<症例>安静時の心機能に比し運動耐容能は良好であったmyotonic dystrophyの1例

概要

論文の詳細を見る
Myotonic dystrophy is a hereditary multisystem disease featuring not only myotonia and muscle atrophy but also cataract, gonadal atrophy, frontal baldness and mental impairment. Involvement of cardiac disease is not uncommon. The most prevalent heart disorder is conduction system disturbance but manifestation of cardiac failure is rare. In this paper a case of myotonic dystrophy, a 44 years old man, who manifested cardiomegaly and unusual ventricular dysfunction without complaining cardiovascular symptoms, is reported. Left ventricular cineangiography demonstrated global severe hypokinesis and ejection fraction was 31% without coronary artery stenosis. He underwent the supine bicycle ergometer symptom-limited maximal exercise with a Swan-Ganz catheter ; the exercise started at a workload of 50 watts and every 3 minutes the workload was increased by 25 watts. He performed the exercise up to 125 watts for 11 minutes and showed an increased stroke index without rise in pulmonary capillary wedge pressure. It is noteworthy that the patient had very good response for exercise intolerance in spite of severely depressed resting ventricular function.
近畿大学の論文
1986-09-25