A rare case of an asymptomatic heterotaxy syndrome associated with dextrocardia is presented in a 45-year-old woman. This anomaly was incidentally discovered on computed tomography and ultrasound of the upper abdomen. Besides the presence of multiple abnormally positioned spleens, right-sided stomach, left-sided liver, short pancreas and venous anomalies, like interrupted left-sided inferior vena cava with azygos continuation, and dextrocardia were seen. Although cardiac abnormalities are frequently associated with heterotaxy syndromes with polysplenia in children, this is far less frequent in adults because of early decease in children with cardiac abnormalities. To the best of our knowledge, this is the third reported case of heterotaxy with polysplenia associated with dextrocardia in an asymptomatic adult patient.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Outcomes of Fontan operation in patients with conotruncal anomalies from Australia and New Zealand Fontan Registry: a review of 1835 patients.
Eur J Cardiothorac Surg
March 2025
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Objectives: The impact of conotruncal anomalies (CTAs), including tetralogy of Fallot, truncus arteriosus, ventriculo-arterial discordance, double outlet right ventricle (DORV), and interrupted aortic arch type B, on long-term outcomes remains poorly described in the Fontan cohort. We sought to review the outcomes of Fontan patients with conotruncal anomalies in Australia and New Zealand.
Methods: We reviewed the data from 1835 patients who underwent a Fontan operation between 1975 and 2023 from the Australia and New Zealand Fontan Registry.
Pheochromocytoma in a patient with heterotaxy syndrome: a case report.
BMC Endocr Disord
March 2025
Advanced Diagnostic and Interventional Radiology Research Center (ADIR), Tehran University of Medical Sciences, Tehran, Iran.
Background: Heterotaxy syndrome is a rare congenital condition characterized by abnormal arrangement of thoracoabdominal organs, often associated with complex cardiac and splenic anomalies. Pheochromocytoma is a rare neuroendocrine tumor that overproduces catecholamines, leading to various complications. The co-occurrence of heterotaxy syndrome and pheochromocytoma has not been previously reported.
View Article and Find Full Text PDFIncidental Detection of Adult Polysplenia Syndrome With Situs Inversus Totalis, Interrupted Inferior Vena Cava, and Bronchiectasis.
Methodist Debakey Cardiovasc J
March 2025
Indira Gandhi Medical College, Shimla, India.
Incidental detection of polysplenia associated with situs inversus totalis in an adult is rarely reported in literature.12 "Heterotaxy" refers to a wide spectrum of anomalies involving dysmorphism of thoracoabdominal organs across the right-left axis of the body. Polysplenia is observed in left isomerism along with bilateral bilobed bronchi and bilateral morphologic left atria.
View Article and Find Full Text PDFThe case presented highlights a unique presentation of coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear anomalies (CHARGE) syndrome along with athymia and heterotaxy to establish a possible association between these 2 well-known patterns of multiple malformations.
View Article and Find Full Text PDFCACNA1G, A Heterotaxy Candidate Gene, Plays a Role in Ciliogenesis and Left-Right Patterning in Xenopus tropicalis.
Genesis
February 2025
Pediatric Genomics Discovery Program, Departments of Pediatrics and Genetics, Yale University School of Medicine, New Haven, Connecticut, USA.
Heterotaxy (HTX) is characterized by an abnormality in the organ arrangement along the Left-Right (LR) axis and is caused by the disruption of LR patterning in early development. LR asymmetry is critical for multiple organs. Specifically, proper LR patterning is crucial for cardiac function and is a cause of congenital heart disease (CHD).
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!