Lung

, Volume 184, Issue 4, pp 205–215 | Cite as

Cardiopulmonary Support in Duchenne Muscular Dystrophy

Article
Accepted:

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked, rapidly progressive myopathy affecting the limb muscles, the respiratory muscles, the heart, the intestines, and the brain. Since about 90% of DMD patients die from muscular respiratory failure or cardiomyopathy, early and adequate therapy is essential. Ventilatory failure from muscle weakness requires mechanical support for ventilation and coughing as soon as there is symptomatic nocturnal hypoventilation. Today noninvasive positive-pressure ventilation (NIPPV) is the method of choice for supportive long-term mechanical ventilation in DMD. For assisted coughing, various methods are available, among which the mechanical in-exsufflator is the most widely used device. There is large nonrandomized clinical trial evidence that NIPPV improves quality of life and prolongs the lives of DMD patients if medical, social, economic, and ethical issues, raised by the availability of long-term NIPPV, are adequately addressed. Cardiac involvement in DMD manifests as impulse generation or impulse conduction abnormalities or cardiomyopathy. Cardiac abnormalities in DMD respond well to adequate therapy. Though DMD is ultimately a fatal disease, quality of life and life expectancy can be markedly improved if cardiopulmonary manifestations are adequately treated.

Keywords

Respiratory failure Mechanical ventilation Neuromuscular Myopathy Heart failure 
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References

  1. 1.
    Ayoub J, Milane J, Targhetta R, et al. (2002) Diaphragm kinetics during pneumatic belt respiratory assistance: a sonographic study in Duchenne muscular dystrophy. Neuromuscul Disord 12:569–575PubMedCrossRefGoogle Scholar
  2. 2.
    Bach JR (2003) Tracheobronchomalacia and noninvasive ventilation? Chest 124:2038PubMedCrossRefGoogle Scholar
  3. 3.
    Backman E, Nylander E (1992) The heart in Duchenne muscular dystrophy: a non-invasive longitudinal study. Eur Heart J 13:1239–1244PubMedGoogle Scholar
  4. 4.
    Bakker E, VanOmmen GJR (1998) Duchenne and Becker muscular dystrophy (DMD and BMD). In: Emery AEH, ed. Neuromuscular disorders: Clinical and molecular genetics. Wiley, New York, pp 59–85Google Scholar
  5. 5.
    Barbe F, Quera-Salva MA, McCann C, et al. (1994) Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy. Eur Respir J 7:1403–1408PubMedCrossRefGoogle Scholar
  6. 6.
    Baydur A, Kanel G (2003) Tracheobronchomalacia and tracheal hemorrhage in patients with Duchenne muscular dystrophy receiving long-term ventilation with uncuffed tracheostomies. Chest 123:1307–1131PubMedCrossRefGoogle Scholar
  7. 7.
    Baydur A, Kanel G, Koss M (2004) Tracheobronchomalacia and noninvasive ventilation revisited. Chest 126:1390–1392PubMedCrossRefGoogle Scholar
  8. 8.
    Bhattacharyya KB, Basu N, Ray TN, Maity B (1997) Profile of electrocardiographic changes in Duchenne muscular dystrophy. J Indian Med Assoc 95:40–42PubMedGoogle Scholar
  9. 9.
    Birnkrant DJ, Pope JF, Eiben RM (1999) Management of respiratory complications of neuromuscular diseases in the pediatric intensive care unit. J Child Neurol 14:139–143PubMedGoogle Scholar
  10. 10.
    Boland BJ, Silbert PL, Groover RV, Wollan PC, Silverstein MD (1996) Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy. Pediatr Neurol 14:7–12PubMedCrossRefGoogle Scholar
  11. 11.
    Breucking E, Reimnitz P, Schara U, Mortier W (2000) Anesthetic complications. The incidence of severe anesthetic complications in patients and families with progressive muscular dystrophy of the Duchenne and Becker types. Anaesthesist 49:187–195PubMedCrossRefGoogle Scholar
  12. 12.
    Breithardt G, Borggrefe M, Karbenn U, et al. (1982) Prevalence of late potentials in patients with and without ventricular tachycardia: correlation with angiographic findings. Am J Cardiol 49:1932–1937PubMedCrossRefGoogle Scholar
  13. 13.
