Background: Heterotaxy is a relatively uncommon congenital anomaly that is usually diagnosed incidentally on\nimaging studies in adults. We present an unusual case of venous thromboembolism in a 26 year old pregnant\nfemale with Heterotaxy syndrome.\nCase presentation: A 26 year-old pregnant female at 13 weeks gestation suffered cardiac arrest with successful\ncardiac resuscitation and return of spontaneous circulation. The cardiac arrest was secondary to massive pulmonary\nembolism requiring thrombolytic therapy and stabilization of hemodynamics. She had extensive evaluation to\ndetermine the etiology for the pulmonary embolism and was noted to have an anatomic variation consistent\nwith heterotaxy syndrome on imaging studies. After thrombolysis the patient was treated with UFH and then\nswitched to enoxaparin without complication until 25 weeks of gestation when she experienced worsening\nabdominal pain with associated headaches, lightheadedness and elevated blood pressures needing elective induction of\nlabor. The infant died shortly after delivery. The anticoagulation was continued for additional 3 months and she\nwas subsequently placed on low dose aspirin to prevent recurrent venous thromboembolic episodes. She is currently\nstable on low dose aspirin and is into her third year after the venous thromboembolism without any recurrence.\nConclusion: To our knowledge, this is the first reported case of venous thromboembolism in pregnancy associated with\nheterotaxy syndrome. A discussion on pathophysiology of venous thromboembolism in pregnancy and heterotaxy\nsyndrome has been undertaken along with treatment approach in such situations
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