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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)
- Abnormality wherein internal thoracoabdominal organs demonstrate abnormal arrangement across left-right axis of body
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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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)
- Abnormality wherein internal thoracoabdominal organs demonstrate abnormal arrangement across left-right axis of body
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
