title: Posterior restoration of left ventricle and mitral valve repair in patients with muscular dystrophy.
abstract: BACKGROUND
  Congestive heart failure (CHF) is major risk factor for survival among patients with muscular dystrophy (MD). The degenerative postero-lateral wall of the left ventricle (LV), which results in systolic dysfunction and functional mitral regurgitation (MR) at the time of CHF, is not well described in MD.
  
  
  METHODS
  We restored the LV and repaired the mitral valves of 6 patients (mean age, 43 ± 9 years) during emergency and elective procedures. Two and 4 patients were in New York Heart Association (NYHA) functional classes III and IV, respectively. One patient required emergency preoperative intraaortic balloon pump support. Before operation, speckle-tracking echocardiography was applied to detect the myocardial lesion. The postero-lateral LV wall that is critically affected in MD was excluded during posterior restoration. Functional MR was repaired using a combination of ring annuloplasty, papillary muscle approximation, and chordal cutting. The LV myocardium between the end of the LV incision line and the mitral annulus was cryoablated to prevent late ventricular arrhythmia.
  
  
  RESULTS
  All patients survived (100%) for a mean follow-up of 59±39 (range; 5 to 101) months. The NYHA functional classes improved to I and II (n=3 each). Left ventricular end-diastolic diameter significantly decreased from 77±13 to 59±4 mm (p=0.0088), whereas ejection fraction did not significantly improve (0.24±0.1 vs 0.29±0.11, p=0.2451), although MR severity was significantly eliminated (3.5±0.5 vs 0.5±0.6, p=0.0003). No cardiac events were associated with CHF or arrhythmia during follow-up.
  
  
  CONCLUSIONS
  Posterior restoration of the LV and mitral repair are useful for treating CHF in patients with MD, and improve survival.
authors.affiliation: Department of Cardiovascular Surgery, Hayama Heart Center, Kanagawa, Japan. Electronic address: hirota@hayamaheart.gr.jp.
authors.forename: Masanori
authors.initials: M
authors.lastname: Hirota
authors.affiliation: Department of Cardiovascular Surgery, Hayama Heart Center, Kanagawa, Japan.
authors.forename: Joji
authors.initials: J
authors.lastname: Hoshino
authors.affiliation: Department of Cardiovascular Surgery, Hayama Heart Center, Kanagawa, Japan.
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Fukada
authors.affiliation: Department of Cardiovascular Surgery, Hayama Heart Center, Kanagawa, Japan.
authors.forename: Taichi
authors.initials: T
authors.lastname: Kondo
authors.affiliation: Department of Cardiovascular Surgery, Hayama Heart Center, Kanagawa, Japan.
authors.forename: Yu
authors.initials: Y
authors.lastname: Takahashi
authors.affiliation: Department of Cardiovascular Medicine, Hayama Heart Center, Kanagawa, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Notomi
authors.affiliation: Department of Cardiovascular Surgery, Hayama Heart Center, Kanagawa, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Isomura
mesh_terms: D000328:Adult; D006348:Cardiac Surgical Procedures / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000150:complications* / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000150:complications* / Q000601:surgery*; D009136:Muscular Dystrophies / Q000150:complications*
keywords: 18; 35