RESUMEN

AIM: Increased diagnostic awareness and specific disease treatments have changed the landscape of transthyretin cardiac amyloidosis (ATTR). Patients with wild-type ATTR (ATTRwt) are increasingly being diagnosed, potentially changing the clinical profile and prognosis compared with existing retrospective data. We aimed to study the clinical characteristics, distribution of red flags and prognosis of contemporary ATTRwt patients. METHODS: From January 1st 2017, to December 31st 2022, 213 consecutive patients were diagnosed with ATTRwt and prospectively followed up. Data on clinical characteristics, biomarkers, echocardiography findings, hospitalization due to worsening heart failure (WHF) and all-cause mortality were collected. RESULTS: A 37% increase in newly diagnosed patients from 2017-2019 (n = 90) vs. 2020-2022 (n = 123) was observed. The majority of patients presented with NAC disease stage I in the latter period (49% in 2017-2019 vs. 58% in 2020-2022, p = .16). Red flags were primarily cardiac-related, including elevated NT-proBNP, impaired left ventricular longitudinal systolic strain with an apical sparing pattern, heart failure with increased left ventricular wall thickness and elevated troponins. NAC disease stage I as well as low NT-proBNP levels (<1000 ng/L) were significantly associated with better survival (both p < .001). When compared with NAC disease stage II + III combined, patients with NAC disease stage I had a significantly lower risk of WHF hospitalization or death (log rank test: p = .0001). Independent predictors of the combined endpoint WHF hospitalization or death were NT-proBNP (HR 1.03 [95% CI 1.00-1.07], p < .049) and prior implantation of permanent pacemaker (HR 2.01 [1.30-3.11], p = .002). CONCLUSION: Increased diagnostic awareness resulted in a 37% increase in newly diagnosed patients in 2020-2022 vs. 2017-2019. As expected all-cause mortality but also the morbidity in terms of risk of hospitalization with WHF were significantly lower in patients with NAC disease stage I, as well as in those with low NT-proBNP levels <1000 ng/L. These findings underline the importance of continuous attention to diagnostic awareness, as early diagnosis is critical for initiating both general and specific ATTR treatment, thus improving prognosis.

Asunto(s)

Neuropatías Amiloides Familiares , Cardiomiopatías , Ecocardiografía , Insuficiencia Cardíaca , Péptido Natriurético Encefálico , Humanos , Masculino , Femenino , Neuropatías Amiloides Familiares/sangre , Neuropatías Amiloides Familiares/diagnóstico , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/mortalidad , Anciano , Pronóstico , Cardiomiopatías/diagnóstico , Cardiomiopatías/sangre , Cardiomiopatías/mortalidad , Péptido Natriurético Encefálico/sangre , Persona de Mediana Edad , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/sangre , Fragmentos de Péptidos/sangre , Anciano de 80 o más Años , Estudios Prospectivos , Hospitalización/estadística & datos numéricos , Biomarcadores/sangre , Estudios Retrospectivos

Asunto(s)

Cardiomiopatías , Muerte Súbita Cardíaca , Genotipo , Fenotipo , Humanos , Muerte Súbita Cardíaca/etiología , Medición de Riesgo , Cardiomiopatías/genética , Cardiomiopatías/mortalidad , Cardiomiopatías/complicaciones

RESUMEN

BACKGROUND: Early identification of immunoglobulin light-chain amyloidosis (AL) is crucial due to its rapid progression. Monoclonal light-chain (M-LC) testing is the first step in the diagnostic workup for patients with suspected cardiac amyloidosis (CA). We aimed to determine whether the time interval between the first CA suspicion and M-LC testing can be related to AL amyloidosis survival outcomes. METHODS: All patients (n = 94) with isolated cardiac AL amyloidosis diagnosed at our center between 2016 and 2020 were included. Those with pre-existing known monoclonal protein (monoclonal gammopathy of undetermined significance or smoldering multiple myeloma) were excluded. Time intervals to diagnostic tests and diagnosis were calculated and assessed for their survival prediction ability. RESULTS: The time interval between first CA suspicion (on echocardiography) and M-LC testing correlated with early mortality, and the best cutoff predicting survival, was 6 weeks. The 26 patients (∼28% of entire cohort) who underwent M-LC-studies >6 weeks after first suspicion more frequently presented Mayo stage IIIb (65% vs. 35%, p = .008), showing poorer overall survival than those (n = 68, 72%) referred for early M-LC studies (median 3 vs. 14 months, p = .039). CONCLUSIONS: Monoclonal protein testing should be the first-step in the diagnostic workup for patients with echocardiographic/other instrumental red flags raising CA suspicion.

Asunto(s)

Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Humanos , Masculino , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/mortalidad , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/diagnóstico , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/patología , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/metabolismo , Femenino , Anciano , Persona de Mediana Edad , Ecocardiografía , Cadenas Ligeras de Inmunoglobulina/sangre , Cadenas Ligeras de Inmunoglobulina/metabolismo , Estudios Retrospectivos , Cardiomiopatías/mortalidad , Cardiomiopatías/diagnóstico , Cardiomiopatías/patología , Cardiomiopatías/metabolismo , Anciano de 80 o más Años

