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Cardiac Disease Burden and Risk of Mortality in Hospitalized Muscular Dystrophy Patients

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Abstract

As treatments of the extra-cardiac complications of muscular dystrophy (MD) improve, males with MD are more likely to develop cardiac disease. The impact of cardiomyopathy or heart failure (HF) and ventricular tachycardia (VT) on hospitalizations and in hospital mortality are not known. We performed an analysis of inpatient admission data for patients with MD using the Pediatric Health Information System database. We selected males who were 6 years or older with diagnosis codes of MD and cardiac disease including cardiomyopathy/HF and VT between 2003 and 2013. We created a logistic regression model to identify predictors of subsequent cardiac arrest or death in MD patients. We also compared hospital charges, lengths of stay and ages among MD patients with or without cardiac disease. Our logistic regression model showed that VT (OR 5.41, 95 % CI 2.83, 10.34) and cardiomyopathy/HF (OR 1.79, 95 % CI 1.05, 3.04) were risk factors for cardiac arrest or death. Of the 84 cardiac arrests or deaths in 3363 MD patients, 49 (58 %) were related to cardiac disease. Nineteen (39 %) of these events occurred in MD patients with VT. The mean hospital charges and the mean length of stay were greater and longer in MD patients with VT compared to those without cardiac disease and those with only cardiomyopathy/HF (p < 0.05). Cardiac disease is a significant burden in hospitalized MD patients. Our results suggest that VT and cardiomyopathy/HF are associated with an increased risk of cardiac arrest or death in MD patients.

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References

  1. Mercuri E, Muntoni F (2013) Muscular dystrophy: new challenges and review of the current clinical trials. Curr Opin Pediatr 25:701–707

    Article  CAS  PubMed  Google Scholar 

  2. Sarnat HB (2007) Neuromuscular Disorders. In: Kleigman RM, Behrman RE, Jenson HB, Stanton BF (eds) Nelson textbook of pediatrics, 18th edn. Saunders Elsevier, Philadelphia, pp 2531–2568

    Google Scholar 

  3. Theadom A, Rodriguez M, Roxburgh R, Balalla S, Higgins C, Bhattacharjee R, Jones K, Krishnamurthi R, Feigin V (2014) Prevalence of muscular dystrophies: a systematic literature review. Neuroepidemiology 43:259–268

    Article  PubMed  Google Scholar 

  4. Emery AE (1991) Population frequencies of inherited neuromuscular diseases—a world survey. Neuromuscul Disord 1:19–29

    Article  CAS  PubMed  Google Scholar 

  5. Mendell JR, Shilling C, Leslie ND, Flanigan KM, al-Dahhak R, Gastier-Foster J, Kneile K, Dunn DM, Duval B, Aoyagi A, Hamil C, Mahmoud M, Roush K, Bird L, Rankin C, Lilly H, Street N, Chandrasekar R, Weiss RB (2012) Evidence-based path to newborn screening for Duchenne muscular dystrophy. Ann Neurol 71:304–313

    Article  CAS  PubMed  Google Scholar 

  6. Spurney CF (2011) Cardiomyopathy of Duchenne muscular dystrophy: current understanding and future directions. Muscle Nerve 44:8–19

    Article  PubMed  Google Scholar 

  7. Fauconnier J, Thireau J, Reiken S, Cassan C, Richard S, Matecki S, Marks AR, Lacampagne A (2010) Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy. PNAS 107:1559–1564

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Finder JD, Birnkrant D, Carl J, Farber HJ, Gozal D, Iannaccone ST, Kovesi T, Kravitz RM, Panitch H, Schramm C, Schroth M, Sharma G, Sievers L, Silvestri JM, Sterni L, American Thoracic Society (2004) Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 170:456–465

    Article  PubMed  Google Scholar 

  9. Moxley RT, Pandya S, Ciafaloni E, Fox DJ, Campbell K (2010) Change in natural history of Duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management. J Child Neurol 9:1116–1129

    Article  Google Scholar 

  10. Eagle M, Baudoin SV, Chandler C, Giddings D, Bullock R, Bushby K (2002) Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 12:926–929

    Article  PubMed  Google Scholar 

  11. Spurney C, Shimizu R, Morgenroth LP, Kolski H, Gordish-Dressman H, Clemens PR, CINRG Investigators et al (2014) Cooperative International Neuromuscular Research Group Duchenne Natural History Study demonstrates insufficient diagnosis and treatment of cardiomyopathy in Duchenne muscular dystrophy. Muscle Nerve 50:250–256

