remained without impairment. Multivariable analysis demonstrated right-sided, C- or D-size defects, extracorporeal membrane oxygenation use, cardiopulmonary resuscitation, and chromosomal anomalies were associated with impairment.
Conclusions: The majority of CDH survivors at our center had mild functional status impairment (FSS ≤ 8) at discharge and 1-year follow-up; however, nearly 15% of patients had moderate impairment during this time period. The feeding domain had the highest level of functional impairment. We observed unchanged or improving functional status longitudinally over 1-year follow-up after hospital discharge. Longitudinal outcomes will guide interdisciplinary management strategies in CDH survivors.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Acquired Patient Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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