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Case Report | Radiology and Medical Imaging Sciences | Volume 15 Issue 5, May 2026 | Pages: 1885 - 1890 | India
Beyond Cyanosis: CT Angiographic Insights into Heterotaxy Syndrome with Left Isomerism in a Post Bilateral Bidirectional Glenn Shunt Patient
Abstract: Heterotaxy syndrome represents a rare spectrum of congenital anomalies characterized by abnormal arrangement of thoracoabdominal organs along the left-right body axis. Left isomerism (polysplenia syndrome) is frequently associated with complex congenital cardiac malformations, interrupted inferior vena cava (IVC) with azygos continuation, and abnormal pulmonary and systemic venous drainage. We present a case of a pediatric patient with known dextrocardia and status post bilateral bidirectional Glenn shunt who underwent CT pulmonary angiography for comprehensive postoperative evaluation. Imaging demonstrated a constellation of classic left isomerism findings: dextrocardia, transverse liver, polysplenia, bilateral morphologic left atrial appendages, common atrium with large atrial septal defect, membranous ventricular septal defect, hypoplastic right ventricle, double outlet right ventricle with supravalvular pulmonary stenosis, and interrupted left-sided IVC with azygos continuation. Bilateral superior vena cavae were identified draining into patent bilateral bidirectional Glenn shunts. Bronchopulmonary findings included bilateral hyparterial bronchi with discordant lung lobation. No pulmonary thromboembolism was detected. This case underscores the indispensable role of multidetector CT angiography in providing comprehensive characterization of complex cardiovascular and thoracoabdominal anatomy in heterotaxy syndrome, particularly in the postoperative setting where echocardiography may be limited.
Keywords: Heterotaxy syndrome, Left isomerism, Polysplenia syndrome, Dextrocardia, Bidirectional Glenn shunt, Double outlet right ventricle, Interrupted inferior vena cava, CT pulmonary angiography, Congenital heart disease
How to Cite?: Dr. Neha Rudraraju, Dr. Madan Manmohan, "Beyond Cyanosis: CT Angiographic Insights into Heterotaxy Syndrome with Left Isomerism in a Post Bilateral Bidirectional Glenn Shunt Patient", Volume 15 Issue 5, May 2026, International Journal of Science and Research (IJSR), Pages: 1885-1890, https://www.ijsr.net/getabstract.php?paperid=MR26529123508, DOI: https://dx.dx.doi.org/10.21275/MR26529123508