Incidencia y causas de hospitalización en pacientes con amiloidosis cardiaca por transtiretina (AC-ATTR) y por cadenas ligeras (AC-AL) / Incidence and causes of hospitalization in patients with transthyretin (ATTR-CA) and light chain (AL-CA) cardiac amyloidosis

Introducción y objetivos: La amiloidosis cardiaca (AC) es una patología asociada a un elevado número de ingresos hospitalarios. Dada la escasa información disponible al respecto, planteamos un análisis de la incidencia y las causas de hospitalización en esta enfermedad.Material y métodosSe evaluaron 143 pacientes (128 por transtiretina [AC-ATTR] y 15 por cadenas ligeras [AC-AL]) incluidos en el Registro de Amiloidosis Cardiaca de Galicia (AMIGAL), recogiendo todas sus hospitalizaciones.ResultadosDurante un seguimiento mediano de 959 días se produjeron 179 hospitalizaciones no programadas (tasa de incidencia [TI] 512,6 ingresos hospitalarios por 1.000 pacientes-año), siendo las más habituales las de causa cardiovascular (n=109, TI 312,2). El motivo individual de ingreso hospitalario más frecuente fue la insuficiencia cardiaca (IC) (n=87, TI 249,2).La AC-AL se asoció con una TI de hospitalizaciones no programadas más elevada que la AC-ATTR (TI 781 vs. 483,2; HR 1,62; p=0,029) a expensas de las de causa no cardiovascular (TI 376 vs. 181,2; HR 2,07; p=0,027). La supervivencia libre de hospitalización no programada al año y a los tres años en la AC-AL fue menor que en la AC-ATTR (46,7 y 20,0% vs. 73,4 y 35,2%, respectivamente; p=0,021). (AU)

Introduction and objetives: Cardiac amyloidosis (CA) is a disorder associated with high number of hospital admissions. Given the scarce information available, we propose an analysis of the incidence and causes of hospitalization in this disease.Material and methodsOne hundred and forty-three patients [128 by transthyretin (ATTR-CA) and 15 by light chains (AL-CA)] included in Registro de Amiloidosis Cardiaca de Galicia (AMIGAL) were evaluated, including all hospitalizations.ResultsDuring a median follow-up of 959 days there were 179 unscheduled hospitalizations [incidence rate (IR) 512.6 admissions per 1000 patients-year], most common due to cardiovascular reasons (n=109, IR 312.2). Most frequent individual cause of hospitalization was heart failure (n=87, TI 249.2).AL-CA was associated with a higher IR of unscheduled hospitalizations than ATTR-CA (IR 781 vs. 483.2; HR 1.62; p=0,029) due to non-cardiovascular admissions (IR 376 vs. 181.2; HR 2.07; p=0.027). Unscheduled admission-free survival at 1 and 3 years in AL-CA was inferior than in ATTR-CA (46.7% and 20.0% vs. 73.4% and 35.2%, respectively; p=0.021). (AU)

[Incidence and causes of hospitalization in patients with transthyretin (ATTR-CA) and light chain (AL-CA) cardiac amyloidosis]. / Incidencia y causas de hospitalización en pacientes con amiloidosis cardiaca por transtiretina (AC-ATTR) y por cadenas ligeras (AC-AL).

INTRODUCTION AND OBJETIVES: Cardiac amyloidosis (CA) is a disorder associated with high number of hospital admissions. Given the scarce information available, we propose an analysis of the incidence and causes of hospitalization in this disease. MATERIAL AND METHODS: One hundred and forty-three patients [128 by transthyretin (ATTR-CA) and 15 by light chains (AL-CA)] included in Registro de Amiloidosis Cardiaca de Galicia (AMIGAL) were evaluated, including all hospitalizations. RESULTS: During a median follow-up of 959 days there were 179 unscheduled hospitalizations [incidence rate (IR) 512.6 admissions per 1000 patients-year], most common due to cardiovascular reasons (n=109, IR 312.2). Most frequent individual cause of hospitalization was heart failure (n=87, TI 249.2). AL-CA was associated with a higher IR of unscheduled hospitalizations than ATTR-CA (IR 781 vs. 483.2; HR 1.62; p=0,029) due to non-cardiovascular admissions (IR 376 vs. 181.2; HR 2.07; p=0.027). Unscheduled admission-free survival at 1 and 3 years in AL-CA was inferior than in ATTR-CA (46.7% and 20.0% vs. 73.4% and 35.2%, respectively; p=0.021). CONCLUSIONS: CA was associated with high incidence of hospitalizations, being heart failure the most frequent individual cause; unscheduled admission-free survival in AL-CA was lower than in ATTR-CA due mostly to non-cardiovascular admissions.

