Mexican position paper for the diagnosis and treatment of cardiac amyloidosis.

La amiloidosis es una enfermedad heterogénea, de origen hereditario o adquirido, que se caracteriza por el depósito anormal de proteínas fibrilares en diversos tejidos. Esta enfermedad puede manifestarse de manera localizada o sistémica, lo que genera una amplia variabilidad en su presentación clínica y, frecuentemente, retrasa el diagnóstico. Por ello, es fundamental la identificación temprana de los signos y síntomas para mejorar el pronóstico. El primer posicionamiento mexicano sobre la amiloidosis cardíaca tiene como objetivo resumir las principales características de la enfermedad y sus subtipos, identificando datos de alarma que incrementen la sospecha de su presencia. Además, busca ofrecer un algoritmo diagnóstico que integre los estudios de imagen y de laboratorio disponibles en nuestro país, con el fin de simplificar la toma de decisiones y llegar a un diagnóstico de manera rápida, práctica y actualizada, basado en recomendaciones sustentadas en evidencia científica. Una vez confirmado el diagnóstico, se abordan los distintos retos terapéuticos, tanto farmacológicos como no farmacológicos, ya que los pacientes no responden al tratamiento convencional de insuficiencia cardíaca. Por ello, el tratamiento debe ser individualizado para cada paciente, teniendo en cuenta la presencia de otras comorbilidades. Finalmente, se presentan los principales factores pronósticos que guiarán un tratamiento adecuado, incluyendo los resultados del asesoramiento genético.

Amyloidosis is a heterogeneous, hereditary or acquired disease characterized by the abnormal deposition of fibrillar proteins in various tissues. The disease can be either localized or systemic, leading to significant variability in its clinical presentation and often causing diagnostic delays. For this reason, early identification of signs and symptoms is essential to improve prognosis. The first Mexican position paper on cardiac amyloidosis aims to summarize the main features of the disease and its subtypes, identifying warning signs that increase clinical suspicion. Additionally, it provides a diagnostic algorithm incorporating the imaging and laboratory studies available in our country, to streamline decision-making and facilitate a fast, practical, and up-to-date diagnosis based on evidence-based recommendations. Once the diagnosis is confirmed, the various therapeutic challenges of this disease, both pharmacological and non-pharmacological, are discussed, as patients do not respond to conventional heart failure treatments. Therefore, treatment must be individualized for each patient, considering the presence of other comorbidities. Finally, the key prognostic factors are outlined to guide appropriate treatment, including results from genetic counseling.

Left ventricular shape index and eccentricity index with ECG-gated Nitrogen-13 ammonia PET/CT in patients with myocardial infarction, ischemia, and normal perfusion.

BACKGROUND: LV geometry with shape index (SI) and eccentricity index (EI) measured by myocardial perfusion positron emission tomography/computed tomography (PET/CT) may allow the evaluation of left ventricular (LV) adverse remodeling. This first study aims to explore the relationship of SI and EI values acquired by Nitrogen-13 ammonia PET/CT in patients with normal perfusion, ischemia, and myocardial infarction. And evaluate the correlations between the variables of LV geometry, and with the variables of LV function. METHODS AND RESULTS: One hundred and forty patients who underwent an electrocardiogram (ECG)-gated PET/CT were selected and classified into 4 groups according to ischemia or infarction burden (normal perfusion, mild ischemia, moderate-severe ischemia, and infarction). The variables were automatically retrieved using dedicated software (QPS/QGS; Cedars-Sinai, Los Angeles, CA, USA). On multicomparison analysis (one-way ANOVA and Dunnett's Test), subjects in the infarction group had significant higher values of SI end-diastolic rest (P < 0.001), and stress (P = 0.003), SI end-systolic rest (P = 0.002) and stress (P < 0.001) as well as statistically significant lower values of EI rest (P < 0.001) and stress (P < 0.001) when compared with all other groups. Regarding Pearson correlation, in the infarcted group all the variables of SI and EI were significantly correlated (P < 0.001) with strong correlation coefficients (>0.60). SI end-systolic correlated significantly with the variables of LV function independently of the group of patients (P < 0.05). CONCLUSIONS: Shape and eccentricity indices differ in patients with myocardial infarction as compared to patients with ischemia or normal perfusion. This encourage further research in their potential for detecting LV adverse remodeling.

