Anaesthetic management of a single stage surgery of anterior chest wall reconstruction and mitral valve replacement in a patient with loeys dietz syndrome

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Introduction This syndrome is a rare genetic disease discovered quite recently in 2005 by Loey and colleagues 1. It is described by the syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by genetic mutations. The anaesthetic management of this patient involves that of plastic surgery and of cardiopulmonary bypass. Retrospective review of patients with LDS types 1-4 undergoing cardiovascular surgery during the years 1991-2016 showed 35 patients underwent 57 cardiac-related operations but no combined plastic and cardiothoracic case has been reported so far2. Methods This is a case report of rare and challenging anesthetic management of combined plastic surgery and cardiothoracic surgery. A 14 year old boy was referred to our centre for severe mitral regurgitation in failure planned for mitral valve replacement. He has underlying Loey's Dietz Syndrome in which he has severe pectus excavatum and thoracolumbar scoliosis. Echocardiography revealed severe mitral regurgitation, ERO 0.49cm2 and dilated annulus of 42cm. There is also mild aortic regurgitation and dilated sinus of valsalva of 42cm. The aortic annulus and sinotubular junction remained in normal range with 24mm and 28 mm respectively. Multidisplinary meeting has concluded to perform single stage surgery to avoid risk of adhesion with preservation of bilateral internal mammary artery to maintain perfusion to reconstruction site. The aortic root left untouched as it hoped by reducing volume from mitral valve replacement, the dilatation will not be worse. Results Patient underwent general anaesthesia with anterior osteomusculocutaneous chest wall island flap raised by plastic team first then proceed with cardiopulmonary bypass for mitral valve replacement (MVR). After good valvular function obtained post replacement, we proceeded with coming off bypass and anterior chest wall reconstruction by plastic team. Patient was sedated and paralysed overnight and extubated the next day. PCA fentanyl started as post-operative pain relief. Discussion The anaesthetic challenges in this case are mounting as we deal with rare congenital disease with multisystem involvement. It also combines both plastic surgery and cardiothoracic anaesthesia management with several parameters that require detailed monitoring. Further study may be required in regards to circuit priming in the case of combined cardiothoracic and plastic operation. This case however serves as a guide as to anaesthetic management in such combined procedure. Should this patient return for further cardiothoracic operation in the future, it will pose another great anaesthetic challenges.