    Brockmeier K, Schmitz L, vonMoers A, et al. (1998) X-chromosomal (p21) muscular dystrophy and left ventricular diastolic and systolic function. Pediatr Cardiol 19:139–144PubMedCrossRefGoogle Scholar
  14. 14.
    Carroll N, Bain RJ, Smith PE, et al. (1991) Domiciliary investigation of sleep-related hypoxaemia in Duchenne muscular dystrophy. Eur Respir J 4:434–440PubMedGoogle Scholar
  15. 15.
    Carter GT, McDonald CM (2000) Preserving function in Duchenne dystrophy with long-term pulse prednisone therapy. Am J Phys Med Rehabil 79:455–458PubMedCrossRefGoogle Scholar
  16. 16.
    Cervellati S, Bettini N, Moscato M, et al. (2004) Surgical treatment of spinal deformities in Duchenne muscular dystrophy: a long term follow-up study. Eur Spine J 13:441–448PubMedCrossRefGoogle Scholar
  17. 17.
    Chenard AA, Becane HM, Tertrain F, deKermadec JM, Weiss YA (1993) Ventricular arrhythmia in Duchenne muscular dystrophy: prevalence, significance and prognosis. Neuromuscul Disord 3:201–206PubMedCrossRefGoogle Scholar
  18. 18.
    Corrado G, Lissoni A, Beretta S, et al. (2002) Prognostic value of electrocardiograms, ventricular late potentials, ventricular arrhythmias, and left ventricular systolic dysfunction in patients with Duchenne muscular dystrophy. Am J Cardiol 89:838–841PubMedCrossRefGoogle Scholar
  19. 19.
    Cox GF, Kunkel LM (1997) Dystrophies and heart disease. Curr Opin Cardiol 12:329–343PubMedGoogle Scholar
  20. 20.
    Curran FJ, Colbert AP (1989) Ventilator management in Duchenne muscular dystrophy and postpoliomyelitis syndrome: twelve years’ experience. Arch Phys Med Rehabil 70:180–185PubMedGoogle Scholar
  21. 21.
    Cziner DG, Levin RI (1993) The cardiomyopathy of Duchenne’s muscular dystrophy and the function of dystrophin. Med Hypotheses 40:169–173PubMedCrossRefGoogle Scholar
  22. 22.
    D’Orsogna L, O’Shea JP, Miller G (1988) Cardiomyopathy of Duchenne muscular dystrophy. Pediatr Cardiol 9:205–213PubMedCrossRefGoogle Scholar
  23. 23.
    De Bruin PF, Ueki J, Bush A, et al. (1997) Diaphragm thickness and inspiratory strength in patients with Duchenne muscular dystrophy. Thorax 52:472–475PubMedCrossRefGoogle Scholar
  24. 24.
    De Bruin PF, Ueki J, Bush A, et al. (2001) Inspiratory flow reserve in boys with Duchenne muscular dystrophy. Pediatr Pulmonol 31:451–457PubMedCrossRefGoogle Scholar
  25. 25.
    deKermadec JM, Becane HM, Chenard A, Tertrain F, Weiss Y (1994) Prevalence of left ventricular systolic dysfunction in Duchenne muscular dystrophy: an echocardiographic study. Am Heart J 127:618–623CrossRefGoogle Scholar
  26. 26.
    Doing AH, Renlund DG, Smith RA (2002) Becker muscular dystrophy-related cardiomyopathy: a favorable response to medical therapy. J Heart Lung Transplant 21:496–498PubMedCrossRefGoogle Scholar
  27. 27.
    Farah MG, Evans EB, Vignos PL (1980) Echocardiographic evaluation of left ventricular function in Duchenne’s muscular dystrophy. Am J Med 69:248–254PubMedCrossRefGoogle Scholar
  28. 28.
    Fenoglio JJ, Pham TD, Harken AH, et al. (1983) Recurrent sustained ventricular tachycardia: structure and ultrastructure of subendocardial regions in which tachycardia originated. Circulation 68:518–533PubMedGoogle Scholar
  29. 29.
    Finder JD, Birnkrant D, Carl J, et al. (2004) American Thoracic Society Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 170:456–465PubMedCrossRefGoogle Scholar
  30. 30.
    Finsterer J, Stöllberger C (2003) The heart in human dystrophinopathies. Cardiology 99:1–19PubMedCrossRefGoogle Scholar
  31. 31.
