title: Mitral valve surgery in a patient with spinal progressive muscular atrophy: report of a case.
abstract: We describe our experience in treating a 69-year-old man with spinal progressive muscular atrophy (SPMA), who underwent a mitral valve replacement. He was admitted for dyspnea, and surgery was indicated for severe mitral insufficiency associated with inferior myocardial infarction. He had been aware of muscle weakness and received a diagnosis of SPMA 18 years previously. Worsening muscle atrophy had led to the need for him to use a wheelchair in his daily life. A preoperative examination revealed markedly reduced pulmonary function (% volume capacity = 44.8%). Because of an acute exacerbation of heart failure, the patient underwent an urgent mitral valve replacement with a 27-mm pericardial bioprosthesis. Although it took 42h to wean him from the mechanical ventilation and he suffered from pulmonary atelectasis after extubation, he was discharged from our hospital in a wheelchair 16 days after surgery. Respiratory management with bilevel positive airway pressure was thus found to be quite useful for patients with neuromuscular disease.
mesh_terms: D000368:Aged; D001705:Bioprosthesis; D002315:Cardiopulmonary Bypass; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D007423:Intra-Aortic Balloon Pumping; D008297:Male; D008943:Mitral Valve; D008944:Mitral Valve Insufficiency / Q000601:surgery*; D009134:Muscular Atrophy, Spinal / Q000150:complications*; D011183:Postoperative Complications / Q000209:etiology; D001261:Pulmonary Atelectasis / Q000209:etiology