Prognostic value of the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure ratio in cardiac amyloidosis.

INTRODUCTION AND OBJECTIVES: The tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/SPAP) ratio is a noninvasive surrogate of right ventricular to pulmonary circulation that has prognostic implications in patients with heart failure (HF) or pulmonary hypertension. Our purpose was to evaluate the prognostic value of the TAPSE/SPAP ratio in patients with cardiac amyloidosis. METHODS: We used the database of the AMIGAL study, a prospective, observational registry of patients with cardiac amyloidosis recruited in 7 hospitals of the Autonomous Community of Galicia, Spain, from January 1, 2018 to October 31, 2022. We selected patients whose baseline TAPSE/SPAP ratio was calculated with transthoracic echocardiography. Long-term survival and survival free of HF hospitalization were assessed by means of 5 different multivariable Cox regression models. Median follow-up was 680 days. RESULTS: We studied 233 patients with cardiac amyloidosis, among whom 209 (89.7%) had transthyretin type. The baseline TAPSE/SPAP ratio correlated significantly with clinical outcomes. Depending on the multivariable model considered, the adjusted hazard ratios estimated per 0.1mm/mmHg increase of baseline TAPSE/SPAP ratio ranged from 0.76 to 0.84 for all-cause mortality. Similarly, the ratios for all-cause mortality of HF hospitalization ranged from 0.79 to 0.84. The addition of the baseline TAPSE/SPAP ratio to the predictive model of the United Kingdom National Amyloidosis Centre resulted in an increase in Harrell's c-statistic from 0.662 to 0.705 for all-cause mortality and from 0.668 to 0.707 for all-cause mortality or HF hospitalization. CONCLUSIONS: Reduced TAPSE/SPAP ratio is an independent adverse prognostic marker in patients with cardiac amyloidosis.

Amiloidosis cardiaca: descripción de una serie de 143 casos / Cardiac amyloidosis: Description of a series of 143 cases

Introducción y objetivos: Recientemente se han producido importantes avances en el diagnóstico y tratamiento de la amiloidosis cardiaca (AC). Nos propusimos realizar una descripción actualizada de sus 2 tipos más frecuentes: la AC por transtirretina (AC-ATTR) y la AC por cadenas ligeras (AC-AL).MétodosRegistro prospectivo de pacientes diagnosticados de AC en 7 hospitales de Galicia entre el 1 de enero de 2018 y el 30 de junio de 2020. Se recogieron variables relativas a características clínicas, pruebas complementarias, supervivencia y causas de muerte.ResultadosSe incluyeron de forma consecutiva 143 pacientes con AC, 128 AC-ATTR (89,5%) y 15 AC-AL (10,5%). La edad media fue de 79,6±7,7 años y un 23,8% fueron mujeres. La mayoría de los pacientes con AC-ATTR se diagnosticaron de forma no invasiva (87,5%). En la exploración física, un 35,7, un 35 y un 7% de los pacientes presentaban el signo de Popeye, contractura de Dupuytren y macroglosia, respectivamente. La supervivencia a los 12 y 24 meses fue del 92,1 y el 76,2% en el grupo AC-ATTR, y del 78,6 y el 61,1% en el grupo AC-AL (p=0,152). La causa de muerte fue cardiovascular en el 80,8% de la cohorte.ConclusionesLa AC-ATTR puede ser diagnosticada en la mayoría de los casos de manera no invasiva y es la forma de AC más frecuente en la práctica clínica habitual. Además, parece observarse un aumento en la supervivencia a corto plazo de la AC que en parte podría deberse a los avances relacionados con su diagnóstico y tratamiento. (AU)

Introduction and objectives: Recently, there have been important advances in the diagnosis and treatment of cardiac amyloidosis (CA). Our aim was to provide an updated description of its 2 most frequent types: the transthyretin CA (ATTR-CA) and the light chain CA (AL-CA).MethodsProspective registry of patients with CA diagnosed in 7 institutions in Galicia (Spain) between January 1, 2018 and June 30, 2020. Variables related to clinical characteristics, complementary tests, survival and causes of death were collected.ResultsOne hundred and forty-three patients with CA were consecutively included, 128 ATTR-CA (89.5%) and 15 AL-CA (10.5%). Mean age was 79.6±7.7 years and 23.8% were women. Most patients with ATTR-CA were diagnosed non-invasively (87.5%). On physical examination, 35.7, 35 and 7% had Popeye's sign, Dupuytren's contracture and macroglossia, respectively. Twelve-month and 24-month survival was 92.1 and 76.2% in the ATTR-CA group, and 78.6 and 61.1% in the AL-CA group (P=.152). The cause of death was cardiovascular in 80.8% of the cohort.ConclusionsATTR-CA can be diagnosed non-invasively in most cases and it is the most common type of CA in routine clinical practice. Furthermore, an increase in the short-term survival of CA appears to be observed, which could be due to advances related to its diagnosis and treatment. (AU)

Syncope in patients with transthyretin amyloid cardiomyopathy: clinical features and outcomes.

