Abstract
Background: Adult congenital heart disease is most commonly complicated by arrhythmias and very rarely by acute coronary syndromes. We present a rare case of ST elevation myocardial infarction (STEMI) in a young 35-year-old patient with congenital transposition of great vessels. Case Presentation: A 35-year-old female with a past medical history of transposition of the great vessels (d-TGA) status post arterial baffle operation in infancy, with prior atrial arrhythmias and severe RV failure, presented to our facility with crushing substernal chest pain radiating down her left arm. ECG was significant for ST elevation in the inferior leads and high sensitivity troponin was elevated. She was taking Rivaroxaban. She was taken emergently to the catheterization lab where she was found to have 100% occlusion of the apical left anterior descending artery secondary to an embolus with no angiographic disease in any other epicardial vessel. Transthoracic echocardiography showed severe dilation of the right ventricle with severe tricuspid regurgitation. The embolic lesion was deemed not amenable to percutaneous intervention and the patient was treated conservatively with a heparin drip. The embolus was presumed to originate from the hyper-trabeculated and distended right ventricle in spite of chronic rivaroxaban therapy and she was started on warfarin. Presently she is stable, awaiting a heart transplant. Discussion: Extensive literature review revealed four reported cases of acute coronary syndrome secondary to emboli in patients with surgically corrected TGA. Patients with d-TGA are most often surgically corrected with an arterial switch procedure. But in Baffle procedures (Mustard or Senning) such as the one in this patient, the right ventricle becomes the systemic ventricle. The most common complications as an adult are right ventricular dilation, severe tricuspid regurgitation and atrial arrhythmias, all three of which were noted in our patient. Conclusion: Our patient developed all three of the most common adult complications of surgically corrected d-TGA, ultimately resulting in the rare and extreme manifestation of an embolic STEMI.
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