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  1. Início
  2. Palavra-chave: Prealbumin
IJCS
Original Article

Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis

Ng Kin Key et al. Adelina Sanches de Melo Joberto Pinheiro Sena Adriano Dias Dourado … Julia Micheli Perez Jose Carlos Raimundo Brito Gilson Feitosa Gilson Soares Feitosa-Filho

Abstract

Background:

Aortic Stenosis (AS) is one of the most common valvular heart diseases in adults, especially the elderly. The persistence of heart failure symptoms and worse outcomes despite valve replacement treatment may be associated with the coexistence of Cardiac Amyloidosis (CA).

Objective:

To determine the prevalence of CA in patients with calcific AS.

Methods:

This was a cross-sectional study. Elderly patients with moderate to severe AS underwent cardiac scintigraphy with bone radiotracer for diagnosis of CA. Patients diagnosed with Transthyretin (TTR) amyloidosis by scintigraphy underwent genetic studies. Groups with altered and unaltered scintigraphy results for continuous variables were compared using an independent t-test for normally distributed variables and a Mann-Whitney test for those with asymmetric distribution. Statistical significance was set at p-value < 0.05

Results:

Forty-one patients with AS underwent pyrophosphate scintigraphy. The mean age of the cases was 79 ± 6 years, 23 (56%) were female, 23 (56%) had classic severe AS pattern, and 8 (19.5%) had low-flow low-gradient AS with reduced Ejection Fraction (EF). Carpal tunnel syndrome was present in 2 (4%) patients, 8 (19%) had polyneuropathy. A RAISE score ≥ 2 was found in 4 patients (9%). Four patients were diagnosed with TTR amyloidosis, one of whom had a Val122I genetic mutation. Three (75%) of the patients diagnosed with AE/AI were male. There were no statistically significant differences between the isolated AS and AS/CA groups.

Conclusion:

The prevalence of CA in patients with moderate to severe AS of probable calcified etiology in our sample was 10%.

  • Amyloidosis
  • Aortic Valve Stenosis
  • Prealbumin
  • Radionuclide Imaging
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Creative Commons Attribution (CC BY) Creative Commons license
22/11/2024 DOI: 10.36660/ijcs.20240064 Visualizações 3
IJCS
Review Article

Evolving Therapies for Transthyretin Cardiac Amyloidosis: New Clinical Trials with Amyloid Fibrils Depleter

Marcus Vinícius Simões et al. Fábio Fernandes André Dabarian Flavio Henrique Valicelli Claudio Tinoco Mesquita

Abstract

Amyloidosis is caused by the extracellular deposition of insoluble protein arrangements, formed from structural changes in different precursor proteins. The prominent clinical and epidemiological relevance of transthyretin amyloid cardiomyopathy (ATTR-CM), whose increased incidence is associated with the aging of the global population, given its high prevalence in the elderly population, has motivated the intensification of the search for specific therapeutic targets capable of altering the natural course of the disease. The past few years have been especially prolific with the emergence of significant new therapies for ATTR-CM, with important trials demonstrating positive results and changing the natural history of an inexorable and deadly disease. Up to now, the available drugs mainly promote stabilization of disease progression in patients with no advanced heart disease but grant no reversal of the structural cardiac disease in most cases. The transthyretin (TTR)-depleters based on monoclonal antibodies directed against the deposited amyloid fibers hold promise to fulfill these expectations. The DepleTTR-CM will test the safety and efficacy of a monoclonal antibody directed against TTR fibrils deposited in the myocardium of patients with ATTR-CM. This will be the first multicenter randomized controlled trial to test this new class of anti-amyloid treatment, the “amyloid fibril depleters”. This is a strategy designed to revert the amyloid accumulation in the cardiac interstitium, which is expected to translate into relevant clinical benefits, with the potential to close a gap in the ATTR-CM treatment strategies, mainly in patients with more severe disease.

  • Amyloidosis
  • deplete
  • Heart Failure
  • Prealbumin
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Creative Commons Attribution (CC BY) Creative Commons license
04/11/2024 DOI: 10.36660/ijcs.20240112 Visualizações 2
IJCS
Original Article

Prognostic Value of the 6-Minute Walk Test and Clinical Characteristics of Patients With Transthyretin Amyloid Cardiomyopathy

Cristhian Espinoza Romero et al. Natalia Melo Pereira Georgina Del Cisne Jadán Luzuriaga Bruno Vaz Kerges Bueno … Suenia Carvalhal Thais Borges Barbara Reis Fábio Fernandes

Abstract

Background:

In transthyretin amyloid cardiomyopathy (ATTR-CM), the assessment of functional capacity is of great importance. A distance < 300m covered in the 6-minute walk test (6MWT) is linked to a poorer prognosis in heart failure (HF) patients. However, in ATTR-CM, this association is not well-established, particularly among its various presentations and variants.

Objectives:

To evaluate functional capacity and, the prognostic value of the 6MWT in patients with ATTR-CM and to identify their clinical characteristics according to the type of ATTR-CM.

