Diagnostic performance of a clinical ultrasound-based algorithm for acute heart failure in patients presenting to the emergency department with dyspnea. / Rendimiento diagnóstico de un algoritmo basado en ecografía clínica para el diagnóstico de insuficiencia cardiaca aguda en pacientes que consultan en urgencias por disnea.

OBJECTIVES: To study the diagnostic performance of an ultrasound-based algorithm that includes the deceleration time (DT) of early mitral filling to establish a diagnosis of acute heart failure (AHF) in patients who come to an emergency department because of dyspnea. MATERIAL AND METHODS: Prospective analysis in a convenience sample of patients who came to a hospital emergency department with acute dyspnea. The algorithm included ultrasound findings and 4 echocardiographic findings as follows: mitral annular plane systolic excursion, Doppler mitral flow velocity, tissue Doppler imaging measure of the lateral annulus, and the DT of early mitral filling. The definitive diagnosis was made by 2 physicians blinded to each other's diagnosis and the ultrasound findings. RESULTS: A total of 166 adult patients with a mean (SD) age of 76 (13) years were included; 79 (48%) were women. AHF was the definitive diagnosis in 62 patients (37%). Diagnostic agreement was good between the 2 physicians (κ = 0.71). The algorithm classified all the patients, and there were no undetermined diagnoses. Diagnostic performance indicators for the ultrasound-based algorithm integrating early DT findings were as follows: area under the receiver operating characteristic curve, 0.91 (95% CI, 0.86-0.96); sensitivity, 87% (95% CI, 76%-94%); specificity, 95% (95% CI, 89%-98%); positive likelihood ratio, 18.1 (95% CI, 7.7-42.8); and negative likelihood ratio, 0.14 (95% CI, 0.07-0.26). CONCLUSION: The ultrasound-based algorithm integrating the DT of early mitral filling performs well for diagnosing AHF in emergency patients with dyspnea. The inclusion of early DT allows all patients to be diagnosed.

OBJETIVO: Analizar el rendimiento diagnóstico de un algoritmo ecográfico que incluye el tiempo de desaceleración precoz del flujo mitral (TD) para establecer el diagnóstico de insuficiencia cardiaca aguda (ICA) en pacientes que consultan en un servicio de urgencias hospitalario (SUH) por disnea. METODO: Análisis prospectivo de una muestra de conveniencia de pacientes que consultan por disnea aguda en un SUH. El algoritmo ecográfico incluyó la ecografía pulmonar y cuatro parámetros ecocardiográficos, se midió MAPSE (desplazamiento sistólico del plano del anillo mitral), medidas doppler de flujo mitral, medidas doppler tisular en el anillo mitral lateral y TD. El diagnóstico final fue asignado por 2 médicos ciegos entre sí y a los hallazgos ecográficos. RESULTADOS: Se incluyeron 166 pacientes adultos, la edad media fue de 76 años (DE 13) y 79 eran mujeres (48%). Hubo 62 pacientes (37%) con un diagnóstico final de ICA. La concordancia entre asignadores fue buena para el diagnóstico de ICA (κ = 0,71). El algoritmo clasificó a todos los pacientes, no hubo ningún diagnóstico indeterminado. El rendimiento diagnóstico del algoritmo mostró un área bajo la curva de 0,91 (IC 95%: 0,86-0,96), sensibilidad del 87% (IC 95%: 76%-94%), especificidad del 95% (IC 95%: 89%-98%), razón de verosimilitud positiva del 18,1 (IC 95%: 7,7-42,8), razón de verosimilitud negativa del 0,14 (IC 95%: 0,07-0,26). CONCLUSIONES: Un algoritmo ecográfico que incluye el TD tiene un buen rendimiento para el diagnóstico de ICA en pacientes que acuden a SUH por disnea. Además, el uso de TD permite clasificar a todos los pacientes.

Effect of SGLT2 inhibitors on anemia and their possible clinical implications.

Sodium-glucose cotransporter 2 inhibitors (SGLT2i) have demonstrated cardiovascular and renal benefits in patients with type 2 diabetes mellitus, heart failure, or chronic kidney disease. Since the first studies with these drugs, an initial increase in hemoglobin/hematocrit levels was observed, which was attributed to an increase in hemoconcentration associated with its diuretic effect, although it was early appearent that these drugs increased erythropoietin levels and erythropoiesis, and improved iron metabolism. Mediation studies found that the increase in hemoglobin was strongly associated with the cardiorenal benefits of these drugs. In this review, we discuss the mechanisms for improving erythropoiesis and the implication of the increase in hemoglobin on the cardiorenal prognostic benefit of these drugs.

Impact of hypochloremia as a prognostic factor in patients with heart failure, a retrospective cohort study.

BACKGROUND: some studies suggest that hypochloremia is a risk factor in the prognosis of heart failure (HF) in patients with recent decompensation. MATERIALS AND METHODS: retrospective cohort study of patients discharged due to HF decompensation who began follow-up in a specialized clinic. Two groups are defined: patients with hypochloremia (chloride < 98 mmol/L) and normochloremic patients (chloride > 98 mmol/L) in the initial assessment within the first month after discharge. The rate of intravenous diuretic rescue, emergency department visits, readmission for HF and cardiovascular (CV) death are compared using a Cox proportional hazards model. RESULTS: 165 patients were included (59% women, mean age 85 years), with 60 (36%) having hypochloremia. Both groups were comparable in terms of baseline characteristics, except for female sex, presence of peripheral artery disease, moderate-to-severe liver disease (more prevalent in the hypochloremia group), PROFUND index, and baseline furosemide dose (higher in patients with hypochloremia). The incidence of the primary event was higher in subjects with hypochloremia than in normochloremic subjects (HR: 1.59, 95% CI 0.97-2.62), mainly due to the need for intravenous diuretic rescue (HR: 1.86, 95% CI 1.07-3.24). CONCLUSIONS: hypochloremia following admission for HF decompensation is associated with a greater need for intravenous diuretic rescue therapy and probably worse overall prognosis across the spectrum of the disease, regardless of left ventricular ejection fraction (LVEF).

