Amyloid cardiomyopathy

Karafiátová, Lucie; Pika, Tomáš

doi:10.5507/bp.2017.001

Cited by 11 publications

(7 citation statements)

References 86 publications

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“…La afectación cardíaca es la principal causa de morbimortalidad en la amiloidosis y es frecuente en la amiloidosis sistémica de cadenas ligeras (AL) y la amiloidosis por depósito de transtiretina, sea ésta de tipo familiar o senil (ATTRv, ATTRwt) 1 , 2 .…”

Section: Introductionunclassified

Características y evolución de los pacientes con amiloidosis sistémica y compromiso cardíaco

Carretero¹,

Aguirre²,

Villanueva³

et al. 2021

ACM

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Objetivos: Determinar la prevalencia de amiloidosis cardíaca en pacientes con amiloidosis sistémica. Comparar la supervivencia según sea que presenten o no compromiso cardíaco. Métodos: Cohorte retrospectiva de pacientes con amiloidosis sistémica del Registro Institucional de Amiloidosis del Hospital Italiano de Buenos Aires, entre 2010 y 2019. Se consideró como compromiso cardíaco la presencia de síntomas o imágenes consistentes con amiloidosis no explicado por otras causas. Se evaluó la muerte por todas las causas. Se calculó la sobrevida mediante Kaplan-Meier. Los factores relacionados con mortalidad se evaluaron con un modelo de regresión de Cox. Se evaluó el trasplante cardíaco en un modelo de regresión de riesgo competitivo. Resultados: La prevalencia de compromiso cardíaco fue del 63%. La incidencia de muerte fue de 14/1,000 personas-meses para el grupo con compromiso cardíaco y de 5/1,000 personas-meses para los pacientes sin compromiso. Los pacientes con compromiso cardíaco tuvieron una sobrevida global a los cinco años de 44% contra 67% en los que no tenían compromiso (p = 0.02). El HR crudo para el compromiso cardíaco fue de 2.09 (p = 0.02). La edad mostró un HR ajustado de 1.06 (p <0.01). El modelo de regresión de riesgos competitivos estableció un sub-HR de 1.86 (IC95%, 0.99-3.49; p = 0.05). Conclusiones: El compromiso cardíaco es un factor pronóstico importante en pacientes con amiloidosis.

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Section: Introductionunclassified

Características y evolución de los pacientes con amiloidosis sistémica y compromiso cardíaco

Carretero¹,

Aguirre²,

Villanueva³

et al. 2021

ACM

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Section: Introductionmentioning

confidence: 99%

“…Amyloidosis is a disease characterized by tissue deposition of insoluble fibrils created by the aggregation of misfolded proteins [1]. This aberrant protein deposition can affect nearly all major organ systems, producing varied clinical presentations which reflect the organs affected by this infiltrative process [1]. Cardiac involvement is common and associated with an increased risk of morbidity and mortality related to the development of congestive heart failure, arrhythmias, and conduction defects [2,3].…”

Section: Introductionmentioning

confidence: 99%

Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR)

Phull

Sanchorawala

Connors

et al. 2018

Amyloid

119

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These data confirm the high prevalence of coexistent MGUS with ATTR amyloidosis in this patient population, with an MGUS rate that is higher than the general population. These findings also highlight the importance of a thorough diagnostic evaluation in patients with amyloidosis to determine the precursor protein, as the clinical course and treatment of AL (light-chain amyloid protein) and ATTR amyloidosis are distinct.

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“…The most significant difficulty in diagnosing the cardiac localization is its late clinical presentation (often after the fifth decade of life) and the wide clinical variability. It manifests itself in the form of a rather severe restrictiveinfiltrative cardiomyopathy [10][11]. A serum monoclonal component is detectable in a significant percentage of patients (identifying that particular type of amyloidosis in which the deposits are characterized precisely by light chains of the immunoglobulins -light-chain amyloidosis, LA -and in which there is often a cardiac involvment).…”

Section: Discussionmentioning

confidence: 99%

Second Level Management of a Complex Case with Suspected Cardiac Amyloidosis: A Challenge for the Clinician

Massimo¹

2019

Ann Heart

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We report the clinical case report of a 67-year-old patient with diagnoses of hypertension, ischemic heart disease and a previous history of Acute Myocardial Infarction (AMI) and nonspecific signs and symptoms (general illness, weight loss, hyporexia, fever, impressive edema at the feet, systemic arterial hypotension). So, we performed a series of laboratory, radiological and instrumental examinations, but the clinical picture remained difficult to interpret. The echocardiographic evaluation led us to a suspicion of infiltrative heart disease, and the magnetic resonance, while confirming the interesting data observed at the ultrasound exam. Therefore, we decided to perform a biopsy, which appeared positive for our suspicion. So, we thought we did not waste any more precious time and, after stabilizing the patient, we sent our patient to a specialized diagnostic center for diagnostic confirmation and the beginning of targeted therapies. Clinical, laboratory and instrumental implications of this differential diagnosis are discussed.

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Amyloid cardiomyopathy

Cited by 11 publications

References 86 publications

Características y evolución de los pacientes con amiloidosis sistémica y compromiso cardíaco

Características y evolución de los pacientes con amiloidosis sistémica y compromiso cardíaco

Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR)

Second Level Management of a Complex Case with Suspected Cardiac Amyloidosis: A Challenge for the Clinician

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