World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM).

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive and fatal condition that requires early diagnosis, management, and specific treatment. The availability of new disease-modifying therapies has made successful treatment a reality. Transthyretin amyloid cardiomyopathy can be either age-related (wild-type form) or caused by mutations in the TTR gene (genetic, hereditary forms). It is a systemic disease, and while the genetic forms may exhibit a variety of symptoms, a predominant cardiac phenotype is often present. This document aims to provide an overview of ATTR-CM amyloidosis focusing on cardiac involvement, which is the most critical factor for prognosis. It will discuss the available tools for early diagnosis and patient management, given that specific treatments are more effective in the early stages of the disease, and will highlight the importance of a multidisciplinary approach and of specialized amyloidosis centres. To accomplish these goals, the World Heart Federation assembled a panel of 18 expert clinicians specialized in TTR amyloidosis from 13 countries, along with a representative from the Amyloidosis Alliance, a patient advocacy group. This document is based on a review of published literature, expert opinions, registries data, patients' perspectives, treatment options, and ongoing developments, as well as the progress made possible via the existence of centres of excellence. From the patients' perspective, increasing disease awareness is crucial to achieving an early and accurate diagnosis. Patients also seek to receive care at specialized amyloidosis centres and be fully informed about their treatment and prognosis.

Comorbidities and clinical symptoms can modify myocardial function: a cross-sectional study with PET/CT / Comorbilidades y síntomas clínicos que pueden modificar la función miocárdica: estudio transversal con PET/TC

Abstract Objective: Associating comorbidities and cardiac symptoms that alter myocardial mechanical function could help clinicians to correctly identify at-risk population. Methods: We conducted a functional open population cross-sectional study of patients referred to a positron emission tomography/computed tomography unit in Mexico City for evaluation of myocardial function, perfusion, and coronary circulation. Ischemia was defined as a sum difference score ≥ 2. Association between comorbidities and cardiac symptoms was tested using logistic regression models and trend analysis. We performed an interaction analysis to evaluate the addition of any accompanying symptoms to comorbid conditions on impairment of myocardial function. Results: One thousand two hundred and seventy-three patients were enrolled, 66.1% male, with a mean age of 62.4 (± 12.7) years, 360 (28.7%) with ischemia, 925 (72.7%) with at least one comorbidity, and 676 (53.1%) had at least one associated cardiac symptom. Patients without ischemia, type 2 diabetes, arterial hypertension, and adverse cardiac symptoms were associated with adverse mechanical, perfusion, and coronary flow parameters. We observed a trend of a cumulative number of comorbidities and cardiac symptoms with increased ischemia and decreased coronary flow. Only in decreased left ventricular ejection fraction, we demonstrated an interaction effect between increased comorbidities and adverse symptoms. Conclusion: The high burden of comorbidities and symptoms in our population alters myocardial function regardless of the level of ischemia.

Resumen Objetivo: La asociación de comorbilidades y síntomas cardíacos que alteran la función miocárdica podría ayudar a los médicos a identificar correctamente a poblaciones de riesgo. Métodos: Se realizó un estudio transversal en población abierta de pacientes referidos a una unidad de PET/CT en la Ciudad de México para evaluación de la función miocárdica, perfusión y circulación coronaria. La isquemia se definió como una suma de diferencia de puntuación (SDS) ≥ 2. La asociación entre las comorbilidades y los síntomas cardíacos se fundamentó mediante modelos de regresión logística y análisis de tendencias. Realizamos un análisis de interacción para evaluar la adición de cualquier síntoma acompañante a condiciones comórbidas en el deterioro de la función miocárdica. Resultados: Se incluyeron 1.273 pacientes, 66,1% del sexo masculino, con una edad media de 62,4 (± 12.7) años, 360 (28,7%) con isquemia, 925 (72,7%) con al menos una comorbilidad y 676 (53,1%) con al menos una menos un síntoma cardíaco asociado. En pacientes sin isquemia, la diabetes mellitus tipo 2, la hipertensión arterial y los síntomas cardíacos adversos se asociaron con parámetros mecánicos, de perfusión y de flujo coronario adversos. Se observó una tendencia con el número acumulado de comorbilidades y síntomas cardíacos con aumento de la isquemia y disminución del flujo coronario. Solo en la disminución de la FEVI se demostró un efecto de interacción entre el aumento de las comorbilidades y los síntomas adversos. Conclusión: La alta carga de comorbilidades y síntomas en nuestra población altera la función miocárdica independientemente del nivel de isquemia.

