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.
Objective:The study aims to estimate the prevalence of cardiac amyloidosis in patients with systemic amyloidosis. Compare survival rates based on whether they show cardiac involvement. Methods: A retrospective cohort study of patients with systemic amyloidosis from the Institutional Amyloidosis Registry of the Hospital Italian of Buenos Aires from 2010 to 2019. Heart involvement is considered to be the presence of symptoms and/or images consistent with amyloidosis, and there is no other reason to explain it. Death for all causes were evaluated. The survival rate was estimated by Kaplan-Meier. Cox regression model was used to evaluate factors related to mortality. Heart transplantation was evaluated in a competitive risk regression model. Results: The prevalence of heart involvement is 63%. For the group with heart involvement, the death rate was 14/1000 person-months, and for patients without damage, the death rate was 5/1000 person-months. The 5-year overall survival rate for patients with heart involvement was 44%, while that for patients without damage was 67% (p = 0.02). Crude hazard ratio (HR) for heart involvement was 2.09 (p= 0.02). Age showed an adjusted HR of 1.06 (p <0.01). The sub-HR estimated by the competitive risk regression model was 1.86 (95% confidence intervals 0.99-3.49) p = 0.05. Conclusion: Cardiac involvement is an important prognostic factor in patients with amyloidosis.
Objetivo: Describir las características clínicas e imagenológicas de los pacientes con trasplante cardiaco por amiloidosis en una institución de la comunidad. Método: Serie de casos descriptiva de pacientes consecutivos receptores de trasplante cardiaco con amiloidosis en un centro médico. Se incluyeron todos los pacientes con diagnóstico de amiloidosis con compromiso cardiaco receptores de trasplante cardiaco en el periodo de noviembre de 2008 a febrero de 2021. Resultados: Se incluyeron 16 pacientes con una edad media de 59.9 años (± 10.2) y el 81.25 % (n = 13) eran de sexo masculino. Según el tipo de amiloidosis, 12 pacientes recibieron el trasplante por amiloidosis por cadenas livianas (AL) y 4 por amiloidosis por transtiretina (ATTR). Las formas más frecuentes de presentación clínica fueron sobrecarga izquierda (50 %) y shock cardiogénico (32 %). La mitad recibieron el trasplante estando en lista de emergencia. La fracción de eyección promedio previa al trasplante fue del 43% (± 16). Presentaron disfunción del ventrículo derecho 14 de los 16 pacientes. El hallazgo más común en la resonancia magnética cardiaca fue el patrón de realce tardío de gadolinio subendocárdico difuso, con anulación del pool sanguíneo. La realización del trasplante cardiaco permitió a la mitad de los pacientes con amiloidosis AL (n = 6) la posibilidad de recibir trasplante de médula ósea en un segundo tiempo. Conclusiones: En la actualidad, el trasplante cardiaco se ha convertido en una opción para pacientes con insuficiencia cardiaca por amiloidosis, tanto AL como ATTR. En los pacientes con amiloidosis AL incluso puede permitir en un segundo tiempo el trasplante de médula ósea.
Objective:The objective of the study was to describe the clinical and imaging characteristics of heart transplantation patients due to amyloidosis in a community institution. Methods: Descriptive case series of consecutive heart transplantation patients with amyloidosis in a medical center. All patients with diagnosis of amyloidosis with cardiac compromise receiving heart transplantation, performed in the period of November 2008-February 2021, were included in the analysis. Results: A total of 16 patients were included in the study. The mean age was 59.9 years (± 10.2). About 81.25% (n = 13) were male. According to the type of amyloidosis, 12 patients were transplanted for AL amyloidosis and four for ATTR amyloidosis. The most frequent clinical presentations were left overload (50%) and cardiogenic shock (32%). The mean ejection fraction before transplantation was 43% (± 16), 14 of the 16 patients had right ventricular dysfunction. The most common finding on cardiac magnetic resonance was the diffuse subendocardial late gadolinium enhancement pattern, with a cancellation of the blood pool. The heart transplantation gave six patients the chance to receive a bone marrow transplantation afterward. Conclusions: Heart transplantation has become an option for patients with heart failure due to AL and ATTR amyloidosis. In patients with AL amyloidosis, it might even allow bone marrow transplantation in a second stage.
Amyloidosis is a systemic disorder produced by the deposition of insoluble protein fibrils that fold and deposit in the myocardium. Patients with amyloidosis and cardiac involvement have higher mortality than patients without cardiac involvement. The two most prevalent forms of amyloidosis associated with cardiac involvement are AL amyloidosis, due to the deposition of immunoglobulin light chains, and ATTR amyloidosis, due to the deposition of the transthyretin (TTR) protein in mutated or senile form. This article aims to review the different cardiac imaging modalities (echocardiography, cardiac magnetic resonance imaging, nuclear medicine and tomography) that allow to determine the severity of cardiac involvement in patients with amyloidosis, the type of amyloidosis and its prognosis. Finally, we suggest a diagnostic algorithm to determine cardiac involvement in amyloidosis adapted to locally available diagnostic tools, with a practical and clinical approach.
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