Situs ambiguous, right/left isomerism, cardiosplenic syndromes, Ivemark syndrome
Definitions
Disturbance of normal left-right asymmetry in position of thoracic & abdominal organs
IMAGING
General Features
Radiographic Findings
Echocardiographic Findings
Ultrasonographic Findings
CT Findings
MR Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Murat SN et al: Diagnosis of heterotaxy syndrome in a patient with multiple congenital cardiac malformations using magnetic resonance imaging. Circ Cardiovasc Imaging. 13(9):e010307, 2020
Broda CR et al: Outcomes in adults with congenital heart disease and heterotaxy syndrome: a single-center experience. Congenit Heart Dis. 14(6):885-94, 2019
Buca DIP et al: Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 51(3):323-30, 2018
Cupers S et al: Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract. 8(1):1004, 2018
Mahmood K et al: Heterotaxy syndrome. J Coll Physicians Surg Pak. 28(3):252-3, 2018
Sanders SP et al: Classifying heterotaxy syndrome: time for a new approach. Circ Cardiovasc Imaging. 11(2):e007490, 2018
Moradi B et al: Fetal echocardiographic evaluation in cases of heterotaxy syndrome. J Clin Ultrasound. 45(7):436-7, 2017
Lambert TE et al: Abnormalities of fetal situs: an overview and literature review. Obstet Gynecol Surv. 71(1):33-8, 2016
Teele SA et al: Heterotaxy syndrome: proceedings from the 10th international PCICS meeting. World J Pediatr Congenit Heart Surg. 6(4):616-29, 2015
Hill SJ et al: Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat. J Pediatr Surg. 49(6):934-7; discussion 937, 2014
Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
Newman B et al: Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol. 44(5):542-51, 2014
Wolla CD et al: Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 7(6):408-16, 2013
Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
Danilov T et al: Bilateral left-sidedness heterotaxy syndrome. J Am Coll Cardiol. 58(1):87, 2011
Valsangiacomo Buechel ER et al: Congenital cardiac defects and MR-guided planning of surgery. Magn Reson Imaging Clin N Am. 19(4):823-40; viii, 2011
Evans WN: Thoracoabdominal situs: a practical approach accompanied by a short history of descriptive terms. Pediatr Cardiol. 31(7):1049-51, 2010
Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
Ghosh S et al: Anomalies of visceroatrial situs. AJR Am J Roentgenol. 193(4):1107-17, 2009
Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
Bartram U et al: Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 88(4):278-90, 2005
Hong YK et al: Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. J Comput Assist Tomogr. 24(5):671-82, 2000
Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Situs ambiguous, right/left isomerism, cardiosplenic syndromes, Ivemark syndrome
Definitions
Disturbance of normal left-right asymmetry in position of thoracic & abdominal organs
IMAGING
General Features
Radiographic Findings
Echocardiographic Findings
Ultrasonographic Findings
CT Findings
MR Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Murat SN et al: Diagnosis of heterotaxy syndrome in a patient with multiple congenital cardiac malformations using magnetic resonance imaging. Circ Cardiovasc Imaging. 13(9):e010307, 2020
Broda CR et al: Outcomes in adults with congenital heart disease and heterotaxy syndrome: a single-center experience. Congenit Heart Dis. 14(6):885-94, 2019
Buca DIP et al: Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 51(3):323-30, 2018
Cupers S et al: Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract. 8(1):1004, 2018
Mahmood K et al: Heterotaxy syndrome. J Coll Physicians Surg Pak. 28(3):252-3, 2018
Sanders SP et al: Classifying heterotaxy syndrome: time for a new approach. Circ Cardiovasc Imaging. 11(2):e007490, 2018
Moradi B et al: Fetal echocardiographic evaluation in cases of heterotaxy syndrome. J Clin Ultrasound. 45(7):436-7, 2017
Lambert TE et al: Abnormalities of fetal situs: an overview and literature review. Obstet Gynecol Surv. 71(1):33-8, 2016
Teele SA et al: Heterotaxy syndrome: proceedings from the 10th international PCICS meeting. World J Pediatr Congenit Heart Surg. 6(4):616-29, 2015
Hill SJ et al: Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat. J Pediatr Surg. 49(6):934-7; discussion 937, 2014
Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
Newman B et al: Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol. 44(5):542-51, 2014
Wolla CD et al: Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 7(6):408-16, 2013
Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
Danilov T et al: Bilateral left-sidedness heterotaxy syndrome. J Am Coll Cardiol. 58(1):87, 2011
Valsangiacomo Buechel ER et al: Congenital cardiac defects and MR-guided planning of surgery. Magn Reson Imaging Clin N Am. 19(4):823-40; viii, 2011
Evans WN: Thoracoabdominal situs: a practical approach accompanied by a short history of descriptive terms. Pediatr Cardiol. 31(7):1049-51, 2010
Lapierre C et al: Segmental approach to imaging of congenital heart disease. Radiographics. 30(2):397-411, 2010
Ghosh S et al: Anomalies of visceroatrial situs. AJR Am J Roentgenol. 193(4):1107-17, 2009
Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
Bartram U et al: Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 88(4):278-90, 2005
Hong YK et al: Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. J Comput Assist Tomogr. 24(5):671-82, 2000
Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
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