Terminology is confusing with multiple terms for similar anatomic combinations
Listed below are terms used for certain combinations of findings (not necessarily synonymous)
Right atrial appendage (RA) isomerism
Asplenia
Bilateral right sidedness
Left atrial appendage (LA) isomerism
Polysplenia
Bilateral left sidedness
Definitions
Abnormality wherein internal thoracoabdominal organs demonstrate abnormal arrangement across left-right axis of body
Implies that laterality of internal organs is neither situs solitus (normal) nor situs inversus (mirror image of situs solitus)
IMAGING
General Features
Ultrasonographic Findings
Echocardiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Liu H et al: Next-generation sequencing in a series of 80 fetuses with complex cardiac malformations and/or heterotaxy. Hum Mutat. 41(12):2167-78, 2020
Buca DIP et al: Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 51(3):323-30, 2018
Gottschalk I et al: Extracardiac anomalies in prenatally diagnosed heterotaxy syndromes. Ultrasound Obstet Gynecol. 47(4):443-9, 2016
Khan MS et al: Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. 99(6):2134-9, 2015
Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
Anagnostopoulos PV et al: Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg. 35(5):871-7; discussion 877-8, 2009
Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Heterotaxy syndrome
Visceral heterotaxy
Situs ambiguous
Terminology is confusing with multiple terms for similar anatomic combinations
Listed below are terms used for certain combinations of findings (not necessarily synonymous)
Right atrial appendage (RA) isomerism
Asplenia
Bilateral right sidedness
Left atrial appendage (LA) isomerism
Polysplenia
Bilateral left sidedness
Definitions
Abnormality wherein internal thoracoabdominal organs demonstrate abnormal arrangement across left-right axis of body
Implies that laterality of internal organs is neither situs solitus (normal) nor situs inversus (mirror image of situs solitus)
IMAGING
General Features
Ultrasonographic Findings
Echocardiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Liu H et al: Next-generation sequencing in a series of 80 fetuses with complex cardiac malformations and/or heterotaxy. Hum Mutat. 41(12):2167-78, 2020
Buca DIP et al: Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 51(3):323-30, 2018
Gottschalk I et al: Extracardiac anomalies in prenatally diagnosed heterotaxy syndromes. Ultrasound Obstet Gynecol. 47(4):443-9, 2016
Khan MS et al: Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. 99(6):2134-9, 2015
Kothari SS: Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol. 7(3):187-92, 2014
Anagnostopoulos PV et al: Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg. 35(5):871-7; discussion 877-8, 2009
Cohen MS et al: Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. 17 Suppl 2:29-43, 2007
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