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Washington University Experience | VASCULAR | Infarct - Embolic | 19A0 Case 19 History
Case 19 History ---- The patient was a 3-year-old boy with hypoplastic left heart syndrome, status post Norwood-Sano operation in early infancy, followed by bidirectional Glenn operation in August 2010. He was admitted to Nebraska Children's Hospital with acute decompensated heart failure on December 22. At that time, he had markedly depressed right ventricular systolic function and tricuspid regurgitation. He underwent attempted tricuspid valve repair on January 18. At that time, he had a thrombus removed from his right ventricle. His hospital course was complicated by hypercoagulability resulting in left internal jugular, left subclavian, and innominate vein thrombi requiring thrombectomy in January 2013. He was transferred to SLCH on maximal cardiopulmonary support on February 20 to evaluate his candidacy for heart transplantation. He was placed on ECMO on February 26; and converted to a CentriMag ventricular assist device on March 4, 2013. His circuit was replaced multiple times due to frequent accumulation of fibrin and thrombi, despite anticoagulation with heparin, AT3 infusion, and antiplatelet agents. He had significant requirement for sedation and intermittent paralysis early in his hospitalization. Head MRI on 2/26 showed diffuse cerebral volume loss, but no acute intracranial abnormality or evidence of thrombosis. Subsequent head CT scans initially demonstrated left parafalcine subdural hematoma on 3/11 and left cerebral convexity subarachnoid hemorrhage on 3/28; and then multiple areas of infarction since 3/31. His last head CTs on April 8 and 11 showed new and evolving right ACA, MCA, PCA territory infarcts and left caudate infarcts. On physical exam, the patient exhibited significant left-sided paresis. His level of alertness progressively decreased over the final days of his life. During his hospitalization, he also developed hypertension, bilateral renal infarction, frequent episodes of tracheitis associated with continued mechanical ventilation, and progressive metabolic acidosis. He had longstanding total parenteral nutrition dependence. The severity of his illness and his extensive organ damage precluded his candidacy for heart transplantation. An echocardiogram obtained on April 14 revealed the presence of a thrombus surrounding the right atrial cannula. Per the CT surgery team, he was not a candidate for removal of his right atrial thrombus due to his fragile clinical status and risk for bleeding. Following extensive discussion, the family decided to redirect care. He passed away on April 14 in the company of his mother and his grandmother. Multiple tracheal aspirates were subsequently positive for pseudomonas aeruginosa and an aspirate obtained on 4/3/13 was positive for Stenotrophomonas maltophilia. ---- Autopsy findings included multiple infarcts ranging from subacute to remote involving the vascular territories of the right MCA, right ACA and left MCA territories, and numerous thromboemboli. A left sided organized subdural hematoma was accompanied by multifocal subarachnoid hemorrhages. In addition to these large infarctions and foci of hemorrhage, the brain also shows moderate diffuse cerebral atrophy, mild cortical neuron loss, and many scattered white matter microcalcifications, consistent with chronic hypoxia/ischemia. There was no evidence of infection or vasculitis.
