Prevalence and Association of Congenital Heart Disease with Hirschsprung’s Disease
DOI:
https://doi.org/10.33192/smj.v76i9.268293Keywords:
Hirschsprung, cardiac screening, neurocristopathy, echocardiographyAbstract
Objective: Neurocristopathies play a role as pathogenesis of Hirschsprung’s disease and congenital heart diseases (CHDs). This study seeks to identify concomitant deformities, syndromes, and/or associations associated with Hirschsprung’s disease warrant evaluation for CHDs through echocardiography.
Materials and Methods: A retrospective analysis was conducted on Hirschsprung’s disease patients at Siriraj Hospital between January 2006 and December 2022. Echocardiograms were performed when clinical symptoms, abnormal chest X-rays (CXR), desaturation, heart murmurs raised suspicions of cardiovascular anomalies.
Results: Among 299 Hirschsprung’s disease patients, 43 (14.4%) exhibited CHDs. The sensitivity of CXR (n=268) and echocardiograms (n=51) in diagnosing CHDs was 48.8% and 100%, respectively. Predominant CHD presentations included patent ductus arteriosus (n=29), atrial septal defects (n=18), and ventricular septal defects (n=15). The presence of concomitant deformities, syndromes and/or associations associated with Hirschsprung’s disease significantly heightened the likelihood of concurrent CHDs (Odds ratio = 23.56, p < 0.001). Patients with Hirschsprung’s disease and concomitant deformities (n=28) (excluding syndromic or chromosomal abnormalities) had 1.73 times the odds of CHDs (p = 0.262) compared to those without concomitant deformities. Patients with Hirschsprung’s disease and Down syndrome (n=34) exhibited 77.78 times higher odds of CHDs (p < 0.001), while those with other syndromes and/or associations (n=6) had 13.03 times higher odds of CHDs (p = 0.005) compared to patients lacking these conditions.
Conclusion: CHDs were identified in 14.4% of Hirschsprung’s disease patients. Echocardiograms should be selectively employed in Hirschsprung’s disease associated with Down syndrome, other syndromes, or concomitant deformities.
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