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Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy.

Loomba R, Shah PH, Anderson RH - Cureus (2015)

Bottom Line: One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs.Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations.In this review, we discuss how these findings can be demonstrated using fetal MRI.

View Article: PubMed Central - HTML - PubMed

Affiliation: Cardiology Dept., Children's Hospital of Wisconsin.

ABSTRACT
Magnetic resonance imaging (MRI) is increasingly used as an investigation during fetal life, particularly for assessment of intracranial masses, congenital diaphragmatic hernia, myelomeningocele, and abdominal masses. As the number of scans increases, so is the variety of congenital malformations being recognized. It is axiomatic that interpretation of the findings is enhanced when attention is paid to the likely findings in the setting of known syndromes, this information then dictating the need for additional acquisition of images. One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs. Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations. In this review, we discuss how these findings can be demonstrated using fetal MRI.

No MeSH data available.


Related in: MedlinePlus

Congenital malformations of the heartBalanced turbo field (bTFE) images of a 34-week gestational age fetus. Panel A demonstrates the aorta arising from the right ventricle as demonstrated by the anterior location of the ventricular mass. The pulmonary artery arises from the left ventricle as demonstrated by the posterior location of the ventricular mass. Panel B is an axial slice demonstrating a ventricular septal defect. Image reprinted without change from Dong, et al. under the creative commons license.
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FIG13: Congenital malformations of the heartBalanced turbo field (bTFE) images of a 34-week gestational age fetus. Panel A demonstrates the aorta arising from the right ventricle as demonstrated by the anterior location of the ventricular mass. The pulmonary artery arises from the left ventricle as demonstrated by the posterior location of the ventricular mass. Panel B is an axial slice demonstrating a ventricular septal defect. Image reprinted without change from Dong, et al. under the creative commons license.

Mentions: Cardiovascular malformations in the setting of heterotaxy can vary from simple to complex. While complex cardiovascular malformations can be found with either right or left isomerism, they are more frequently observed with right isomerism. Atrioventricular septal defects and double outlet right ventricle are frequently noted with right isomerism, and ventricular imbalance may often necessitate a univentricular approach to palliation. Other lesions, such as Tetralogy of Fallot (Figure 11), may also be seen. Pulmonary venous connections are, by definition, always anomalous in the setting of right isomerism, even if the pulmonary veins return to the heart. This is because the connections must be anatomically anomalous in the setting of isomeric right atrial appendages. In about half of the cases with right isomerism, nonetheless, the pulmonary veins will drain into an extracardiac confluence. Left isomerism is typically associated with septal defects, coarctation of the aorta, and interruption of the inferior caval vein with azygos continuation (Figures 9, 11). The pulmonary veins in this setting are often connected to the heart in symmetrical fashion. A left-sided superior caval vein may be present with either right or left isomerism (Figures 11-12). In right isomerism, however, the vein will drain to the roof of the left-sided atrium, whereas in left isomerism, it typically drains through the coronary sinus. A right-sided aortic arch may also be present (Figure 12). Discordant ventriculoarterial connections can be found with either variant, but are more common with right isomerism (Figure 13). Left-handed, rather than right-handed, ventricular topology can also be found with either variant. The heart may be in either the left or right chest, while the cardiac apex may point leftward or rightward (Figure 14) [22, 24, 45].


Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy.

Loomba R, Shah PH, Anderson RH - Cureus (2015)

Congenital malformations of the heartBalanced turbo field (bTFE) images of a 34-week gestational age fetus. Panel A demonstrates the aorta arising from the right ventricle as demonstrated by the anterior location of the ventricular mass. The pulmonary artery arises from the left ventricle as demonstrated by the posterior location of the ventricular mass. Panel B is an axial slice demonstrating a ventricular septal defect. Image reprinted without change from Dong, et al. under the creative commons license.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4494530&req=5

FIG13: Congenital malformations of the heartBalanced turbo field (bTFE) images of a 34-week gestational age fetus. Panel A demonstrates the aorta arising from the right ventricle as demonstrated by the anterior location of the ventricular mass. The pulmonary artery arises from the left ventricle as demonstrated by the posterior location of the ventricular mass. Panel B is an axial slice demonstrating a ventricular septal defect. Image reprinted without change from Dong, et al. under the creative commons license.
Mentions: Cardiovascular malformations in the setting of heterotaxy can vary from simple to complex. While complex cardiovascular malformations can be found with either right or left isomerism, they are more frequently observed with right isomerism. Atrioventricular septal defects and double outlet right ventricle are frequently noted with right isomerism, and ventricular imbalance may often necessitate a univentricular approach to palliation. Other lesions, such as Tetralogy of Fallot (Figure 11), may also be seen. Pulmonary venous connections are, by definition, always anomalous in the setting of right isomerism, even if the pulmonary veins return to the heart. This is because the connections must be anatomically anomalous in the setting of isomeric right atrial appendages. In about half of the cases with right isomerism, nonetheless, the pulmonary veins will drain into an extracardiac confluence. Left isomerism is typically associated with septal defects, coarctation of the aorta, and interruption of the inferior caval vein with azygos continuation (Figures 9, 11). The pulmonary veins in this setting are often connected to the heart in symmetrical fashion. A left-sided superior caval vein may be present with either right or left isomerism (Figures 11-12). In right isomerism, however, the vein will drain to the roof of the left-sided atrium, whereas in left isomerism, it typically drains through the coronary sinus. A right-sided aortic arch may also be present (Figure 12). Discordant ventriculoarterial connections can be found with either variant, but are more common with right isomerism (Figure 13). Left-handed, rather than right-handed, ventricular topology can also be found with either variant. The heart may be in either the left or right chest, while the cardiac apex may point leftward or rightward (Figure 14) [22, 24, 45].

Bottom Line: One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs.Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations.In this review, we discuss how these findings can be demonstrated using fetal MRI.

View Article: PubMed Central - HTML - PubMed

Affiliation: Cardiology Dept., Children's Hospital of Wisconsin.

ABSTRACT
Magnetic resonance imaging (MRI) is increasingly used as an investigation during fetal life, particularly for assessment of intracranial masses, congenital diaphragmatic hernia, myelomeningocele, and abdominal masses. As the number of scans increases, so is the variety of congenital malformations being recognized. It is axiomatic that interpretation of the findings is enhanced when attention is paid to the likely findings in the setting of known syndromes, this information then dictating the need for additional acquisition of images. One such syndrome is so-called "visceral heterotaxy", in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs. Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations. In this review, we discuss how these findings can be demonstrated using fetal MRI.

No MeSH data available.


Related in: MedlinePlus