Efecto de los inhibidores del cotransportador sodio-glucosa tipo 2 sobre la anemia: posibles implicaciones clínicas / Effect of SGLT2 inhibitors on anemia and their possible clinical implications

Los inhibidores del cotransportador sodio-glucosa tipo 2 (iSGLT2) han demostrado su beneficio cardiovascular y renal en pacientes con diabetes mellitus tipo 2, insuficiencia cardiaca (IC) o enfermedad renal crónica (ERC). Desde los primeros estudios, con estos fármacos se objetivó un incremento inicial de los niveles de hemoglobina/hematocrito que se atribuyó a un aumento de la hemoconcentración asociados a su efecto diurético, aunque pronto se constató que aumentaban los niveles de eritropoyetina (EPO) y eritropoyesis, mejorando el metabolismo férrico. Los estudios de mediación objetivaron que el incremento de hemoglobina se asociaba estrechamente con los beneficios cardiorrenales de estas sustancias. En la presente revisión se discuten los mecanismos de mejora de la eritropoyesis y la implicación del aumento de hemoglobina sobre el beneficio pronóstico cardiorrenal de estos medicamentos.(AU)

Sodium-glucose cotransporter 2 inhibitors (SGLT2i) have demonstrated cardiovascular and renal benefits in patients with type 2 diabetes mellitus, heart failure, or chronic kidney disease. Since the first studies with these drugs, an initial increase in hemoglobin/hematocrit levels was observed, which was attributed to an increase in hemoconcentration associated with its diuretic effect, although it was soon seen that these drugs increased erythropoietin levels and erythropoiesis, and improved iron metabolism. Mediation studies found that the increase in hemoglobin was strongly associated with the cardiorenal benefits of these drugs. In this review, we discuss the mechanisms for improving erythropoiesis and the implication of the increase in hemoglobin on the cardiorenal prognostic benefit of these drugs.(AU)

Rendimiento diRendimiento diagnóstico de un algoritmo basado en ecografía clínica para el diagnóstico de insuficiencia cardiaca aguda en pacientes que consultan en urgencias por disnea / Diagnostic performance of a clinical ultrasound-based algorithm for acute heart failure in patients presenting to the emergency department with dyspnea

Objetivos. Analizar el rendimiento diagnóstico de un algoritmo ecográfico que incluye el tiempo de desaceleración precoz del flujo mitral (TD) para establecer el diagnóstico de insuficiencia cardiaca aguda (ICA) en pacientes que consultan en un servicio de urgencias hospitalario (SUH) por disnea. Métodos. Análisis prospectivo de una muestra de conveniencia de pacientes que consultan por disnea aguda en un SUH. El algoritmo ecográfico incluyó la ecografía pulmonar y cuatro parámetros ecocardiográficos, se midió MAPSE (desplazamiento sistólico del plano del anillo mitral), medidas doppler de flujo mitral, medidas doppler tisular en el anillo mitral lateral y TD. El diagnóstico final fue asignado por 2 médicos ciegos entre sí y a los hallazgos ecográficos. Resultados. Se incluyeron 166 pacientes adultos, la edad media fue de 76 años (DE 13) y 79 eran mujeres (48%). Hubo 62 pacientes (37%) con un diagnóstico final de ICA. La concordancia entre asignadores fue buena para el diagnóstico de ICA (κ = 0,71). El algoritmo clasificó a todos los pacientes, no hubo ningún diagnóstico indeterminado. El rendimiento diagnóstico del algoritmo mostró un área bajo la curva de 0,91 (IC 95%: 0,86-0,96), sensibilidad del 87% (IC 95%: 76%-94%), especificidad del 95% (IC 95%: 89%-98%), razón de verosimilitud positiva del 18,1 (IC 95%: 7,7-42,8), razón de verosimilitud negativa del 0,14 (IC 95%: 0,07-0,26). Conclusiones. Un algoritmo ecográfico que incluye el TD tiene un buen rendimiento para el diagnóstico de ICA en pacientes que acuden a SUH por disnea. Además, el uso de TD permite clasificar a todos los pacientes. (AU)

Objective. To study the diagnostic performance of an ultrasound-based algorithm that includes the deceleration time (DT) of early mitral filling to establish a diagnosis of acute heart failure (AHF) in patients who come to an emergency department because of dyspnea. Methods. Prospective analysis in a convenience sample of patients who came to a hospital emergency department with acute dyspnea. The algorithm included ultrasound findings and 4 echocardiographic findings as follows: mitral annular plane systolic excursion, Doppler mitral flow velocity, tissue Doppler imaging measure of the lateral annulus, and the DT of early mitral filling. The definitive diagnosis was made by 2 physicians blinded to each other’s diagnosis and the ultrasound findings. Results. A total of 166 adult patients with a mean (SD) age of 76 (13) years were included; 79 (48%) were women. AHF was the definitive diagnosis in 62 patients (37%). Diagnostic agreement was good between the 2 physicians (κ = 0.71). The algorithm classified all the patients, and there were no undetermined diagnoses. Diagnostic performance indicators for the ultrasound-based algorithm integrating early DT findings were as follows: area under the receiver operating characteristic curve, 0.91 (95% CI, 0.86-0.96); sensitivity, 87% (95% CI, 76%-94%); specificity, 95% (95% CI, 89%-98%); positive likelihood ratio, 18.1 (95% CI, 7.7-42.8); and negative likelihood ratio, 0.14 (95% CI, 0.07-0.26). Conclusions. The ultrasound-based algorithm integrating the DT of early mitral filling performs well for diagnosing AHF in emergency patients with dyspnea. The inclusion of early DT allows all patients to be diagnosed. (AU)

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)

Levosimendán ambulatorio repetitivo como puente al trasplante cardiaco / Repetitive ambulatory levosimendan as a bridge to heart transplantation