Methods:

This was a retrospective, single-center study that included patients with a confirmed diagnosis of ATTR-CM who performed the 6MWT. Median follow-up was 19 months (8 – 29). The significance level was set at P < 0.05.

Results:

A total of 32 patients were analyzed; 59.4% had the hereditary form (ATTRh), of which 63.2% were Val142Ile. Mean left ventricular ejection fraction (LVEF) was lower in the ATTRh group when compared to the wild-type form (ATTRwt) [42 ±11 versus 52± 9 %; p = 0.029]. Median distance walked in the 6MWT was 328 m (269 – 397; IQR) in ATTRwt and 304 m (246 – 387; IQR) in ATTRh, with no difference between groups (p = 0.833). In patients with a distance <300 m in the 6MWT, survival was lower (28.8 vs 36.3 months; p = 0.041).

Conclusions:

A distance <300 m walked in the 6MWT was linked to shorter event-free survival in patients with ATTR-CM. Functional capacity of most patients was very low, and no differences were observed in the distance covered between the wild-type form and the hereditary form of ATTR.

  • Amyloidosis
  • Heart Failure
  • Prealbumin
  • Walk Test
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Creative Commons Attribution (CC BY) Creative Commons license
04/11/2024 DOI: 10.36660/ijcs.20240091 Visualizações 1
IJCS
Viewpoint

Heart Rate Control or Rhythm Control in Patients With Atrial Fibrillation and Cardiac Amyloidosis

Lígia Lopes Balsalobre Trevizan et al. Sandrigo Mangini

Introduction

Cardiac amyloidosis (CA) manifests as a gradually progressive infiltrative condition characterized by the accumulation of insoluble protein aggregates within the myocardium and cardiac conduction system. The majority of CA cases are associated with precursor proteins, particularly immunoglobulin light chains (AL-CA) and transthyretin (ATTR-CA), which represent over 95% of cases. ATTR precursor substances may originate from a broad spectrum, encompassing both wild-type (ATTRwt) and variant (ATTRv) genes.

Arrhythmias in CA result from a combination of factors. The deposition of amyloid fibrils and infiltration into the myocardium cause thickening of the atrial and ventricular walls, leading to compromised relaxation. Consequently, this cascade results in increased filling pressures and atrial dilation, making individuals more susceptible to atrial fibrillation (AF) and other atrial arrhythmias. While left atrial (LA) enlargement has historically been linked with AF, new-onset AF can occur even without LA enlargement, suggesting a complex pathophysiology of AF in CA, where various processes affect LA structure, function, and mechanics in different ways, thereby heightening the AF risk. The usual manifestation of LA remodeling involves substantial infiltration of the atrial walls, leading to gradual impairment of atrial function and heightened stiffness, often observed without significant LA dilation.

[…]

  • Amyloidosis
  • Atrial Fibrillation
  • Heart Failure
  • Prealbumin
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Creative Commons Attribution (CC BY) Creative Commons license
09/08/2024 DOI: 10.36660/ijcs.20240050 Visualizações 2

Como citar este artigo

EndNote RIS

Formato de Documento Eletrônico (Vancouver)

Key NK, Melo AS, Sena JP, Dourado AD, Perez JM, Brito JCR, Feitosa G, et al. Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis. International Journal of Cardiovascular Sciences 2024;37:e20240064.

Formato de Documento Eletrônico (ABNT)

Key, Ng Kin; Melo, Adelina Sanches de; Sena, Joberto Pinheiro; Dourado, Adriano Dias; Perez, Julia Micheli; Brito, Jose Carlos Raimundo; Feitosa, Gilson; Feitosa-Filho, Gilson Soares. Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis. International Journal of Cardiovascular Sciences, v. 37, e20240064, nov. 2024.

Formato de Documento Eletrônico (APA)

Key, N. K., Melo, A. S. , Sena, J. P., Dourado, A. D., Perez, J. M., Brito, J. C. R., Feitosa, G., & Feitosa-Filho, G. S. (2024). Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis. International Journal of Cardiovascular Sciences, 37, e20240064.

Formato de Documento Eletrônico (ISO)

Key, Ng Kin and Melo, Adelina Sanches de and Sena, Joberto Pinheiro and Dourado, Adriano Dias and Perez, Julia Micheli and Brito, Jose Carlos Raimundo and Feitosa, Gilson and Feitosa-Filho, Gilson Soares. Prevalence of Cardiac Amyloidosis in Elderly Patients With Aortic Stenosis. International Journal of Cardiovascular Sciences [online]. 2024, vol. 37, [citado 2026-10-14], e20240064. Disponível em: <https://journals.cardiol.br/article/prevalence-of-cardiac-amyloidosis-in-elderly-patients-with-aortic-stenosis-2/>. ISSN 2359-4802

EndNote RIS

Formato de Documento Eletrônico (Vancouver)

Simões MV, Fernandes F, Dabarian A, Valicelli FH, Mesquita CT. Evolving Therapies for Transthyretin Cardiac Amyloidosis: New Clinical Trials with Amyloid Fibrils Depleter. International Journal of Cardiovascular Sciences 2024;37:e20240112.

