Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia
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In brief
In brief
This CDH Study Group analysis of 6,357 infants found that 7% had isolated aortic arch anomalies, most commonly coarctation. These patients showed higher mortality (58% vs 24%) associated with defect severity rather than aortic pathology itself, as only 17% required aortic intervention.
Written by the GCMD Library team from the article.
Abstract
Purpose
Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients.
Methods
Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality.
Results
Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2–5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65–1.50; p = 0.90). Only 17% (n = 28) of CDH + IAAA patients underwent aortic intervention.
Conclusions
IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.