BACKGROUND: We aimed to describe the clinical characteristics, underlying causes and outcomes of syncope in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). METHODS: The clinical profile and underlying causes of syncopal episodes were reviewed in a cohort of 128 patients with ATTR-CM enrolled from January 2018 to June 2020 in a prospective multicentre registry in 7 hospitals of Galicia (Spain). After enrollment, patients were followed during a median period of 520 days. The effect of syncope on all-cause mortality was assessed by means of multivariate Cox´s regression. RESULTS: Thirty (23.4%) patients had a history of previous syncope as a clinical antecedent before being enrolled in the prospective phase of the registry, and 4 (3.1%) experienced a first episode of syncope thereafter. The estimated incidence density rate of syncope during the prospective follow-up period after registry enrollment was 71.9 episodes per 1000 patients-year (95% Confidence Interval (CI) 32.8-111.1). The estimated overall prevalence of syncope was 26.6% (95% CI 18.9%-34.2%). Cardiac arrhythmias (n = 11, 32.3%), structural diseases of the heart or great vessels (n = 5, 14.7%), a neurally mediated reflex (n = 6, 17.6%), and orthostatic hypotension (n = 4, 11.8%) were identified as probable underlying causes of syncope; in 8 (23.6%) patients, syncope remained unexplained. Patients with syncope had increased non-adjusted all-cause mortality than patients without it (univariate hazard-ratio 3.37; 95% CI 1.43-7.94). When other independent predictors of survival were added to the survival model, this association was no longer statistically significant (multivariate hazard-ratio 1.81, 95% CI 0.67-4.84). CONCLUSIONS: Syncope is frequent in patients with ATTR-CM. This study could not demonstrate an independent association between syncope and mortality in those individuals.Abbreviations: ATTR-CM: Transthyretin amyloid cardiomyopathy; CI: Confidence Interval; HF: Heart Failure; HR: Hazard Ratio; IQR: Interquartile rank; LVEF: Left Ventricular Ejection Fraction; NTproBNP: N-terminal pro-brain natriuretic peptide; SD: Standard Deviation; 99mTc-DPD: technetium-99m-labeled 3,3-diphosphono-1,2-propanodicarboxylic acid.

Cardiac amyloidosis: Description of a series of 143 cases. / Amiloidosis cardiaca: descripción de una serie de 143 casos.

INTRODUCTION AND OBJECTIVES: Recently, there have been important advances in the diagnosis and treatment of cardiac amyloidosis (CA). Our aim was to provide an updated description of its 2 most frequent types: the transthyretin CA (ATTR-CA) and the light chain CA (AL-CA). METHODS: Prospective registry of patients with CA diagnosed in 7 institutions in Galicia (Spain) between January 1, 2018 and June 30, 2020. Variables related to clinical characteristics, complementary tests, survival and causes of death were collected. RESULTS: One hundred and forty-three patients with CA were consecutively included, 128 ATTR-CA (89.5%) and 15 AL-CA (10.5%). Mean age was 79.6±7.7 years and 23.8% were women. Most patients with ATTR-CA were diagnosed non-invasively (87.5%). On physical examination, 35.7, 35 and 7% had Popeye's sign, Dupuytren's contracture and macroglossia, respectively. Twelve-month and 24-month survival was 92.1 and 76.2% in the ATTR-CA group, and 78.6 and 61.1% in the AL-CA group (P=.152). The cause of death was cardiovascular in 80.8% of the cohort. CONCLUSIONS: ATTR-CA can be diagnosed non-invasively in most cases and it is the most common type of CA in routine clinical practice. Furthermore, an increase in the short-term survival of CA appears to be observed, which could be due to advances related to its diagnosis and treatment.

Beta-Blocker Exposure and Survival in Patients With Transthyretin Amyloid Cardiomyopathy.