    Article  PubMed  PubMed Central  Google Scholar 

  12. Barber BJ, Andrews JG, Lu Z, West N, Meaney FJ, Price ET, Gray A, Sheehan DW, Pandya S, Yang M, Cunniff C (2013) Oral corticosteroids and onset of cardiomyopathy in Duchenne muscular dystrophy. J Pediatr 163:1080–1084

    Article  CAS  PubMed  Google Scholar 

  13. Finsterer J, Stollberger C (2003) The heart in human dystrophinopathies. Cardiology 99:1–19

    Article  PubMed  Google Scholar 

  14. Nigro G, Comi LI, Politano L, Bain RJ (1990) The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy. Int J Cardiol 26:271–277

    Article  CAS  PubMed  Google Scholar 

  15. Connuck DM, Sleeper LA, Colan SD, Cox GF, Towbin JA, Lowe AM, Wilkinson JD, Orav EJ, Cuniberti L, Salbert BA, Lipshultz SE, Pediatric Cardiomyopathy Registry Study Group (2008) Characteristics and outcomes of cardiomyopathy in children with Duchenne or Becker muscular dystrophy: a comparative study from the pediatric cardiomyopathy registry. Am Heart J 155:998–1005

    Article  PubMed  PubMed Central  Google Scholar 

  16. Duboc D, Meune C, Pierre B, Wahbi K, Eymard B, Toutain A, Berard C, Vaksmann G, Weber S, Becane HM (2007) Perindopril preventive treatment on mortality in Duchenne muscular dystrophy: 10 years’ follow-up. Am Heart J 154:595–602

    Article  Google Scholar 

  17. Wagner KR, Lechtzin N, Judge DP (2007) Current treatment of adult Duchenne muscular dystrophy. Biochim Biophys Acta 1772:229–237

    Article  CAS  PubMed  Google Scholar 

  18. Yanagisawa A, Miyagawa M, Yotsukura M, Tsuya T, Shirato C, Ishihara T, Aoyagi T, Ishikawa K (1992) The prevalence and prognostic significance of arrhythmias in Duchenne type muscular dystrophy. Am Heart J 124:1244–1250

    Article  CAS  PubMed  Google Scholar 

  19. Chenard AA, Becane HM, Tertrain F, de Kermadec JM, Weiss YA (1993) Ventricular arrhythmia in Duchenne muscular dystrophy: prevalence, significance and prognosis. Neuromuscul Disord 3:201–206

    Article  CAS  PubMed  Google Scholar 

  20. Groh WJ (1985) Arrhythmias in the muscular dystrophies. Heart Rhythm 9:1890–1895

    Article  Google Scholar 

  21. Diegoli M, Grasso M, Favalli V, Seria A, Gambarin FI, Klersy C, Pasott M, Agozzino E, Scelsi L, Ferlini A, Febo O, Piccoli G, Tavazzi L, Narula J, Arbustini E (2011) Diagnostic work-up and risk stratification in X-linked dilated cardiomyopathies caused by dystrophin defects. J Am Coll Cardiol 58:925–934

    Article  PubMed  Google Scholar 

  22. Muscular Dystrophy. Center for disease control and prevention website. http://www.cdc.gov/ncbddd/musculardystrophy/facts.html. Updated 14 Apr 2015. Accessed 9 Sep 2015

  23. Gulati S, Saxena A, Kumar V, Kalra V (2005) Duchenne muscular dystrophy: prevalence and patterns of cardiac involvement. Indian J Pediatr 72:389–393

    Article  PubMed  Google Scholar 

  24. Corrado G, Lissoni A, Beretta S, Terenghi L, Tadeo G, Foglia-Manzillo G, Tagliagambe LM, Spata M, Santarone M (2002) Prognostic value of electrocardiograms, ventricular late potentials, ventricular arrhythmias, and left ventricular systolic dysfunction in patients with Duchenne muscular dystrophy. Am J Cardiol 89:838–841

    Article  PubMed  Google Scholar 

  25. Singh SN, Fisher SG, Carson PE, Fletcher RD, The Department of Veteran Affairs CHF-STAT Investigators (1998) Prevalence and significance of nonsustained ventricular tachycardia in patients with premature ventricular contractions and heart failure treated with vasodilator therapy. J Am Coll Cardiol 32:942–947

