Posterior restoration of left ventricle and mitral valve repair in patients with muscular dystrophy.

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.