    Finsterer J, Stöllberger C, Blazek G, Spahits E (2001) Cardiac involvement in myotonic dystrophy, Becker muscular dystrophy and mitochondrial myopathy: a five year follow-up. Can J Cardiol 17:1061–1069PubMedGoogle Scholar
  32. 32.
    Frankel KA, Rosser RL (1976) The pathology of the heart in progressive muscular dystrophy. Epimyocardial fibrosis. Hum Pathol 7:375–386PubMedGoogle Scholar
  33. 33.
    Gaffney JF, Kingston WJ, Metlay LA, Gramiak R (1989) Left ventricular thrombus and systemic emboli complicating the cardiomyopathy of Duchenne’s muscular dystrophy. Arch Neurol 46:1249–1252PubMedGoogle Scholar
  34. 34.
    Gomez-Merino E, Bach JR (2002) Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil 81:411–415PubMedCrossRefGoogle Scholar
  35. 35.
    Gozal D (2000) Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy. Pediatr Pulmonol 29:141–150PubMedCrossRefGoogle Scholar
  36. 36.
    Gozal D, Thiriet P (1999) Respiratory muscle training in neuromuscular disease: long-term effects on strength and load perception. Med Sci Sports Exerc 31:1522–1527PubMedCrossRefGoogle Scholar
  37. 37.
    Hukins CA, Hillman DR (2000) Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med 161:166–170PubMedGoogle Scholar
  38. 38.
    Ishihara T (2003) Cardiopulmonary failure in Duchenne muscular dystrophy—pathophysiology and management. Rinsho Shinkeigaku 43:761–764PubMedGoogle Scholar
  39. 39.
    Ishikawa K (1997) Cardiac involvement in progressive muscular dystrophy of the Duchenne type. Jpn Heart J 38:l63–180Google Scholar
  40. 40.
    Ishikawa Y, Bach JR (2000) Nocturnal oxygenation and prognosis in Duchenne muscular dystrophy. Am J Respir Crit Care Med 161:675–676PubMedGoogle Scholar
  41. 41.
    Kanzato N, Suehara M, Mayumi H, et al. (2000) Tracheopulmonary-subcutaneous fistula associated with anaerobic subcutaneous abscess on mechanical ventilator support in a patient with Duchenne muscular dystrophy. Rinsho Shinkeigaku 40:490–493PubMedGoogle Scholar
  42. 42.
    Kawai H, Adachi K, Kimura C, et al. (1990) Secretion and clinical significance of atrial natriuretic peptide in patients with muscular dystrophy. Arch Neurol 47:900–904PubMedGoogle Scholar
  43. 43.
    Kawai M, Shintani M, Komiya T, Yatabe K (1995) Nocturnal hypoxia index: a new pulse oxymetry index of nocturnal hypoventilation in neuromuscular disorders. Rinsho Shinkeigaku 35:1003–1007Google Scholar
  44. 44.
    Klefbeck B, Svartengren K, Camner P, et al. (2001) Lung clearance in children with Duchenne muscular dystrophy or spinal muscular atrophy with and without CPAP (continuous positive airway pressure). Exp Lung Res 27:469–484PubMedCrossRefGoogle Scholar
  45. 45.
    Koessler W, Wanke T, Winkler G, et al. (2001) 2 Years’ experience with inspiratory muscle training in patients with neuromuscular disorders. Chest 120:765–769PubMedCrossRefGoogle Scholar
  46. 46.
    Kubo M, Matsuoka S, Taguchi Y, Akita H, Kuroda Y (1993) Clinical significance of late potentials in patients with Duchenne muscular dystrophy. Pediatr Cardiol 14:214–219PubMedCrossRefGoogle Scholar
  47. 47.
    Lanza GA, Russo AD, Giglio V, et al. (2001) Impairment of cardiac autonomic function in patients with Duchenne muscular dystrophy: relationship to myocardial and respiratory function. Am Heart J 141:808–812PubMedCrossRefGoogle Scholar
  48. 48.
    Lyager S, Steffensen B, Juhl B (1995) Indicators of need for mechanical ventilation in Duchenne muscular dystrophy and spinal muscular atrophy. Chest 108:779–785PubMedGoogle Scholar
  49. 49.
    MacDuff A, Grant IS (2003) Critical care management of neuromuscular disease, including long-term ventilation. Curr Opin Crit Care 9:106–112PubMedCrossRefGoogle Scholar
  50. 50.