Ischemia-guided vs routine non-culprit vessel angioplasty for patients with ST segment elevation myocardial infarction and multi-vessel disease: the IAEA SPECT STEMI trial.

BACKGROUND: In patients with multi-vessel disease presenting with ST elevation myocardial infarction (STEMI), the efficacy and safety of ischemia-guided, vs routine non-culprit vessel angioplasty has not been adequately studied. METHODS: We conducted an international, randomized, non-inferiority trial comparing ischemia-guided non-culprit vessel angioplasty to routine non-culprit vessel angioplasty, following primary PCI for STEMI. The primary outcome was the between-group difference in percent ischemic myocardium at follow-up stress MPI. All MPI images were processed and analyzed at a central core lab, blinded to treatment allocation. RESULTS: In all, 109 patients were enrolled from nine countries. In the ischemia-guided arm, 25/48 (47%) patients underwent non-culprit vessel PCI following stress MPI. In the routine non-culprit PCI arm, 43/56 (77%) patients underwent angioplasty (86% within 6 weeks of randomization). The median percentage of ischemic myocardium on follow-up imaging (mean 16.5 months) was low, and identical (2.9%) in both arms (difference 0.13%, 95%CI - 1.3%-1.6%, P < .0001; non-inferiority margin 5%). CONCLUSION: A strategy of ischemia-guided non-culprit PCI resulted in low ischemia burden, and was non-inferior to a strategy of routine non-culprit vessel PCI in reducing ischemia burden. Selective non-culprit PCI following STEMI offers the potential for cost-savings, and may be particularly relevant to low-resource settings. (CTRI/2018/08/015384).

Comorbidities and cardiac symptoms can modify myocardial function regardless of ischemia: a cross-sectional study with PET/CT.

Objective: Associating comorbidities and cardiac symptoms that alter myocardial mechanical function could help clinicians to correctly identify at-risk population. Methods: We conducted a functional open population cross-sectional study of patients referred to a positron emission computed tomography/computed tomography unit in Mexico City for evaluation of myocardial function, perfusion, and coronary circulation. Ischemia was defined as a sum difference score (SDS) > 2. Association between comorbidities and cardiac symptoms was tested using logistic regression models and trend analysis. We performed an interaction analysis to evaluate the addition of any accompanying symptoms to comorbid conditions on impairment of myocardial function. Results: One thousand two hundred and seventy-three patients were enrolled, 66.1% male, with a mean age of 62.4 (± 12.7) years, 360 (28.7%) with ischemia, 925 (72.7%) with at least one comorbidity, and 676 (53.1%) had at least one associated cardiac symptom. Patients without ischemia, type 2 diabetes, arterial hypertension, and adverse cardiac symptoms were associated with adverse function, perfusion, and coronary flow parameters. We observed a trend of a cumulative number of comorbidities and cardiac symptoms with increased ischemia and decreased coronary flow. Only in decreased LVEF, we demonstrated an interaction effect between increased comorbidities and adverse symptoms. Conclusions: The high burden of comorbidities and symptoms in our population alter myocardial function regardless of the level of ischemia.

Role of molecular imaging in the diagnosis of prosthetic aortic valve endocarditis by Bacillus licheniformis: a case report.

