Becker's Muscular Dystrophy and Orthotopic Heart Transplantation: Perioperative Considerations


  • Christopher B. Komanapalli
  • Valerie Sera
  • Matthew S. Slater
  • Melodie Burdette
  • Uttam Tripathy
  • Glenn Brady
  • Lars Hegnell
  • Pasala S. Ravichandran
  • Ray E. Hershberger
  • Howard K. Song



Patients with Becker's and Duchenne's muscular dystrophy occasionally have myocardial involvement leading to end-stage heart failure. Heart transplantation is established as an effective therapy. Achieving successful outcomes in this challenging group requires special consideration during the perioperative period to limit preoperative deconditioning, minimize anesthesia complications, and rapidly institute rehabilitation with appropriate precautions. We reviewed our recent experience with Becker's muscular dystrophy patients and discuss the management of perioperative issues specific to this patient group.


Bennun M, Goldstein B, Finkelstein Y, Jedeikin R. 2000. Continuous propofol anaesthesia for patients with myotonic dystrophy. Br J Anaesth85:407--9.nBittner RE, Shorny S, Streubel B, Hubner C, Voit T, Kress W. 1995. Serum antibodies to the deleted dystrophin sequence after cardiac transplantation in a patient with Becker's muscular dystrophy. N Engl J Med333:732--3.nChrzanowski L, Kasprzak JD, Trzos E, et al. 2003. Different expressions of X-linked cardiomyopathy in monozygotic triplets with Becker's dystrophy. Int J Cardiovasc Imaging19:377--80.nConraads VM, Beckers PJ, Vorlat A, Vrints CJ. 2002. Importance of physical rehabilitation before and after cardiac transplantation in a patient with myotonic dystrophy: a case report. Arch Phys Med Rehabil83:724--6.nCoral-Vazquez R, Cohn RD, Moore SA, et al. 1999. Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: a novel mechanism for cardiomyopathy and muscular dystrophy. Cell98:465--74.nDonofrio PD, Challa VR, Hackshaw BT, Mills SA, Cordell AR. 1989. Cardiac transplantation in a patient with muscular dystrophy and cardiomyopathy. Arch Neurol46:705--7.nEmery AE. 2002. The muscular dystrophies. Lancet359:687--95.nFinsterer J, Bittner RE, Grimm M. 1999. Cardiac involvement in Becker's muscular dystrophy, necessitating heart transplantation, 6 years before apparent skeletal muscle involvement. Neuromuscul Disord9:598--600.nHaller RG, Lewis SF. 1984. Pathophysiology of exercise performance in muscle disease. Med Sci Sports Exerc16:456--9.nHoffman EP, Fischbeck KH, Brown RH, et al. 1988. Characterization of dystrophin in muscle-biopsy specimens from patients with Duchenne's or Becker's muscular dystrophy. N Engl J Med318:1363--8.nLeprince P, Heloire F, Eymard B, Leger P, Duboc D, Pavie A. 2002. Successful bridge to transplantation in a patient with Becker muscular dystrophy-associated cardiomyopathy. J Heart Lung Transplant21:822--4.nMuntoni F. 2003. Cardiomyopathy in muscular dystrophies. Curr Opin Neurol16:577--83.nPetrof BJ, Shrager JB, Stedman HH, Kelly AM, Sweeney HL. 1993. Dystrophin protects the sarcolemma from stresses developed during muscle contraction. Proc Natl Acad Sci U S A90:3710--4.nRees W, Schuler S, Hummel M, Hetzer R. 1993. Heart transplantation in patients with muscular dystrophy associated with end-stage cardiomyopathy. J Heart Lung Transplant12:804--7.nRirie DG, Shapiro F, Sethna NF. 1998. The response of patients with Duchenne's muscular dystrophy to neuromuscular blockade with vecuronium. Anesthesiology88:351--4.nRoland EH. 2000. Muscular dystrophy. Pediatr Rev21:233--7; quiz 238.nRuiz-Cano MJ, Delgado JF, Jimenez C, et al. 2003. Successful heart transplantation in patients with inherited myopathies associated with end-stage cardiomyopathy. Transplant Proc35:1513--5.nStamler JS, Meissner G. 2001. Physiology of nitric oxide in skeletal muscle. Physiol Rev81:209--37.n



How to Cite

Komanapalli, C. B., Sera, V., Slater, M. S., Burdette, M., Tripathy, U., Brady, G., Hegnell, L., Ravichandran, P. S., Hershberger, R. E., & Song, H. K. (2006). Becker’s Muscular Dystrophy and Orthotopic Heart Transplantation: Perioperative Considerations. The Heart Surgery Forum, 9(2), E604-E606.




Most read articles by the same author(s)