RESUMEN

BACKGROUND: Light chain cardiac amyloidosis (AL-CA) is associated with a high incidence of mortality. Big endothelin-1 (ET-1), the precursor of endothelial-vasoconstrictive ET-1, is closely related to the concentration of bioactive ET-1. Association between big ET-1 and prognosis of AL-CA has not yet been documented. The purpose of this study was to evaluate the prognostic value of big ET-1 for poor outcomes in moderate to severe AL-CA. METHODS: Big ET-1 levels were determined on admission in patients with newly diagnosed AL-CA with modified Mayo 2004 stage II or III. Primary outcome was all-cause mortality. The secondary outcomes included death from cardiac cause and the composite of the primary outcome or hospitalisations due to worsening heart failure. RESULTS: Overall, 141 patients were retrospectively included (57 stage II, 34 stage IIIa, 50 stage IIIb). During a median follow-up time of 25.7 months, 84 (59.6%) patients died. Patients with big ET-1 levels of ≤0.88 pmol/L had longer survival than those with >0.88 pmol/L (median survival time: 34.1 months vs 15.3 months, log-rank p<0.001), which was also observed in the validation cohort (log-rank p=0.026). Higher big ET-1 levels were predictive for all-cause mortality after multivariable adjustment (HR 1.91, 95% CI 1.05 to 3.49, p=0.035). Big ET-1 levels added an incremental prognostic value over modified Mayo 2004 stage (C-index: from 0.671 to 0.696, p=0.025; integrated discrimination improvement 0.168, p=0.047). CONCLUSIONS: Big ET-1 is a strong and independent predictor of mortality in patients with moderate to severe AL-CA, which may indicate a possible role for risk stratification in patients with this disease.

Asunto(s)

Biomarcadores , Cardiomiopatías , Endotelina-1 , Humanos , Endotelina-1/sangre , Masculino , Femenino , Anciano , Pronóstico , Estudios Retrospectivos , Persona de Mediana Edad , Cardiomiopatías/sangre , Cardiomiopatías/mortalidad , Cardiomiopatías/diagnóstico , Biomarcadores/sangre , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/sangre , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/mortalidad , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/diagnóstico , Índice de Severidad de la Enfermedad , Valor Predictivo de las Pruebas , Factores de Riesgo , Medición de Riesgo/métodos

RESUMEN

The prognostic implications of late gadolinium-enhanced (LGE) magnetic resonance imaging (MRI) in the context of cardiac sarcoidosis (CS) have attracted considerable attention. Nevertheless, a subset of studies has undistinguished confirmed and suspected CS cases, thereby engendering interpretative ambiguities. In this meta-analysis, we evaluated the differences in cardiac MRI findings and their prognostic utility between confirmed and suspected CS. A literature search was conducted using PubMed, Web of Science, and Cochrane libraries to compare the findings of cardiac MRI and its prognostic value in CS and suspected CS. A meta-analysis was performed to compare the prevalence of LGE MRI, odds ratios, and hazard ratios for predicting cardiac events in both groups. A total of 21 studies encompassing 24 different populations were included in the meta-analysis (CS: 393 cases, suspected CS: 2151 cases). CS had a higher frequency of LGE of the left ventricle (87.2% vs. 36.4%, p < 0.0001) and right ventricle (62.1% vs. 23.8%, p = 0.04) than suspected CS. In patients with suspected CS, the presence of left ventricular LGE was associated with higher all-cause mortality [odds ratio: 5.70 (95%CI: 2.51-12.93), p < 0.0001, I2 = 8%, p for heterogeneity = 0.37] and ventricular arrhythmia [odds ratio: 15.51 (95%CI: 5.65-42.55), p < 0.0001, I2 = 0, p for heterogeneity = 0.94]. In contrast, in CS, not the presence but extent of left ventricular LGE was a significant predictor of outcome (hazard ratio = 1.83 per 10% increase of %LGE (95%CI: 1.43-2.34, p < 0.001, I2 = 15, p for heterogeneity = 0.31). The presence of left ventricular LGE was a strong prognostic factor in suspected sarcoidosis. However, the extremely high prevalence of left ventricular LGE in confirmed CS suggests that the quantitative assessment of LGE is useful for prognostic estimation.

Asunto(s)

Cardiomiopatías , Medios de Contraste , Imagen por Resonancia Magnética , Miocardio , Valor Predictivo de las Pruebas , Sarcoidosis , Humanos , Sarcoidosis/diagnóstico por imagen , Sarcoidosis/mortalidad , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/mortalidad , Pronóstico , Factores de Riesgo , Persona de Mediana Edad , Femenino , Miocardio/patología , Masculino , Adulto , Oportunidad Relativa , Anciano , Distribución de Chi-Cuadrado , Gadolinio , Función Ventricular Izquierda

RESUMEN

Anemia is prevalent in transthyretin amyloid cardiomyopathy (ATTR-CM), but its prognostic significance remains uncertain because of conflicting data mainly in patients not receiving disease-modifying therapy. Additionally, the effect of anemia on morbidity in this population has not been studied. This retrospective study included 270 patients diagnosed with ATTR-CM, receiving disease-modifying treatment (tafamidis), of which 30% (n = 80) were anemic (defined as a hemoglobin level <13 g/100 ml for males and <12 g/100 ml for females according to the World Health Organization). At baseline, patients with anemia were on average older (mean age 79 vs 77 years), more likely to be female (21% vs 12%), and exhibited higher symptom severity based on the New York Heart Association class (42% in class III vs 27%) compared with those without anemia. Additionally, they had a worse Columbia score (mean score 3 vs 5) and Columbia stage (12% in late-stage vs 7.1%) than those without anemia. Kaplan-Meier analysis indicates that anemia was associated with a higher likelihood of mortality, all-cause, and cardiovascular (CV) hospitalizations (p <0.05). However, in the Cox regression analysis, after adjusting for baseline age, ATTR genotype, and Columbia score, anemia was only associated with a higher risk of all-cause hospitalizations (hazard ratio 1.9 (1.3 to 2.7), p <0.001) and CV-related hospitalizations (hazard ratio 1.9 (1.2 to 2.9), p = 0.006). In conclusion, this study indicates that anemic patients with ATTR-CM have higher risks of CV and all-cause hospitalizations compared with nonanemic ATTR-CM patients. Further research is needed to understand how treating anemia may improve outcomes in this high-risk patient population.

