Case reports in cardiology · 2026
Dextrocardia in Heterotaxy Syndrome (Polysplenia Variant) in a 36-Year-Old Ethiopian Woman: A Case Report and Literature Review.
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Tukeni Kedir Negesso, Dekema Ramadan Jemal, Shukri Mohammed Kedir, Mohammed Jafer Yasin, Jima Michael Deyu, Kebede Merid Lemma
Аннотация
BACKGROUND: Heterotaxy syndrome-polysplenia variant (left isomerism) with dextrocardia is a rare constellation of laterality defects characterized by left-isomerism, multiple splenic nodules, and associated vascular and visceral anomalies. This report describes an adult Ethiopian woman who presented with nonspecific abdominal pain and was found to have imaging features diagnostic of heterotaxy (polysplenia variant).
CASE PRESENTATION: A 36-year-old Ethiopian female presented with a three-day history of mild to moderate, nonradiating anterior abdominal pain. Physical examination revealed the apical impulse on the right and mild left upper quadrant tenderness. Contrast-enhanced CT of the chest and abdomen demonstrated dextrocardia with the cardiac apex directed to the right, right-sided aortic arch, interrupted inferior vena cava with azygos continuation, bilobed lungs consistent with left isomerism, multiple splenic nodules clustered in the left upper quadrant (polysplenia), and a short pancreas. Routine laboratory tests were unremarkable. The final diagnosis was heterotaxy syndrome-polysplenia variant (left isomerism) with dextrocardia. The patient was managed conservatively for presumed costochondritis, counseled about her anatomical variation, advised to carry a medical alert card, and remained symptom-free at two-week follow-up.
CONCLUSION: Imaging features in this patient are most consistent with heterotaxy syndrome (polysplenia variant) rather than isolated situs inversus totalis. Clear, consistent diagnostic terminology (heterotaxy-polysplenia variant with dextrocardia) is essential for accurate communication, appropriate counseling, and safe planning of future interventions. Recognition of such cases in underreported regions supports improved diagnostic awareness and tailored care.
CASE PRESENTATION: A 36-year-old Ethiopian female presented with a three-day history of mild to moderate, nonradiating anterior abdominal pain. Physical examination revealed the apical impulse on the right and mild left upper quadrant tenderness. Contrast-enhanced CT of the chest and abdomen demonstrated dextrocardia with the cardiac apex directed to the right, right-sided aortic arch, interrupted inferior vena cava with azygos continuation, bilobed lungs consistent with left isomerism, multiple splenic nodules clustered in the left upper quadrant (polysplenia), and a short pancreas. Routine laboratory tests were unremarkable. The final diagnosis was heterotaxy syndrome-polysplenia variant (left isomerism) with dextrocardia. The patient was managed conservatively for presumed costochondritis, counseled about her anatomical variation, advised to carry a medical alert card, and remained symptom-free at two-week follow-up.
CONCLUSION: Imaging features in this patient are most consistent with heterotaxy syndrome (polysplenia variant) rather than isolated situs inversus totalis. Clear, consistent diagnostic terminology (heterotaxy-polysplenia variant with dextrocardia) is essential for accurate communication, appropriate counseling, and safe planning of future interventions. Recognition of such cases in underreported regions supports improved diagnostic awareness and tailored care.