title: Cardiac surgery in type-1-myotonic muscular dystrophy (Steinert syndrome) associated to Barlow disease.
abstract: 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 degrees 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.
authors.affiliation: Experimental Surgery Unit, Cardiac Surgery, Department of Heart and Vessels, Careggi Hospital, Viale Morgagni 85, 50134, Florence, Italy.
authors.forename: Sandro
authors.initials: S
authors.lastname: Gelsomino
authors.forename: Roberto
authors.initials: R
authors.lastname: Lorusso
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Billè
authors.forename: Giuseppe
authors.initials: G
authors.lastname: De Cicco
authors.forename: Ugo
authors.initials: U
authors.lastname: Da Broi
authors.forename: Carlo
authors.initials: C
authors.lastname: Rostagno
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Stefàno
authors.forename: Gian Franco
authors.initials: GF
authors.lastname: Gensini
mesh_terms: D000328:Adult; D000368:Aged; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D002315:Cardiopulmonary Bypass / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D006324:Heart Arrest, Induced / Q000009:adverse effects / Q000379:methods; D006801:Humans; D007036:Hypothermia, Induced / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D008945:Mitral Valve Prolapse / Q000209:etiology / Q000601:surgery*; D009223:Myotonic Dystrophy / Q000150:complications* / Q000601:surgery; D013997:Time Factors; D016896:Treatment Outcome