Amyloid deposits repeatedly involv...

Amyloid deposits repeatedly involve the heart and cause disturbances in conduction and impulse formation in patients with familial amyloid polyneuropathy (FAP). Seven patients with FAP required pacemaker treatment during eight years. The greatest in quantity frequent bradyarrhythmias requiring pacing were sinus node dysfunction with junctional failure. Our seven patients had attacks and symptoms of bradyarrhythmias. A pacemaker relieved symptoms of bradycardias with return of dislocation of electrode, exit shut up and relatively high threshold. However, pacing did not improve the ultimate prognosis of FAP, because of progressive inanition of FAP. In our series, pacing should have been started earlier before advanced stage according to the ECG findings as in other diseases with bradycardias.

(Chest 1989; 96:80-84)

Familial amyloid polyneuropathy is inherited in an autosomal method and the amyloid deposits involve motor, sensory and autonomic nervous regularitys Familial amyloid polyneuropathy is a progressive disease with gradual deterioration, and the prognosis of this disease is grave.

In a small number of patients with this disease, the heart is widely involved by means of amyloid infiltration, which causes disturbances in impulse formation and in conduction, requiring pacemaker treatment. The condition is place in limited areas in the world. Many reports have been from Portugal and Sweden.[1-7] It has also been construct in localized areas in Japan. Ogawa Village, Nagano, is individual of the regions of concentration of this disease in Japan.

In this report, we describe clinical features and ECG abnormalities that required pacemaker treatment, findings of atrial pacing trials His bundle electrocardiography and pacemaker implantation, and complications in pacemaker treatment in patients with FAP from Ogawa Village, Nagano, Japan.

Patients and Methods

Between February 1979 and October 1987 we examined 88 patients with FAP originating from Ogawa Village, Nagano, Japan. Seven of these patients unraveled symptomatic bradyarrhythmias. They were from five of 34 pedigrees which have been reported previously through Kito et al.[8] All patients be affected byed from polyneuropathy and showed characteristic clinical manifestations with progressive polyneuropathy. These patients had episodes of elision or Adams-Stokes' attacks from bradyarrhythmias, further none of these patients had a history of myocardial infarction or any other heart diseases and were not receiving antiarrhythmic medications.

mien of amyloid was confirmed according to sural nerve biopsy in these seven patients. The previously recorded ECG and regular [i]or[/i] melodious movement strips were collected and analyzed. Twelve-lead ECG and bedside monitorings were recorded at various times in all patients. Holter ECG recordings were made in brace cases.

Atrial pacing experiments were performed in three patients and His packet electrocardiography (HBE) was recorded in five patients during temporary pacing. At the same time, an endocardial biopsy was done in five patients.

Pacemakers were implanted transvenously after temporary pacing in all seven patients. The clincal features of the seven patients and analysis of 244 ECG recordings including the 12-lead ECG Holter ECG recordings, and bedside monitor recordings are not absented in this report. Data from atrial pacing proofs HBE and pacemaker implantation, and follows of endocardial biopsy are also presented

Results

The patients were pair men and five women aged 38 to 49 years advanced in years (mean 40). The charge of disease occurred at 29 to 41 years olden (mean 35). The duration of affection to the time of pacemaker implantation was 72 to 132 month (mean 104) (Table 1) The retrograde investigation of family tree revealed more than single family member affected with FAP in each family.

Clinical Features

The initial symptoms began in the lower extremities with progressive los of sensation, weakness, superficial dullnes and pain. The following signs and symptoms were muscular atrophy, flaccid paralysis, [TABULAR DATA OMITTED] trophic disturbances of skin (bull formation and ulceration of skin), sensory dullnes in the head chest, loss of weight, malnutrition, and emaciation in all patients. Various manifestations in the autonomic nervous schemes were noted in all patients. These included urinary bladder and sphincter dysfunctions, impotence, nausea and vomiting, diarrhea and constipation, and orthostatic hypotension. All seven patients sustained from characteristic progressive familial amyloid polyneuropathy and were bedridden fit to muscle weakness.

Five patients had Adams-Stokes' attacks and pair patients had syncopal attacks from bradyarrhythmias. Dizziness from orthostatic hypotension was noted in five patients (Table 1)

ECG Findings

Twelve-lead ECG revealed minimal R progression with intelligent S wave in the right precordial lead and small R progression in the left precordial lead. Various disturbances in impulse formation and conduction was observ in all cases (Table 2)