Unexpected cardiac death due to a slit-like left coronary ostium with associated high take-off of the right coronary art
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Unexpected cardiac death due to a slit-like left coronary ostium with associated high take-off of the right coronary artery in a previously healthy child David S. Priemer • Saar Danon • Miguel A. Guzman
Accepted: 4 September 2014 / Published online: 16 October 2014 Ó Springer Science+Business Media New York 2014
Case report A previously healthy 6 year-old boy with no significant past medical or family history suddenly began vomiting in the evening after spending part of the day working with his father. He continued to vomit through the night and was taken to his pediatrician, who immediately drove him to the local emergency department. Physical examination and laboratory findings were consistent with cardiogenic shock. Electrocardiography showed diffusely low voltage, and echocardiography revealed poor left ventricular function. Hemodynamic stability could not be maintained despite IV fluids, dopamine, and multiple attempts at cardiopulmonary resuscitation. The patient was placed on cardiopulmonary bypass and transfer to a tertiary medical facility was arranged. After approximately 3 h on bypass, transport arrived and the patient was converted to a portable Extracorporeal Membrane Oxygenation (ECMO) circuit and transferred. On arrival, examination revealed a diffusely edematous, unresponsive patient without spontaneous respirations, palpable pulses, or audible heart sounds. He had multiple ventricular dysrhythmias with repeated attempts at defibrillation. ECMO cannulation was converted from femoral
D. S. Priemer M. A. Guzman (&) Department of Pathology, Saint Louis University, Saint Louis, MO, USA e-mail: [email protected]; [email protected] S. Danon Department of Pediatrics, Saint Louis University, Saint Louis, MO, USA S. Danon Cardinal Glennon Medical Center, Saint Louis, MO, USA
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vessels to the neck vessels, and cardiac catheterization was performed to decompress the left heart by trans-septal puncture of the atrial septum and static balloon septostomy. Hemodialysis was added to the ECMO circuit due to severe multiorgan failure, but ultimately the condition of the patient did not improve. He developed pulseless electrical activity and neurologic exams eventually confirmed brain death. Announcement of death came on the fifth day from the onset of symptoms, with 57 h and 19 min on ECMO. The suspected cause of death prior to autopsy was fulminant myocarditis versus an undetected anatomic abnormality. At autopsy, external examination revealed a well-nourished and well-developed boy. Findings included labial cyanosis as well as abdominal distention. Internal examination was most remarkable for findings observed in the heart. The heart had a weight of 170 g (99th percentile [1]) with a thickened left ventricular wall (1.6 cm); the right ventricle was within normal limits (0.4 cm). Sectioning of the ventricles showed patchy fibrosis throughout the anterior and lateral walls of the left ventricle and the interventricular septum as well as hemorrhagic and necrotic changes of the left pa
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