Asunto(s)

Neuropatías Amiloides Familiares , Anemia , Cardiomiopatías , Humanos , Femenino , Masculino , Anemia/epidemiología , Anemia/complicaciones , Anciano , Estudios Retrospectivos , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/mortalidad , Cardiomiopatías/mortalidad , Cardiomiopatías/complicaciones , Cardiomiopatías/epidemiología , Pronóstico , Anciano de 80 o más Años , Morbilidad/tendencias , Tasa de Supervivencia/tendencias , Benzoxazoles/uso terapéutico

RESUMEN

BACKGROUND: Positron emission tomography/computed tomography (PET/CT) with 18F-florbetapir, a novel amyloid-targeting radiotracer, can quantify left ventricular (LV) amyloid burden in systemic light-chain (AL) amyloidosis. However, its prognostic value is not known. OBJECTIVES: The authors' aim was to evaluate the prognostic value of LV amyloid burden quantified by 18F-florbetapir PET/CT, and to identify mechanistic pathways mediating its association with outcomes. METHODS: A total of 81 participants with newly diagnosed AL amyloidosis underwent 18F-florbetapir PET/CT imaging. Amyloid burden was quantified using 18F-florbetapir LV uptake as percent injected dose. The Mayo stage for AL amyloidosis was determined using troponin T, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain levels. Major adverse cardiac events (MACE) were defined as all-cause death, heart failure hospitalization, or cardiac transplantation within 12 months. RESULTS: Among participants (median age, 61 years; 57% males), 36% experienced MACE, increasing from 7% to 63% across tertiles of LV amyloid burden (P < 0.001). LV amyloid burden was associated with MACE (HR: 1.46; 95% CI: 1.16-1.83; P = 0.001). However, this association became nonsignificant when adjusted for Mayo stage. In mediation analysis, the association between LV amyloid burden and MACE was mediated by NT-proBNP (P < 0.001), a marker of cardiomyocyte stretch and heart failure, and a component of Mayo stage. CONCLUSIONS: In this first study to link cardiac 18F-florbetapir uptake to subsequent outcomes, LV amyloid burden estimated by percent injected dose predicted MACE in AL amyloidosis. This effect was not independent of Mayo stage and was mediated primarily through NT-proBNP. These findings provide novel insights into the mechanism linking myocardial amyloid deposits to MACE.

Asunto(s)

Compuestos de Anilina , Glicoles de Etileno , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Péptido Natriurético Encefálico , Fragmentos de Péptidos , Tomografía Computarizada por Tomografía de Emisión de Positrones , Valor Predictivo de las Pruebas , Radiofármacos , Humanos , Femenino , Masculino , Persona de Mediana Edad , Anciano , Radiofármacos/administración & dosificación , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/diagnóstico por imagen , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/metabolismo , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/mortalidad , Pronóstico , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/metabolismo , Fragmentos de Péptidos/sangre , Factores de Riesgo , Función Ventricular Izquierda , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/metabolismo , Factores de Tiempo , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/metabolismo , Biomarcadores/sangre , Trasplante de Corazón/efectos adversos , Medición de Riesgo , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/metabolismo , Cardiomiopatías/mortalidad , Cadenas Ligeras de Inmunoglobulina/metabolismo

RESUMEN

BACKGROUND: Results from ATTR-ACT (Safety and Efficacy of Tafamidis in Patients With Transthyretin Cardiomyopathy) indicate that tafamidis prolongs survival and reduces cardiovascular hospitalizations in cardiac transthyretin amyloidosis (ATTR-CA). However, real-world data supporting these findings are scarce. Thus, we sought to characterize the clinical outcome of patients with ATTR-CA treated with tafamidis in a real-world setting and assess the prognostic role of the New York Heart Association (NYHA) classification. METHODS AND RESULTS: We conducted a retrospective observational study, enrolling a consecutive sample of patients with ATTR-CA (wild-type or variant) treated with tafamidis. Clinical outcome was tracked through follow-up visits or phone calls. Primary outcomes were death and major adverse cardiac events (MACE), a composite end point of death and hospitalizations for acute cardiac decompensation, myocardial infarction, severe arrythmias, or stroke. Kaplan-Meier analysis estimated overall and MACE-free survival including NYHA subgroups (NYHA I/II versus NYHA III). One hundred sixty-seven patients with ATTR-CA (94.6% wild-type) were enrolled and followed for a median of 539 [323-869] days. Median overall survival was not reached. Estimated 1-year, 2-year, and 5-year overall survival among the whole cohort was 93.5%, 85.9%, and 70.2%, respectively. Overall survival was higher in the NYHA I/II subgroup (P=0.002). Median MACE-free survival time was 1082 (95% CI, 962-1202) days. MACE-free survival was higher in the NYHA I/II subgroup (P<0.001). With respective hazard ratios of 5.85 (95% CI, 1.48-23.18; P=0.012) and 3.95 (95% CI, 1.99-7.84; P<0.001), NYHA III was an independent predictor of death and MACE. CONCLUSIONS: Treatment of ATTR-CA with tafamidis led to substantial improvements of clinical outcome. NYHA classification at treatment initiation is a reliable tool to provide patients with individualized prognostic information.