    Mallory GB (2004) Pulmonary complications of neuromuscular disease. Pediatr Pulmonol Suppl 26:138–140PubMedCrossRefGoogle Scholar
  51. 51.
    Matsumura T, Saito T, Miyai I, Nozaki S, Kang J (1998) Excess caloric intake induced severe hypercapnia in a patient with Duchenne muscular dystrophy on non-invasive positive pressure ventilation. Rinsho Shinkeigaku 38:822–825PubMedGoogle Scholar
  52. 52.
    Matsumura T, Saito T, Miyai I, Nozaki S, Kang J (1999) Effective milrinone therapy to a Duchenne muscular dystrophy patient with advanced congestive heart failure. Rinsho Shinkeigaku 39:643–648PubMedGoogle Scholar
  53. 53.
    Matsumura T, Saito T, Miyai I, Nozaki S, Kang J (2000) Electrolyte abnormalities and metabolic acidosis in two Duchenne muscular dystrophy patients with advanced congestive heart failure. Rinsho Shinkeigaku 40:439–445PubMedGoogle Scholar
  54. 54.
    Melacini P, Vianello A, Villanova C, et al. (1996) Cardiac and respiratory involvement in advanced stage Duchethe mascular dystrophy. Neuromuscul Disord 6:367–376PubMedCrossRefGoogle Scholar
  55. 55.
    Miller F, Moseley CF, Koreska J, Levison H (1988) Pulmonary function and scoliosis in Duchenne dystrophy. J Pediatr Orthop 8:133–137PubMedGoogle Scholar
  56. 56.
    Miller G, D’Orsogna L, O’Shea JP (1989) Autonomic function and the sinus tachycardia of Duchenne muscular dystrophy. Brain Dev 11:247–250PubMedGoogle Scholar
  57. 57.
    Miske LJ, Hickey EM, Kolb SM, Weiner DJ, Panitch HB (2004) Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough. Chest 125:1406–1412PubMedCrossRefGoogle Scholar
  58. 58.
    Moriuchi T, Fujii Y, Kagawa N, Hizawa K (1991) Autopsy study on the weight of the heart, liver, kidney and brain in Duchenne muscular dystrophy. Tokushima J Exp Med 38:5–13PubMedGoogle Scholar
  59. 59.
    Moriuchi T, Kagawa N, Mukoyama M, Hizawa (1991) Autopsy analysis of the muscular dystrophies. Tokushima J Exp Med 40:83–93Google Scholar
  60. 60.
    Nagai T (1989) Prognostic evaluation of congestive heart failure in patients with Duchenne muscular dystrophy—retrospective study using non-invasive cardiac function tests. Jpn Circ J 53:406–415PubMedGoogle Scholar
  61. 61.
    Nakayama T, Saito Y, Yatabe K, Uchiyama T, Kawai M (1999) The usefulness of tracheostomy in Duchenne muscular dystrophy ventilated by a chest respirator. Rinsho Shinkeigaku 39:606–609PubMedGoogle Scholar
  62. 62.
    Negri SM, Cowan MD (1998) Becker muscular dystrophy with bundle branch reentry ventricular tachycardia. J Cardiovasc Electrophysiol 9:652–654PubMedGoogle Scholar
  63. 63.
    Nigro G, Comi LI, Politano L, Bain RJ (1990) The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy. Int J Cardiol 26:271–277PubMedCrossRefGoogle Scholar
  64. 64.
    Nigro G, Comi LI, Politano L, et al. (1995) Evaluation of the cardiomyopathy in Becker muscular dystrophy. Muscle Nerve 18:283–291PubMedCrossRefGoogle Scholar
  65. 65.
    Nishimura T, Yanagisawa A, Sakata H, et al. (2001) Thallium-201 single photon emission computed tomography (SPECT) in patients with Duchenne’s progressive muscular dystrophy: a histopathologic correlation study. Jpn Circ J 65:99–105PubMedCrossRefGoogle Scholar
  66. 66.
    Orlov YSK, Brodsky MA, Allen BJ, et al. (1994) Cardiac manifestations and their management in Becker’s muscular dystrophy. Am Heart J 128: 193–196PubMedCrossRefGoogle Scholar
  67. 67.
    Oya Y, Ogawa M, Kawai M (2004) Respiratory system elastance and resistance measured by proportional assist ventilation in patients with respiratory muscle weakness. Rinsho Shinkeigaku 44:268–273PubMedGoogle Scholar
  68. 68.
