Lung function in school‐aged congenital diaphragmatic hernia patients; a longitudinal evaluation

Abstract

Objective Children with congenital diaphragmatic hernia (CDH) are at risk for pulmonary morbidity. Data on longitudinal evaluation of lung function in CDH are scarce. We hypothesized that CDH patients would have impaired lung function that worsens over time. We evaluated lung function and its determinants at ages 8 and 12 years. Methods Dynamic and static lung volumes, and diffusion capacity were measured. Extracorporeal membrane oxygenation (ECMO) treatment, the standardized European neonatal treatment protocol, patch repair, duration of ventilation, type of initial mechanical ventilation, and nitric oxide treatment were entered as covariates in linear mixed models with standard deviation score (SDS) lung function parameters (FEV1, FEF 25‐75, and K CO) as dependent variables. Results Seventy‐six children (27 ECMO‐treated) born between 1999 and 2009 performed 113 reliable lung function tests. Severity of airflow obstruction deteriorated significantly from age 8 to 12 years: estimated mean difference (95% confidence interval [CI]) SDS FEV1 was −0.57 (−0.79 to −0.36) and SDS FEF25‐75 was −0.63 (−0.89 to −0.37), both P < .001. Static lung volumes were within normal range and unchanged over time: estimated mean difference (95% CI) SDS TLC −0.27 (−0.58 to 0.04); P = .085. SDS KCO was below normal at 8 and 12 years and remained stable: −0.06 (−0.22 to 0.35); P = .648. These observations were irrespective of ECMO treatment. FEV1 and FEF25‐75 were negatively associated with duration of ventilation (P < .001). Baseline data were not related with TLC or KCO. Conclusions CDH patients should be followed into adulthood as they are at risk for worsening airflow obstruction and decreased diffusion capacity at school age, irrespective of ECMO treatment.

Quality of life was similar in children with congenital diaphragmatic hernia and oesophageal atresia and related to respiratory morbidity

“…high ratio FEV1/peak flow) or restrictive pattern, with less frequent typical obstructive pattern (low FEV1/FVC), 25,26 especially for children with tracheobronchial fistula. 27 As previously observed in children with AO, 16 Previous studies have shown the impact of high neonatal respiratory morbidity on the QoL scores of children with CDH. 8,9,13 As in our study, Sheikh et al also observed lower scores in parents of children with a history of oxygen therapy need at 30 days of life.…”

“…Those results are consistent with the description of abnormal lung function parameters after CDH repair, which may worsen over time, showing that this affection is a complex developmental lung disease 1,4,24 . On the other hand, children with OA usually have a history of barky cough and a lung function profile of central airway obstruction (low peak expiratory flow; high ratio FEV1/peak flow) or restrictive pattern, with less frequent typical obstructive pattern (low FEV1/FVC), 25,26 especially for children with tracheobronchial fistula 27 …”

Patient-Reported Outcome Measures and Clinical Outcomes in Children with Foregut Anomalies

“…For this study, we selected children who completed a Pediatric Quality of Life Inventory (PedsQL, a PROM for HS) and/or Dutch-Child-AZL-TNO-Quality-of-Life (DUX-25, a PROM for QoL) questionnaire during follow-up assessments at eight years of age. Standardized assessments of health and daily functioning were performed as standard of care using previously described instruments and methods [ 11 , 12 , 13 , 14 ]. Although our follow-up protocol was subject to changes in regular care throughout the study period, evaluation methods were considered interchangeable and the same outcomes were measured over the years.…”

Pediatric pulmonology 2019 year in review: rare and diffuse lung disease

“…The second paper reported on 113 pulmonary function test (PFT) results performed at 8 and 12 years of age by 76 children born with CDH between 1999 and 2009. 9 Mean forced expiratory volume in one second (FEV 1 ) and forced expiratory flow (FEF 25%-75% ) declined between the 8-and 12-year measures, suggesting worsening obstruction on average. Mean static lung volumes were within normal limits and stable, while mean K CO was below normal but stable across the two time points.…”

“…Lower exercise capacity was associated with longer initial hospital stay and lower diffusion capacity for carbon monoxide corrected for alveolar volume (K CO ). The second paper reported on 113 pulmonary function test (PFT) results performed at 8 and 12 years of age by 76 children born with CDH between 1999 and 2009 9 . Mean forced expiratory volume in one second (FEV 1 ) and forced expiratory flow (FEF 25%–75% ) declined between the 8‐ and 12‐year measures, suggesting worsening obstruction on average.…”