Asunto(s)

Neuropatías Amiloides Familiares , Humanos , Masculino , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/mortalidad , Neuropatías Amiloides Familiares/terapia , Neuropatías Amiloides Familiares/tratamiento farmacológico , Femenino , Estudios Retrospectivos , Anciano , Benzoxazoles/uso terapéutico , Persona de Mediana Edad , Resultado del Tratamiento , Cardiomiopatías/mortalidad , Anciano de 80 o más Años , Factores de Tiempo , Hospitalización/estadística & datos numéricos , Pronóstico

RESUMEN

AIMS: Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is often accompanied by atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia (AT), which are difficult to control because beta-blockers and antiarrhythmic drugs can worsen heart failure (HF). This study aimed to investigate the outcomes of catheter ablation (CA) for AF/AFL/AT in patients with ATTRwt-CM and propose a treatment strategy for CA. METHODS AND RESULTS: A cohort study was conducted on 233 patients diagnosed with ATTRwt-CM, including 54 who underwent CA for AF/AFL/AT. The background of each arrhythmia and the details of the CA and its outcomes were investigated. The recurrence-free rate of AF/AFL/AT overall in ATTRwt-CM patients with multiple CA was 70.1% at 1-year, 57.6% at 2-year, and 44.0% at 5-year follow-up, but CA significantly reduced all-cause mortality [hazard ratio (HR): 0.342, 95% confidence interval (CI): 0.133-0.876, P = 0.025], cardiovascular mortality (HR: 0.378, 95% CI: 0.146-0.981, P = 0.045), and HF hospitalization (HR: 0.488, 95% CI: 0.269-0.889, P = 0.019) compared with those without CA. There was no recurrence of the cavotricuspid isthmus (CTI)-dependent AFL, non-CTI-dependent simple AFL terminated by one linear ablation, and focal AT originating from the atrioventricular (AV) annulus or crista terminalis eventually. Twelve of 13 patients with paroxysmal AF and 27 of 29 patients with persistent AF did not have recurrence as AF. However, all three patients with non-CTI-dependent complex AFL not terminated by a single linear ablation and 10 of 13 cases with focal AT or multiple focal ATs originating beyond the AV annulus or crista terminalis recurred even after multiple CA. CONCLUSION: The outcomes of CA for ATTRwt-CM were acceptable, except for multiple focal AT and complex AFL. Catheter ablation may be aggressively considered as a treatment strategy with the expectation of improving mortality and hospitalization for HF.

Asunto(s)

Neuropatías Amiloides Familiares , Fibrilación Atrial , Aleteo Atrial , Cardiomiopatías , Ablación por Catéter , Humanos , Ablación por Catéter/efectos adversos , Masculino , Aleteo Atrial/cirugía , Aleteo Atrial/etiología , Femenino , Fibrilación Atrial/cirugía , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/fisiopatología , Anciano , Neuropatías Amiloides Familiares/cirugía , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/mortalidad , Cardiomiopatías/mortalidad , Cardiomiopatías/terapia , Resultado del Tratamiento , Persona de Mediana Edad , Recurrencia , Taquicardia Supraventricular/cirugía , Taquicardia Supraventricular/etiología , Taquicardia Supraventricular/fisiopatología , Taquicardia Supraventricular/diagnóstico , Estudios Retrospectivos , Prealbúmina/genética , Prealbúmina/metabolismo

RESUMEN

BACKGROUND: Although peripartum cardiomyopathy (PPCM) is a fatal disease affecting young patients and fetuses, little is known about its recent prognosis and risk factors. This study investigated temporal trends in clinical characteristics and outcomes for PPCM in a nationwide multicenter registry. METHODS AND RESULTS: The study population comprised 340 patients (mean age, 33 years) who were diagnosed with PPCM between January 2000 and September 2022 in 26 tertiary hospitals in South Korea. PPCM was defined as heart failure with left ventricular ejection fraction ≤45% and no previously known cardiac disease. The main study outcomes included time to the first occurrence of all-cause death, heart transplantation, and cardiovascular hospitalization. The diagnosis of PPCM cases increased notably during the study period (P<0.001). However, clinical outcomes showed no significant improvement (all-cause death for 10 years: 0.9% [2000-2010] versus 2.3% [2011-2022], P=0.450; all-cause death and heart transplantation for 10 years: 3.6% [2000-2010] versus 3.0% [2011-2022] P=0.520; all-cause death, heart transplantation, and cardiovascular hospitalization for 10 years: 11.7% [2000-2010] versus 19.8% [2011-2022], P=0.240). High body mass index (hazard ratio [HR], 1.106 [95% CI, 1.024-1.196]; P=0.011), the presence of gestational diabetes (HR, 5.346 [95% CI, 1.778-16.07]; P=0.002), and increased baseline left ventricular end-diastolic dimension (HR, 1.078 [95% CI, 1.002-1.159]; P=0.044) were significant risk factors for poor prognosis. CONCLUSIONS: While the incidence of PPCM has increased over the past 20 years, the prognosis has not improved significantly. Timely management and close follow-up are necessary for high-risk patients with PPCM with high body mass index, gestational diabetes, or large left ventricular end-diastolic dimension.