Introducción y objetivos:El levosimendán ambulatorio repetitivo es una opción como puente al trasplante cardiaco (TxC), aunque la evidencia sobre su eficacia y su seguridad es escasa. El objetivo del registro LEVO-T es describir a los pacientes en lista de TxC que reciben levosimendán, sus pautas y los eventos clínicos durante el seguimiento, en comparación con los que no lo reciben. Métodos: Se revisó en retrospectiva a los pacientes en lista de espera para TxC electivo de 14 centros españoles desde 2015 hasta 2020. Resultados: Se incluyó a 1.015 pacientes consecutivos; los 238 (23,4%) que recibieron levosimendán mostraron más ingresos por insuficiencia cardiaca (IC) el año anterior y peor perfil clínico. Las dosis fijas por necesidades clínicas fueron la pauta más frecuente. Dos pacientes (0,8%) presentaron arritmias ventriculares no mortales. No hubo diferencias en hospitalizaciones por IC entre los que comenzaron levosimendán en los primeros 30 días después de inclusión y los que no (el 33,6 frente al 34,5%; p=0,848). De estos últimos, 102 (32,9%) pasaron a levosimendán después de un ingreso por IC, y la tasa de ingresos por IC/mes varió de 0,57 antes del levosimendán a 0,21 después. El análisis mediante emparejamiento por puntuación de propensión no mostró diferencias entre los pacientes con y sin levosimendán en la supervivencia a 1 año tras la inclusión en lista (HR=1,03; IC95%, 0,36-2,97; p=0,958) ni en la supervivencia tras el TxC (HR=0,97; IC95%, 0,60-1,56; p=0,958). Conclusiones: El levosimendán ambulatorio repetitivo como puente al trasplante cardiaco es un tratamiento frecuente y seguro que podría reducir ingresos por IC. (AU)

Introduction and objectives: Repetitive ambulatory doses of levosimendan are an option as a bridge to heart transplantation (HT), but evidence regarding the safety and efficacy of this treatment is scarce. The objective of the LEVO-T Registry is to describe the profile of patients on the HT list receiving levosimendan, prescription patterns, and clinical outcomes compared with patients not on levosimendan. Methods: We retrospectively reviewed all patients listed for elective HT from 2015 to 2020 from 14 centers in Spain. Results: A total of 1015 consecutive patients were included, of whom 238 patients (23.4%) received levosimendan. Patients treated with levosimendan had more heart failure (HF) admissions in the previous year and a worse clinical profile. The most frequent prescription pattern were fixed doses triggered by the patients’ clinical needs. Nonfatal ventricular arrhythmias occurred in 2 patients (0.8%). No differences in HF hospitalizations were found between patients who started levosimendan in the first 30 days after listing and those who did not (33.6% vs 34.5%; P=.848). Among those who did not, 102 patients (32.9%) crossed over to levosimendan after an HF admission. These patients had a rate of 0.57 HF admissions per month before starting levosimendan and 0.21 afterwards. Propensity score matching analysis showed no differences in survival at 1 year after listing between patients receiving levosimendan and those who did not (HR, 1.03; 95%CI, 0.36-2.97; P=.958) or in survival after HT (HR, 0.97; 95%CI, 0.60-1.56; P=.958). Conclusions: Repetitive levosimendan in an ambulatory setting as a bridge to heart transplantation is commonly used, is safe, and may reduce HF hospitalizations. (AU)

Aspectos epidemiológicos, manejo clínico y resultados a corto plazo en pacientes mayores diagnosticados de insuficiencia cardiaca aguda en urgencias en España: resultados del estudio EDEN-34 / Epidemiological aspects, clinical management and short-term outcomes in elderly patients diagnosed with acute heart failure in the emergency department in Spain: results of the EDEN-34 study

Objetivo: Estimar la incidencia de diagnóstico de insuficiencia cardiaca aguda (ICA) en pacientes mayores en los servicios de urgencias (SU), la confirmación diagnóstica de la ICA en pacientes hospitalizados y los eventos adversos a corto plazo. Método: Se incluyeron a todos los pacientes de ≥65 años atendidos en 52 SU españoles durante una semana y se seleccionaron los diagnosticados de ICA. En los hospitalizados se recogieron los diagnosticados de ICA al alta. Como eventos adversos, se recogió la mortalidad intrahospitalaria y a 30 días, y evento adverso combinado (muerte u hospitalización) a 30 días posalta. Se calcularon las odds ratio (OR) ajustadas de las características demográficas, de estado basal y a la llegada al SU asociadas con mortalidad y evento adverso posalta a 30 días. Resultados: Se incluyeron 1.155 pacientes con ICA (incidencia anual: 26,5 por 1.000 habitantes ≥65 años, IC95%: 25,0-28,1). En el 86%, el diagnóstico de ICA constaba al alta. La mortalidad global a 30 días fue del 10,7%, la intrahospitalaria del 7,9% y el evento combinado posalta del 15,6%. La mortalidad intrahospitalaria y a 30 días se asoció con hipotensión arterial (OR ajustada: 74,0, IC95%: 5,39-1.015.; y 42,6, 3,74-485, respectivamente) e hipoxemia (2,14, 1,27-3,61; y 1,87, 1,19-2,93) a la llegada a urgencias y con precisar ayuda en la deambulación (2,24, 1,04-4,83; y 2,48, 1,27-4,86) y la edad (por cada incremento de 10 años; 1,54, 1,04-2,29, y 1,60, 1,13-2,28). Conclusiones: La ICA es un diagnóstico frecuente en los pacientes mayores que consultan en los SU. El deterioro funcional, la edad, la hipotensión e hipoxemia son los factores que más se asocian a mortalidad.(AU)

