The heart in Portuguese amyloidosis

Abstract

Summary:A systematic investigation was performed in patients with familial amyloidotic polyneuropathy, Portuguese type (AFp) to assess the pattern and incidence of cardiac involvement. Of 327 patients investigated, ECG abnormalities were present in 285 (87.2%). Low We conclude that the ECG is the most precise, sensitive and clinically useful method for detecting cardiac amyloidosis in patients with AFp.

Echocardiographie Assessment of the Course of Heart Disease in Familial Amyloidotic Polyneuropathy

“…Our cross-sec tional study shows that échocardiographie abnormalities such as increased thicknesses of the ventricular walls, hyperrefractile myocardial echoes, and decreased dia stolic ventricular function are common in all stages of FAP, whereas the systolic function expressed as left ven tricular fractional shortening is essentially normal. These findings are in accordance with those in previous crosssectional échocardiographie studies on FAP [25,26], but at variance with the findings in a series of Portuguese FAP patients where there were few échocardiographie abnormalities [9], Our study also shows that frequency and extent of the morphologic abnormalities increase with increasing severity of the systemic disease. Our lon gitudinal study shows a clear, albeit modest, increase of frequency and extent of the échocardiographie findings during the follow-up period.…”

“…Hereditary amyloidosis has been de scribed in a number of kindreds from around the world. Most of these kindreds have a symmetrical polyneuropa thy as the main feature of the amyloidosis, but many differ with respect to cardiac, intestinal, renal, and other organ involvement [2], The commonest of these heredi tary syndromes is familial amyloidotic polyneuropathy (FAP) which during the last decades has been reported in heart failure is found only occasionally [7][8][9], Informa tion on the evolution of the heart disease in FAP and its relation to the severity of the progressive neurological symptoms is, however, still sparse. This study was under taken to elucidate the course of the heart disease in FAP by means of cross-sectional and serial échocardiographie studies.…”

Liver transplantation for hereditary transthyretin amyloidosis

“…10,12,13,76 In some centers, preoperative or perioperative pacemaker insertion has been used in the majority of patients, whereas in other centers, pacemakers are not used routinely but are reserved for those with a history of syncope or arrhythmia. It is noteworthy that in the Swedish series, no patient with normal AV conduction on the preoperative electrocardiogram (ECG) developed an arrhythmia needing pacemaker treatment, and a 24-hour ECG did not identify the patients who developed circulatory disturbances.…”

“…In later stages of the disease, multiple organs are affected, including the gastrointestinal tract, kidneys, heart, and eyes. [9][10][11][12][13][14][15][16] Autonomic nervous system dysfunction is often present early during the course of the disease, causing severe orthostatic hypotension and impotence. 6,[16][17][18][19][20][21] In the final stage of the disease, the patients are severely incapacitated, bedridden or bound to a wheelchair, malnourished, and have urinary and fecal incontinence.…”

Reduced Myocardial 123-Iodine Metaiodobenzylguanidine Uptake

“…13 In contrast to other systemic amyloidosis, cardiac manifestations are not the dominant clinical picture in TTR-FAP. However, previous studies involving small groups of patients suggest that cardiovascular abnormalities may be detected at initial stages of the disease, sometimes preceding the neurological manifestations, offering an opportunity for early clinical diagnosis.…”