Background: Infective endocarditis is a challenging diagnosis that usually requires cardiovascular image confirmation as part of the approach. 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) is an imaging technique more sensible for the diagnosis of prosthetic valve endocarditis (PVE) when echocardiography is inconclusive. Case summary: We present the case of a 35-year-old man who had a previous Bentall-De Bono procedure 4 years prior that included biological, national institute of cardiology (INC)-type, locally manufactured aortic valve replacement and woven Dacron tube graft implantation in the ascending aorta. He was admitted because of dyspnoea, oedema, fever, and syncope. A complete auriculoventricular blockade was diagnosed, requiring cardiac pacing. Also, infective endocarditis (IE) was suspected. Blood cultures showed the isolation of Bacillus licheniformis. Transthoracic echocardiography, transoesophageal echocardiography, and CT angiography were inconclusive for IE. Treatment was initiated with intravenous (IV) antibiotic therapy, and an extensive protocol for IE, including molecular imaging modalities, was ordered. 99mTc-Ubiquicidin scintigraphy was acquired without abnormal findings. Images of 18F-FDG-PET/CT revealed abnormally intense heterogeneous uptake in the prosthetic aortic annulus in a classic pattern. Applying the modified 2015 Duke criteria for PET/CT, PVE was confirmed. Discussion: Although the other imaging modalities were negative, the high clinical suspicion made it mandatory to continue the study protocol, remarking on the utility of 18F-FDG-PET/CT on patients categorized as having 'possible' endocarditis, as in our patient.

13N-Ammonia myocardial blood flow quantitation in patient with aneurismal coronary artery disease.

Aneurysmal coronary artery disease includes coronary artery aneurysms and ectasia; this condition has been associated with poor long-term outcomes. Few studies have explored myocardial blood flow 13N-ammonia PET/CT MPI added value. We present a 45-year-old man who came to the emergency department with chest pain. After a physical examination and laboratory studies, he was diagnosed with very high-risk unstable angina and referred to the catheterization laboratory. Coronary angiography showed the culprit lesion in the LCx and was treated by angioplasty and stent. LAD was found with coronary artery ectasia (TIMI 2 flow grade) and the RCA with aneurysmal disease in the proximal and middle segments (TIMI 3 flow grade). Medical treatment was decided for these findings and the patient was discharged. Two weeks later, we performed a 13N-ammonia PET/CT MPI founding apical, inferior, and inferoseptal severe ischemia, and reduced hyperemic coronary blood flow and coronary flow reserve in the RCA territory. Flow was normal in the LAD territory. Although coronary angiography remains the gold standard for evaluating these coronary abnormalities, it does not show the physiological compromise. Therefore 13N-ammonia PET/CT MPI should be performed as a complementary noninvasive imaging approach.

Assessment of Atypical Cardiovascular Risk Factors Using Single Photon Emission Computed Tomography in Mexican Women.

BACKGROUND: Ischemic heart disease (IHD) is a health care problem in women that increases morbimortality, particularly in developing countries. There is limited information regarding atypical risk factors associated with IHD in Mexican women. AIM: To explore risk factors in women that could contribute to IHD and myocardial dysfunction using the single photon emission computed tomography (SPECT) myocardial perfusion study (MPS). METHODS: We designed a cross-sectional study in which we evaluated atypical and typical risk factors using a clinical questionnaire. We performed a SPECT-MPS to evaluate the presence of ischemia/infarction, decreased left ventricular ejection fraction, systolic dyssynchrony and diastolic function by peak filling rate and time to peak filling rate. RESULTS: 172 women were included, 64 with IHD. Adverse events during pregnancy (premature birth and miscarriage), rheumatoid arthritis, gynecological conditions (menopause and age of first menstruation) and low educational level, together with previously known typical risk factors were associated with infarction or ischemia and ventricular dysfunction. Potential associated factors for systolic dyssynchrony were rheumatoid arthritis (OR: 2.90, 95% CI: 0.95-8.66, p = 0.054) and history of premature birth (OR: 0.13, 95% CI: 0.01-0.66, p <0.01). Although those women with arterial hypertension and smoking shown an increased risk for dyssynchrony, these factors were not statistically significant. Low-educational level (OR 2.16, 95% CI 1.1-4.18, p = 0.019) was associated with decreased peak filling rate. CONCLUSION: The presence of atypical risk factors in women could lead to decreased myocardial function, particularly in women at risk of developing IHD.

