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Washington University Experience | VASCULAR | Infarct, watershed | 7A0 Case 7 History
Case 7 History ---- The patient was a 4 year old girl with a history of complex congenital heart disease characterized by double outlet right ventricle with subpulmonic ventricular septal defect (Taussig-Bing anomaly) and side-by-side great vessels. Her past surgical history includes an arterial switch operation with VSD closure and baffle of left ventricle to neo-aorta on day of life 7. During recovery from that operation she went into cardiac arrest associated with severe right ventricular dysfunction and required extra-corporeal membrane oxygenation (ECMO). She also developed endocarditis with vancomycin resistant enterococcus at that time, which was treated with Linezolid for 6 weeks. She was discharged on day of life 19. A cardiac catheterization one year later (10/5/05) showed a subaortic fibromuscular tunnel, 2+ aortic insufficiency and circumferential stenosis of the proximal left pulmonary artery. A subaortic muscle resection was performed 9 days later without complications. Echocardiograms and catheterizations throughout the next 2 years showed worsening aortic insufficiency, severe left ventricular outflow tract obstruction, and moderate, stable right ventricular outflow tract obstruction. The patient underwent aortic valve repair with resection of subaortic stenosis on 1/13/09. After the patient was taken off bypass, she had poor left ventricular function requiring epinephrine and dopamine drips. She was transferred to the Cardiac Intensive Care Unit. There, she had an episode of ventricular tachyarrhythmia and ventricular fibrillation, received chest compressions and then was placed on ECMO. Due to concern for left atrial hypertension, a vent was placed into the left atrium. Even with that, there was significant difficulty in obtaining adequate mean arterial blood pressure. She had several episodes of ventricular tachycardia and ventricular fibrillation requiring frequent defibrillation. During that time, she was noted to have significant pulmonary edema with bloody frothy secretions. An echocardiogram was performed, which revealed a distended left atrium and left ventricle, and no contraction of the left ventricular free wall. Paradoxical motion of the intraventricular septum was noted. At that time, the left atrial vent was replaced with a larger one. During this procedure, which occurred at the bedside, the patient had several episodes of ventricular tachycardia and ventricular fibrillation requiring defibrillation. With the larger vent in place, pulmonary edema subsided clinically and the peak airway pressure went down. On January 15, she was taken back to the operating room for a mediastinal exploration in order to evacuate the left pleural effusion and assess her cardiac function by epicardial echocardiogram. The echocardiogram showed poor left ventricular function. After this procedure, her ECMO flow started to decrease to around 1 liter per minute, which was associated with a significant decrease in blood pressure. She was therefore re-explored emergently. Her venous cannula was found to be dislodged almost completely with air entering into the ECMO circuit. This required clamping of the ECMO circuit in order to remove the air from the arterial line. During the de-airing maneuvers, she remained without perfusion for 17 minutes. In spite of the adequate ECMO flow, her systolic blood pressure remained around 60. Overnight from January 15 to January 16, she was noted to have asymmetric pupils and was taken to the CT scanner on an emergent basis. The CT scan revealed multiple regions of low attenuation throughout the cerebrum in the watershed areas as well as in the cerebellum consistent with multiple infarcts. She continued to be hypotensive with multiple organ dysfunction. The patient’s family decided to withdraw support and she passed away on January 16th.
