Published online May 1, 2006
PEDIATRICS Vol. 117 No. 5 May 2006, pp. 1864 (doi:10.1542/peds.2005-3189)
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Therapeutic Nihilism in Duchenne Cardiomyopathy

Jeffrey Rein, MD, FAAP
El Rio Neighborhood Health Center
Tucson, AZ 85719

To the Editor.

The recent American Academy of Pediatrics clinical report on cardiovascular supervision in Duchenne muscular dystrophy1 was overly nihilistic. Advances in diagnostic and therapeutic interventions were dismissed or ignored. The authors call for early recognition of cardiac dysfunction and acknowledge the limitations of echocardiography in scoliotic patients but do not mention B-type natriuretic peptide. Assay of serum B-type natriuretic peptide is widely available and may identify patients with cardiac involvement even before symptoms develop.2,3 Several studies, including some cited in their report, suggest the possibility of significant benefit from both angiotensin-converting enzyme (ACE) inhibition and ß blockade in the treatment of Duchenne cardiomyopathy.4–6 There are no reports of significant unanticipated risk from these therapies.

Unfortunately, optimal studies have not been conducted and may never be. The toll of cardiac disease on patients with muscular dystrophy is high, however, and safe, rational, and apparently effective modalities for evaluating and treating these patients are readily available. We should not dismiss their use out of hand.

REFERENCES

  1. American Academy of Pediatrics, Section on Cardiology and Cardiac Surgery. Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy. Pediatrics. 2005;116 :1569 –1573[Abstract/Free Full Text]
  2. Mori K, Manabe T, Nii M, Hayabuchi Y, Kuroda Y, Tatara K. Plasma levels of natriuretic peptide and echocardiographic parameters in patients with Duchenne's progressive muscular dystrophy. Pediatr Cardiol. 2002;23 :160 –166[CrossRef][Web of Science][Medline]
  3. Kashiwagi S, Akaike M, Kawai H, Adachi K, Saito S. Estimation of cardiac function by plasma concentration of brain natriuretic peptide in patients with Duchenne muscular dystrophy [in Japanese]. Rinsho Shinkeigaku. 1996;36 :7 –11[Medline]
  4. Ishikawa Y, Bach JR, Ishikawa Y, Minami R. A management trial for Duchenne cardiomyopathy. Am J Phys Med Rehabil. 1995;74 :345 –350[Web of Science][Medline]
  5. Shaddy RE, Tani LY, Gidding SS, et al. Beta-blocker treatment of dilated cardiomyopathy with congestive heart failure in children: a multi-institutional experience. J Heart Lung Transplant. 1999;18 :269 –274[CrossRef][Web of Science][Medline]
  6. Ishikawa Y, Bach JR, Minami R. Cardioprotection for Duchenne's muscular dystrophy. Am Heart J. 1999;137 :895 –902[CrossRef][Web of Science][Medline]
PEDIATRICS (ISSN 1098-4275). ©2006 by the American Academy of Pediatrics

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Related articles in Pediatrics:

Therapeutic Nihilism in Duchenne Cardiomyopathy: In Reply
Linda Cripe and Larry Markham
Pediatrics 2006 117: 1865. [Extract] [Full Text]  




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