| Summary: | Abstract Background The Maroteaux-Lamy syndrome (Mucopolysaccharidosis type VI) is a rare, inherited metabolic disease that results in progressive tissue accumulation of dermatan-sulfated glycosaminoglycans and inflammatory consequences that almost always affects the heart valves. From the anesthesia point of view, managing the airway and ventilation might be a serious challenge due to specific features of the syndrome. Additionally, it is more than probable that the surgical team will perform a non-straightforward procedure. Case presentation A 42-year-old male with Maroteaux-Lamy syndrome was referred to our department with shortness of breath, due to severe aortic stenosis, and at least moderate mitral valve regurgitation. The patient was initially scheduled for aortic valve replacement. After multiple attempts with video assisted laryngoscopy, the endotracheal intubation was achieved with the aid of fiberoptic bronchoscopy, while the ventilation succeeded only with laryngeal mask. The somatic features of the syndrome that made the anesthesia induction extremely difficult, also affected the surgical procedure. Suboptimal exposure of the mitral valve, patch enlargement of the aortic root to host the bigger possible prosthesis, and the hard decision to replace the mitral valve even with a marginal indication were the intraoperative challenges for the surgical team. Finally, the patient underwent a successful double valve replacement with aortic root enlargement and 18 months postoperatively remains improved. Conclusion Patients with Maroteaux-Lamy syndrome represent a challenge for both anesthesiologists and cardiac surgeons. The whole team should be well prepared to deal with difficulties in airway management, ventilation and surgical valve exposure. The cardiac surgeon should be ready to offer additional procedures and even replace “prematurely” a moderately diseased valve in order to avoid a dangerous reoperation. The limited knowledge on the natural history of the Maroteaux-Lamy syndrome valvulopathy and the difficulties in anesthesia induction support this approach.
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