The relationship of pulmonary artery pressure and survival in congenital diaphragmatic hernia

Dillon, Peter W.; Cilley, Robert E.; Mauger, David T.; Zachary, Christopher; Meier, Andreas H.

doi:10.1016/j.jpedsurg.2003.11.010

Cited by 188 publications

(150 citation statements)

References 16 publications

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“…14 This improvement was not sufficient to reduce mortality or need for ECMO, and appears to be transient. 14,21,22 These studies suggest that early treatment with INO might provide short-term improvement in oxygenation in patients with CDH and severe pulmonary hypertension, and buy neonatologists and pediatric surgeons valuable time in the "bridge" period immediately before ECMO cannulation. However, the safety and efficacy of prolonged INO therapy in patients with CDH has not been thoroughly investigated in studies published to date.…”

Section: Resultsmentioning

confidence: 99%

Inhaled Nitric Oxide Use in Neonates With Congenital Diaphragmatic Hernia

Campbell¹,

Herbst

Briden³

et al. 2014

Pediatrics

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Section: Resultsmentioning

confidence: 99%

Inhaled Nitric Oxide Use in Neonates With Congenital Diaphragmatic Hernia

Campbell¹,

Herbst

Briden³

et al. 2014

Pediatrics

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“…Earlier reports suggest that as much as 25% of the observed mortality in CDH is due to potentially treatable aspects of the underlying pathophysiology. 47,56,57,63,64 Future investigations, either observational cohort studies or randomized clinical trials, should stratify analyses based on the categories of illness severity, including anatomic indices and physiologic responses to therapy, rather than simply 'isolated CDH' or 'CDH with other anomalies'. Determination of the appropriate therapies may be aided by the development of a 'supportive care grid', in which severity of illness and current level of cardio-respiratory support are plotted vs measures of cardiac performance, tissue perfusion and acid-base status.…”

Section: Discussionmentioning

confidence: 99%

“…27 Although there may be short-term benefit from the selective use of iNO, the routine use of iNO for all CDH infants is not supported by results from successful centers. 11,46,47 Furthermore, meta-analysis of randomized clinical trials evaluating the rescue use of iNO in neonates with CDH suggests that iNO may be associated with worse outcomes (that is, higher rates of ECMO use or death). 48 Sedation, paralysis and environment.…”

Section: Congenital Diaphragmatic Hernia Jw Logan Et Almentioning

confidence: 99%

Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies

et al. 2007

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Objectives: Recent reports suggest that specific care strategies improve survival of infants with congenital diaphragmatic hernia (CDH). This review presents details of care from centers reporting high rates of survival among CDH infants.Study Design: We conducted a MEDLINE search (1995 to 2006) and searched all citations in the Cochrane Central Register of Controlled Trials. Studies were included if they contained reports of >20 infants with symptomatic CDH, and >75% survival of isolated CDH.Result: Thirteen reports from 11 centers met inclusion criteria. Overall survival, including infants with multiple anomalies, was 603/763 (79%; range: 69 to 93%). Survival for isolated CDH was 560/661 (85%; range: 78 to 96%). The frequency of extracorporeal membrane oxygenation (ECMO) use for isolated CDH varied widely among reporting centers 251/622 (40%; range: 11 to 61%), as did survival for infants with isolated CDH placed on ECMO: 149/206 (73%; range: 33 to 86%). There was no suggestion of benefit from use of antenatal glucocorticoids given after 34 weeks gestation or use of postnatal surfactant. Low mortality was frequently attributed to minimizing lung injury and adhering to center-specific criteria for ECMO.Conclusion: Use of strategies aimed at minimizing lung injury, tolerance of postductal acidosis and hypoxemia, and adhering to center-specific criteria for ECMO were strategies most consistently reported by successful centers. The literature lacks randomized clinical trials of these or other care strategies in this complex patient population; prospective studies of safety and long-term outcome are needed. Keywords: congenital diaphragmatic hernia; pulmonary hypertension; pulmonary hypoplasia; inhaled nitric oxide; extracorporeal membrane oxygenation; systematic review Introduction Congenital diaphragmatic hernia (CDH) is a rare malformation observed in approximately 1 in 3000 live births, with estimates of postnatal survival ranging from 50 to 70% despite advances in neonatal care. 1 The combination of pathophysiologic derangements associated with CDH, including lung hypoplasia, lung dysmaturity and pulmonary hypertension (PPHN), requires a multidisciplinary approach to care. 2,3 The complex nature of clinical management and the relatively infrequent presentation of infants with this rare malformation to single centers has lead to difficulty identifying consistent strategies for CDH management. A recent report from the Canadian Neonatal Research Network indicates better than predicted survival in Network centers, with significant improvement in the higher mortality odds strata, and 90% survival in the three centers that cared for at least 12 infants over a 22-month period. Details of management strategies are not provided, but the report suggests that survival is best achieved by caring for CDH infants at centers that have developed a standardized approach to care, with adequate numbers of CDH infants to develop expertise. 4 Literature review reveals a limited number of controlled clinical trials examining inter...

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“…As stated before, vascular abnormalities might contribute to the development of pulmonary hypertension in CDH patients in the neonatal period. In the first 3 weeks of life, pulmonary hypertension resolves spontaneously in almost half of the CDH patients without the need for ECMO [87].…”

Section: Pulmonary Hypertension and Follow-upmentioning

confidence: 99%

“…Furthermore, postoperative intolerance of enteral feedings and prolonged hospitalization were found predominantly in patients with symptomatic GERD [105]. There might [79] or might not [87] be an association between GERD and preoperative thoracic position of the stomach.…”

Section: Prediction Of Gerd In Cdh Survivorsmentioning

confidence: 99%

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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The relationship of pulmonary artery pressure and survival in congenital diaphragmatic hernia

Cited by 188 publications

References 16 publications

Inhaled Nitric Oxide Use in Neonates With Congenital Diaphragmatic Hernia

Inhaled Nitric Oxide Use in Neonates With Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies

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

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