Objective: To estimate the incidence of acute heart failure (AHF) diagnosis in elderly patients in emergency departments (ED), diagnostic confirmation in hospitalized patients, and short-term adverse events. Methods: All patients aged ≥65 years attended in 52 Spanish EDs during 1 week were included and those diagnosed with AHF were selected. In hospitalized patients, those diagnosed with AHF at discharge were collected. As adverse events, in-hospital and 30-day mortality, and combined adverse event (death or hospitalization) at 30 days post-discharge were collected. Adjusted odds ratios (OR) for association of demographic variables, baseline status and constants at ED arrival with mortality and 30-day post-discharge adverse event were calculated. Results: We included 1,155 patients with AHF (annual incidence: 26.5 per 1000 inhabitants ≥65 years, 95%CI: 25.0-28.1). In 86% the diagnosis of AHF was known at discharge. Overall 30-day mortality was 10.7% and in-hospital mortality was 7.9%, and the combined event in 15.6%. In-hospital and 30-day mortality was associated with arterial hypotension (adjusted OR: 74.0, 95%CI: 5.39-1015. and 42.6, 3.74-485, respectively and hypoxemia (2.14, 1.27-3.61; and 1.87, 1.19-2.93) on arrival at the ED and requiring assistance with ambulation (2.24, 1.04-4.83; and 2.48, 1.27-4.86) and age (per 10-year increment; 1.54, 1.04-2.29; and 1.60, 1.13-2.28). The combined post-discharge adverse event was not associated with any characteristic. Conclusions: AHF is a frequent diagnosis in elderly patients consulting in the ED. The functional impairment, age, hypotension and hypoxemia are the factors most associated with mortality.(AU)

Clinical suspicion, diagnosis and management of cardiac amyloidosis: update document and executive summary.

In recent years, the interest in cardiac amyloidosis has grown exponentially. However, there is a need to improve our understanding of amyloidosis in order to optimise early detection systems. Therefore, it is crucial to incorporate solutions to improve the suspicion, diagnosis and follow-up of cardiac amyloidosis. In this sense, we designed a tool following the different phases to reach the diagnosis of cardiac amyloidosis, as well as an optimal follow-up: a) clinical suspicion, where the importance of the "red flags" to suspect it and activate the diagnostic process is highlighted; 2) diagnosis, where the diagnostic algorithm is mainly outlined; and 3) follow-up of confirmed patients. This is a practical resource that will be of great use to all professionals caring for patients with suspected or confirmed cardiac amyloidosis, to improve its early detection, as well as to optimise its accurate diagnosis and optimal follow-up.

Pregnancy in women with dilated cardiomyopathy genetic variants.

INTRODUCTION AND OBJECTIVES: Limited information is available on the safety of pregnancy in patients with genetic dilated cardiomyopathy (DCM) and in carriers of DCM-causing genetic variants without the DCM phenotype. We assessed cardiac, obstetric, and fetal or neonatal outcomes in this group of patients. METHODS: We studied 48 women carrying pathogenic or likely pathogenic DCM-associated variants (30 with DCM and 18 without DCM) who had 83 pregnancies. Adverse cardiac events were defined as heart failure (HF), sustained ventricular tachycardia, ventricular assist device implantation, heart transplant, and/or maternal cardiac death during pregnancy, or labor and delivery, and up to the sixth postpartum month. RESULTS: A total of 15 patients, all with DCM (31% of the total cohort and 50% of women with DCM) experienced adverse cardiac events. Obstetric and fetal or neonatal complications were observed in 14% of pregnancies (10 in DCM patients and 2 in genetic carriers). We analyzed the 30 women who had been evaluated before their first pregnancy (12 with overt DCM and 18 without the phenotype). Five of the 12 (42%) women with DCM had adverse cardiac events despite showing NYHA class I or II before pregnancy. Most of these women had a history of cardiac events before pregnancy (80%). Among the 18 women without phenotype, 3 (17%) developed DCM toward the end of pregnancy. CONCLUSIONS: Cardiac complications during pregnancy and postpartum were common in patients with genetic DCM and were primarily related to HF. Despite apparently good tolerance of pregnancy in unaffected genetic carriers, pregnancy may act as a trigger for DCM onset in a subset of these women.

Can catheter-based renal denervation reduce frequency of hospitalization in patients who have resistant hypertension and heart failure with reduced ejection fraction?

Hypertension is one of the most powerful and modifiable risk factors for the development, progression and even decompensation of heart failure. Uncontrolled hypertension increases to frequency of heart failure hospitalizations by increase sympathetic tone. Catheter-based renal denervation has been shown to reduce blood pressure in the treatment of multidrug-resistant hypertension. We report the improvement in clinical status after renal denervation in a 47-year-old male patient with a history of hypertension, chronic ischemic heart failure, and recurrent hospitalizations for acute hypertensive pulmonary edema despite optimal medical therapy.

Therapeutic management, adherence, and clinical outcomes of heart failure in Andalucía. ANDALIC Protocol. / Manejo terapéutico, adherencia y resultados clínicos de la insuficiencia cardiaca en Andalucía. Protocolo ANDALIC.

Heart failure is a prevalent syndrome with high mortality rates, representing a significant economic burden in terms of healthcare. The lack of systematic information about the treatment and adherence of patients with heart failure limits the understanding of these aspects and potentially the improvement of clinical outcomes. OBJECTIVE: To describe the clinical characteristics, therapeutic management, adherence, persistence and clinical results, as well as the association between these variables, in a cohort of patients with heart failure in Andalusia. DESIGN: This study will be an observational, population-based, retrospective cohort study. Data of patients discharged from an Andalusian hospital with a diagnosis of heart failure between 2014 and 2023 will be extracted from the Andalusian population health database. ANALYSIS: The statistical analysis will incorporate the following strategies: 1) Descriptive analysis of the characteristics of the population cohort, adherence measures, and clinical outcomes. 2) Bivariate analyses to study the association of covariates with adherence, persistence and clinical results. 3) Multivariate logistic regression and Cox regression analysis including relevant covariates. 4) To evaluate changes over time, multivariate Poisson regression models will be used. By conducting this comprehensive study, we aim to gain valuable insights into the clinical characteristics, treatment management, and adherence of heart failure patients in Andalusia, as well as to identify factors that may influence clinical outcomes. These findings could be critical both for the development of optimized strategies that improve medical care and quality of life of patients and for mitigating the health burden of HF in the region.

The association between pre-existing heart failure and cancer incidence: A systematic review and meta-analysis.

