INTRODUCTION: Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease characterized by a ventricular septal defect, overriding of the aorta, right ventricular outflow tract obstruction, and right ventricular hypertrophy(1). Surgical repair is the only means for patients to improve their survival rate(2). Although adults with surgically repaired TOF can still present with cyanotic spells, they can also present complications of chronic cyanosis, including erythrocytosis and blood hyperviscosity(1).
Cardiac myxoma is the most frequent primary cardiac neoplasm, nonetheless, the cardiac tumor is very rare. 1 The diagnosis of these tumors can be incidental or due to symptoms secondary to complications, like embolization, or valvular dysfunction. 2 The management will depend on the size and characteristic of the mass. Embolic stroke is a dramatic and severe complication of cardiac myxoma. 2,3 CASE PRESENTATION: A 31 year-old female with a PMHx of well controlled Crohn's disease, presented to our institution via helicopter for a higher level of care, after receiving TPA for an ischemic stroke. A mechanical thrombectomy was planned and her NIHSS was 9. After she arrived, a CT cerebral perfusion scan showed an acute ischemic infarction in the territory of the right MCA with right superior M2 occlusion. There was a mild mass effect on the right lateral ventricle without evidence of brain herniation. The patient was started on NS 3% and mannitol.On the 2nd day of hospitalization the patient suddenly desaturated requiring emergent intubation. A chest x-ray demonstrated flash pulmonary edema. The patient's mechanical ventilation was optimized for ARDS and she was turned prone. On the same day the patient was started on multiple pressors. On day 3 a copious amount of clear fluid was suctioned from the ET tube, analyzed as transudate with no signs of infection.The patient's cerebral edema also worsened and a right decompressive craniectomy was required. The patient began to go into multiorgan failure due to continued hypoperfusion. Her LFTs trended upwards as her kidney function declined. On day 4 an Echocardiogram showed a normal left ventricular size with decreased EF 25%-30%. A large mobile mass was attached to the interatrial septum obstructing the mitral valve with moderate mitral regurgitation and pulmonary hypertension. This mass was suggestive of an atrial myxoma. CT surgery was immediately consulted, intervention was scheduled for the following day. Unfortunately the patient coded the same day after she was turned from prone to supine. Multiple rounds of CPR were performed, however the patient expired.DISCUSSION: We suspect that the change in position caused increased outflow obstruction by the myxoma resulting in cardiac arrest. Due to the large size of the mass and the rapid progression of the patient's symptoms, it is unlikely an early diagnosis would have significantly changed her prognosis. CONCLUSIONS:The severe and dramatic presentation of this case, showed the relevance of this rare pathology and the devastating consequences of the natural course of this disease. In a retrospective analysis of the case, no personal history suggested this diagnosis. In the setting of a sudden ischemic stroke shortly followed or accompanied by flash pulmonary edema, should arouse clinical suspicions for some embolic pathology that may be causing outflow obstruction such as an atrial myxoma.
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