    Article  CAS  PubMed  Google Scholar 

  26. Von Olshausen K, Schafer A, Mehmel HC, Schwarz F, Senger J, Kubler W (1984) Ventricular arrhythmias in idiopathic dilated cardiomyopathy. Br Heart J 51:195–201

    Article  Google Scholar 

  27. Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemns PA, Cripe L, Kaul A, Kinnett K, McDonal C, Pandya S, Poysky J, Shapiro F, Tomezsko J, Constantin C, DMD Care Considerations Working Group (2010) Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol 9:77–93

    Article  PubMed  Google Scholar 

  28. Spurney CF (2011) Cardiac complications of neuromuscular disorders. In: Bertorini TE (ed) Neuromuscular diseases: management and therapy, 1st edn. Saunders Elsevier, Philadelphia, pp 33–50

    Google Scholar 

  29. Kadish A, Dyer A, Daubert JP, Quigg R, Estes NAM, Anderson KP, Calkins H, Hoch D, Goldberger J, Shalaby A, Sanders WE, Schaecter A, Levine JH, Defibrillators in Non-Ischemic Cardiomyopathy Treatment Evaluation (DEFINITE) Investigators (2004) Prophylactic defibrillators implantation in patients with nonischemic dilated cardiomyopathy. N Engl J Med 350:2151–2158

    Article  CAS  PubMed  Google Scholar 

  30. Grimm W, Jurgen Hoffman, Muller HH, Maisch B (2002) Implantable defibrillator event rates in patients with idiopathic dilated cardiomyopathy, nonsustained ventricular tachycardia on holter and a left ventricular ejection fraction below 30 %. J Am Coll Cardiol 39:780–787

    Article  PubMed  Google Scholar 

  31. Moss AJ, Zareba W, Hall J, Klein H, Wilber DJ, Cannom DS, Daubert JP, Higgins SL, Brown MW, Andrews ML, Multicenter Automatic Defibrillator Implantation Trial II Investigators (2002) Prophylactic implantation of a defibrillator in patients with myocardial infarction and reduced ejection fraction. N Engl J Med 346:877–883

    Article  PubMed  Google Scholar 

  32. Bell CF, Kurosky SK, Candrilli SD (2015) Muscular dystrophy-related hospitalizations among male pediatric patients in the United States. Hosp Pract 1995:1–6

    Google Scholar 

  33. McNally EM, Kaltman JR, Benson DW, Canter CE, Cripe LH, Duan D, Finder JD, Hoffman EP, Judge DP, Kertesz N, Kinnett K, Kirsch R, Metzger JM, Pearson GD, Rafael-Fortney JA, Raman SV, Spurney CF, Targum SL, Wagner KR, Markham LW (2015) Contemporary cardiac issues in Duchenne muscular dystrophy. Circulation 131:1590–1598

    Article  PubMed  PubMed Central  Google Scholar 

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Acknowledgments

This project was supported by Award Number UL1TR000075 from the NIH National Center for Advancing Translational Sciences. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the National Center for Advancing Translational Sciences or the National Institutes of Health. The authors would like to acknowledge Jacob Hartz, MD, MPH (Division of Cardiology, Children’s National) for his assistance with data collection.

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Authors and Affiliations

  1. Division of Cardiology, Lurie Children’s Hospital of Chicago, 225 E Chicago Avenue, Chicago, IL, 60611, USA

    Ann R. Punnoose

  2. Division of Cardiology, Children’s National Health System, Washington, DC, USA

    Jonathan R. Kaltman & Christopher F. Spurney

  3. Office of Clinical Research Management, Children’s National Health System, Washington, DC, USA

    William Pastor

  4. Division of Biostatistics and Study Methodology, Children’s National Health System, Washington, DC, USA

    Robert McCarter & Jianping He

  5. Division of Cardiology, Children’s Hospital of Wisconsin, 8915 W. Connell Ct, P. O. Box 1997, Milwaukee, WI, 53226, USA

    Ann R. Punnoose

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  1. Ann R. Punnoose
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Correspondence to Ann R. Punnoose.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The study was approved by the Institutional Review Board at the Children’s National Health System.

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Punnoose, A.R., Kaltman, J.R., Pastor, W. et al. Cardiac Disease Burden and Risk of Mortality in Hospitalized Muscular Dystrophy Patients. Pediatr Cardiol 37, 1290–1296 (2016). https://doi.org/10.1007/s00246-016-1432-5

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  • DOI: https://doi.org/10.1007/s00246-016-1432-5

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