INTRODUCTION AND OBJECTIVES: Cardiovascular diseases (CVD) and cancer are some of the most recognized causes of mortality and morbidity worldwide. Cancer is the second leading cause of death in heart failure (HF) populations. Recent studies have hypothesized that HF might promote the development and progression of cancer. We aim to analyze and discuss the most recent evidence on the relationship between HF and cancer development. METHODS: From inception to November 2022, we searched PubMed, Web of Science and ClinicalTrials.gov for relevant articles on patients with HF and a subsequent cancer diagnosis that reported outcomes of overall and site-specific cancer incidence, or mortality. RESULTS: Of 2401 articles identified in our original search, 13 articles met our criteria. Studies reporting risk rate estimates were summarized qualitatively. Studies reporting hazard ratios (HRs), or relative risks were combined in a meta-analysis and revealed that HF was associated with an increased overall cancer incidence with a HR=1.30(95%CI:1.04-1.62) compared with individuals without HF. Subgroup analyses by cancer type revealed increased risk for lung cancer (HR=1.87;95%CI:1.28-2.73), gastrointestinal cancer (HR=1.22;95%CI:1.03-1.45), hematologic cancer (HR=1,60;95%CI:1.23-2.08) and female reproductive cancer (HR=1.67;95%CI:1,27-2.21). Mortality from cancer was higher in HF patients compared with non-HF subjects with a HR=2.17 (95%CI:1,23-3.84). CONCLUSIONS: Our systematic review and meta-analysis revealed that HF may result in a subsequent increase in cancer incidence as well as in cancer-related mortality. The most common cancer subtypes in HF patients were lung, female reproductive system, and hematologic cancers. Further research is needed to understand this association better and to provide the best cardiological and oncological care.

[Primary care and natriuretic peptides: design of a care process as a pathway to improve the diagnosis of heart failure]. / Atención primaria y péptidos natriuréticos: diseño de un proceso asistencial para la mejora en el diagnóstico de la insuficiencia cardiaca.

INTRODUCTION: There are few data about the optimal use of natriuretic peptides (NP) in the Primary Care (PC) setting. The aim to assess how, through a common coordinated PC-hospital care pathway, the use of NPs in patients with suspected heart failure (HF) is improved. MATERIAL AND METHODS: Analytical, experimental, prospective, non-randomized study. An intervention group composed of 22 PC physicians from 2 health centers is provided with face-to-face training and a consensual protocol is attached with a cut-off point of NT-proBNP> 300 pg/mL as pathological. The control group is made up of the rest of PC physicians in the healthcare area. The aim is to compare the use and results of PN in both groups. Propensity analysis is performed so thar the patient populations with requested PN are comparable. RESULTS: From June 2021 to March 2022, NP was requested in 103 and 105 patients in the intervention/control groups. Both populations were similar, with equal HF risk. Symptomatology was present in 100% of intervention vs 41% of asymptomatic patients in the control group (p <0.001). ECG was performed in 100% vs 33.3%, p <0.001. Optimal NP indication in 76.7% vs 29.5%, p <0.001. In the intervention group more patients with NT-proBNP> 300 pg/mL are referred to cardiology consultations (76.6% vs 27.2%, p 0.001). CONCLUSION: The optimal indication for NP and its interpretation as a diagnostic tool for HF, in the PC setting seems not to be appropriate, but improvable with a coordinated and multidisciplinary intervention approach.

A systematic review and meta-analysis of mortality in chronic Chagas cardiomyopathy versus other cardiomyopathies: higher risk or fiction?

INTRODUCTION AND OBJECTIVES: Although multiple studies suggest that chronic Chagas cardiomyopathy (CCC) has higher mortality than other cardiomyopathies, the absence of meta-analyses supporting this perspective limits the possibility of generating robust conclusions. The aim of this study was to systematically evaluate the current evidence on mortality risk in CCC compared with that of other cardiomyopathies. METHODS: PubMed/Medline and EMBASE were searched for studies comparing mortality risk between patients with CCC and those with other cardiomyopathies, including in the latter nonischemic cardiomyopathy (NICM), ischemic cardiomyopathy, and non-Chagas cardiomyopathy (nonCC). A random-effects meta-analysis was performed to combine the effects of the evaluated studies. RESULTS: A total of 37 studies evaluating 17 949 patients were included. Patients with CCC had a significantly higher mortality risk compared with patients with NICM (HR, 2.04; 95%CI, 1.60-2.60; I2, 47%; 8 studies) and non-CC (HR, 2.26; 95%CI, 1.65-3.10; I2, 71%; 11 studies), while no significant association was observed compared with patients with ischemic cardiomyopathy (HR, 1.72; 95%CI, 0.80-3.66; I2, 69%; 4 studies) in the adjusted-measures meta-analysis. CONCLUSIONS: Patients with CCC have an almost 2-fold increased mortality risk compared with individuals with heart failure secondary to other etiologies. This finding highlights the need for effective public policies and targeted research initiatives to optimally address the challenges of CCC.

Prevalence of moderate-severe aortic stenosis in patients with cardiac amyloidosis in a referral center.

BACKGROUND: Aortic stenosis (AS) is currently the most common valvular disease, with an estimated prevalence of over 4% in octogenarians. OBJECTIVE: To describe the prevalence of moderate-severe aortic stenosis (AS) in patients with wild type transthyretin amyloidosis (ATTRwt). Also, describe the clinical features, echocardiographic characteristics and clinical evolution. METHOD: Retrospective cohort of patients with diagnosis of ATTRwt, belonging to Hospital Italiano de Buenos Aires Institutional Amyloidosis Registry, from 30/11/2007 to 31/05/2021. Patients follow up was carried out through the institution clinical history. The prevalence of moderate-severe AE was estimated and presented as a percentage with its 95% confidence interval (95% CI). The characteristics were compared by groups according to whether or not they had moderate-severe AS. RESULTS: 104 patients with ATTRwt were included. Median follow up was 476 days [interquartile range: 192-749]. Moderate-severe AS prevalence at the ATTRwt time of diagnosis was 10.5% (n = 11; 95% CI: 5-18%). The median age of patients with AS moderate-severe at the time of diagnosis of ATTRwt was 86 years [78-91] and the male sex predominated (82%). Most of the patients had a history of heart failure (n = 8) and atrial fibrillation (n = 8) prior to the diagnosis of ATTRwt. Most of the patients were subclassified as low flow low gradient severe AS group (n = 7). Four patients underwent some intervention on the aortic valve. During follow-up, 5 patients (46%) were hospitalized for decompensated heart failure and 4 (36%) died. CONCLUSIONS: In our cohort, the coexistence of both pathologies had a similar prevalence as reported in the international literature. It was an elderly population with a high percentage of atrial fibrillation and history of heart failure. Most of the patients presented with severe AS with low flow low gradient.

