Characterisation of computed tomography angiography findings in paediatric patients with heterotaxy
Carlos F Ugas Charcape1, Larry R Alpaca Rodriguez2, Irma A Matos Rojas2
1Department of Diagnostic Imaging, Instituto Nacional de Salud del Niño San Borja, Av. Javier Prado Este 3101, 15037, Lima, Peru. cugas@insnsb.gob.pe.
Insights
Heterotaxy, a condition of abnormal organ arrangement, often involves complex heart defects. CT angiography effectively visualizes these cardiovascular and extracardiac anomalies in children, aiding diagnosis.
Area of Science:
- Medical Imaging
- Pediatric Cardiology
- Radiology
Background:
- Heterotaxy is a congenital condition characterized by abnormal left-right organ positioning.
- It is frequently associated with complex congenital heart disease.
Purpose of the Study:
- To characterize cardiac and extracardiac findings in pediatric heterotaxy using CT angiography.
- To compare findings between different types of isomerism.
Main Methods:
- Retrospective analysis of 64 pediatric patients with heterotaxy.
- Evaluation using CT angiography within a 52-month period.
Main Results:
- Right atrial isomerism (69%) and left atrial isomerism (28%) were identified.
- Common cardiac defects included complete atrioventricular canal (80%) and total anomalous pulmonary venous return (67%).
- Specific extracardiac findings correlated with isomerism type, such as asplenia with right isomerism and polysplenia with left isomerism.
Conclusions:
- CT angiography is valuable for assessing cardiovascular and extracardiac manifestations of heterotaxy.
- Heterotaxy presents with diverse anatomical variations and side-related findings.
Background:
Heterotaxy refers to the abnormal arrangement of organs across the left-right axis and is typically associated with complex cardiovascular malformations.
Objective:
To characterise the range of cardiac and extracardiac CT angiography findings in children with heterotaxy using the latest nomenclature consensus and to compare the different types of isomerism.
Materials And Methods:
We retrospectively analysed the data of 64 consecutive paediatric patients referred to our tertiary paediatric cardiovascular centre who underwent CT angiography for the evaluation of known or suspected heterotaxy within a 52-month period.
Results:
Right atrial isomerism was identified in 44 (69%) children, while left atrial isomerism was identified in 18 (28%) children. Atrial appendage anatomy and situs could not be determined in 2 children (3%). Associated heart defects included complete atrioventricular canal (CAVC) in 51 (80%) children, total anomalous pulmonary venous return in 43 (67%) and pulmonary atresia in 23 (36%). The bronchial branching pattern corresponded to atrial appendage morphology in all children except in the two in whom atrial appendage morphology could not be defined. In children with right atrial isomerism, the most common associated abnormalities were CAVC (n=41, 93%) and asplenia (n=34, 77%), while in those with left atrial isomerism, the most common associated abnormalities were polysplenia (n=17, 94%) and an interrupted inferior vena cava with azygos continuation (n=15, 83%).
Conclusion:
CT angiography provides useful cardiovascular and extracardiac data on heterotaxy, which frequently involves a pattern of side-related findings but has great anatomical variability.
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