Collaboration between the National Association of Cardiologists of Mexico, the Mexican Society of Cardiology, and the University of Guanajuato. COVID-19 and Cardiac Imaging Personnel: Review of Sanitary Practices in Early 2021 / Colaboración de la Asociación Nacional de Cardiólogos de México y de la Sociedad Mexicana de Cardiología con la Universidad de Guanajuato. COVID-19 y personal de imagen cardiaca: Revisión de prácticas sanitarias al inicio de 2021

The authors of the image chapters of the National Association of Cardiologists of Mexico (ANCAM) and the Mexican Society of Cardiology (SMC), as well as personnel from the Department of Medicine and Nutrition of the University of Guanajuato, together with prominent experts in cardiovascular imaging from Mexico, have collaborated in the review, analysis and expansion of the various health strategies published in the first year of the coronavirus disease 2019 (COVID-19) pandemic, to safely perform cardiac imaging studies. This update aims to reduce the risk of COVID-19 transmission among patients and health-care personnel in the CT, MRI, and nuclear cardiology services. This work was expanded with supplementary information available free of charge on the website www.ancam-imagen.com.

Los capítulos de imagen de la Asociación Nacional de Cardiólogos de México (ANCAM) y de la Sociedad Mexicana de Cardiología (SMC), así como personal del Departamento de Medicina y Nutrición de la Universidad de Guanajuato, en conjunto con destacados expertos de la imagen cardiovascular en México, han colaborado en la revisión, análisis y ampliación de las diversas estrategias sanitarias publicadas en los primeros 15 meses de la pandemia de enfermedad por coronavirus 2019 (COVID-19) para realizar con seguridad los estudios de imagen cardiaca; esta actualización tiene como objetivo principal disminuir el riesgo de transmisión de la COVID-19 entre los pacientes y el personal de salud en los servicios de tomografía, resonancia y cardiología nuclear. Este trabajo se amplió con información suplementaria disponible sin costo en el sitio www.ancam-imagen.com.

Effectiveness and Safety of Extracorporeal Shockwave Myocardial Revascularization in Patients With Refractory Angina Pectoris and Heart Failure.

Extracorporeal shockwave myocardial revascularization (ESMR) is a therapy for refractory angina pectoris. Our aim was to assess the efficacy and safety of ESMR in the management of patients with stable coronary artery disease (CAD) and heart failure as well as its effects on inflammation and angiogenesis. In this single-arm prospective trial, we included 48 patients with CAD, myocardial ischemia assessed by radionuclide imaging, echocardiographic evidence of left ventricular systolic dysfunction and without revascularization options. Changes in angina grading score, myocardial perfusion, left ventricular ejection fraction, and six-minute walk test after ESMR therapy were used for efficacy assessment. Changes of inflammation and angiogenesis biomarkers were also evaluated. ESMR therapy was performed using a commercially available cardiac shockwave generator system (Cardiospec; Medispec). After 9 weeks of ESMR therapy, a significant improvement was found regarding the initial angina class, severity of ischemia, left ventricular ejection fraction, and six-minute walk test in most patients. No deleterious side effects after treatment were detected. Regarding biomarkers, endothelial progenitor cells and angiopoietin-3 were significantly increased whereas IL-18 and TGF-ß were significantly decreased after ESMR in the total group. Notably, VEGF, IL-1ß, and lipoxin A4 levels were significantly increased only in patients with myocardial ischemia improvement. In conclusion, ESMR therapy is safe and effective in most but not all patients with CAD and heart failure. ESMR is associated with increased markers of angiogenesis and decreased markers of inflammation. Myocardial ischemia improvement after ESMR is associated with increased markers of angiogenesis and pro-resolving mediators.

Diastolic dyssynchrony assessment by gated myocardial perfusion-SPECT in subjects who underwent cardiac resynchronization therapy.