Asunto(s)

Cardiomiopatías , Periodo Periparto , Complicaciones Cardiovasculares del Embarazo , Sistema de Registros , Humanos , Femenino , Adulto , Embarazo , República de Corea/epidemiología , Cardiomiopatías/epidemiología , Cardiomiopatías/terapia , Cardiomiopatías/fisiopatología , Cardiomiopatías/mortalidad , Complicaciones Cardiovasculares del Embarazo/epidemiología , Complicaciones Cardiovasculares del Embarazo/terapia , Factores de Riesgo , Factores de Tiempo , Trasplante de Corazón/tendencias , Trasplante de Corazón/estadística & datos numéricos , Pronóstico , Función Ventricular Izquierda , Volumen Sistólico , Causas de Muerte/tendencias , Hospitalización/tendencias , Hospitalización/estadística & datos numéricos , Trastornos Puerperales/epidemiología , Trastornos Puerperales/terapia , Trastornos Puerperales/mortalidad , Trastornos Puerperales/fisiopatología , Estudios Retrospectivos , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/fisiopatología , Incidencia

RESUMEN

BACKGROUND: Cardiac amyloidosis (CA) is frequently found in older patients with aortic stenosis (AS). However, the prevalence of AS among patients with CA is unknown. The objective was to study the prevalence and prognostic impact of AS among patients with CA. METHODS AND RESULTS: We conducted a retrospective analysis of a prospective registry comprising 976 patients with native aortic valves who were confirmed with wild type transthyretin amyloid (ATTRwt), hereditary variant transthyretin amyloid (ATTRv), or immunoglobulin light-chain (AL) CA. CA patients' echocardiograms were re-analyzed focusing on the aortic valve. Multivariable Cox regression analysis was performed to assess the mortality risk associated with moderate or greater AS in ATTRwt CA. The crude prevalence of AS among patients with CA was 26% in ATTRwt, 8% in ATTRv, and 5% in AL. Compared with population-based controls, all types of CA had higher age- and sex-standardized rate ratios (SRRs) of having any degree of AS (AL: SRR, 2.62; 95% Confidence Interval (CI) [1.09-3.64]; ATTRv: SRR, 3.41; 95%CI [1.64-4.60]; ATTRwt: SRR, 10.8; 95%CI [5.25-14.53]). Compared with hospital controls, only ATTRwt had a higher SRR of having any degree of AS (AL: SRR, 0.97, 95%CI [0.56-1.14]; ATTRv: SRR, 1.27; 95%CI [0.85-1.44]; ATTRwt: SRR, 4.01; 95%CI [2.71-4.54]). Among patients with ATTRwt, moderate or greater AS was not associated with increased all-cause death after multivariable adjustment (hazard ratio, 0.71; 95%CI [0.42-1.19]; P=0.19). CONCLUSIONS: Among patients with CA, ATTRwt but not ATTRv or AL is associated with a higher prevalence of patients with AS compared with hospital controls without CA, even after adjusting for age and sex. In our population, having moderate or greater AS was not associated with a worse outcome in patients with ATTRwt.

Asunto(s)

Estenosis de la Válvula Aórtica , Cardiomiopatías , Sistema de Registros , Humanos , Masculino , Femenino , Prevalencia , Anciano , Estudios Retrospectivos , Estenosis de la Válvula Aórtica/epidemiología , Estenosis de la Válvula Aórtica/mortalidad , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Pronóstico , Cardiomiopatías/epidemiología , Cardiomiopatías/mortalidad , Anciano de 80 o más Años , Neuropatías Amiloides Familiares/epidemiología , Neuropatías Amiloides Familiares/mortalidad , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/genética , Neuropatías Amiloides Familiares/diagnóstico , Factores de Riesgo , Ecocardiografía , Persona de Mediana Edad , Amiloidosis/epidemiología , Amiloidosis/mortalidad , Amiloidosis/diagnóstico , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/epidemiología , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/mortalidad , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , Prealbúmina/genética , Válvula Aórtica/diagnóstico por imagen

RESUMEN

BACKGROUND: Chronic myocardial injury is a condition defined by stably elevated cardiac biomarkers without acute myocardial ischemia. Although studies from high-income countries have reported that chronic myocardial injury predicts adverse prognosis, there are no published data about the condition in sub-Saharan Africa. METHODS: Between November 2020 and January 2023, adult patients with chest pain or shortness of breath were recruited from an emergency department in Moshi, Tanzania. Medical history and point-of-care troponin T (cTnT) assays were obtained from participants; those whose initial and three-hour repeat cTnT values were abnormally elevated but within 11% of each other were defined as having chronic myocardial injury. Mortality was assessed thirty days following enrollment. RESULTS: Of 568 enrolled participants, 81 (14.3%) had chronic myocardial injury, 73 (12.9%) had acute myocardial injury, and 412 (72.5%) had undetectable cTnT values. Of participants with chronic myocardial injury, the mean (± sd) age was 61.5 (± 17.2) years, and the most common comorbidities were CKD (n = 65, 80%) and hypertension (n = 60, 74%). After adjusting for CKD, thirty-day mortality rates (38% vs. 36%, aOR 1.03, 95% CI: 0.52-2.03, p = 0.931) were similar between participants with chronic myocardial injury and those with acute myocardial injury, but significantly greater (38% vs. 13.6%, aOR 3.63, 95% CI: 1.98-6.65, p<0.001) among participants with chronic myocardial injury than those with undetectable cTnT values. CONCLUSION: In Tanzania, chronic myocardial injury is a poor prognostic indicator associated with high risk of short-term mortality. Clinicians practicing in this region should triage patients with stably elevated cTn levels in light of their increased risk.

