Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia

Trachsel, Daniel; Selvadurai, Hiran; Bohn, Desmond; Langer, Jacob C.; Coates, Allan L.

doi:10.1002/ppul.20193

Cited by 139 publications

(129 citation statements)

References 33 publications

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“…There was no correlation between the extent of pulmonary hypoplasia, assessed during the operation and by pre-and postoperative chest Xrays, and reduction of airflow rate [59]. Although FEV 1 improves in approximately half of the patients after bronchodilator therapy [68,69], it remains significantly lower when compared to healthy subjects [69]. Furthermore, there is evidence for more peripheral airway obstruction in CDH patients [69] possibly due to structural abnormalities in distal airways [69].…”

Section: Pulmonary Functionmentioning

confidence: 94%

“…Some longterm follow-up studies indicate a higher incidence of restrictive lung function impairment among CDH patients compared to the healthy population [63,65] especially when there was a large diaphragmatic defect [65], while others cannot confirm this finding [59,66]. Overall CDH patients appear not to have an important reduction of total lung capacity in their school-and adolescence years [66,68,69].…”

Section: Pulmonary Functionmentioning

confidence: 97%

“…Spirometric testing of CHD patients revealed evidence of obstructive airway disease [64] when compared with healthy controls [48,62,64,[66][67][68][69]. Some authors describe that one-third of the CDH patients have reduced forced expiratory volume in 1 s (FEV 1 ) and forced vital capacity (FVC) [62,69].…”

Section: Pulmonary Functionmentioning

confidence: 99%

“…It has been speculated that there must be some degree of emphysema because of the finding of a high residual volume total lung capacity (RV/TLC) ratio, despite the lack of airway dysfunction [66]. Others suggest that the high RV/TLC ratio (index for the amount of trapped air after full exhalation) is associated with chest wall deformity [68]. Trachsel et al demonstrated that CDH patients with a chest wall deformity (moderate to severe pectus excavatum) had a significantly higher RV/TLC ratio (40.4 vs. 27.9%).…”

Section: Pulmonary Functionmentioning

confidence: 99%

“…The diffusion capacity (i.e. rate at which a gas diffuses through the alveolar-capillary membrane) corrected for alveolar volume (DLCO/V 0 A) however, appears to be normal in CDH patients compared to healthy subjects [68,69] suggesting that the alveolar membrane is not damaged [69].…”

Section: Pulmonary Functionmentioning

confidence: 99%

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The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

et al. 2008

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Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a mortality rate of approximately 40-50%, depending on case selection. It has been suggested that new therapeutic modalities such as nitric oxide (NO), high frequency oxygenation (HFO) and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation. When these new therapies indeed prove to be beneficial, a larger number of children with severe forms of CDH might survive, resulting in an increase of CDH-associated complications and/or consequences. In follow-up studies of infants born with CDH, many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. CDH has also been associated with persistent pulmonary vascular abnormalities, resulting in pulmonary hypertension in the neonatal period.

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Section: Pulmonary Functionmentioning

confidence: 94%

Section: Pulmonary Functionmentioning

confidence: 97%

Section: Pulmonary Functionmentioning

confidence: 99%

Section: Pulmonary Functionmentioning

confidence: 99%

Section: Pulmonary Functionmentioning

confidence: 99%

See 3 more Smart Citations

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

et al. 2008

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Congenital diaphragmatic hernia in 22q11.2 deletion syndrome

Unolt

DiCairano

Schlechtweg

et al. 2016

American J of Med Genetics Pt A

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We report the important association of congenital diaphragmatic hernia (CDH) and 22q11.2 deletion syndrome (22q11.2DS). The prevalence of CDH in our cohort of patients with 22q11.2DS is 0.8% (10/1246), which is greater than in the general population (0.025%). This observation suggests that 22q11.2DS should be considered when a child or fetus presents with CDH, in particular when other clinical findings associated with the 22q11.2DS are present, such as congenital cardiac defects. Furthermore, this finding may lead to the identification of an additional locus for diaphragmatic hernia in the general population. © 2016 Wiley Periodicals, Inc.

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Antenatal and postnatal lung and vascular anatomic and functional studies in congenital diaphragmatic hernia: Implications for clinical management

Keller

2007

American J of Med Genetics Pt C

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Congenital diaphragmatic hernia is characterized by fetal and neonatal lung hypoplasia as well as vascular hypoplasia. Antenatal imaging studies have been performed that attempt to quantify the degree of hypoplasia and its impact on infant prognosis. Prenatal and perinatal growth of the lung and vasculature are interdependent and their continued coordinated growth is critical for survival after birth in this patient population. Lung protection strategies appear to improve survival in newborns with diaphragmatic hernia, but a subset of infants remain who demonstrate sufficiently severe lung hypoplasia that we are unable to provide support long-term after birth. Fetal intervention is a strategy designed to enhance fetal lung growth towards improving survival in this most severely affected group, though other therapies to enhance postnatal lung and vascular growth should be concurrently investigated. However, any of these interventions will require careful selection of those infants at risk for poor outcome and thorough follow up, since long-term morbidity is significant in children with diaphragmatic hernia.

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Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia

Cited by 139 publications

References 33 publications

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

Congenital diaphragmatic hernia in 22q11.2 deletion syndrome

Antenatal and postnatal lung and vascular anatomic and functional studies in congenital diaphragmatic hernia: Implications for clinical management

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