Cardiac surgery in type-1-myotonic muscular dystrophy (Steinert syndrome) associated to Barlow disease

• Published in: Interactive cardiovascular and thoracic surgery 2008-04, Vol.7 (2), p.222-226
• Main Authors: Gelsomino, Sandro, Lorusso, Roberto, Bille, Giuseppe, De Cicco, Giuseppe, Da Broi, Ugo, Rostagno, Carlo, Stefano, Pierluigi, Gensini, Gian Franco
• Format: Article
• Language: English
• Subjects: Adult; Aged; Cardiac Surgical Procedures - adverse effects; Cardiopulmonary Bypass - adverse effects; Female; Follow-Up Studies; Heart Arrest, Induced - adverse effects; Heart Arrest, Induced - methods; Humans; Hypothermia, Induced - adverse effects; Male; Middle Aged; Mitral Valve Prolapse - etiology; Mitral Valve Prolapse - surgery; Myotonic Dystrophy - complications; Myotonic Dystrophy - surgery; Time Factors; Treatment Outcome
• ISSN: 1569-9293; 1569-9285
• DOI: 10.1510/icvts.2007.171611

a Experimental Surgery Unit, Cardiac Surgery, Department of Heart and Vessels, Careggi Hospital, Viale Morgagni 85, 50134, Florence, Italy b Cardiac Surgery, Civic Hospital, Brescia, Italy c Anesthesia, Santa Maria della Misericordia Hospital, Udine, Italy *Corresponding author. Tel.: [phone]; fax: [phone]. E-mail address : sandro.gelsomino{at}libero.it (S. Gelsomino). No data exist in the English-language literature about patients with Barlow disease associated to Steinert syndrome and little is known about the employment of hypothermic cardiopulmonary bypass (CPB) and hyperkalemic cardioplegia in these patients. We present our experience with six patients affected by myxomatous degeneration associated to Steinert disease undergoing complex mitral valve repair. In all patients we employed mild hypothermic CPB (31 °C) and myocardial protection was achieved, in the entire cohort, by the use of blood hyperkalemic cold cardioplegia. The postoperative course was uneventful in all patients and neither shivering nor generalized muscle contraction were observed. Furthermore, all patients have remained well on an outpatient basis. Hypothermic CPB and hyperkalemic cardioplegia can be safely employed in patients with Steinert syndrome requiring complex cardiac surgery. Further large studies are necessary to confirm our findings. Key Words: Myotonic muscular dystrophy; Steinert disease; Mitral repair; Barlow disease