Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden
Familial amyloidosis with polyneuropathy (FAP) was first reported from Portugal in 1952, but since then this syndrome has been recognized in many countries including Sweden. In this investigation cardiac and pulmonary functions in the Swedish variety of FAP were studied. A retrospective survey of th...
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Umeå universitet, Klinisk fysiologi
1982
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ftumeauniv:oai:DiVA.org:umu-141062 2023-10-09T21:54:35+02:00 Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden Olofsson, Bert-Ove 1982 application/pdf http://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-141062 eng eng Umeå universitet, Klinisk fysiologi Umeå : Umeå universitet Umeå University medical dissertations, 0346-6612 86 http://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-141062 info:eu-repo/semantics/openAccess amyloidosis atrioventricular conduction ECG echocardiography hemodynamics pulmonary diffusing capacity Clinical Medicine Klinisk medicin Doctoral thesis, comprehensive summary info:eu-repo/semantics/doctoralThesis text 1982 ftumeauniv 2023-09-22T13:49:53Z Familial amyloidosis with polyneuropathy (FAP) was first reported from Portugal in 1952, but since then this syndrome has been recognized in many countries including Sweden. In this investigation cardiac and pulmonary functions in the Swedish variety of FAP were studied. A retrospective survey of the ECG findings in 71 patients showed a high prevalence of atrioventricular (38%) and intraventricular (41%) conduction defects, and also a high prevalence of atrial fibrillation (14%). In several patients a progression in the conduction defects to advanced disturbances could be observed and 10 out of 71 patients (14%) in the present series required pacemaker treatment. A histopathological study of the atrioventricular part of the conduction system showed marked amyloid infiltration in each case, which may explain the high prevalence of conduction defects. In an échocardiographie study which emcompassed 22 consecutive patients, all but those two patients with the shortest duration of symptomatic disease showed abnormal features. The most frequent and characteristic findings were hypertrophy of the interventricular septum (86%) and a hyperrefractile appearance of the iryocardium (68%). This unusual association of échocardiographie features is considered almost diagnostic of cardiac amyloidosis. A hemodynamic study showed an essentially normal systolic heart function, but in several patients there were signs of impaired diastolic function with increased myocardial rigidity. Several patients showed signs of obstruction of the ventricular outflow tracts. This finding, as well as the échocardiographie features, is in accordance with altered anatomical and functional properties of the interventricular septum. The major pulmonary function abnormalities were decreased maximum respiratory pressure which indicate that the neuropathy in FAP involves the respiratory musculature, and impaired diffusing capacity consistent with an alveo-capillary block caused by amyloid deposits. digitalisering@umu Doctoral or Postdoctoral Thesis Northern Sweden Umeå University: Publications (DiVA) |
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Umeå University: Publications (DiVA) |
op_collection_id |
ftumeauniv |
language |
English |
topic |
amyloidosis atrioventricular conduction ECG echocardiography hemodynamics pulmonary diffusing capacity Clinical Medicine Klinisk medicin |
spellingShingle |
amyloidosis atrioventricular conduction ECG echocardiography hemodynamics pulmonary diffusing capacity Clinical Medicine Klinisk medicin Olofsson, Bert-Ove Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden |
topic_facet |
amyloidosis atrioventricular conduction ECG echocardiography hemodynamics pulmonary diffusing capacity Clinical Medicine Klinisk medicin |
description |
Familial amyloidosis with polyneuropathy (FAP) was first reported from Portugal in 1952, but since then this syndrome has been recognized in many countries including Sweden. In this investigation cardiac and pulmonary functions in the Swedish variety of FAP were studied. A retrospective survey of the ECG findings in 71 patients showed a high prevalence of atrioventricular (38%) and intraventricular (41%) conduction defects, and also a high prevalence of atrial fibrillation (14%). In several patients a progression in the conduction defects to advanced disturbances could be observed and 10 out of 71 patients (14%) in the present series required pacemaker treatment. A histopathological study of the atrioventricular part of the conduction system showed marked amyloid infiltration in each case, which may explain the high prevalence of conduction defects. In an échocardiographie study which emcompassed 22 consecutive patients, all but those two patients with the shortest duration of symptomatic disease showed abnormal features. The most frequent and characteristic findings were hypertrophy of the interventricular septum (86%) and a hyperrefractile appearance of the iryocardium (68%). This unusual association of échocardiographie features is considered almost diagnostic of cardiac amyloidosis. A hemodynamic study showed an essentially normal systolic heart function, but in several patients there were signs of impaired diastolic function with increased myocardial rigidity. Several patients showed signs of obstruction of the ventricular outflow tracts. This finding, as well as the échocardiographie features, is in accordance with altered anatomical and functional properties of the interventricular septum. The major pulmonary function abnormalities were decreased maximum respiratory pressure which indicate that the neuropathy in FAP involves the respiratory musculature, and impaired diffusing capacity consistent with an alveo-capillary block caused by amyloid deposits. digitalisering@umu |
format |
Doctoral or Postdoctoral Thesis |
author |
Olofsson, Bert-Ove |
author_facet |
Olofsson, Bert-Ove |
author_sort |
Olofsson, Bert-Ove |
title |
Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden |
title_short |
Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden |
title_full |
Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden |
title_fullStr |
Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden |
title_full_unstemmed |
Cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern Sweden |
title_sort |
cardio-pulmonary function in familial amyloidosis with polyneuropathy : a clinical study of cases from northern sweden |
publisher |
Umeå universitet, Klinisk fysiologi |
publishDate |
1982 |
url |
http://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-141062 |
genre |
Northern Sweden |
genre_facet |
Northern Sweden |
op_relation |
Umeå University medical dissertations, 0346-6612 86 http://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-141062 |
op_rights |
info:eu-repo/semantics/openAccess |
_version_ |
1779318214854967296 |