DOI QR코드

DOI QR Code

Cardiac function associated with home ventilator care in Duchenne muscular dystrophy

  • Lee, Sangheun (Division of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Lee, Heeyoung (Division of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Eun, Lucy Youngmin (Division of Pediatric Cardiology, Department of Pediatrics, Yonsei University College of Medicine) ;
  • Gang, Seung Woong (Department of Rehabilitation Medicine, Yonsei University College of Medicine)
  • 투고 : 2017.08.01
  • 심사 : 2017.09.28
  • 발행 : 2018.02.15

초록

Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Results: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older ($16.25{\pm}1.85years$) than those in the nonventilator group ($14.73{\pm}1.36years$) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity ($1,038{\pm}620.41mL$) than the nonventilator group ($1,455{\pm}603.2mL$). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio ($1.7{\pm}0.44$) was lower in the home ventilator group than in the nonventilator group ($2.02{\pm}0.62$. The mitral valve annular systolic velocity was higher in the home ventilator group (estimated ${\beta}$, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Conclusion: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.

키워드

참고문헌

  1. Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Cripe L, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol 2010;9:77-93. https://doi.org/10.1016/S1474-4422(09)70271-6
  2. Deconinck N, Dan B. Pathophysiology of duchenne muscular dystrophy: current hypotheses. Pediatr Neurol 2007;36:1-7. https://doi.org/10.1016/j.pediatrneurol.2006.09.016
  3. Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Cripe L, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol 2010;9:177-89. https://doi.org/10.1016/S1474-4422(09)70272-8
  4. Gomez-Merino E, Bach JR. Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil 2002;81:411-5. https://doi.org/10.1097/00002060-200206000-00003
  5. Eagle M, Baudouin SV, Chandler C, Giddings DR, Bullock R, Bushby K. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 2002;12:926-9. https://doi.org/10.1016/S0960-8966(02)00140-2
  6. Finder JD, Birnkrant D, Carl J, Farber HJ, Gozal D, Iannaccone ST, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004;170:456-65. https://doi.org/10.1164/rccm.200307-885ST
  7. El-Aloul B, Altamirano-Diaz L, Zapata-Aldana E, Rodrigues R, Malvankar-Mehta MS, Nguyen CT, et al. Pharmacological therapy for the prevention and management of cardiomyopathy in Duchenne muscular dystrophy: a systematic review. Neuromuscul Disord 2017;27:4-14. https://doi.org/10.1016/j.nmd.2016.09.019
  8. Kwon SW, Kang SW, Kim JY, Choi EY, Yoon YW, Park YM, et al. Outcomes of cardiac involvement in patients with late-stage Duchenne muscular dystrophy under management in the pulmonary rehabilitation center of a tertiary referral hospital. Cardiology 2012;121:186-93. https://doi.org/10.1159/000336810
  9. Raman SV, Hor KN, Mazur W, Halnon NJ, Kissel JT, He X, et al. Eplerenone for early cardiomyopathy in Duchenne muscular dystrophy: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 2015;14:153-61. https://doi.org/10.1016/S1474-4422(14)70318-7
  10. Stein CA. Eteplirsen approved for Duchenne muscular dystrophy: The FDA faces a difficult choice. Mol Ther 2016;24:1884-5. https://doi.org/10.1038/mt.2016.188
  11. Viollet L, Thrush PT, Flanigan KM, Mendell JR, Allen HD. Effects of angiotensin-converting enzyme inhibitors and/or beta blockers on the cardiomyopathy in Duchenne muscular dystrophy. Am J Cardiol 2012;110:98-102. https://doi.org/10.1016/j.amjcard.2012.02.064
  12. Cox GF, Kunkel LM. Dystrophies and heart disease. Curr Opin Cardiol 1997;12:329-43.
  13. Barber BJ, Andrews JG, Lu Z, West NA, Meaney FJ, Price ET, et al. Oral corticosteroids and onset of cardiomyopathy in Duchenne muscular dystrophy. J Pediatr 2013;163:1080-4.e1. https://doi.org/10.1016/j.jpeds.2013.05.060
  14. Kantor PF, Lougheed J, Dancea A, McGillion M, Barbosa N, Chan C, et al. Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines. Can J Cardiol 2013;29:1535-52. https://doi.org/10.1016/j.cjca.2013.08.008
  15. Sohn DW, Chai IH, Lee DJ, Kim HC, Kim HS, Oh BH, et al. Assessment of mitral annulus velocity by Doppler tissue imaging in the evaluation of left ventricular diastolic function. J Am Coll Cardiol 1997;30:474-80. https://doi.org/10.1016/S0735-1097(97)88335-0
  16. Sarnat HB. Muscular dystrophies. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, editors. Nelson textbook of pediatrics. 20th ed. Philadelphia (PA): Elsevier Saunders, 2015:2976-9.
  17. Jo WH, Eun LY, Jung JW, Choi JY, Gang SW. Early marker of myocardial deformation in children with Duchenne muscular dystrophy assessed using echocardiographic myocardial strain analysis. Yonsei Med J 2016;57:900-4. https://doi.org/10.3349/ymj.2016.57.4.900
  18. Mehmood M, Ambach SA, Taylor MD, Jefferies JL, Raman SV, Taylor RJ, et al. Relationship of right ventricular size and function with respiratory status in Duchenne muscular dystrophy. Pediatr Cardiol 2016;37:878-83. https://doi.org/10.1007/s00246-016-1362-2
  19. Villanova M, Brancalion B, Mehta AD. Duchenne muscular dystrophy: life prolongation by noninvasive ventilatory support. Am J Phys Med Rehabil 2014;93:595-9. https://doi.org/10.1097/PHM.0000000000000074
  20. Finsterer J, Stollberger C. The heart in human dystrophinopathies. Cardiology 2003;99:1-19. https://doi.org/10.1159/000068446

피인용 문헌

  1. Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy vol.60, pp.3, 2018, https://doi.org/10.1002/mus.26623
  2. Is It Possible for Children in Duchenne Muscular Dystrophy to Preserve Cardiac Function with Medical Assistance? vol.7, pp.11, 2020, https://doi.org/10.3390/children7110249