Lung function in survivors of congenital diaphragmatic hernia at school age

AbstractEvaluation of lung function parameters and additional use of prostaglandin E1 (PGE1) for the stabilisation of cardiopulmonary function in patients with congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PHT). Observational study.Between 2007 and 2009 8 patients with CDH have been treated in our pediatric intensive care unit (gestational age 34 + 0 – 40 + 4 weeks, birth weight 2 160–3 840 g). All patients required respiratory support. Gentle mechanical ventilation adapted to the degree of pulmonary hypoplasia based on serially measurements of lung function parameters to find appropriate ventilator settings has been performed.Functional residual capacity (FRC) and compliance of the respiratory system in all patients were markedly reduced. A FRC between 9.3–10.6 ml/kg and compliance between 1.1–1.8 ml/kPa/kg indicated pronounced hypoplasia of the lungs. Doppler flow patterns through the arterial duct were classified into left-to-right, right-to-left and bidirectional shunting and correlated to the degree of PHT. The additional use of PGE1 to reopen the arterial duct and to stabilize right ventricular function led to an amelioration of severe PHT and preoperative stabilisation in 2 newborns with pronounced pulmonary hypoplasia. All patients underwent successful surgery, and did not show any complications after 2 years follow-up.Measurements of lung function parameters and adaptation of mechanical ventilation to the degree of pulmonary hypoplasia and additional therapy with PGE1 may help to improve the outcome in CDH patients.

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MR lung perfusion measurements in adolescents after congenital diaphragmatic hernia: correlation with spirometric lung function tests

European Radiology ◽

10.1007/s00330-021-08315-9 ◽

2021 ◽

Author(s):

Verena Groß ◽

Katrin Zahn ◽

Kristina Maurer ◽

Lucas Wessel ◽

Thomas Schaible ◽

...

Keyword(s):

Blood Flow ◽

Lung Function ◽

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Pulmonary Blood Flow ◽

Lung Perfusion ◽

Control Group ◽

Lung Function Tests ◽

Function Tests ◽

Perfusion Measurements

Abstract Objectives To evaluate whether lung perfusion continues to be reduced in 10-year-old children after congenital diaphragmatic hernia (CDH) and whether lung perfusion values correlate with spirometric lung function measurements. Methods Fifty-four patients after CDH repair received dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI)-based lung perfusion measurements at the age of 10 years (10.2 ± 1.0 years). Additionally, a control group of 10 children has been examined according to the same protocol. Lung spirometry was additionally available in 43 patients of the CDH group. A comparison of ipsilateral and contralateral parameters was performed. Results Pulmonary blood flow (PBF) was reduced on the ipsilateral side in CDH patients (60.4 ± 23.8 vs. 93.3 ± 16.09 mL/100 mL/min; p < 0.0001). In comparison to the control group, especially the ratio of ipsilateral to contralateral, PBF was reduced in CDH patients (0.669 ± 0.152 vs. 0.975 ± 0.091; p < 0.0001). There is a positive correlation between ipsilateral pulmonary blood flow, and spirometric forced 1-s volume (r = 0.45; p = 0.0024). Conclusions Pulmonary blood flow impairment persists during childhood and correlates with spirometric measurements. Without the need for ionizing radiation, MRI measurements seem promising as follow-up parameters after CDH. Key Points • Ten-year-old children after congenital diaphragmatic hernia continue to show reduced perfusion of ipsilateral lung. • Lung perfusion values correlate with lung function tests after congenital diaphragmatic hernia.

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Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia

European Journal of Pediatric Surgery ◽

10.1055/s-0038-1676586 ◽

2018 ◽

Vol 29 (01) ◽

pp. 120-124 ◽

Cited By ~ 3

Author(s):

Robin Wigen ◽

Wenming Duan ◽

Theo Moraes ◽

Priscilla Chiu

Keyword(s):

Lung Function ◽

Pulmonary Function ◽

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Pulmonary Function Tests ◽

Forced Expiratory Volume ◽

Patch Repair ◽

Recorded Data ◽

Pulmonary Morbidity

Purpose The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. Methods A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. Results Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. Conclusion Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood.

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