Evaluation of Cardiac Malposition, Cardiac Segmental Approach, and Heterotaxy Syndrome

Cardiac CT is a powerful tool for diagnosis and management of patients with complex congenital heart disease. Utilizing a comprehensive, systematic descriptive scheme is essential to consistently describe features of a wide variety of defects, ensure consistent evaluation from patient to patient, and communicate these abnormalities in a succinct and complete way to multidisciplinary teams. Complete and thorough diagnosis of all cardiac defects and associated lesions allows for optimal interventional planning and counseling of the patient and caregivers. We present our method for describing the wide constellation of anatomic and cardiac defects typically found in complex patients, especially helpful in those with heterotaxy syndrome. The basic components that should be described, when possible, are cardiac position, pulmonary and abdominal (visceral) situs, systemic and pulmonary venous connections, atrial situs (or arrangement), ventricular situs and atrioventricular relationships, outflow tracts and ventriculoarterial alignments, and the great arteries.