Diagnostic and prognostic contribution of DPD scintigraphy in transthyretin V30M cardiac amyloidosis.

BACKGROUND: Early diagnosis and prognostic stratification of cardiac transthyretin amyloidosis are crucial. Although 99mTc 3,3-diphosphono-1,2-propanedicarboxylic acid (DPD) scintigraphy is the preferred method for the non-invasive diagnosis, its accuracy appears to be limited in transthyretin amyloidosis protein (ATTR) V30M mutation. Furthermore, its prognostic value in this mutation is unknown. This study investigated the diagnostic value of DPD scintigraphy to detect ATTR cardiomyopathy in V30M mutation and explored its prognostic value regarding mortality. METHODS: A total of 288 ATTR V30M mutation carriers (median age: 46 years; 49% males) without myocardial thickening (defined as septal thickness ≥13mm) attributable to other causes and who underwent DPD scintigraphy were enrolled. ATTR cardiomyopathy was defined by septal thickness ≥13mm and at least one of the criteria: late heart-to-mediastinum (H/M) 123I-metaiodobenzylguanidine (MIBG) uptake ratio <1.60; electrical heart disease or biopsy-documented amyloidosis. RESULTS: ATTR cardiomyopathy was identified in 41 (14.2%) patients and cardiac DPD uptake in 34 (11.8%). During a mean follow-up of 33.6 ± 1.2 months, 16 patients died (5.6%). Mortality was 14 times higher in patients with ATTR cardiomyopathy, 13 times higher in those with DPD uptake and 10 times higher in those with late H/M MIBG <1.60. The combined assessment of septal thickness and cardiac DPD uptake improved risk stratification: patients without septal thickening and without DPD retention had an excellent prognosis while those who presented either or both of them had a significantly worse prognosis, with 5-year mortality rates ranging from 39.9 to 53.3%. CONCLUSIONS: DPD scintigraphy is useful for prognostic stratification of ATTR V30M mutation carriers. Patients without septal thickening and no DPD uptake present the best prognosis compared to those with any signs of cardiac involvement.

conTemporary reflectiOns regarding heart failure manaGEmenT - How to ovERcome the PorTuguese barriers (TOGETHER-PT).

INTRODUCTION AND OBJECTIVES: Heart failure (HF) is a complex clinical syndrome that is a significant burden in hospitalisations, morbidity, and mortality. Although a significant effort has been made to better understand its consequences and current barriers in its management, there are still several gaps to address. The present work aimed to identify the views of a multidisciplinary group of health care professionals on HF awareness and literacy, diagnosis, treatment and organization of care, identifying current challenges and providing insights into the future. METHODS: A steering committee was established, including members of the Heart Failure Study Group of the Portuguese Society of Cardiology (GEIC-SPC), the Heart Failure Study Group of the Portuguese Society of Internal Medicine (NEIC-SPMI) and the Cardiovascular Study Group (GEsDCard) of the Portuguese Association of General and Family Medicine (APMGF). This steering committee produced a 16-statement questionnaire regarding different HF domains that was answered to by a diversified group of 152 cardiologists, internists, general practitioners, and nurses with an interest or dedicated to HF using a five-level Likert scale. Full agreement was defined as ≥80% of level 5 (fully agree) responses. RESULTS: Globally, consensus was achieved in all but one of the 16 statements. Full agreement was registered in seven statements, namely 3 of 4 statements for patient education and HF awareness and 2 in 4 statements of both HF diagnosis and healthcare organization, with proportions of fully agree responses ranging from 82.9% to 96.7%. None of the HF treatment statements registered full agreement but 3 of 4 achieved ≥80% of level 4 (agree) responses. CONCLUSION: This document aims to be a call-to-action to improve HF patients' quality of life and prognosis, by promoting a change in HF care in Portugal.

The value of multiparametric prediction scores in heart failure varies with the type of follow-up after discharge: a comparative analysis.

AIMS: Multiple prediction score models have been validated to predict major adverse events in patients with heart failure. However, these scores do not include variables related to the type of follow-up. This study aimed to evaluate the impact of a protocol-based follow-up programme of patients with heart failure regarding scores accuracy for predicting hospitalizations and mortality occurring during the first year after hospital discharge. METHODS AND RESULTS: Data from two heart failure populations were collected: one composed of patients included in a protocol-based follow-up programme after an index hospitalization for acute heart failure and a second one-the control group-composed of patients not included in a multidisciplinary HF management programme after discharge. For each patient, the risk of hospitalization and/or mortality within a period of 12 months after discharge was calculated using four different scores: BCN Bio-HF Calculator, COACH Risk Engine, MAGGIC Risk Calculator, and Seattle Heart Failure Model. The accuracy of each score was established using the area under the receiver operating characteristic curve (AUC), calibration graphs, and discordance calculation. AUC comparison was established by the DeLong method. The protocol-based follow-up programme group included 56 patients, and the control group, 106 patients, with no significant differences between groups (median age: 67 years vs. 68.4 years; male sex: 58% vs. 55%; median ejection fraction: 28.2% vs. 30.5%; functional class II: 60.7% vs. 56.2%, I: 30.4% vs. 31.9%; P = not significant). Hospitalization and mortality rates were significantly lower in the protocol-based follow-up programme group (21.4% vs. 54.7%; P < 0.001 and 5.4% vs. 17.9%; P < 0.001, respectively). When applied to the control group, COACH Risk Engine and BCN Bio-HF Calculator had, respectively, good (AUC: 0.835) and reasonable (AUC: 0.712) accuracy to predict hospitalization. There was a significant reduction of COACH Risk Engine accuracy (AUC: 0.572; P = 0.011) and a non-significant accuracy reduction of BCN Bio-HF Calculator (AUC: 0.536; P = 0.1) when applied to the protocol-based follow-up programme group. All scores showed good accuracy to predict 1 year mortality (AUC: 0.863, 0.87, 0.818, and 0.82, respectively) when applied to the control group. However, when applied to the protocol-based follow-up programme group, a significant predictive accuracy reduction of COACH Risk Engine, BCN Bio-HF Calculator, and MAGGIC Risk Calculator (AUC: 0.366, 0.642, and 0.277, P < 0.001, 0.002, and <0.001, respectively) was observed. Seattle Heart Failure Model had non-significant reduction in its acuity (AUC: 0.597; P = 0.24). CONCLUSIONS: The accuracy of the aforementioned scores to predict major events in patients with heart failure is significantly reduced when they are applied to patients included in a multidisciplinary heart failure management programme.