ANTECEDENTES: La estenosis aórtica (EA) es actualmente la enfermedad valvular más frecuente, con una prevalencia estimada de más del 4 % en octogenarios. OBJETIVO: Describir la prevalencia de estenosis aórtica (EA) moderada-grave en pacientes con amiloidosis por transtiretina wild type (ATTRwt). Además, describir las características clínicas, ecocardiográficas y la evolución en este grupo de pacientes. MÉTODO: Estudio de cohorte retrospectiva de pacientes con diagnóstico de ATTRwt, pertenecientes al Registro Institucional de Amiloidosis del Hospital Italiano de Buenos Aires, en el periodo del 30/11/2007 al 31/05/2021. El seguimiento de los pacientes se realizó a través de la historia clínica electrónica de la institución. Se estimó la prevalencia de EA moderada-grave, que se presenta como porcentaje con su intervalo de confianza del 95% (IC 95%). Se compararon las características por grupos según tuvieran o no EA moderada-grave. RESULTADOS: Se incluyeron 104 pacientes con diagnóstico de ATTRwt. La mediana de seguimiento fue de 476 días [rango intercuartílico: 192-749]. La prevalencia de EA moderada-grave al momento del diagnóstico de ATTRwt fue del 10.5% (n = 11; IC95%: 5-18%). La mediana de edad de los pacientes con EA fue de 86 años [78-91] y predominó el sexo masculino (81.8%). La mayoría de los pacientes tenían el antecedente de insuficiencia cardiaca (n = 8) y fibrilación auricular (n = 8). Predominaron los pacientes con EA grave de bajo flujo y bajo gradiente (n = 7). Cuatro pacientes fueron sometidos a alguna intervención en la válvula aórtica. Durante el seguimiento, 5 pacientes (46%) tuvieron internaciones por insuficiencia cardiaca descompensada y 4 (36%) fallecieron. CONCLUSIONES: En nuestra cohorte, la coexistencia de ambas patologías tuvo una prevalencia similar a la reportada en la literatura internacional. Se trató de una población añosa con alto porcentaje de fibrilación auricular y antecedente de insuficiencia cardiaca. La mayoría presentaron EA grave de bajo flujo y bajo gradiente.

Response predictors to cardiac resynchronization therapy in chronic heart failure: a 10-year-cardiovascular center experience.

BACKGROUND: Cardiac resynchronization therapy (CRT) has been established as an effective therapy for heart failure with reduced ejection fraction. Randomized clinical trials have shown its impact on mortality and HF hospitalizations, as well as improvement of symptoms and quality of life. OBJECTIVES: Finding clinical, electrocardiographic, and echocardiographic variables that may predict the response to cardiac resynchronization therapy (CRT). METHODS: We performed a single-center, observational, analytic, and retrospective study that included 102 patients with heart failure (HF) diagnosis who underwent CRT according to guideline-directed therapy from January 2010 to April 2020 in a third-level center. CRT response was defined as an improvement of New York Heart Association functional class in at least 1 category associated with a recovery of ≥ 5% in the left ventricular ejection fraction (LVEF). RESULTS: Our study population was 102 patients of which 61 (59.8%) were men. The mean age at HF diagnosis was 54 ± 18.7 years. Ischemic heart disease was the etiology in 37 (36.3%) cases. Fifty-one (50%) patients were classified as responders. Responders had wider QRS, and lower LVEF and right ventricular fractional area change at baseline. After CRT, responders had a greater reduction of QRS duration, and improvement in LVEF, global longitudinal strain, and echocardiographic dyssynchrony parameters. Multivariate regression analysis showed that left bundle branch block (LBBB), left ventricular end-diastolic volume (LVEDV), tricuspid annular plane systolic excursion (TAPSE), and baseline difference of pre-ejection periods were predictors of a positive response to CRT in this population. CONCLUSIONS: LBBB, TAPSE, LVEDV, and pre-ejection time difference are independent variables that can predict adequate response to CRT.

ANTECEDENTES: La terapia de resincronización cardíaca (TRC) se ha establecido como una terapia efectiva para la insuficiencia cardíaca con fracción de eyección reducida. Ensayos clínicos aleatorizados han demostrado su impacto en la mortalidad y hospitalizaciones por insuficiencia cardíaca, así como la mejora de los síntomas y la calidad de vida. OBJETIVOS: Determinar las variables clínicas, electrocardiográficas y ecocardiográficas que puedan predecir la respuesta a la terapia de resincronización cardíaca (TRC). MÉTODO: Estudio unicéntrico, observacional, analítico, retrospectivo, que incluyó 102 pacientes con diagnóstico de IC sometidos a TRC y terapia dirigida por guías, de enero de 2010 a abril de 2020, en un centro de tercer nivel. La respuesta a TRC fue definida como mejoría de la clase funcional de la New York Heart Association en al menos 1 categoría, asociado con una recuperación ≥ 5% en la fracción de expulsión del ventrículo izquierdo (FEVI). RESULTADOS: Incluimos a 102 pacientes, 61 (59.8%) fueron hombres. El promedio de edad al diagnóstico de IC fue 54 ± 18.7 años. La cardiopatía isquémica fue la etiología en 37 (36.3%) pacientes. 51 (50%) pacientes, fueron clasificados como respondedores. Los respondedores presentaron QRS amplio, menor FEVI y menor fracción de acortamiento del ventrículo derecho al inicio del estudio. Después de la TRC, los respondedores tuvieron una mayor reducción en la duración del QRS, mejoría en la FEVI, strain longitudinal global y parámetros de disincronía ecocardiográfica. El análisis de regresión multivariado mostró que el bloqueo de rama izquierdo (BRI), el volumen telediastólico del ventrículo izquierdo (VTDVI) la excursión sistólica del plano anular tricuspídeo (TAPSE) y la diferencia basal del período expulsivo fueron predictores de respuesta positiva a TRC. CONCLUSIONES: BRI, TAPSE, VTDVI y la diferencia basal de períodos preexpulsivos son variables independientes que predicen respuesta adecuada a TRC.

