Online Images in the Medical Sciences| Volume 355, ISSUE 1, e1, January 2018

Vascular and Splenic Abnormalities in Heterotaxy Syndrome

Published:September 20, 2017DOI:
      A 37-year-old woman with paroxysmal atrial fibrillation, presented to the emergency department with palpitations. Previously, a pulmonary venous isolation (PVI) was attempted to control similar symptomatic episodes owing to atrial fibrillation. However, PVI was unsuccessful owing to abnormal inferior vena cava (IVC) anatomy. Magnetic resonance venography demonstrated an interrupted IVC with azygous continuation into the abdomen and pelvis. The hepatic veins drained directly into the right atrium (Figure A). The azygos veins drained the right renal vein and iliac veins, whereas the hemiazygos drained the left renal veins and then drained into the azygos vein. Other than the venous anomalies, the patient also had polysplenia (Figure B), a midline liver and intestinal malrotation leading to the diagnosis of heterotaxy syndrome (HTx) with left atrial isomerism (LAI). A transthoracic echocardiogram showed no associated structural heart disease and another PVI was planned. With the help of interventional radiology, transhepatic access to the right atrium was obtained. Transesophageal echo was used to guide the transseptal access to the left atrium and PVI was performed successfully with good result and complete isolation of all pulmonary veins.
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        • Sutherland M.J.
        • Ware S.M.
        Disorders of left-right asymmetry: heterotaxy and situs inversus.
        Am J Med Genet C Semin Med Genet. 2009; 151: 307-317
        • Gilljam T.
        • McCrindle B.W.
        • Smallhorn J.F.
        • et al.
        Outcomes of left atrial isomerism over a 28-year period at a single institution.
        J Am Coll Cardiol. 2000; 36: 908-916
        • Kothari S.
        Non-cardiac issues in patients with heterotaxy syndrome.
        Ann Pediatr Cardiol. 2014; 7: 187