title: Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography.
abstract: Heterotaxy and situs abnormalities describe an abnormal arrangement of visceral organs in the thoracoabdominal cavity across the normal left-right axis of the body. It is associated with a high occurrence of congenital heart and abdominal defects, including anomalous pulmonary venous connections, systemic venous abnormalities, asplenia, and intestinal malrotation. Without proper diagnosis and surgical intervention, the prognosis of patients with heterotaxy syndrome and associated congenital defects is extremely poor. Complex intracardiac and extracardiac lesions are common in heterotaxy and can be difficult to assess by echocardiography. CT angiography (CTA) is a useful tool in this setting to accurately assess intracardiac and extracardiac abnormalities in this population for medical or surgical management. The intention of this pictorial essay is to review the most common cardiovascular defects involved with heterotaxy syndrome in addition to emphasizing the utility of CTA in the identification and classification of anomalies seen in these patients. This review briefly defines most common terminology used in situs abnormalities as well as presents CT images and 3-dimensional reconstructions of common anomalies associated with situs abnormalities. In summary, this review should prepare radiologists and pediatric cardiologists to describe heterotaxy and situs abnormalities in addition to recognizing the utility of CTA in these patients.
authors: Department of Radiology and Radiological Science, Medical University of South, Carolina, Ashley River Tower, MSC 226, 25 Courtenay Dr, Charleston, SC 29425, USA.
authors: Christopher D
authors: CD
authors: Wolla
authors: Department of Radiology and Radiological Science, Medical University of South, Carolina, Ashley River Tower, MSC 226, 25 Courtenay Dr, Charleston, SC 29425, USA; Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South, Carolina, Charleston, SC, USA.
authors: Anthony M
authors: AM
authors: Hlavacek
authors: Department of Radiology and Radiological Science, Medical University of South, Carolina, Ashley River Tower, MSC 226, 25 Courtenay Dr, Charleston, SC 29425, USA; Department of Medicine, Medical University of South Carolina, Charleston, SC, USA. Electronic address: schoepf@musc.edu.
authors: U Joseph
authors: UJ
authors: Schoepf
authors: Department of Radiology and Radiological Science, Medical University of South, Carolina, Ashley River Tower, MSC 226, 25 Courtenay Dr, Charleston, SC 29425, USA; Department of Diagnostic and Interventional Radiology, University Hospital Frankfurt, Frankfurt am Main, Frankfurt, Germany.
authors: Andreas M
authors: AM
authors: Bucher
authors: Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South, Carolina, Charleston, SC, USA.
authors: Shahryar
authors: S
authors: Chowdhury
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D002648:Child; D017023:Coronary Angiography / Q000379:methods*; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging*; D059446:Heterotaxy Syndrome / Q000000981:diagnostic imaging*; D006801:Humans; D007231:Infant, Newborn; D014057:Tomography, X-Ray Computed / Q000379:methods*
keywords: CT angiography; Heterotaxy; Isomerism; Situs abnormalities; Situs ambiguus; Situs inversus