Evaluation of vital signs through a mobile application in patients with heart failure: a opportunity for remote titration?

BACKGROUND: Virtual consultations have increased exponentially, but a limitation is the inability to assess vital signs (VS). This is particularly useful in patients with heart failure (HF) for titrating prognosis-modifying medication. This issue could potentially be addressed by a tool capable of measuring blood pressure (BP) and heart rate (HR) accurately, remotely, and conveniently. Mobile phones equipped with transdermal optical imaging technology could meet these requirements. OBJECTIVE: To evaluate the accuracy of a transdermal optical imaging-based app for estimating VS compared to clinical assessment in patients with HF. METHODS: A prospective cohort study included patients evaluated in an HF outpatient unit between February and April 2022. BP and HR were simultaneously assessed using the app and clinical examination (BP with an automated sphygmomanometer and HR by brachial palpation). Three measurements were taken by both the app and clinic for each patient, by two independent blinded physicians. RESULTS: Thirty patients were included, with 540 measurements of BP and HR. The mean age was 66 (± 13) years, 53.3% were male. The mean left ventricular ejection fraction was 37 ± 15, with 63.3% having previous hospitalizations for HF, and 63.4% in NYHA class II-III. The mean difference between the app measurement and its clinical reference measurement was 3.6 ± 0.5 mmHg for systolic BP (SBP), 0.9 ± -0.2 mmHg for diastolic BP (DBP), and 0.2 ± 0.4 bpm for HR. When averaging the paired mean differences for each patient, the mean across the 30 patients was 2 ± 6 mmHg for SBP, -0.14 ± 4.6 mmHg for DBP, and 0.23 ± 4 bpm for HR. CONCLUSION: The estimation of BP and HR by an app with transdermal optical imaging technology was comparable to non-invasive measurement in patients with HF and met the precision criteria for BP measurement in this preliminary study. The use of this new transdermal optical imaging technology provides promising data, which should be corroborated in larger cohorts.

ANTECEDENTES: Las consultas virtuales aumentaron exponencialmente, pero presentan como limitación la imposibilidad de valorar los signos vitales (SV), siendo especialmente útiles en los pacientes con insuficiencia cardiaca (IC) para titular medicación que modifica pronóstico. Este problema podría potencialmente solucionarse mediante una herramienta que pueda medir la presión arterial (PA) y frecuencia cardiaca (FC) de manera precisa, accesible y remota. Los teléfonos móviles equipados con tecnología de imágenes ópticas transdérmicas podrían cumplir con estos requisitos. OBJETIVO: Evaluar la precisión de una app basada en imagen óptica transdérmica para estimar SV en relación con la valoración clínica en pacientes con IC. MÉTODOS: Estudio de cohorte prospectivo, se incluyeron pacientes evaluados en una unidad ambulatoria de IC de febrero a abril del 2022. Se valoró simultáneamente la PA y FC mediante la app y el examen clínico (PA con un esfigmomanómetro automatizado y FC por palpación braquial). Se realizaron tres mediciones por app y clínica en cada paciente, por dos médicos independientes, encontrándose ciegos a los resultados. RESULTADOS: Se incluyeron 30 pacientes, con 540 mediciones de TA y de FC. Edad media de 66 (± 13) años, el 53.3% de sexo masculino. La fracción de eyección del ventrículo izquierdo media fue de 37 ± 15, con hospitalizaciones previas por IC el 63.3%, en CF II-III el 63.4%. La diferencia media entre la medición de la app y su medición de referencia clínica fue de 3.6 ± 0.5 mmHg para PA sistólica (PAS), 0.9 ± ­0.2 mmHg para PA diastólica (PAD) y 0.2 ± 0.4 lpm para FC. Cuando se promedian las diferencias medias emparejadas para cada paciente, la media entre los 30 pacientes es de 2 ± 6 mmHg para PAS, ­0.14 ± 4.6 mmHg para PAD y 0.23 ± 4 lpm para FC. CONCLUSIÓN: La estimación de PA y FC por una app con tecnología de imagen óptica transdérmica fue comparable a la medición no invasiva en pacientes con IC, y cumple los criterios de precisión de la medición de PA en este estudio preliminar. La utilización de esta nueva tecnología de imagen óptica transdérmica brinda datos prometedores, que deberán ser corroborados en cohortes de mayor tamaño.

Experience with the Kosmos ultrasonographic tool in the approach and treatment of ambulatory patients of a heart failure clinic: a single-center cross-sectional study.

BACKGROUND: In Mexico, the epidemiology of heart failure is still not well understood. However, it is known that the primary cause of hospital admissions in patients with heart failure is pulmonary and systemic congestion. OBJECTIVE: To estimate congestion status and assess cardiac function using portable ultrasound in patients with heart failure. METHOD: A cross-sectional observational study was conducted. Patients who attended the Heart Failure Clinic at the Ignacio Chávez National Cardiology Institute in Mexico City between May and August 2022 were selected. They underwent ultrasonographic evaluation using a portable device to assess pulmonary and systemic congestion, as well as cardiac function and structure. RESULTS: One-hundred patients diagnosed with heart failure were prospectively included during the study period; 76% were male, with an average age of 59 years (range: 50-68 years). The recorded LVEF median was 34% (IQR: 27-43.5%). When evaluating pulmonary congestion, 78% of the patients showed a pattern A and 22% a pattern B. Following the VExUS protocol, 92% of the patients were at grade 0, 2% at grade 1, and 6% at grade 2. CONCLUSIONS: The use of the portable ultrasound facilitated the quantitative characterization of the echocardiographic features of the studied population. This device could provide better clinical characterization which, in turn, might allow for optimized drug prescription for heart failure and dose adjustments of diuretics based on echocardiographic congestion findings.

