Amilóidose cardíaca. Uma doença de muitas faces e diferentes prognósticos

Barretto, Antônio Carlos Pereira; Précoma, Dalton Bertolim; Serro-Azul, João Batista; Wajngarten, Maurı́cio; Pierri, Humberto; Pivotto, L; Nussbacher, Amit; Gebara, Otavio C.E.; Bellotti, Giovanni

doi:10.1590/s0066-782x1997000800003

Cited by 7 publications

(7 citation statements)

References 10 publications

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“…A amiloidose pode colaborar na obstrução das artérias coronárias, ocasionando na morte do tecido cardíaco6. Pacientes com distúrbio de condução do ramo direito tiveram uma evolução para bloqueio atrioventricular (BAVT) colaborando com acometimento cardíaco 7 .…”

Section: Aspectos Clínicosunclassified

Amiloidose Cardíaca: Etiologia, Aspectos Clínicos, Diagnóstico E Tratamento

Júnior

Silva²,

Cerqueira³

2019

rsc

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O presente estudo teve como objetivo apresentar a etiologia, aspectos clínicos, métodos de diagnóstico e tratamentos da amiloidose cardíaca (AC). Através de uma revisão narrativa foram selecionados artigos científicos publicados no idioma português utilizando o termo “amiloidose cardíaca” nas bases de dados Bireme, Periódicos CAPES e SciELO. A AC é o acumulo de fibrilas proteicas nas paredes cardíacas e/ou nas paredes dos vasos sanguíneos que causam o enrijecimento das paredes cardíacas e/ou a obstrução dos vasos sanguíneos, podendo ser mais comum em amiloidose primária, senil e hereditária. Trata-se de uma doença com prognóstico reservado, sendo importante que haja um diagnóstico precoce, para que o portador tenha uma sobrevida maior com os tratamentos disponíveis. Atualmente, existem diferentes formas de diagnósticos, sendo alguns deles: o ecodopplercardiograma, eletrocardiograma, a ressonância magnética e a biópsia do tecido cardíaco. O indivíduo com AC pode apresentar insuficiência cardíaca, hipotensão ortostática, disfunção autonômica e outros sintomas, chegando a óbito. Entre os tratamentos disponíveis para aumentar a sobrevida dos indivíduos, existem a terapia com fármacos, transplante de células-tronco com auxílio de quimioterapia e transplante de órgãos.

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Section: Aspectos Clínicosunclassified

Amiloidose Cardíaca: Etiologia, Aspectos Clínicos, Diagnóstico E Tratamento

Júnior

Silva²,

Cerqueira³

2019

rsc

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“…5). Restrictive heart failure is one of the major forms of heart amyloidosis presentation 11 . Other deposits with the same microscopic characteristics were found in the pulmonary, hepatic, and splenic vessels, in the glomerular loops, and in the tongue.…”

Section: Autopsymentioning

confidence: 99%

The patient is a 65-year-old woman with heart failure and proteinuria

2000

Arq. Bras. Cardiol.

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A 65-year-old woman sought medical assistance due to dyspnea on mild exertion and edema.She complained that 6 months earlier, dyspnea on moderate exertion appeared, rapidly evolving to dyspnea on mild exertion and even at rest. Simultaneously, dizziness and edema of the lower limbs appeared and she lost 10 kg. She denied precordial pain, palpitation, and syncope during this time, as well as hypertension and diabetes mellitus. Twenty-five years earlier, she underwent a partial thyroidectomy because of multinodular goiter.On physical examination, her heart rate was 74 bpm, her blood pressure 110/70 mmHg, and the jugular venous pressure was increased (++/4+). Examination of the lungs revealed crepitant rales at both bases. Heart examination showed reduction in the intensity of heart sounds. The liver was tender on palpation and could be felt 4cm from the right costal margin and 6cm from the xiphoid process. Marked edema of the lower limbs occurred.Electrocardiogram showed atrial rhythm with a heart rate of 63 bpm, QRS duration of 0.08s; QRS axis of +120º backwards, a low voltage in the frontal plane, a small progression of the R wave from V 1 to V 3 , a probably inactive lateral area, and diffuse alterations of ventricular repolarization ( fig. 1).Chest X-ray showed a moderate increase in the cardiac silhouette (++/4+) and bilateral pleural effusion.Laboratory tests were as follows: hemoglobin -14g/ dL; hematocrit -42%; glycemia -105mg/dL; creatinine -0.8 mg/dL; total cholesterol -155 mg/dL (HDL -27mg/dL; LDL -105mg/dL; VLDL -23mg/dL); triglycerides -117mg/dL. Urine analysis revealed density of 1028, pH of 5.0, proteinuria of 3.2g/L, and a normal sediment except for 660 cylinders/mL.The diagnosis of heart failure due to cardiomyopathy with ventricular dilation was established and the investigation for anatomical and etiologic characterization of the clinical findings was started. The patient was prescribed 80mg of furosemide, 50mg of hydrochlorothiazide in association with 5mg of amiloride and 20mg of enalapril.One week later, she sought medical assistance due to severe dyspnea even at rest and orthopnea. On physical exa-

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“…Amyloidosis has a mortality rate of 100% in two years, after the onset of symptoms 5,32 , and the prognosis is worse in those with restrictive cardiomyopathy 33 .…”

Section: Etiology Of Congestive Heart Failurementioning

confidence: 99%