Long term respiratory outcomes of congenital diaphragmatic hernia, esophageal atresia, and cardiovascular anomalies

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Summary

Intrathoracic congenital malformations may be associated with long-term pulmonary morbidity. This certainly is the case for congenital diaphragmatic hernia, esophageal atresia and cardiac and aortic arch abnormalities. These conditions have variable degrees of impaired development of both the airways and lung vasculature, with a postnatal impact on lung function and bronchial reactivity. Pulmonary complications are themselves frequently associated to non-pulmonary morbidities, including gastrointestinal and orthopaedic complications. These are best recognized in a structured multidisciplinary follow-up clinic so that they can be actively managed.

Introduction

Lung development is a lengthy process, starting during fetal life, and continuing in the early postnatal years. A wide range of pathologies may interfere with normal lung development, some of them leading to permanent sequelae. In this article the long term pulmonary consequences of three intra-thoracic malformations are discussed, all of them potentially being diagnosed in the prenatal period.

Section snippets

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) occurs sporadically with an incidence of 1 in 2200 live births, which means that in the EU-27 (5 421 000 births per annum) one baby with CDH is born every 4 h. The vast majority of cases are left-sided, 13% are right-sided, and bilateral lesions, complete agenesis and other rarities comprise <2%. The presence of associated anomalies, which occurs in ∼40%, is an independent predictor of neonatal death. In isolated CDH, several tertiary centers report a rate

Esophageal atresia

Esophageal atresia (EA) is an uncommon malformation with an estimated incidence of one in 3000 (Figure 3). It is often associated with tracheo-esophageal fistula (TEF). Suggestive prenatal signs are the absence of a small stomach bubble, pouch sign (transient filling of the proximal esophagus); late onset polyhydramnios because of swallowing problems quite frequently raises suspicion. The condition is often associated with intrauterine growth restriction. As other pathologies are often

Pulmonary outcome in congenital cardiac and aortic arch anomalies

Congenital cardiac and aortic arch anomalies are often associated with pulmonary complications that may persist despite partial or complete surgical repair. These complications are heterogeneous, but dominated by airway compression, altered lung function, and increased airway responsiveness.

Airway compression is a relatively common complication of congenital cardiac and aortic arch anomalies. The obstruction may be the result of an anomalous relationship between the tracheobronchial tree and

Conflict of interest statement

None declared.

Funding sources

JD, JT and MR are supported by the European Commission FP 6 (eurostec LSHC-CT-2006-037409) and FP7 Marie Curie Industria-Academia Partnership and Pathways (251356), the Fonds Wetenschappelijk Onderzoek Vlaanderen (1.8.012.07.N.02).

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