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Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome

 Yong Kook Hong  ;  Young Whan Park  ;  Sok Jong Ryu  ;  Je Whan Won  ;  Jae Young Choi  ;  Jun Hee Sul  ;  Sung Kyu Lee  ;  Bum Koo Cho  ;  Kyu Ok Choe 
 Journal of Computer Assisted Tomography, Vol.24(5) : 671-682, 2000 
Journal Title
 Journal of Computer Assisted Tomography 
Issue Date
Abnormalities, Multiple/pathology* ; Angiocardiography ; Cardiac Catheterization ; Child, Preschool ; Echocardiography ; Female ; Heart Atria/abnormalities ; Heart Defects, Congenital/complications ; Heart Defects, Congenital/pathology* ; Heart Defects, Congenital/surgery ; Humans ; Magnetic Resonance Imaging* ; Male ; Viscera/abnormalities*
PURPOSE: The authors' goal was to assess the diagnostic accuracy and clinical effect of MRI compared with echocardiography and catheterization in the evaluation of cardiac defects with situs ambiguous. METHOD: Twenty-two patients with visceral heterotaxy syndrome were included. RESULTS: Because situs determined by the relation between the pulmonary artery and bronchi showed most predominantly a tendency toward lateralization, this was regarded as the standard reference of situs determination. For the purpose of this study, patients were classified as having right isomerism (n = 13) or left isomerism groups (n = 9). MRI has several advantages compared with echocardiography or cardiac angiography for examining patients with situs ambiguous. (1) The bronchial, pulmonary arterial, and splenic situs can be readily determined, and discrepancies (n = 2) can be assessed easily. (2) Venoatrial connections are adequately imaged. In particular, all types of total and partial anomalous pulmonary venous return are delineated, regardless of whether restrictions of pulmonary blood flow or pulmonary venous obstructions are involved (n = 4). The courses of vertical veins were easily identified, and the prearterial position was revealed in only one of seven right isomerisms with total anomalous pulmonary venous return. The drain pattern of the hepatic vein can be visualized using three-dimensional spatial information and is useful for total cavopulmonary connection design. (3) Associated complicated cardiac anomalies, particularly the size or peripheral stenosis of the pulmonary arteries, may be evaluated, and this information is useful for palliative shunt operations. CONCLUSION: Because of its wide field of view and imaging, which is not restricted by associated anomalies, a thorough understanding of the cardiovascular anatomy of the situs ambiguous can be achieved using MRI, which is of considerable value in the surgical correction of this complicated anomaly. MRI can obviate or facilitate catheterization in these critically ill patients.
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1. College of Medicine (의과대학) > Dept. of Thoracic and Cardiovascular Surgery (흉부외과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Park, Young Hwan(박영환) ORCID logo https://orcid.org/0000-0001-9802-8017
Choi, Jae Young(최재영) ORCID logo https://orcid.org/0000-0002-1247-6669
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