Cardiac amyloidosis presenting as sick sinus syndrome and intractable heart failure: report of a case. 1993

Y T Hwang, and C D Tseng, and J J Hwang, and K L Hsu, and F T Chiang, and Y Z Tseng, and S W How
Department of Internal Medicine, College of Medicine, National Taiwan University, Taipei, R.O.C.

Cardiac amyloidosis, an uncommon disease, has been reported to manifest as congestive heart failure (CHF) and/or various arrhythmias. Herein, we report a case of CHF and sick sinus syndrome. The patient, a 66-year-old man, was admitted to the National Taiwan University Hospital because of dizzy spells and recurrent syncope. Electrocardiogram showed a sinoatrial block, first degree atrioventricular block, right bundle branch block and low-voltage Q wave, R wave and S wave (QRS) complex. Prolonged corrected sinus node recovery time was documented by an atrial pacing study. A permanent pacemaker was implanted for the patient's bradyarrhythmia, but he developed progressive heart failure. Echocardiography revealed a normal-sized ventricular chamber, concentric left ventricular hypertrophy with a "granular sparkling" appearance of the myocardium, and impaired diastolic and systolic function of the left ventricle. Despite aggressive treatment, the patient expired due to intractable heart failure. Postmortem needle aspiration revealed amyloidosis involving the heart, lung and skin. We conclude that cardiac amyloidosis should be considered in elderly patients with conduction disturbance and unexplained congestive heart failure.

UI MeSH Term Description Entries
D008297 Male Males
D009202 Cardiomyopathies A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS). Myocardial Disease,Myocardial Diseases,Myocardial Diseases, Primary,Myocardial Diseases, Secondary,Myocardiopathies,Primary Myocardial Disease,Cardiomyopathies, Primary,Cardiomyopathies, Secondary,Primary Myocardial Diseases,Secondary Myocardial Diseases,Cardiomyopathy,Cardiomyopathy, Primary,Cardiomyopathy, Secondary,Disease, Myocardial,Disease, Primary Myocardial,Disease, Secondary Myocardial,Diseases, Myocardial,Diseases, Primary Myocardial,Diseases, Secondary Myocardial,Myocardial Disease, Primary,Myocardial Disease, Secondary,Myocardiopathy,Primary Cardiomyopathies,Primary Cardiomyopathy,Secondary Cardiomyopathies,Secondary Cardiomyopathy,Secondary Myocardial Disease
D006333 Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION. Cardiac Failure,Heart Decompensation,Congestive Heart Failure,Heart Failure, Congestive,Heart Failure, Left-Sided,Heart Failure, Right-Sided,Left-Sided Heart Failure,Myocardial Failure,Right-Sided Heart Failure,Decompensation, Heart,Heart Failure, Left Sided,Heart Failure, Right Sided,Left Sided Heart Failure,Right Sided Heart Failure
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000686 Amyloidosis A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits. Amyloidoses
D012804 Sick Sinus Syndrome A condition caused by dysfunctions related to the SINOATRIAL NODE including impulse generation (CARDIAC SINUS ARREST) and impulse conduction (SINOATRIAL EXIT BLOCK). It is characterized by persistent BRADYCARDIA, chronic ATRIAL FIBRILLATION, and failure to resume sinus rhythm following CARDIOVERSION. This syndrome can be congenital or acquired, particularly after surgical correction for heart defects. Sinus Node Dysfunction,Sick Sinus Node Syndrome,Sinus Node Disease,Dysfunction, Sinus Node,Dysfunctions, Sinus Node,Sinus Node Diseases,Syndrome, Sick Sinus

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