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Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging combination

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Published in Thoracic and Cardiovascular Surgeon. 2011, vol. 59, no. 5, p. 313-6
Abstract Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.
Keywords Abnormalities, MultipleAdolescentAortic Coarctation/complications/diagnosis/surgeryAortic Valve/abnormalities/surgeryAortic Valve Insufficiency/complications/diagnosis/surgeryBlood Vessel Prosthesis ImplantationCardiac Surgical ProceduresCardiopulmonary BypassEchocardiography, DopplerFemaleHeart Failure/etiologyHeart Septal Defects, Ventricular/complications/diagnosis/surgeryHeart Valve Prosthesis ImplantationHumansIsolated Noncompaction of the Ventricular Myocardium/complications/diagnosis/surgeryMagnetic Resonance ImagingTreatment Outcome
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PMID: 21432759
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Article (Published version) (846 Kb) - document accessible for UNIGE members only Limited access to UNIGE
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Research groups Chirurgie cardio-vasculaire (105)
L'hypertension pulmonaire (pédiatrie) (228)
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MYERS, Patrick Olivier et al. Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging combination. In: Thoracic and Cardiovascular Surgeon, 2011, vol. 59, n° 5, p. 313-6. https://archive-ouverte.unige.ch/unige:24921

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Deposited on : 2013-01-03

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