Adams-Stokes発作を示した心アミロイドーシスの1例

A case of cardiac amyloidosis with Adams-Stokes seizure

　心アミロイドーシスの臨床症状は他の心疾患と同様な症状を呈するために生前診断するのは難しい。我々は以前に心アミロイドーシスの生前診断に超音波検査が有力な手段であることを報告^1,2）したが，今回この方法により生前診断したアダムスーストークス発作及びウッ血性心不全をきたした心アミロイドーシスの1例を経験したので報告する。

The diagnosis of cardiac amyloidosis is often difficult during life. Because of marked clinical and hemodynamic similarities to other cardiac disease, the diagnosis may remain observed even after clinically significant cardiovascular decompen-sation occurs. Presiously we reported that cardiacechocardiogram play an important role of its diagnosis and differentiation from other cardiac disease. Namely characteristic findings of cardiac amyloidosis by echocardiogram is, 1) Significant symmetrical LV hypertrophy. 2) Reduced septal and posterior LV wall motion. 3) Highly reflective echoes throughout the myocardium in strongly infiltrated case. This time, we experienced a case of cardiac amyloidosis detected by this method. A 77 years old woman was admitted with complaints of drowsy consciousness, dyspnea & cold sweat. An electrocardiogram showed a complete A-V block right axis deviation & QS pattern in V_1-4. A chest X-ray film revealed large pleural effusion cardiomegaly. An echocardiography demonstrat-ed significant symmetrical LV hypertrophy, mark-edly reduced septal and posterior LV wall motion, high density echoes in LA wall & slightly peri-cardial effusion behind the posterior LV wall. After admission, she was recovered successfuly and doing well but suddenly died of venticular fibril-lation.