Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

Stefanutti, Giorgio; Filippone, Marco; Tommasoni, Nicola; Midrio, Paola; Zucchetta, Pietro; Moreolo, Giorgio Svaluto; Toffolutti, Tiziana; Baraldi, Eugenio; Gamba, Piergiorgio

doi:10.1016/j.jpedsurg.2003.12.006

Cited by 80 publications

(74 citation statements)

References 34 publications

(27 reference statements)

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“…The electrocardiogram showed evidence for right ventricular hypertrophy in six of the 14 children; however, this could also be related to cardiac malposition due to the underlying pulmonary hypoplasia. In a group of 24 survivors of mild to moderate CDH, studied by Stefanutti et al [63], no echocardiographic signs of pulmonary hypertension were found. Recently a nearly normal resting and exercise cardiorespiratory function has been reported in CDH survivors aged 10-16 years without evidence for exercise-induced pulmonary hypertension measured by echocardiography and maximal exercise testing [72].…”

Section: Pulmonary Hypertension and Follow-upmentioning

confidence: 89%

“…Bronchodilators and inhaled steroids were, at least transiently, used by the majority of CDH patients, during the first year of life and thereafter [61,62]. 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: 95%

“…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%

“…A negative correlation has been demonstrated between the duration of ventilation and pulmonary function at follow-up [63,69], illustrated by the fact that CDH patients who had been ventilated for more than 7 days had significantly lower FEV 1 and vital capacity (VC) than CDH patients who had been ventilated for less than 7 days [69]. This could reflect the severity of the pulmonary disease [63], but can also be the consequence of ventilator induced barotrauma.…”

Section: Pulmonary Functionmentioning

confidence: 99%

“…This could reflect the severity of the pulmonary disease [63], but can also be the consequence of ventilator induced barotrauma. Sakurai et al [49] found microscopic evidence of significant pulmonary injury related to the pressure effects of mechanical ventilation in deceased infants with CDH, implying destruction of the alveolar-capillary membrane.…”

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 Hypertension and Follow-upmentioning

confidence: 89%

Section: Fetal Tracheal Occlusionmentioning

confidence: 95%

Section: Pulmonary Functionmentioning

confidence: 99%

Section: Pulmonary Functionmentioning

confidence: 99%

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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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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Functional echocardiography in the fetus with non-cardiac disease

et al. 2013

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We describe the hemodynamic changes observed in fetuses with extra cardiac conditions such as intrauterine growth restriction, tumors, twin-twin transfusion syndrome, congenital infections, and in fetuses of mothers with diabetes. In most fetuses with mild extra cardiac disease, the alterations in fetal cardiac function remain subclinical. Cardiac function assessment has however helped us to achieve a better understanding of the pathophysiology of these diseases. In fetuses at the more severe end of the disease spectrum, functional echocardiography may help in guiding clinical decision-making regarding the need for either delivery or fetal therapy. The growth-restricted fetus represents a special indication for routine cardiac function assessment, as in utero hemodynamic changes may help optimize the timing of delivery. Moreover, in intrauterine growth restriction, the altered hemodynamics causes cardiovascular remodeling, which can result in an increased risk of postnatal cardiovascular disease.

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Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

Cited by 80 publications

References 34 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

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

Functional echocardiography in the fetus with non-cardiac disease

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