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Improvement of the Cardiac Function by Nasal Bilevel Positive Airway Pressure in a 67-year-old Woman with Myotonic Dystrophy



Yasunori Kakuta, MD1; Schinichi Okabe, MD2; Jun Demachi, MD2; Kunio Shirato, MD2; Wataru Hida, MD2. Nishitaga National Hospital1 and Tohoku University School of Medicine2, Sendai, Japan.

Introduction: In myotonic dystrophy (DM) nocturnal hypoventilation due to diaphragmatic weakness causes severe desaturation. The effect on the cardiac function of this chronic respiratory failure, however, has not received much attention, because the abnormality of the cardiac function evaluated by echocardiography is minor. Here, we report a case of DM, which showed that the nocturnal desaturation was harmful to the function of the cardiac muscle and that ventilation support overcame this problem.

Case summary: A 67-year-old woman with DM received nasal bilevel positive airway pressure (BiPAP) during the night to treat her chronic respiratory failure. The clinical course before and after treatment is summarized below.

  Before BiPAP 0.5 Y after 1 Y after
% of nocturnal SpO2<90% 90 19 14
Diurnal PaO2 (Torr) 45.3 53.3 58.5
Diurnal PaCO2 (Torr) 64.2 59.2 57.5
Left ventricular ejection fraction 0.56 0.67 0.61
Brain natriuretic peptide (pg/ml) 172 125 51

Discussion: As the plasma brain natriuretic peptide level was raised before BiPAP, we can presume the abnormality of cardiac function. It is likely due to the respiratory failure, because BiPAP reduced it. The reason why we chose nasal BiPAP is that it is less expensive and easier to apply to such patients of DM than volume controlled noninvasive positive pressure ventilation.

Conclusion: Cardiac dysfunction is present in DM with chronic respiratory failure even though the change of cardiac function by echocardiography is slight, and ventilation support is useful to improve this abnormality.

OF-01-124
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