Asunto(s)

Servicio de Urgencia en Hospital , Troponina T , Humanos , Masculino , Femenino , Tanzanía/epidemiología , Persona de Mediana Edad , Estudios Prospectivos , Troponina T/sangre , Anciano , Pronóstico , Adulto , Biomarcadores/sangre , Enfermedad Crónica , Cardiomiopatías/sangre , Cardiomiopatías/epidemiología , Cardiomiopatías/mortalidad

RESUMEN

BACKGROUND: Although tafamidis treatment improves prognosis in patients with wild-type transthyretin amyloid cardiomyopathy, an optimal surrogate marker monitoring its therapeutic effect remains unclear. This study investigated the association between changes in cardiac biomarkers, high-sensitivity cardiac troponin T (hs-cTnT) and B-type natriuretic peptide (BNP) during the first year after tafamidis treatment and clinical outcomes. METHODS AND RESULTS: In 101 patients with wild-type transthyretin amyloid cardiomyopathy receiving tafamidis at our institution, change in cardiac biomarkers from baseline to 1 year after tafamidis administration and its association with composite outcomes (composite of all-cause death and hospitalization attributable to heart failure) was assessed. During the follow-up period (median, 17 months), 16 (16%) patients experienced composite outcomes. The hs-cTnT level significantly decreased at 1 year after tafamidis treatment, unlike the BNP level. The frequencies of increased hs-cTnT and BNP levels were significantly higher in those with composite outcomes than in those without (44% versus 15%; P=0.01). Kaplan-Meier survival analysis showed that patients in whom both hs-cTnT and BNP levels increased at 1 year after tafamidis had a higher probability of composite outcomes compared with those with decreased hs-cTnT and BNP levels (log-rank P<0.01). Cox regression analysis identified increased hs-cTnT and BNP levels at 1 year after tafamidis administration as an independent predictor of higher cumulative risk of composite outcomes. CONCLUSIONS: Deterioration in cardiac biomarkers during the first year after tafamidis treatment predicted a worse prognosis, suggesting the utility of serial assessment of cardiac biomarkers for monitoring the therapeutic response to tafamidis in patients with wild-type transthyretin amyloid cardiomyopathy.

Asunto(s)

Neuropatías Amiloides Familiares , Benzoxazoles , Biomarcadores , Cardiomiopatías , Péptido Natriurético Encefálico , Troponina T , Humanos , Masculino , Femenino , Biomarcadores/sangre , Péptido Natriurético Encefálico/sangre , Anciano , Neuropatías Amiloides Familiares/sangre , Neuropatías Amiloides Familiares/tratamiento farmacológico , Neuropatías Amiloides Familiares/mortalidad , Neuropatías Amiloides Familiares/diagnóstico , Benzoxazoles/uso terapéutico , Troponina T/sangre , Cardiomiopatías/sangre , Cardiomiopatías/tratamiento farmacológico , Cardiomiopatías/mortalidad , Cardiomiopatías/diagnóstico , Resultado del Tratamiento , Factores de Tiempo , Persona de Mediana Edad , Anciano de 80 o más Años , Insuficiencia Cardíaca/sangre , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/mortalidad , Estudios Retrospectivos , Prealbúmina/metabolismo

RESUMEN

BACKGROUND: The 6-minute walk test (6MWT) represents a comprehensive functional assessment that is commonly used in patients with heart failure; however, data are lacking in patients with transthyretin cardiac amyloidosis (ATTR-CA). OBJECTIVES: This study aimed to assess the prognostic importance of the 6MWT in patients with ATTR-CA. METHODS: A retrospective analysis of patients diagnosed with ATTR-CA at the National Amyloidosis Centre who underwent a baseline 6MWT between 2011 and 2023 identified 2,141 patients, of whom 1,118 had follow-up at 1 year. RESULTS: The median baseline 6MWT distance was 347 m (Q1-Q3: 250-428 m) and analysis by quartiles demonstrated an increased death rate with each distance reduction (deaths per 100 person-years: 6.3 vs 9.2 vs 13.6 vs 19.0; log-rank P < 0.001). A 6MWT distance of <350 m was associated with a 2.2-fold higher risk of mortality (HR: 2.15; 95% CI: 1.85-2.50; P < 0.001), with a similar increased risk across National Amyloidosis Centre disease stages (P for interaction = 0.761) and genotypes (P for interaction = 0.172). An absolute (reduction of >35 m) and relative worsening (reduction of >5%) of 6MWT at 1 year was associated with an increased risk of mortality (HR: 1.80; 95% CI: 1.51-2.15; P < 0.001 and HR: 1.89; 95% CI: 1.59-2.24; P < 0.001, respectively), which was similar across the aforementioned subgroups. When combined with established measures of disease progression (N-terminal pro-B-type natriuretic peptide progression and outpatient diuretic intensification), each incremental increase in progression markers was associated with an increased death rate (deaths per 100 person-years: 7.6 vs 13.9 vs 22.4 vs 32.9; log-rank P < 0.001). CONCLUSIONS: The baseline 6MWT distance can refine risk stratification beyond traditional prognosticators. A worsening 6MWT distance can stratify disease progression and, when combined with established markers, identifies patients at the highest risk of mortality.

Asunto(s)

Neuropatías Amiloides Familiares , Cardiomiopatías , Prueba de Paso , Humanos , Masculino , Femenino , Estudios Retrospectivos , Pronóstico , Prueba de Paso/métodos , Anciano , Neuropatías Amiloides Familiares/mortalidad , Neuropatías Amiloides Familiares/fisiopatología , Neuropatías Amiloides Familiares/diagnóstico , Cardiomiopatías/fisiopatología , Cardiomiopatías/mortalidad , Cardiomiopatías/diagnóstico , Persona de Mediana Edad , Estudios de Seguimiento