    Pahnucci L, Mongini T, Chiado-Piat L, Doriguzzi C, Fubini A (2000) Dystrophinopathy expressing as either cardiomyopathy or Becker dystrophy in the same family. Neurology 54:529–530Google Scholar
  69. 69.
    Perloff JK, Roberts SC, deLeon AC Jr, O’Doherty D (1967) Distinctive electrocardiogram of Duchenne’s progressive muscular dystrophy. Am J Med 42:170–188CrossRefGoogle Scholar
  70. 70.
    Perloff JK, Henze E, Schelbert HR (1984) Alterations in regional myocardial metabolism, perfusion, and wall motion in Duchenne muscular dystrophy studied by radionuclide imaging. Circulation 69:33–42PubMedGoogle Scholar
  71. 71.
    Phillips MF, Smith PE, Carroll N, Edwards RH, Calverley PM (1998) Nocturnal oxygenation and prognosis in Duchenne muscular dystrophy. Am J Respir Crit Care Med 160:198–202Google Scholar
  72. 72.
    Piazza C, Bolzoni A, Cavaliere S, Peretti G (2003) Tracheocoele in a Duchenne muscular dystrophy patient. Case report. Acta Otorhinolaryngol Ital 23:194–198Google Scholar
  73. 73.
    Quinlivan R, Ball J, Dunckley M, et al. (1995) Becker muscular dystrophy presenting with complete heart block in the sixth decade. J Neurol 242:398–400PubMedCrossRefGoogle Scholar
  74. 74.
    Quinlivan RM, Lewis P, Marsden P, et al. (1996) Cardiac function, metabolism and perfusion in Duchenne and Becker muscular dystrophy. Neuromuscul Disord 6:237–246PubMedCrossRefGoogle Scholar
  75. 75.
    Raphael JC, Chevret S, Chastang C, Bouvet F (1994) Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy. Lancet 343:1600–1604PubMedCrossRefGoogle Scholar
  76. 76.
    Raphael JC, Chevret S, Auriant I, Clair B, Gajdos P (1998) Long-term ventilation at home in adults with neurological diseases. Rev Mal Respir 15:495–505PubMedGoogle Scholar
  77. 77.
    Raphael JC, Dazord A, Jaillard P, et al. (2002) Assessment of quality of life for home ventilated patients with Duchenne muscular dystrophy. Rev Neurol (Paris) 158:453–460Google Scholar
  78. 78.
    Rees W, Schuler S, Hummel M, Hetzer R (1993) Heart transplantation in patients with muscular dystrophy associated with end-stage cardiomyopathy. J Heart Lung Transplant 12:804–807PubMedGoogle Scholar
  79. 79.
    Riggs T (1990) Cardiomyopathy and pulmonary emboli in terminal Duchenne’s muscular dystrophy. Am Heart J 119:690–693PubMedCrossRefGoogle Scholar
  80. 80.
    Rittoo DB, Morris P (1995) Tracheal occlusion in the prone position in an intubated patient with Duchenne muscular dystrophy. Anaesthesia 50:719–721PubMedGoogle Scholar
  81. 81.
    Rodillo E, Noble-Jamieson CM, Aber V, et al. (1989) Respiratory muscle training in Duchenne muscular dystrophy. Arch Dis Child 64(5):736–738PubMedCrossRefGoogle Scholar
  82. 82.
    Rumbak MJ, Walker RM (2001) Should patients with neuromuscular disease be denied the choice of the treatment of mechanical ventilation? Chest 119:683–684PubMedCrossRefGoogle Scholar
  83. 83.
    Saito M, Kawai H, Akaike M, et al. (1996) Cardiac dysfunction with Becker muscular dystrophy. Am Heart J 132:642–647PubMedCrossRefGoogle Scholar
  84. 84.
    Saito T, Takenaka M, Miyai I, et al. (2001) Coagulation and fibrinolysis disorder in muscular dystrophy. Muscle Nerve 24:399–402PubMedCrossRefGoogle Scholar
  85. 85.
    Saitoh M, Kasagi S, Miyakoda H, Kotake H, Mashiba H (1991) Evaluation of cardiac complications in patients with Duchenne muscular dystrophy using echocardiography—a comparison between patients with and without mechanical ventilation. No To Shinkei 43:37–41PubMedGoogle Scholar
  86. 86.
