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.

Prognostic Role of Contraindicated Drugs in Hospitalized Patients with Decompensated Heart Failure.

Due to the ageing population, patients often present to the hospital with a high burden of comorbidities and polypharmacy. For patients admitted with decompensated heart failure (HF), the evidence on the effects of contraindicated drugs on long-term mortality is scarce. Therefore, we aimed to investigate the effect of contraindicated medications on outcomes of patients admitted with decompensated HF. We analyzed all consecutive patients from the National Heart Failure Audit admitted to two tertiary centers with acutely decompensated HF between April 2020 and October 2021. We included medication classes listed as contraindicated (class III) in the most recent European and American guidelines on the management of HF. The primary outcome measure was in-hospital mortality. The secondary outcome measure was overall mortality. Overall, 716 patients admitted with acute HF were included. One-fifth (n = 156, 21.8%) were on at least one contraindicated medication at admission. The prevalence of comorbidities was comparable between medication groups. During hospitalization, the use of nonsteroidal anti-inflammatory drugs (NSAIDs) was associated with increased in-hospital mortality (29% versus 9%, P = 0.013). On multivariable analyses, NSAID use was independently associated with worse in-hospital mortality (hazard ratio, 6.86; 95% confidence interval, 1.61-25.5; P = 0.005). However, other contraindicated medications were not associated with adverse outcomes. Postdischarge, the use of erythropoietin during admission was associated with increased mortality (54% versus 31%, P = 0.031). NSAID use is associated with increased in-hospital mortality for patients admitted with acute HF. However, inpatient use of other contraindicated medications was not associated with adverse in-hospital outcomes. Further studies are needed to confirm these results in larger and prospective cohorts. SIGNIFICANCE STATEMENT: Use of nonsteroidal anti-inflammatory drugs is associated with a worse in-hospital mortality in patients with decompensated heart failure. The prognostic role of other contraindicated medications remains still uncertain.

Acute Mitral Regurgitation Due to Chordae Tendineae Rupture: A Rare Presentation of Cardiac Amyloidosis.

BACKGROUND In cardiac amyloidosis (CA), misfolded proteins deposit in the extracellular space of cardiac tissue. These deposits classically cause restrictive cardiomyopathy with diastolic dysfunction. Although there are at least 30 proteins known to cause amyloid aggregates, 2 main types make up most diagnosed cases: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Since CA is considered a rare condition, it is often underdiagnosed or recognized in the advanced stages. Once amyloid deposits involve the heart tissue, they are associated with a worse outcome and higher mortality rates, especially in patients presenting symptoms of heart failure. CASE REPORT We report a case of a 22-year-old man presenting with acute severe mitral regurgitation, secondary to posterior mitral leaflet chordae tendineae rupture (CTR). Surgical mitral valve replacement with a mechanical prosthesis was performed, and cardiac tissue biopsy samples were obtained. After surgery, the patient improved significantly but suddenly presented with hemodynamic deterioration, until he died due to severe hemodynamic compromise and multiorgan failure. Although the etiology of the CTR was not established before surgical intervention, the histopathological analysis suggested CA. CONCLUSIONS CA diagnosis can be complex, especially in a 22-year-old-man with atypical clinical and imaging manifestations. In this patient, other differential diagnoses were considered, since CA presenting in a young patient is a rare phenomenon and acute mitral regurgitation secondary to CTR presents more frequently in other heart conditions. Furthermore, rapid postoperative deterioration resulted in the patient's death before biopsy samples were available because suspicion of amyloidosis had not been raised until that point.

On stethoscopes, patient records, artificial intelligence, and zettabytes: A glimpse into the future of digital medicine in Mexico / Sobre estetoscopios, expedientes clínicos, inteligencia artificial y zettabytes: una mirada al futuro de la medicina digital en México

Abstract Science and technology are modifying medicine at a dizzying pace. Although access in our country to the benefits of innovations in the area of devices, data storage and artificial intelligence are still very restricted, the advance of digital medicine offers the opportunity to solve some of the biggest problems faced by medical practice and public health in Mexico. The potential areas where digital medicine can be disruptive are accessibility to quality medical care, centralization of specialties in large cities, dehumanization of medical treatment, lack of resources to access evidence-supported treatments, and among others. This review presents some of the advances that are guiding the new revolution in medicine, discusses the potential barriers to implementation, and suggest crucial elements for the path of incorporation of digital medicine in Mexico.

Resumen La ciencia y la tecnología han modificado la medicina a un ritmo vertiginoso. Si bien el acceso en México a los beneficios de las innovaciones en el área de dispositivos, almacenamiento de datos e inteligencia artificial aún es muy restringido, el avance de la medicina digital ofrece la oportunidad de solventar algunos de los problemas más grandes que enfrenta la práctica médica y la salud pública en este país. Las potenciales áreas en las que la medicina digital puede resultar innovadora son la accesibilidad a cuidados médicos de calidad, la centralización de las especialidades en grandes urbes, la deshumanización del trato médico, la falta de recursos para acceder a tratamientos avalados por evidencia, entre otros. Esta revisión presenta algunos de los avances que guían la nueva revolución en la medicina, revisa el potencial y las posibles barreras para su aplicación, además de sugerir elementos cruciales para el trayecto de incorporación de la medicina digital en México.

