[Cardiopulmonary failure in Duchenne muscular dystrophy--pathophysiology and management].
As there is no cure in patients with Duchenne muscular dystrophy (DMD) yet, we must pay attention to manage cardiopulmonary complications in DMD. They died at 18.2 years old in 1984 in my hospital. From autopsy findings, respiratory failure occupied 75%, and left-sided heart failure occupied 12.5%. First of all, we had to know the relationship between cardiac system and respiratory system. Right-sided heart catheterization revealed that respiratory failure patients were divided into Forrester's subset 1 (left ventricular function was within normal limits). So, it is unnecessary to give digitalis and/or diuretics for patients with respiratory failure. They only need respirator treatment. We tried cuirass ventilation since 1984. This respirator elongated their lives about 3 years. Since 1991 NIPPV was introduced in Japan, this treatment elongated their lives about 5.5 years. Nowadays TIPPV with tracheostomy is not first choice of treatment but we select this treatment not so unwillingly as before. As for left-sided heart failure, BNP (brain natriuretic peptide) is now considered useful parameter for left ventricular function. Japanese clinical researcher proposed treatment based on Values of BNP in left-sided heart failure. In 1980s, from the onset of heart failure until death was only 16 months, we feel that better results already accomplished. Kawai reported that average age at death in Japan was 26.8 years old in 2002. Our efforts must be done more and more until cure of this disease can be found.
Ishihara
Tadayuki
T
Department of Neurology, National Hakone Hospital.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D015415:Biomarkers / Q000097:blood; D002423:Cause of Death; D002648:Child; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000401:mortality; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011175:Positive-Pressure Respiration / Q000379:methods; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000628:therapy; D016277:Ventricular Function, Left
D015415:Biomarkers; D020097:Natriuretic Peptide, Brain