RESUMEN

BACKGROUND: Risk stratification for patients with non-ischemic cardiomyopathy (NICM) remains challenging as previous studies predicting life-threatening ventricular arrhythmia (LTVA) events were conducted before the establishment of the current standard treatment. We investigated the prognostic value of non-sustained ventricular tachycardia (NSVT) in NICM patients among recent studies. METHODS: MEDLINE, Embase were searched from January 2000 to October 2023. The risk of NSVT on LTVA and mortality was assessed using a random-effects model for patients with NICM. A meta-regression analysis was employed to identify sources of heterogeneity. The systematic review and meta-analysis were carried out according to the PRISMA guidelines. RESULTS: A total of 18 studies were identified, including 5238 pooled participants. Meta-analysis demonstrated that the presence of NSVT was considered a significant prognostic indicator for LTVA events [hazard ratio (HR): 2.90; 95 % CI; 2.31-3.64] with low heterogeneity (I2: 19 %) and for mortality (HR; 2.28; 95%CI; 1.26-4.13) with high heterogeneity (I2: 69 %). The prognostic value of NSVT for LTVA was not affected by either ejection fraction or medications at baseline. CONCLUSION: NSVT remained an important predictor of LTVA events even in patients receiving healthcare in contemporary eras. Detection of NSVT helps us to identify the high-risk patients with NICM.

Asunto(s)

Cardiomiopatías , Taquicardia Ventricular , Humanos , Taquicardia Ventricular/etiología , Taquicardia Ventricular/mortalidad , Cardiomiopatías/complicaciones , Cardiomiopatías/etiología , Cardiomiopatías/mortalidad , Pronóstico , Medición de Riesgo

Asunto(s)

Cardiomiopatías , Insuficiencia Cardíaca , Taquicardia por Reentrada en el Nodo Atrioventricular , Humanos , Taquicardia por Reentrada en el Nodo Atrioventricular/fisiopatología , Taquicardia por Reentrada en el Nodo Atrioventricular/diagnóstico , Taquicardia por Reentrada en el Nodo Atrioventricular/complicaciones , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/complicaciones , Cardiomiopatías/fisiopatología , Cardiomiopatías/complicaciones , Cardiomiopatías/mortalidad , Factores de Riesgo , Masculino , Electrocardiografía

RESUMEN

INTRODUCTION: Septic shock is a severe form of sepsis that has a high mortality rate, and a substantial proportion of these patients will develop cardiac dysfunction, often termed septic cardiomyopathy (SCM). Some SCM patients may develop frank cardiac failure, termed sepsis-related cardiogenic shock (SeRCS). Little is known of SeRCS. This study describes baseline characteristics of patients with SCM and SeRCS compared to patients with septic shock without cardiac dysfunction. We compare clinical outcomes among SCM, SeRCS, and septic shock, and identify risk factors for the development of SCM and SeRCS. METHODS: Septic patients admitted to the ICU with an echocardiogram obtained within 72 hours were included. Left ventricular ejection fraction of ≤55% was used to define SCM, and cardiac index ≤2.1 L/min/m2 among patients with SCM defined SeRCS. Machine learning was used to identify risk factors for development of SCM and SeRCS. Logistic regression was used to compare mortality among groups. RESULTS: Among 1229 patients, 977 patients had septic shock without cardiac dysfunction, 207 had SCM, and 45 had SeRCS. In patients with septic shock, the strongest predictor for developing SCM and SeRCs was a prior history of cardiac dysfunction. Mortality did not significantly differ among the three groups. CONCLUSIONS: SCM and SeRCS affect a minority of patients with septic shock, disproportionately affecting individuals with a history of cardiac disease. We did not identify a mortality difference associated with SCM or SeRCS. Additional work is needed to define further subtypes and treatment options for this patient population.

Asunto(s)

Cardiomiopatías , Choque Cardiogénico , Choque Séptico , Humanos , Masculino , Femenino , Choque Cardiogénico/mortalidad , Choque Cardiogénico/complicaciones , Choque Cardiogénico/etiología , Anciano , Cardiomiopatías/mortalidad , Cardiomiopatías/complicaciones , Estudios Retrospectivos , Persona de Mediana Edad , Choque Séptico/mortalidad , Choque Séptico/complicaciones , Factores de Riesgo , Sepsis/mortalidad , Sepsis/complicaciones , Ecocardiografía , Anciano de 80 o más Años

RESUMEN

BACKGROUND AND AIMS: Patients with atrial fibrillation (AF) are at increased risks of cardiovascular diseases and mortality, but risks according to age at diagnosis have not been reported. This study investigated age-specific risks of outcomes among patients with AF and the background population. METHODS: This nationwide population-based cohort study included patients with AF and controls without outcomes by the application of exposure density matching on the basis of sex, year of birth, and index date. The absolute risks and hazard rates were stratified by age groups and assessed using competing risk survival analyses and Cox regression models, respectively. The expected differences in residual life years among participants were estimated. RESULTS: The study included 216 579 AF patients from year 2000 to 2020 and 866 316 controls. The mean follow-up time was 7.9 years. Comparing AF patients with matched controls, the hazard ratios among individuals ≤50 years was 8.90 [95% confidence interval (CI), 7.17-11.0] for cardiomyopathy, 8.64 (95% CI, 7.74-9.64) for heart failure, 2.18 (95% CI, 1.89-2.52) for ischaemic stroke, and 2.74 (95% CI, 2.53-2.96) for mortality. The expected average loss of life years among individuals ≤50 years was 9.2 years (95% CI, 9.0-9.3) years. The estimates decreased with older age. CONCLUSIONS: The findings show that earlier diagnosis of AF is associated with a higher hazard ratio of subsequent myocardial disease and shorter life expectancy. Further studies are needed to determine causality and whether AF could be used as a risk marker among particularly younger patients.

