ReviewIndian journal of thoracic and cardiovascular surgery2021
The heterotaxy syndrome: associated congenital heart defects and management.
Review in Indian journal of thoracic and cardiovascular surgery, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 46 citations in OpenAlex.
- Biallelic DAW1 variants reveal a tissue-specific role in heterotaxy without primary ciliary dyskinesia.European journal of human genetics : EJHG · 2026Article
- Functional genomics iniScience · 2026Article
- Laparoscopic transhiatal management of a rare case of esophageal lung and medialized stomach associated with heterotaxy - a technical case report.International journal of surgery case reports · 2026Article
- Compound heterozygous DAW1 variants reveal tissue-specific roles in left-right patterning and congenital heart disease without primary ciliary dyskinesia.medRxiv : the preprint server for health sciences · 2026Article
- Ivemark syndrome in an adult with complete atrioventricular septal defect and a single ventricle with L-transposition of the great arteries: a case report.Journal of medical case reports · 2026Article
- Successful preterm cesarean delivery in a woman with right atrial isomerism: a case report.BMC pregnancy and childbirth · 2026Article
- Isomerism of the right atrial appendages with bilateral eparterial bronchi: clinical challenges and surgical management guided by 3D cardiac computed tomography.Indian journal of thoracic and cardiovascular surgery · 2025Article
- Molecular and Functional Divergence of Zebrafish Sox Paralogs Controlling Endoderm Formation and Left-Right Patterning.Genome biology and evolution · 2025Article
- Integrative Functional Genomics Identifies ARHGAP10 in the 4q31.2 Locus as a Novel Congenital Heart Disease and Ciliopathy Gene.bioRxiv : the preprint server for biology · 2025Article
- Unilateral Lung Agenesis: A Systematic Review of Prevalence, Anatomical Variants, and Clinical Implications.Diagnostics (Basel, Switzerland) · 2025Review
- Article
- Addressing immunization gaps in children with congenital heart disease - a narrative review.Medicine and pharmacy reports · 2025Review
- Unilateral Lung Agenesis and Left Isomerism in a Neonate: A Case Report of Multi-system Anomalies.Cureus · 2025Article
- The Heterotaxy Gene CCDC11 Is Important for Cytokinesis via RhoA Regulation.Cytoskeleton (Hoboken, N.J.) · 2025Article
- What Happens If Hepatic Veins Drain to the Pulmonary Venous Atrium?: Depends on the Physiology!JACC. Case reports · 2025Article
- Coexistence of coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear anomalies (CHARGE) syndrome and heterotaxy in a newborn with athymia.The journal of allergy and clinical immunology. Global · 2025Article
- Pheochromocytoma in a patient with heterotaxy syndrome: a case report.BMC endocrine disorders · 2025Article
- Heterotaxy syndrome, dextrocardia, ureteropelvic obstruction, endometriosis, and pulmonary hypertension in an adult with congenital heart defects: a case report.Journal of medical case reports · 2025Article
- Heterotaxy syndrome with biliary atresia: a case report.The Pan African medical journal · 2025Article
- Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Heterotaxy syndrome (HS) constitutes a spectrum of anomalies arising from embryological errors that result in abnormalities of lateralization involving thoraco-abdominal viscera and culminate in loss of normal asymmetric arrangement of these organs. Besides the unique challenges involved in planning and execution of surgical procedures aimed at correction or palliation of these anomalies, they have the potential to cause profound physiological and immunological consequences in the individual patient due to their cardiac and extra-cardiac manifestations. This article aims to review the literature on this rare and extraordinary subset of developmental anomalies with the intention of familiarizing the reader on the modes of presentation, manifestations, and the variations thereof while dealing with this anomaly. In our institutional experience with HS, 75 consecutive patients were seen between January 2011 and September 2018. Of these, 48 (64%) were confirmed to have isomerism of right atrial appendages (IRAA) and the rest had isomerism of left atrial appendages (ILAA). The cardiac and extra-cardiac manifestations of these patients were listed out. Fifty-four patients (34 with IRAA and 20 with ILAA) underwent 83 surgical procedures. While 49 patients were palliated on the univentricular pathway, 5 underwent biventricular repair. The in-hospital mortality was 7 (13%) in both groups combined (5 for patients with IRAA and 2 for ILAA). In conclusion, the surgical management of HS is associated with satisfactory outcomes in current era.
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