OBJECTIVE: To investigate a potential association between beta-blocker exposure and survival in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). METHODS: In this real-world prospective registry of 128 consecutive patients with ATTR-CM recruited in 7 institutions in Galicia (Spain), survival of 65 patients who received beta blockers on registry enrollment was compared with that of 63 untreated controls by means of both unweighted Cox regression and Cox regression with inverse probability of treatment weighting. Tolerance to and adverse effects of beta blockers were recorded. Median study follow-up was 520 days. RESULTS: Patients with ATTR-CM who received beta blockers showed statistically significant lower all-cause mortality than untreated controls as evaluated by either unweighted Cox regression (hazard ratio, 0.31; 95% CI, 0.12 to 0.79) or Cox regression with inverse probability of treatment weighting (hazard ratio, 0.18; 95% CI, 0.08 to 0.41; P<.001). Several sensitivity analyses confirmed the internal validity of these results. The overall frequency of beta-blocker suspension due to adverse effects was 25% (95% CI, 15.5% to 34.5%). CONCLUSION: In this real-world, prospective, multi-institutional registry, patients with ATTR-CM who received beta blockers had lower all-cause mortality than untreated controls.

Differences in the Clinical Profile and Management of Atrial Fibrillation According to Gender. Results of the REgistro GallEgo Intercéntrico de Fibrilación Auricular (REGUEIFA) Trial.

To analyze the clinical profile and therapeutic strategy in atrial fibrillation (AF) according to gender in a contemporaneous patient cohort a prospective, multicenter observational study was performed on consecutive patients diagnosed with AF and assessed by cardiology units in the region of Galicia (Spain). A total of 1007 patients were included, of which 32.3% were women. The mean age of the women was significantly greater than that of the men (71.6 versus 65.7 years; p < 0.001), with a higher prevalence of hypertension (HTN) and valve disease. Women more often reported symptoms related to arrhythmia (28.2% in EHRA class I versus 36.4% in men), with a poorer level of symptoms (EHRA classes IIb and III). Thromboembolic risk was significantly higher among women (CHA2DS2-VASc 3 ± 1.3 versus 2 ± 1.5), in the same way as bleeding risk (HAS-BLED 0.83 ± 0.78 versus 0.64 ± 0.78) (p < 0.001), and women more often received anticoagulation therapy (94.1% versus 87.6%; p = 0.001). Rhythm control strategies proved significantly less frequent in women (55.8% versus 66.6%; p = 0.001), with a lesser electrical cardioversion (ECV) rate (18.4% versus 27.3%; p = 0.002). Perceived health status was poorer in women. Women were older and presented greater comorbidity than men, with a greater thromboembolic and bleeding risk. Likewise, rhythm control strategies were less frequent than in men, despite the fact that women had poorer perceived quality of life and were more symptomatic.

Cystatin C provides more information than other renal function parameters for stratifying risk in patients with acute coronary syndrome.

INTRODUCTION AND OBJECTIVES: The protein cystatin C has a stable plasma concentration and is eliminated exclusively by the kidneys. The aim of this study was to determine the prognostic value of cystatin C in patients with acute coronary syndrome (ACS). METHODS: The prospective study included 203 hospitalized ACS patients. Clinical evaluation during the first 24 hours of hospitalization included a hemogram and measurement of creatinine, cystatin C, total and fractionated cholesterol and markers of myocardial necrosis. The glomerular filtration rate (GFR) was estimated using the MDRD (Modification of Diet in Renal Disease) equation. A comparison was made between two groups of patients divided according to a serum cystatin-C level above or below 0.95 mg/L. The mean follow-up period was 151 days. RESULTS: In total, 90 patients (44.3%) had a cystatin-C level < or =0.95 mg/L and 113 (55.7%) had a level >0.95 mg/L. Those with a cystatin-C level >0.95 mg/L had poorer in-hospital outcomes, including more frequent heart failure (51.3% vs. 13.3%; P=.001) and higher in-hospital mortality (17.6% vs. 3.3%; P=.001), as well as higher mortality throughout follow-up (22.0% vs. 5.6%; P=.001). Multivariate analysis adjusted for age, ejection fraction and troponin-I and high-sensitivity C-reactive protein concentrations showed that cystatin C was the most powerful independent predictor of a cardiovascular event (relative risk=1.91; 95% confidence interval, 1.03-3.53). Patients with a GFR >60 mL/1.73 m(2) and a cystatin-C level >0.95 mg/L had higher in-hospital mortality (10.2% vs. 3.9%; P=.001). CONCLUSIONS: Measurement of cystatin C in high-risk ACS patients may be clinically useful for risk stratification during hospitalization, particularly in those with a normal GFR.

