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Myocardial Dysfunction in Maternally Inherited Diabetes and Deafness

  1. Sandra P. Silveiro, MD,
  2. Luis Henrique Canani, MD,
  3. Ana Luiza Maia, MD,
  4. Jagdish W. Butany, MD and
  5. Jorge L. Gross, MD
  1. From the Endocrine Division, Hospital de Clínicas de Porto Alegre, Federal University of Rio Grande do Sul, Porto Alegre, Brazil

    The pattern and degree of myocardial involvement in maternally inherited diabetes and deafness (MIDD) is unclear (1–3). A recent French multicenter study that examined 54 patients with MIDD described left ventricular hypertrophy (LVH) in 8 and congestive heart failure (CHF) in 2 patients (2). Here, we reported a patient with mtDNA 3243 mutation who developed the full clinical, echocardiographic, and radiologic picture of CHF.

    A 54-year-old man with diabetes since age 23 years was diagnosed as having MIDD based on the findings of deafness, familial history of diabetes, and short stature involving his younger brother and mother. His older brother also presented diabetes but not short stature or deafness. Insulin was started when the patient was age 38 years, and he never presented ketosis. …

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