Asunto(s)

Fibrilación Atrial , Humanos , Fibrilación Atrial/mortalidad , Fibrilación Atrial/epidemiología , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico , Masculino , Femenino , Persona de Mediana Edad , Anciano , Adulto , Factores de Edad , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/epidemiología , Incidencia , Factores de Riesgo , Anciano de 80 o más Años , Cardiomiopatías/mortalidad , Cardiomiopatías/epidemiología , Cardiomiopatías/diagnóstico , Accidente Cerebrovascular Isquémico/epidemiología , Accidente Cerebrovascular Isquémico/mortalidad , Estudios de Casos y Controles

RESUMEN

AIMS: Primary prevention patients with ischaemic cardiomyopathy and chronic total occlusion of an infarct-related coronary artery (CTO) are at a particularly high risk of implantable cardioverter-defibrillator (ICD) therapy occurrence. The trial was designed to evaluate the efficacy of preventive CTO-related substrate ablation strategy in ischaemic cardiomyopathy patients undergoing primary prevention ICD implantation. METHODS AND RESULTS: The PREVENTIVE VT study was a prospective, multicentre, randomized trial including ischaemic patients with ejection fraction ≤40%, no documented ventricular arrhythmias (VAs), and evidence of scar related to the coronary CTO. Patients were randomly assigned 1:1 to a preventive substrate ablation before ICD implantation or standard therapy with ICD implantation only. The primary outcome was a composite of appropriate ICD therapy or unplanned hospitalization for VAs. Secondary outcomes included the primary outcome's components, the incidence of appropriate ICD therapies, cardiac hospitalization, electrical storm, and cardiovascular (CV) mortality. Sixty patients were included in the study. During the mean follow-up of 44.7 ± 20.7 months, the primary outcome occurred in 5 (16.7%) patients undergoing preventive substrate ablation and in 13 (43.3%) patients receiving only ICD [hazard ratio (HR): 0.33; 95% confidence interval (CI): 0.12-0.94; P = 0.037]. Patients in the preventive ablation group also had fewer appropriate ICD therapies (P = 0.039) and the electrical storms (Log-rank: P = 0.01). While preventive ablation also reduced cardiac hospitalizations (P = 0.006), it had no significant impact on CV mortality (P = 0.151). CONCLUSION: Preventive ablation of the coronary CTO-related substrate in patients undergoing primary ICD implantation is associated with the reduced risk of appropriate ICD therapy or unplanned hospitalization due to VAs.

Asunto(s)

Ablación por Catéter , Oclusión Coronaria , Desfibriladores Implantables , Isquemia Miocárdica , Prevención Primaria , Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Oclusión Coronaria/mortalidad , Oclusión Coronaria/terapia , Oclusión Coronaria/prevención & control , Oclusión Coronaria/complicaciones , Resultado del Tratamiento , Estudios Prospectivos , Isquemia Miocárdica/complicaciones , Isquemia Miocárdica/mortalidad , Taquicardia Ventricular/prevención & control , Taquicardia Ventricular/terapia , Taquicardia Ventricular/mortalidad , Cardiomiopatías/mortalidad , Cardiomiopatías/complicaciones , Cardiomiopatías/terapia , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiología , Factores de Riesgo , Cardioversión Eléctrica/instrumentación , Cardioversión Eléctrica/efectos adversos , Cardioversión Eléctrica/mortalidad , Infarto del Miocardio/mortalidad , Infarto del Miocardio/prevención & control , Infarto del Miocardio/complicaciones , Enfermedad Crónica , Factores de Tiempo

RESUMEN

AIMS: Predicting mortality in severe AL cardiac amyloidosis is challenging due to elevated biomarker levels and limited thresholds for stratifying severe cardiac damage. METHODS AND RESULTS: This prospective, observational, cohort study included de novo, confirmed cardiac AL amyloidosis patients at the Henri Mondor National Reference Centre. The goal was to identify predictors of mortality to enhance prognostic stratification and improve informed decision-making regarding therapy. Over the 12-year study period, among the 233 patients included, 133 were NYHA III-IV and 179 Mayo 2004 III. The independent predictors for mortality identified were hsTnT, NT-proBNP, cardiac output, and conjugated bilirubin. A novel prognostic, conditional stratification, Mondor amyloidosis cardiac staging (MACS) was developed with biomarker cut-off values for Stage 1: hsTnT ≤ 107 ng/L and NT-proBNP ≤ 3867 ng/L (n = 77; 33%); for stage 2 NT-proBNP > 3867 ng/L (n = 72; 30%). For stage 3, if troponin >107 ng/L, regardless of NT-proBNP then CB 4 µmol/L, was added (n = 41; 17.5%) and stage 4: CB > 4 µmol/L (n = 43; 18.5%). The median overall survival was 8 months 95% CI [2-24]. At 1 year, 102 (44%) patients died and the Kaplan-Meier median survival with MACS Stage 1 was not reached, while stage 2 was 15.2 months (95% CI [11-18]) and stage 3, 6.6 months (95% CI [1-13]). Notably, among European stage II patients, 17.1%, n = 8 were MACS stage 3 and European stage IIIb 21.4% (n = 23) were MACS stage 4. Importantly, among European stage IIIb patients 42.2% (n = 29) were classified MACS stage 4 and 12.5% n = 9 were only MACS stage 2. CONCLUSIONS: The Mondor prognostic staging system, including conjugate bilirubin may significantly improve prognostic stratification for patients with severe cardiac amyloidosis.

Asunto(s)

Cardiomiopatías , Humanos , Masculino , Femenino , Estudios Prospectivos , Pronóstico , Cardiomiopatías/sangre , Cardiomiopatías/mortalidad , Cardiomiopatías/diagnóstico , Anciano , Persona de Mediana Edad , Biomarcadores/sangre , Tasa de Supervivencia/tendencias , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/mortalidad , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/sangre , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/diagnóstico , Estudios de Seguimiento , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre