A case of restrictive cardiomyopathy due to severe kyphosis
의약학 > 내과학
( Ju Yang Jung ) , ( Hyo Jung Lee ) , ( Hong Seok Lim )
대한내과학회 추계학술발표논문집 2011년, 제2011권 제1호, 136(총1쪽)
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    Restrictive cardiomyopathy is a myocardial disease characterized by diastolic filling defect with increased stiffness of ventricles. The patients present with dyspnea, fatigue, limited exercise capacity and need to be assessed in relation to familial involvement and potential genetic basis, like endomyocardial fibrosis, amyloidosis, sarcoidosis, hemochromatosis, Glycogen storage disease. A 59-year-old man visited our emergency room for dyspnea aggravated for recent 2 years. He had severe kyphosis caused by fall down injury when he was 3 years old. His height is 148 cm, weight is 50 kg, his back is bend. On the physical examination, Jugular venous distension was noted, the heart sounds were regular without murmur, but presystolic gallop was checked. The breath sounds were coarse with rales. The chest radiography showed Severe kyphosis of T-L spine, an enlarged cardiac silhouette pulmonary congestion. Electrocardiography showed regular sinus tachycardia, right atrial enlargement, left ventricular hypertrophy. The echocardiography revealed concentric left ventricular hypertrophy (end-diastolic interventricular septum thickness=14 mm, end-diastolic posterior wall thickness=17 mm) and on the doppler study, ratio of early mitral inflow to late filling velocity(E/A) was 0.9, early diastolic mitral annular velocity (E`) was decreased(0.03 cm/sec) with increase E/E`` ratio(19), suggesting restrictive physiology of diastolic dysfunction with elevated LV filling pressure. On chest CT, there was left-shifted thoracic cage due to kyphosis with tortuous aorta and atelectasis in left lower lobe. On MRI of heart and vessel, we could find restrictive diastolic dysfunction, due to limited space of thoracic cage and there was no evidence of myocardial disease like fibrosis, infilatration or storage disease. He didn`t have any family history of disease. We concluded restrictive cardiomyopathy due to physiologic restriction of small thoracic cage without any pathologic condition and started diuretics and antiplatelet agent, his symptoms improved gradually.
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