The sick sinus syndrome in familial amyloidosis with polyneuropathy

doi:10.1016/0167-5273(83)90217-6

International Journal of Cardiology

Volume 4, Issue 1, August 1983, Pages 71-73

https://doi.org/10.1016/0167-5273(83)90217-6 Get rights and content

Abstract

We report five cases of dysfunction of the sinus node in patients suffering from familial amyloidosis with polyneuropathy. The implantation of a pacemaker resulted in symptomatologic relief in all.

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Cited by (15)

2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy
2019, Heart Rhythm
Citation Excerpt :
This cardiotoxic infiltrative milieu is hypothesized to be the fundamental driver of conduction abnormalities, atrial and ventricular arrhythmias. Although widespread involvement is not uncommon, with sinus node dysfunction well-recognized,426–428 infranodal conduction system disease appears to be the primary conduction abnormality, as evidenced by HV interval prolongation.418,429 The disease is associated with the risk of sudden death in a number of cohorts.418
Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
Liver transplantation for hereditary transthyretin amyloidosis
2000, Liver Transplantation
Transthyretin (TTR) amyloidosis is the most commonform of hereditary amyloidosis. It is a systemic amyloidosis caused by an amyloidogenic variant TTR (ATTR), of which the methionine for valine at position 30 (ATTR Val30Met) gives rise to a fatal neuropathic amyloidosis. Because more than 95% ofTTR is produced by the liver, a liver transplantation should abolish the liver's production of amyloidogenic mutant TTR and thereby halt amyloid formation. The first liver transplantation for hereditary TTR amyloidosis was performed in Sweden in 1990 on a patient with ATTR Val30Met amyloidosis, and the result was encouraging. Today, liver transplantation for TTR amyloidosis is an established treatment. However, the disease is rarely seen except in a few endemic areas; therefore, most transplantation centers only receive a few cases. Because the disease phenotype varies with different TTR mutations and variability is even encountered for the same mutation, an evaluation of patients for transplantation must include an investigation of all organs that may be affected by the disease and may impact on the morbidity and mortality of the procedure. The aim of this review is to present the results of liver transplantation for TTR amyloidosis and give recommendations for patient evaluation and selection based on the literature and our experience with the disease.
Comparison of electrocardiographic findings in patients with AL (primary) amyloidosis and in familial amyloid polyneuropathy and anginal pain and their relation to histopathologic findings
2000, American Journal of Cardiology
Citation Excerpt :
Another mechanism that contributed to the chest pain appeared to be strikingly limited coronary microvascular dilatory capacity, which was noted in cardiac amyloidosis in association with restrictive cardiomyopathy,24 because 1 patient with only mild to moderate narrowing of the small coronary vessels also showed effort angina in our study. One of the most important cardiac manifestations in FAP is a high incidence of cardiac conduction disturbances,9–12 and chest pain related to myocardial ischemia is a very unusual feature in this disease.8 In our study, some patients with AL amyloidosis showed anginal pain with variable degrees of amyloid deposition into intramural coronary arteries, whereas none of the patients with FAP had occlusive disease of the small coronary vessels.
To assess the prevalence of chest pain and ischemic electrocardiographic (ECG) changes and relate them to histopathologic findings of coronary arteries in cardiac amyloidosis, 33 patients with AL (primary) amyloidosis and 60 patients with familial amyloid polyneuropathy (FAP) were examined. Five patients (15%) with AL amyloidosis had recurrent anginal pain with exertion and 2 of them also experienced anginal pain after orthostatic hypotension. The chest pain was associated with transient downsloping or horizontal ST-segment depression with or without T-wave inversion in right precordial leads, whereas the remaining patients with AL amyloidosis and all patients with FAP did not show anginal pain or ischemic ST-T changes. Histologic sections of coronary arteries were obtained in 12 patients with AL amyloidosis, including 4 of the 5 patients who had angina pectoris and in 25 patients with FAP. Three patients with anginal pain had variable degrees of stenoses of the intramural coronary arteries by amyloid deposition predominantly in the media with normal or nearly normal epicardial arteries. One patient with AL amyloidosis who had effort angina showed marked stenosis and complete occlusion of the small coronary vessels by transmural amyloid deposition. The remaining 8 patients with AL amyloidosis and 25 with FAP without chest pain did not exhibit any stenosis or occlusion of both the epicardial and intramural vessels. These findings suggest that ischemic ST-T changes with chest pain are not so rare in patients with AL amyloidosis, and that markedly decreased myocardial oxygen supply due to diffuse stenotic or occlusive disease of the small coronary vessels by amyloid deposition contributes to the development of clinically significant ischemic heart disease in these patients.
Electrophysiologic evaluation of the cardiac conduction system and its autonomic regulation in familial amyloid polyneuropathy
1985, The American Journal of Cardiology
A high incidence of cardiac conduction disturbances is a salient feature in familial amyloid polyneuropathy (FAP) and amyloid infiltration of the conduction system has been found. In patients with FAP autonomic nervous dysfunction involving gastrointestinal, urogenital and cardiovascular systems has been described. However, the present study is the first to evaluate the results of autonomic tests and pharmacologic intervention in the autonomic nervous regulation of the cardiac conduction system during an invasive electrophysiologic study. Seven patients with FAP and severe disturbances of the conduction system, evaluated concerning indications for pacemaker treatment, were studied; in 3 of them the parasympathetic regulation was found to be abolished. In 1 patient the observations indicated either vagal overactivity or denervation oversensitivity of the receptors. The function of the β adrenoceptors was intact in all patients, but a disturbance of sympathetic innervation could not be excluded. Dysfunction of autonomic nervous regulation of the cardiac conduction system was thus found in 4 of 7 patients. However, most conduction abnormalities were irreversible, indicating amyloid infiltration of the conduction system as the predominant pathophysiologic mechanism, in accordance with the findings of pathoanatomic studies.
Twins with implanted pacemakers: Is there an increased mortality risk for the co-twin? A follow-up study based on the Danish Twin Registry and the Danish Pacemaker Register
2005, Europace
The long-term impact of liver transplantation on kidney function in familial amyloidotic polyneuropathy patients
2005, Transplant International

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Brief reportThe sick sinus syndrome in familial amyloidosis with polyneuropathy

Abstract

Am J Cardiol

Am J Cardiol

Brief report
The sick sinus syndrome in familial amyloidosis with polyneuropathy