Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects

Vanamo, Kari; Rintala, Risto J.; Sovijärvi, A.; Jääskeläinen, J; Turpeinen, M.; Lindahl, Harry; Louhimo, I

doi:10.1016/s0022-3468(96)90095-0

Cited by 94 publications

(88 citation statements)

References 14 publications

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“…In the study from Marven et al [66], CDH patients perceived their own fitness to be worse than the healthy control group. Other studies demonstrate that around 20% of interviewed CDH patients consider themselves less fit than their healthy peers [59,65,73]. It seems most likely that the decreased V 0 O 2 max found by Zaccara et al is due to a lower degree of physical fitness.…”

Section: Pulmonary Functionmentioning

confidence: 88%

“…In other studies, however, none of the participating children had significant respiratory symptoms [63,64], suggesting that CDH patients were doing well, regarding long-term pulmonary outcome. Despite the subjective impression that CDH survivors older than 2 years generally were doing well, objective data are scarce, due to a limited number of included patients [62,65].…”

Section: Fetal Tracheal Occlusionmentioning

confidence: 99%

“…It is hypothesized that reduced-lung tissue and consequent alveolar distension may result in early airway closure as found in the aging emphysematous lung, which could be an additional risk factor in the development of chronic obstructive disease on the longer term [48,71]. 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: 99%

“…It has been demonstrated that in survivors of left side hernia repair, mean perfusion of the ipsilateral lung was lower than that in healthy children [48,[62][63][64][65] and in the contralateral lung [48,62,71]. Patients who were ventilated for 4 days or more had poorer perfusion than patients who had required shorter ventilatory support [48].…”

Section: Lung Perfusionmentioning

confidence: 99%

“…It has been suggested that CDH patients with a clinically asymmetric chest and scoliosis are prone for pulmonary function impairment [65,68]. In other CDH patients with pectus excavatum, a reduced lung function compared to normal CDH patients has been demonstrated [70,145].…”

Section: Chest Wall Deformitiesmentioning

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: 88%

Section: Fetal Tracheal Occlusionmentioning

confidence: 99%

Section: Pulmonary Functionmentioning

confidence: 99%

Section: Lung Perfusionmentioning

confidence: 99%

Section: Chest Wall Deformitiesmentioning

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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The lungs in congenital diaphragmatic hernia: Do we understand?

IJsselstijn

Tibboel

1998

Pediatr. Pulmonol.

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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 sequelae in survivors of congenital diaphragmatic defects

Cited by 94 publications

References 14 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

The lungs in congenital diaphragmatic hernia: Do we understand?

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

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