ANTECEDENTES: En México aún es muy poco conocida la epidemiología de la insuficiencia cardiaca, sin embargo se sabe que la principal causa de ingresos hospitalarios en los pacientes con insuficiencia cardiaca es la congestión pulmonar y sistémica. OBJETIVO: Estimar el estado de congestión y evaluar la función cardiaca mediante el ultrasonido portátil en pacientes con insuficiencia cardiaca tratados en un centro de tercer nivel en México. MÉTODO: Se llevó a cabo un estudio observacional transversal. Se seleccionaron pacientes que acudieron a la Clínica de Insuficiencia Cardiaca del Instituto Nacional de Cardiología Ignacio Chávez en la Ciudad de México entre mayo y agosto de 2022. Se les sometió a una evaluación ultrasonográfica mediante un dispositivo portátil para valorar la congestión pulmonar y sistémica, así como la función y estructura cardiaca. RESULTADOS: Se incluyeron de forma prospectiva 100 pacientes diagnosticados con insuficiencia cardiaca en el periodo de estudio. El 76% fueron hombres, con una edad mediana de 59 años (RIQ: 50-68 años). La mediana del FEVI registrada fue del 34% (RIQ: 27.0-43.5%). Al evaluar la congestión pulmonar, el 78% de los pacientes presentaron un patrón A y el 22% un patrón B. Siguiendo el protocolo VExUS, el 92% de los pacientes mostraron un grado 0, el 2% un grado 1 y el 6% un grado 2. CONCLUSIONES: El uso del ultrasonido portátil facilitó la caracterización cuantitativa de las características ecocardiográficas de la población estudiada. Este dispositivo podría ofrecer una mejor caracterización clínica que, a su vez, permita una optimización en la prescripción de medicamentos para la insuficiencia cardiaca y el ajuste de dosis de diuréticos según los hallazgos ecocardiográficos de congestión.

Diferencias clínicas entre la amiloidosis cardiaca por transtirretina y la cardiopatía hipertensiva / Clinical differences between transthyretin cardiac amyloidosis and hypertensive heart disease

Introducción: Un porcentaje importante de pacientes finalmente diagnosticados de amiloidosis cardIaca por transtirretina (ATTR) fueron previamente diagnosticados de cardiopatía hipertensiva (CHTA), ya que ambas enfermedades suelen cursar con insuficiencia cardíaca (IC) con fracción de eyección preservada (ICFEp) e hipertrofia ventricular. Nuestros objetivos fueron evaluar las diferencias clínicas, electrocardiográficas y ecocardiográficas, y analizar si existe un pronóstico diferencial entre ambas entidades nosológicas. Material y métodos: Se incluyeron retrospectivamente todos los pacientes con CHTA a los que se solicitó una gammagrafía cardíaca con 99mTc-Difosfonatos (GDPD) y estudio de cadenas ligeras en sangre y orina para despistaje de ATTR en nuestro centro, en el periodo 2016-2021. Para el análisis, se excluyeron aquellos diagnosticados de otros tipos de amiloidosis. Resultados: Se analizaron un total de 72 pacientes: 33 fueron diagnosticados de ATTR y 39 de CHTA, finalmente. Los pacientes con ATTR presentaron mayores niveles de troponina I ultrasensible (TnI-US) y propéptido natriurético cerebral N-terminal (NT-ProBNP); en electrocardiograma (ECG) presentaron más frecuentemente patrón de seudoinfarto y alteraciones de la conducción; en ecocardiograma transtorácico (ETT) presentaron mayor grado de hipertrofia ventricular, disfunción ventricular izquierda y parámetros de peor función diastólica, con presiones de llenado más elevadas. En el seguimiento a 4 años, el grupo de ATTR mostró mayor necesidad de marcapasos (MCP), sin evidenciarse evidencias en cuanto a mortalidad, desarrollo de fibrilación auricular o más ingresos por IC. Conclusiones: En nuestra serie, los pacientes con ATTR presentaron diferencias clínicas, electrocardiográficas y ecocardiográficas respecto a aquellos con CHTA, con mayor riesgo necesidad de MCP en el seguimiento.(AU)

Introduction: A significant percentage of patients eventually diagnosed with cardiac transthyretin amyloidosis (TTRA) was previously diagnosed with hypertensive heart disease (HHD), since both conditions usually present with heart failure (HF) with preserved ejection fraction (HFpEF) and ventricular hypertrophy. Our objectives were to evaluate the clinical, electrocardiographic and echocardiographic differences, and to analyse whether there exists a differential prognosis between these two nosological entities. Materials and methods: We retrospectively included all patients with HHD for whom a cardiac scintigraphy with 99mTc-diphosphonate (GDPD) and a free light chains test in blood and urine were ordered for ATTR screening in our centre, in the period between 2016 and 2021. Those diagnosed with other types of amyloidosis were excluded from the analysis. Results: A total of 72 patients were analyzed: 33 were finally diagnosed with TTRA and 39 with CHTA. Patients with TTRA had higher levels of ultrasensitive troponin I (TnI-US) and N-terminal brain natriuretic propeptide (NT-ProBNP); in electrocardiography (ECG) they presented a pseudo-infarction pattern more frequently as well as conduction disturbances; in echocardiography (TTE) they presented a higher degree of ventricular hypertrophy, left ventricular dysfunction and worse diastolic function parameters, with elevated filling pressures. In the 4-year follow-up, the ATTR group showed greater need for pacemaker (PCM), with no evidence regarding mortality, development of atrial fibrillation (AF), or more admissions for heart failure (HF). Conclusions: In our series, patients with TTRA showed clinical, electrocardiographic and echocardiographic differences compared to patients with HHD, with increased risk of need for PCM.(AU)