La cistatina C aporta más información que otros parámetros de función renal en la estratificación del riesgo de los pacientes con síndrome coronario agudo / Cystatin C provides more information than other renal function parameters for stratifying risk in patients with acute coronary syndrome

Introducción y objetivos. La cistatina C es una proteína con una concentración plasmática estable y eliminación exclusivamente renal. El objetivo del presente estudio es evaluar el valor pronóstico de la cistatina C en pacientes con síndrome coronario agudo. Métodos. Estudiamos prospectivamente a 203 pacientes ingresados por síndrome coronario agudo. Se realizó una determinación analítica a las 24 h del ingreso que incluía creatinina, cistatina C, hemograma, colesterol total y fraccionado y marcadores de necrosis miocárdica. Se estimó la tasa de filtrado glomerular mediante la ecuación MDRD. Se compararon dos grupos según las concentraciones séricas de cistatina C (> 0,95 y < 0,95 mg/l). Se llevó a cabo un seguimiento medio de 151 días. Resultados. Noventa (44,3%) pacientes tenían cistatina C ≤ 0,95 mg/l y 113 (55,7%), > 0,95 mg/l. Aquellos con cistatina C > 0,95 mg/l presentaron peor evolución hospitalaria con más insuficiencia cardiaca (el 51,3 frente al 13,3%; p = 0,001), mayor mortalidad hospitalaria (el 17,6 frente al 3,3%; p = 0,001) y durante el seguimiento (el 22 frente al 5,6%; p = 0,001). En un modelo multivariable ajustado por edad, fracción de eyección, tropo-nina I y proteína C reactiva ultrasensible, la cistatina C demostró ser el predictor independiente más potente de complicaciones cardiovasculares (RR = 1,91; intervalo de confianza del 95%, 1,03-3,53). Los pacientes con cistatina C > 0,95 y tasa de filtración > 60/ml/1,73 m2 presentaron mayor mortalidad hospitalaria (el 10,2 frente al 3,9%; p = 0,001). Conclusiones. La determinación de cistatina C en el síndrome coronario agudo de alto riesgo podría ser un buen elemento clínico en la estratificación de su riesgo durante la hospitalización, en particular en pacientes con filtrado glomerular normal (AU)

Introduction and Objectives. The protein cystatin C has a stable plasma concentration and is eliminated exclusively by the kidneys. The aim of this study was to determine the prognostic value of cystatin C in patients with acute coronary syndrome (ACS). Methods. The prospective study included 203 hospitalized ACS patients. Clinical evaluation during the first 24 hours of hospitalization included a hemogram and measurement of creatinine, cystatin C, total and fractionated cholesterol, and markers of myocardial necrosis. The glomerular filtration rate (GFR) was estimated using the MDRD (Modification of Diet in Renal Disease) equation. A comparison was made between 2 groups of patients divided according to a serum cystatin-C level above or below 0.95 mg/L. The mean follow-up period was 151 days. Results. In total, 90 patients (44.3%) had a cystatin-C level ≤0.95 mg/L and 113 (55.7%) had a level >0.95 mg/L. Those with a cystatin-C level >0.95 mg/L had poorer in-hospital outcomes, including more frequent heart failure (51.3% vs 13.3%; P=.001) and higher in-hospital mortality (17.6% vs 3.3%; P=.001), as well as higher mortality throughout follow-up (22.0% vs 5.6%; P=.001). Multivariate analysis adjusted for age, ejection fraction and troponin-I, and high-sensitivity C-reactive protein concentrations showed that cystatin C was the most powerful independent predictor of a cardiovascular event (relative risk =1.91; 95% confidence interval, 1.03-3.53). Patients with a GFR >60 mL/1.73 m2 and a cystatin-C level >0.95 mg/L had higher in-hospital mortality (10.2% vs 3.9%; P=.001). Conclusions. Measurement of cystatin C in high-risk ACS patients may be clinically useful for risk stratification during hospitalization, particularly in those with a normal GFR. cystatin-C level >0.95 mg/L had poorer in-hospital outcomes, including more frequent heart failure (51.3% vs. 13.3%; P=.001) and higher in-hospital mortality (17.6% vs. 3.3%; P=.001), as well as higher mortality throughout follow-up (22.0% vs. 5.6%; P=.001). Multivariate analysis adjusted for age, ejection fraction and troponin-I and high-sensitivity C-reactive protein concentrations showed that cystatin C was the most powerful independent predictor of a cardiovascular event (relative risk=1.91; 95% confidence interval, 1.03-3.53). Patients with a GFR >60 mL/1.73 m2 and a cystatin-C level >0.95 mg/L had higher in-hospital mortality (10.2% vs. 3.9%; P=.001). Conclusions. Measurement of cystatin C in high-risk ACS patients may be clinically useful for risk stratification during hospitalization, particularly in those with a normal GFR (AU)