Myofibroblastoma presenting as aortic intramural hemathoma (CROSBI ID 651661)
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Podaci o odgovornosti
Planinc, Mislav ; Blažeković, Robert ; Barić, Davor ; Sutlić, Željko ; Rudež, Igor
engleski
Myofibroblastoma presenting as aortic intramural hemathoma
A 48-year-old otherwise healthy male patient was admitted to local hospital after echocardiographic suspicion of intramural hemathoma of ascending aorta which was made in outpatient clinic. He complained on chest pain and diswcomfort lasting two previous weeks. Echocardiography revealed thickened aortic root and ascending part of the aorta, with mild aortic regurgitation, circular pericardial effusion up to 1 cm and preserved systolic function. Multisliced computed-tomography (MSCT) was performed showing intramural hemathoma of ascending aorta in length of 26 mm with width of the aorta around 38 mm without signs of aortic dissection. He was referred to our center for urgent surgical procedure. Additional transthoracic echo and MSCT were done with same findings as in referring hospital. With such findings indication to proceed with surgery was made and Bentall operation was planned. After axillary artery cannulation, median sternotomy and pericardiotomy were performed in standard fashion. Inspection and palpation revealed extremely firm aortic root and dense white wax-like layer of ascending aorta. Following cannulation of right atrium and placing patient on CPB, aorta was cross-clamped and opened higher then usually but direct antegrade cardioplegia could not be delivered so retrograde cardioplegia was used to arrest the heart. After standard removal of aortic valve leaflets, very thickened aortic root was dissected. Both coronary ostia were very fragile and firmly adherent to surrounding tissue so button couldn't have been made. At that point saphenous vein grafts were harvested and coronary artery bypass grafts were created on LAD, RCA and Cx. After aortic cross clamp was released, intraoperative echocardiography showed global hypokinesis of the heart. Thereafter two unsuccessful attempts to wean the patient from CPB were made and V-A ECMO was established. Evewn with that support low cardiac output syndrome ocurred and patient succumbed during the procedure. Patohistological analysis of specimen of ascending aorta and right atrium found inflamed myofibroblastoma of the heart to be the cause of aortic thickening and tissue covering aortic root and right atrium.
Myofibroblastoma
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114-115.
2014.
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