BACKGROUND: Left ventricular diastolic dyssynchrony (LVDD) can be assessed by gated myocardial perfusion single-photon emission computed tomography (GMP-SPECT). LVDD is an area of interest in subjects who underwent cardiac resynchronization therapy (CRT). The aim of this post hoc analysis was to assess the role of LVDD in subjects with CRT who were followed up at 6-month period. MATERIAL & METHODS: Left ventricular diastolic dyssynchrony was assessed by GMP-SPECT at baseline and after CRT procedure in 160 subjects from 10 different cardiological centers. CRT procedure was performed as per current guidelines. Outcomes were defined as improvement in ≥1 New York Heart Association (NYHA) class, left ventricular ejection fraction (LVEF) by 5%, and reduction in end-systolic volume (ESV) by 15% and 5% points in Minnesota Living with Heart Failure Questionnaire. LVDD was defined as diastolic phase standard deviation ≥40 ± 14°. RESULTS: Improvement in NYHA functional class occurred in 105 (65.6%), LVEF in 74 (46.3%), decrease in ESV in 86 (53.8%), and Minnesota score in 85 (53.1%) cases. Baseline LV diastolic standard deviation was 53.53° ± 20.85 and at follow-up 40.44° ± 26.1283; (P < 0.001). LVDD was not associated with improvement in clinical outcomes at follow-up. CONCLUSION: CRT improves both systolic and diastolic dyssynchrony values at 6-month follow-up. LVDD at baseline is correlated with cardiac functionality at follow-up, but not with overall favorable clinical outcomes.

Optimal Medical Treatment vs. Invasive Approach in Patients with Significantly Obstructive Coronary Artery Disease and Ischemia.

INTRODUCTION: Stable ischemic heart disease (SIHD) is a condition that develops in subjects after myocardial infarction. Evidence suggests that optimal medical treatment (OMT) is not inferior to intervention (INT) using percutaneous coronary intervention (PCI) or coronary artery bypass graft (CABG). AIM: To compare clinical outcomes in subjects with SIHD who only received OMT and those who received INT+OMT. METHODS: We retrospectively examined subjects with SIHD who underwent myocardial perfusion study-SPECT/CT in a reference center in Mexico. We assigned two branches: INT+OMT (subjects with previous PCI or CABG) and OMT (subjects with antiplatelet drugs, ß-blockers, renin-angiotensin-system blockade, nitrates, calcium-channel blockers, and aggressive lipid-lowering therapy). Clinical outcomes at follow-up were angina relief, functional class improvement, hospitalization, myocardial reinfarction and death from any cause. RESULTS: We included 100 subjects; 51 with OMT and 49 with INT+OMT. 54 subjects had 1 affected vessel and 46 more than 2. INT+OMT group had up to 14 fold likelihood (95% CI: 3.38-63.35) of achieving angina relief and 2.2 fold likelihood (95% CI: 0.92-5.57, p = 0.077) for functional class improvement. No differences were found in hospitalization, myocardial infarction and death from any cause compared to OMT. CONCLUSIONS: Subjects with OMT have no higher risk of adverse clinical outcomes compared to INT+OMT. However, the INT+OMT provides angina relief and functional class improvement compared to OMT.

Comorbid conditions in individuals assessed by SPECT: Study of a reference cardiology center in Mexico City.

BACKGROUND: There is an increasing prevalence of comorbidities in patients with ischemic heart disease (IHD) in developing countries. The aim of this work is to assess the prevalence of comorbidities and associated factors for IHD among patients at a reference cardiology center. DESIGN AND METHODS: This was a cross-sectional study. A complete clinical history which focused on the main comorbidities, previous myocardial infarction, and the main reason of referral was assessed. A single-photon emission computed tomography (SPECT) myocardial perfusion study (MPS) with two protocols was performed. RESULTS: We included 1998 patients, 64.2% male, median age 63 (I.R.: 56-71) years. 1514 (75.8%) subjects had at least one associated comorbidity. The main comorbidity was diabetes (T2D) (772: 38.6%), followed by systemic hypertension (737: 36.9%), smoking (518: 25.9%), and dyslipidemia (517: 25.9%). 806 (40.3%) had histories of previous myocardial infarctions. The main cause of referral was angina (923: 46.2%). We identified 1330 (66.5%) abnormal MPS. 460 (23%) had ischemia, 292 (14.6%) infarction, and 578 (28.9%) ischemia and infarction. CONCLUSION: An increased prevalence of comorbidities was found in patients who were studied in the Nuclear Cardiology Department (NCD): most of them had traditional risk factors attributable to myocardial infarction. A great percentage were newly diagnosed with both ischemia and infarction.