title: Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging combination.
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.
authors: Division of Cardiovascular Surgery, Geneva University Hospitals and Medical School, Geneva, Switzerland. patrick.myers@hcuge.ch
authors: P O
authors: PO
authors: Myers
authors: C
authors: C
authors: Tissot
authors: M
authors: M
authors: Cikirikcioglu
authors: A
authors: A
authors: Kalangos
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D001017:Aortic Coarctation / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation; D006348:Cardiac Surgical Procedures; D002315:Cardiopulmonary Bypass; D015150:Echocardiography, Doppler; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D008279:Magnetic Resonance Imaging; D016896:Treatment Outcome