Replacement of the descending thoracic aorta for massive aneurysm in neonatal Marfan syndrome.
We describe a patient with neonatal Marfan syndrome presenting with massive ascending and descending thoracic aortic aneurysm. Because of rapidly progressive respiratory distress due to tracheobronchial compression, emergency replacement of the descending thoracic aorta with a 12-mm PTFE vascular prosthesis was undertaken at 1 month of age. The postoperative course was complicated by bilateral tension pneumothorax contributing to irreversible respiratory failure. The unique clinico-pathological features and the relevant surgical implications of the case are discussed.
Department of Cardiovascular Surgery, Universityof Verona Medical School, Italy.
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Luciani
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Faggian
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Mazzucco
D015901:Angiography, Digital Subtraction; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D004630:Emergencies; D017809:Fatal Outcome; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D011030:Pneumothorax / Q000209:etiology; D011138:Polytetrafluoroethylene; D011183:Postoperative Complications / Q000209:etiology; D012131:Respiratory Insufficiency / Q000209:etiology
D011138:Polytetrafluoroethylene