Cardiac screening in congenital spine disorders

Children with congenital spine disorders are known to have a high prevalence of congenital heart disease. The purpose of this work is to examine the presentation of these cardiac defects. It was our hypothesis that the anomalies are clinically obvious; thus, screening with electrocardiogram (ECG) or echocardiogram for occult disease would prove unnecessary after orthopaedic referral.

The records and radiographs of 190 consecutive children known to have a congenital vertebral abnormality (excluding myelodysplasia and sacral agenesis) were retrospectively reviewed. Twenty-four children presented to the orthopaedist with a known cardiac anomaly. Of the remaining children, 64 underwent cardiac screening with echocardiogram, 27 underwent screening ECG and pediatric or cardiology evaluation, and 75 underwent evaluation by the orthopaedist only. Level of evidence was case series, level IV.

Twenty-four children presented to the orthopaedist with an established diagnosis of congenital heart disease. One child, who underwent echocardiogram, was detected to have a bicuspid aortic valve. This prevalence is consistent with what would be expected in screening the general population with echocardiogram. None of the 27 children screened with ECG and pediatric or cardiology consultation, and none of the 75 who underwent evaluation only by the orthopaedist were detected to have a cardiac abnormality. Also, no additional cardiac abnormalities have been found with follow-up of 2 to 17 years.

The presentation of congenital heart disease associated with congenital spine disorders is general clinically evident and made before the referral to the orthopaedist. Routine referral by the orthopaedist for ECG or echocardiogram is not supported by this work.