Prevalence of Cardiac Anomalies in Children with Syndromic and Non-syndromic Craniosynostosis

Craniosynostosis mostly occurs as a single abnormality; however, it may rarely occur concomitantly with other congenital abnormalities known as syndromic craniosynostosis. Cardiac anomalies are among the most common ones occurring coincidentally with craniosynostosis. Nevertheless, the information about the exact prevalence of cardiac anomalies in craniosynostosis has not been well-understood yet. We aimed to assess the prevalence of different cardiac anomalies that coincidence with craniosynostosis.

This cross-sectional study was done on 145 patients with craniosynostosis from January 2015 to December 2019. 103 patients with a single-suture involvement were placed in the non-syndromic group, and the remaining 42 with pansynostosis or the clinical manifestations of Apert, Pfeiffer, Crouzon, and Carpenter syndromes in the syndromic group. The prevalence of cardiac anomalies was evaluated and compared between the groups.

The prevalence of congenital cardiac anomalies was 22.3% and 50% in non-syndromic and syndromic cases, respectively (P = 0.001). Syndromic boys predominantly presented cardiac anomalies (P = 0.85), whereas non-syndromic girls were mostly affected by cardiac anomalies (P = 0.75). Age was not associated with congenital cardiac anomalies, neither in non-syndromic (P = 0.31) nor in syndromic (P = 0.26) patients. The number of affected sutures was not associated with cardiac anomalies (P > 0.05). Tricuspid regurgitation (TR) (16.7%), patent ductus arteriosus (PDA) (14.3%), and ventricular septal defect (VSD) (11.9%) were the most prominent anomalies found among the syndromic patients, while TR (8.7%), atrial septal defect (ASD) (3.9%), and PDA (3.9%) were common among the non-syndromic ones.

Congenital heart disease is a prevalent abnormality among children with craniosynostosis. Therefore, cardiac assessment in craniosynostosis is strongly recommended.