Telemedicina como instrumento de consulta cardiológica durante la pandemia COVID-19 / Telemedicine as an instrument for cardiological consultation during the COVID-19 pandemic

Resumen La telemedicina es una herramienta subutilizada en nuestros sistemas de atención sanitaria. Se trata de un recurso tecnológico que optimiza los servicios de salud, ahorra recursos, expande la capacidad de atención especializada a lugares remotos, descongestiona servicios médicos tradicionales y es un instrumento invaluable de enseñanza e investigación. La pandemia por COVID-19 nos obliga a extender su uso y supone una oportunidad para diseñar una adecuada implementación.

Abstract Telemedicine is an underused instrument along our healthcare systems. It´s a technological tool that optimizes resources, save money, expands our capacities, decongests our traditional medical services and is an invaluable help for teaching and research. The COVID-19 pandemic is forcing us to expand its use and it gives us the opportunity to design an appropriate implementation.

Stress myocardial blood flow correlates with ventricular function and synchrony better than myocardial perfusion reserve: A Nitrogen-13 ammonia PET study.

BACKGROUND: Cardiac PET quantifies stress myocardial blood flow (MBF) and perfusion reserve (MPR), while ECG-gated datasets can measure components of ventricular function simultaneously. Stress MBF seems to outperform MPR in the detection of significant CAD. However, it is uncertain which perfusion measurement is more related to ventricular function. We hypothesized that stress MBF correlates with ventricular function better than MPR in patients studied for suspected myocardial ischemia. METHODS: We studied 248 patients referred to a rest and adenosine-stress Nitrogen-13 ammonia PET. We performed a multivariate analysis using systolic function (left ventricular ejection fraction, LVEF), diastolic function (mean filling rate in diastole, MFR/3), and synchrony (Entropy) as the outcome variables, and stress MBF, MPR, and relevant covariates as the predictors. Secondarily, we repeated the analysis for the subgroup of patients with and without a previous myocardial infarction (MI). RESULTS: 166 male and 82 female patients (mean age 63 ± 11 and 67 ± 11 year, respectively) were included. 60% of the patients presented hypertension, 57% dyslipidemia, 21% type 2 diabetes mellitus, 45% smoking, and 34.7% a previous MI. Mean stress MBF was 1.99 ± 0.75 mL/g/min, MPR = 2.55 ± 0.89, LVEF = 61.6 ± 15%, MFR/3 = 1.12 ± 0.38 EDV/s, and Entropy = 45.6 ± 11.3%. There was a significant correlation between stress MBF (P < .001) and ventricular function. This was stronger than the one for MPR (P = .063). Sex, age, diabetes, and extent of previous MI were also significant predictors. Results were similar for the analyses of the 2 subgroups. CONCLUSION: Stress MBF is better correlated with ventricular function than MPR, as evaluated by Nitrogen-13 ammonia PET, independently from other relevant cardiovascular risk factors and clinical covariates. This relationship between coronary vasodilatory capacity and ventricular function is sustained across groups with and without a previous MI.

Endocarditis infecciosa en un paciente con válvula aórtica bicúspide: Reporte y revisión de un caso / Infective endocarditis in a patient with bicuspid aortic valve: a case report and review

Resumen La endocarditis infecciosa es una patología que requiere un diagnóstico y tratamiento oportuno para prevenir sus graves complicaciones y, ante todo, evitar la muerte del paciente. En el presente artículo, documentamos el abordaje diagnóstico y terapéutico de un caso de endocarditis, en el escenario de una cardiopatía congénita, válvula aórtica bicúspide, atendida en el Instituto Nacional de Cardiología "Ignacio Chávez".

Abstract Infective endocarditis is a condition that requires an early diagnosis and treatment to prevent its severe complications and, above all, avoid death. In this article we document the diagnosis and therapeutics of a case of infective endocarditis in the setting of a congenital heart disease, bicuspid aortic valve, referred to the National Institute of Cardiology "Ignacio Chávez".

Anomalía de Ebstein / Ebstein's anomaly

La anomalía de Ebstein es una cardiopatía congénita compleja, caracterizada por el adosamiento de los velos valvulares tricuspídeos posterior y septal al endocardio ventricular derecho, lo que condiciona el desplazamiento del orificio valvular hacia la porción apical del ventrículo derecho, dando por resultado una atrialización de dicho ventrículo. El primer caso reportado en la literatura data del año 1866 por el médico Wilhelm Ebstein. Las manifestaciones clínicas son muy variables; entre las más frecuentes destacan: cianosis progresiva, disnea, insuficiencia cardiaca, palpitaciones y arritmias. El abordaje del paciente debe incluir electrocardiograma, radiografía de tórax y ecocardiograma transtorácico, siendo este último el estándar de oro para establecer el diagnóstico. Únicamente aquellos pacientes que cumplan con los criterios establecidos deberán someterse a tratamiento quirúrgico. Dada la alta similitud de la anomalía de Ebstein con otras displasias de la válvula tricúspide, es fundamental llevar a cabo una evaluación multidisciplinaria para establecer un diagnóstico y tratamiento precisos.

Ebstein's anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle, specifically tethering of the posterior and septal tricuspid valve leaflets to the right ventricle endocardium resulting in an apical displacement of tricuspid ring. The first reported case dates back to 1866 by Wilhelm Ebstein. Clinical presentation varies among patients. The most common symptoms are: progressive cyanosis, exertional dyspnea, heart failure, palpitations and arrhythmias. Patient approach should include electrocardiogram, chest X-ray and transthoracic echocardiogram, the latter being the gold standard for diagnosis. Only those patients who meet established criteria will undergo surgical treatment. Given the high similarity of Ebstein's anomaly with other dysplasias of the tricuspid valve, it is essential to conduct a multidisciplinary evaluation to establish an accurate diagnosis and treatment.