Formato de Documento Eletrônico (ABNT)

Simões, Marcus Vinícius; Fernandes, Fábio; Dabarian, André; Valicelli, Flavio Henrique; Mesquita, Claudio Tinoco. Evolving Therapies for Transthyretin Cardiac Amyloidosis: New Clinical Trials with Amyloid Fibrils Depleter. International Journal of Cardiovascular Sciences, v. 37, e20240112, nov. 2024.

Formato de Documento Eletrônico (APA)

Simões, M. V., Fernandes, F., Dabarian, A., Valicelli, F. H., & Mesquita, C. T. (2024). Evolving Therapies for Transthyretin Cardiac Amyloidosis: New Clinical Trials with Amyloid Fibrils Depleter. International Journal of Cardiovascular Sciences, 37, e20240112.

Formato de Documento Eletrônico (ISO)

Simões, Marcus Vinícius and Fernandes, Fábio and Dabarian, André and Valicelli, Flavio Henrique and Mesquita, Claudio Tinoco. Evolving Therapies for Transthyretin Cardiac Amyloidosis: New Clinical Trials with Amyloid Fibrils Depleter. International Journal of Cardiovascular Sciences [online]. 2024, vol. 37, [citado 2026-10-14], e20240112. Disponível em: <https://journals.cardiol.br/article/evolving-therapies-for-transthyretin-cardiac-amyloidosis-new-clinical-trials-with-amyloid-fibrils-depleter-2/>. ISSN 2359-4802

EndNote RIS

Formato de Documento Eletrônico (Vancouver)

Romero CE, Pereira NM, Luzuriaga GDCJ, Bueno BVK, Carvalhal S, Borges T, Reis B, et al. Prognostic Value of the 6-Minute Walk Test and Clinical Characteristics of Patients With Transthyretin Amyloid Cardiomyopathy. International Journal of Cardiovascular Sciences 2024;37:e20240091.

Formato de Documento Eletrônico (ABNT)

Romero, Cristhian Espinoza; Pereira, Natalia Melo; Luzuriaga, Georgina Del Cisne Jadán; Bueno, Bruno Vaz Kerges; Carvalhal, Suenia; Borges, Thais; Reis, Barbara; Fernandes, Fábio. Prognostic Value of the 6-Minute Walk Test and Clinical Characteristics of Patients With Transthyretin Amyloid Cardiomyopathy. International Journal of Cardiovascular Sciences, v. 37, e20240091, nov. 2024.

Formato de Documento Eletrônico (APA)

Romero, C. E., Pereira, N. M., Luzuriaga, G. D. C. J., Bueno, B. V. K., Carvalhal, S., Borges, T., Reis, B., & Fernandes, F. (2024). Prognostic Value of the 6-Minute Walk Test and Clinical Characteristics of Patients With Transthyretin Amyloid Cardiomyopathy. International Journal of Cardiovascular Sciences, 37, e20240091.

Formato de Documento Eletrônico (ISO)

Romero, Cristhian Espinoza and Pereira, Natalia Melo and Luzuriaga, Georgina Del Cisne Jadán and Bueno, Bruno Vaz Kerges and Carvalhal, Suenia and Borges, Thais and Reis, Barbara and Fernandes, Fábio. Prognostic Value of the 6-Minute Walk Test and Clinical Characteristics of Patients With Transthyretin Amyloid Cardiomyopathy. International Journal of Cardiovascular Sciences [online]. 2024, vol. 37, [citado 2026-10-14], e20240091. Disponível em: <https://journals.cardiol.br/article/prognostic-value-of-the-6-minute-walk-test-and-clinical-characteristics-of-patients-with-transthyretin-amyloid-cardiomyopathy-2/>. ISSN 2359-4802

EndNote RIS

Formato de Documento Eletrônico (Vancouver)

Trevizan LLB, Mangini S. Heart Rate Control or Rhythm Control in Patients With Atrial Fibrillation and Cardiac Amyloidosis. International Journal of Cardiovascular Sciences 2024;37:e20240050.

Formato de Documento Eletrônico (ABNT)

Trevizan, Lígia Lopes Balsalobre; Mangini, Sandrigo. Heart Rate Control or Rhythm Control in Patients With Atrial Fibrillation and Cardiac Amyloidosis. International Journal of Cardiovascular Sciences, v. 37, e20240050, ago. 2024.

Formato de Documento Eletrônico (APA)

Trevizan, L. L. B., & Mangini, S. (2024). Heart Rate Control or Rhythm Control in Patients With Atrial Fibrillation and Cardiac Amyloidosis. International Journal of Cardiovascular Sciences, 37, e20240050.

Formato de Documento Eletrônico (ISO)

Trevizan, Lígia Lopes Balsalobre and Mangini, Sandrigo. Heart Rate Control or Rhythm Control in Patients With Atrial Fibrillation and Cardiac Amyloidosis. International Journal of Cardiovascular Sciences [online]. 2024, vol. 37, [citado 2026-10-14], e20240050. Disponível em: <https://journals.cardiol.br/article/heart-rate-control-or-rhythm-control-in-patients-with-atrial-fibrillation-and-cardiac-amyloidosis-2/>. ISSN 2359-4802

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