    Shinoe T, Kawai M (1996) Tracheal deformity observed in tracheostomized Duchenne muscular dystrophy patients who can speak with appropriately inflated cuff of tracheal tube. Rinsho Shinkeigaku 36:355–357PubMedGoogle Scholar
  87. 87.
    Shneerson JM, Simonds AK (2002) Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J 20:480–487PubMedCrossRefGoogle Scholar
  88. 88.
    Simonds AK (2003) Home ventilation. Eur Respir J Suppl 47:38s–46sPubMedCrossRefGoogle Scholar
  89. 89.
    Suarez AA, Pessolano FA, Monteiro SG, et al. (2002) Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease. Am J Phys Med Rehabil 81:506–511PubMedCrossRefGoogle Scholar
  90. 90.
    Takenaka A, Yokota M, Iwase M, et al. (1993) Discrepancy between systolic and diastolic dysfunction of the left ventricle in patients with Duchenne muscular dystrophy. Eur Heart J 14:669–676PubMedGoogle Scholar
  91. 91.
    Topin N, Matecki S, Le Bris S, et al. (2002) Dose-dependent effect of individualized respiratory muscle training in children with Duchenne muscular dystrophy. Neuromuscul Disord 12:576–583PubMedCrossRefGoogle Scholar
  92. 92.
    van Kesteren RG, Kampelmacher MJ (2000) Mechanical ventilation in neuromuscular diseases: do not start too early, but certainly not too late. Ned Tijdschr Geneeskd 144:1249–1252Google Scholar
  93. 93.
    Vianello A, Bevilacqua M, Salvador V, Cardaioli C, Vincenti E (1994) Long-term nasal intermittent positive pressure ventilation in advanced Duchenne’s muscular dystrophy. Chest 105:445–448PubMedGoogle Scholar
  94. 94.
    Vianello A, Arcaro G, Gallan F, Ori C, Bevilacqua M (2004) Pneumothorax associated with long-term non-invasive positive pressure ventilation in Duchenne muscular dystrophy. Neuromuscul Disord 14:353–355PubMedCrossRefGoogle Scholar
  95. 95.
    Vignos PJ Jr (1977) Respiratory function and pulmonary infection in Duchenne muscular dystrophy. Isr J Med 13:207–214Google Scholar
  96. 96.
    Wakai S, Minami R, Kameda K, et al. (1988) Electron microscopic study of the biopsied cardiac muscle in Duchenne muscular dystrophy. J Neurol Sci 84:167–175PubMedCrossRefGoogle Scholar
  97. 97.
    Winkler G, Zifko U, Nader A, et al. (2000) Dose-dependent effects of inspiratory muscle training in neuromuscular disorders. Muscle Nerve 23:1257–1260PubMedCrossRefGoogle Scholar
  98. 98.
    Yamamoto T, Kawai M (1994) Spontaneous pneumothorax in Duchenne muscular dystrophy. Rinsho Shinkeigaku 34:552–556PubMedGoogle Scholar
  99. 99.
    Yanagisawa A, Yokota N, Myagawa M, et al. (1992) Plasma levels of natriuretic peptide in patients with Duchenne’s progressive muscular dystrophy. Am Heart J 120:1154–1158CrossRefGoogle Scholar
  100. 100.
    Yasuma F, Kato T, Matsuoka Y, Konagaya M (2001) Row-a-boat phenomenon: respiratory compensation in advanced Duchenne muscular dystrophy. Chest 119:1836–1839PubMedCrossRefGoogle Scholar
  101. 101.
    Yotsukura M, Miyagawa M, Tsuya T, Ishihara T, Ishikawa A (1992) A 10-year follow-up study by orthogonal Frank lead ECG on patients with progressive muscular dystrophy of the Duchenne type. J Electrocardiol 25:345–353PubMedCrossRefGoogle Scholar
  102. 102.
    Yotsukura M, Sasaki K, Kachi E, et al. (1995) Circadian rhythm and variability of heart rate in Duchenne type progressive muscular dystrophy. Am J Cardiol 765:947–951CrossRefGoogle Scholar
  103. 103.
    Yotsukura M, Yamamoto A, Kajiwara T, et al. (1999) QT dispersion in patients with Duchenne type progressive muscular dystrophy. Am Heart J 137:672–677PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  1. 1.Krankenstalt RudolfstiftungViennaAustria

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