Effectiveness and Safety of Sacubitril/Valsartan in Patients with Chronic Kidney Disease-A Real-World Experience.

BACKGROUND: data regarding the effectiveness and safety of sacubitril/valsartan in heart failure and reduced ejection fraction (HFrEF) patients with chronic kidney disease (CKD) are scarse. OBJECTIVE: to evaluate the effectiveness and safety of sacubitril/valsartan in HFrEF and CKD in a real-world population. METHODS: we included consecutive ambulatory HFrEF patients that initiated sacubitril/valsartan between February 2017 and October 2020, stratified by CKD (KDIGO stage 5 excluded). PRIMARY OUTCOMES: the incidence rate per 100 patient-years and the annualized length of stay (LOS) of acute decompensated HF hospitalizations (HFH). SECONDARY OUTCOMES: all-cause mortality, NYHA improvement, and titration of sacubitril/valsartan. RESULTS: We included 179 patients, 77 with CKD, those being older (72 ± 10 vs. 65 ± 12 years, p < 0.001), had higher NT-proBNP (4623 ± 5266 vs. 1901 ± 1835 pg/mL, p < 0.001), and high anaemia incidence (p < 0.001). After 19 ± 11 months, a significant reduction in HFH adjusted incidence rate (57.5% decrease in CKD vs. 74.6%, p = 0.261) was observed, with 5 days there was a reduction in annualized LOS in both groups (p = 0.319). NYHA improved similarly in both groups (p = 0.670). CKD patients presented non-significant higher all-cause mortality (HR = 2.405, 95%CI: [0.841; 6.879], p = 0.102). Both groups had similar sacubitril/valsartan maximum dose achievement and drug withdrawal. CONCLUSION: sacubitril/valsartan was effective on reducing HFH and LOS without affecting all-cause mortality in a CKD real-world population.

Effectiveness of the contrast technique as recovery after effort according to professional athletes / Eficácia da técnica de contraste como recuperação pós-esforço segundo atletas profissionais

Abstract Introduction: Alterations caused in the metabolism of those who practice physical exercise regularly generate health benefits, however, in athletes, these alterations can cause muscle damage, so post-recovery recovery methods are extremely important for their physiological maintenance. Objective: To analyze the effectiveness of the contrast therapy technique with post-exercise recovery according to professional athletes. Methods: A questionnaire was applied that addressed the technical scientific knowledge of professional athletes over 18 years of age, of both sexes, about post-exerciseffort recovery techniques (PERT), personal information, and ethical issues, on an online platform. Descriptive statistical analysis was performed, with values presented in percentages and an association through the Chi-Square test on the knowledge of PERT and other independent variables. Results: In total, 63 athletes, 15 women and 48 men, answered the online questionnaire, 71.4% were between 18 and 30 years old, including swimmers, footballers, and athletics practitioners, and 85.7% had more than three years' experience in the sport and 57.1% competed at an international level. Considering the main PERTs, 92.1% were aware, 58.7% knew more than four, 96.8% frequently used at least one PERT, and 65.1% had used it for more than three years. Knowledge of PERTs is associated with age (p = 0.001), education (p = 0.001), practice time (p = 0.001), hours of daily training (0.001), and competitive level (p = 0.03). With respect to the use of the contrast technique, 36.5% of the respondents had already used it, and 34.9% found it effective. Conclusion: The athletes who used the contrast technique reported a good perception of recovery.

Resumo Introdução: Alterações causadas no metabolismo de quem pratica exercício físico constantemente geram benefícios à saúde. Em atletas, porém, causam danos musculares e por isso métodos de recuperação pós-esforço são de extrema importância para a manutenção fisiológica de atletas. Objetivo: Analisar a eficácia da técnica de contraste com recuperação pós-esforço segundo atletas profissionais. Métodos: Aplicou-se um questionário online que abordou o conhecimento técnico científico de atletas profissionais acima de 18 anos, de ambos os sexos, sobre técnicas de recuperação pós-esforço (TRPE), informações pessoais e questões éticas. Realizou-se análise estatística descritiva, onde os valores foram apresentados em percentuais, e associação através do teste qui-quadrado sobre o conhecimento das TRPE e demais variáveis independentes. Resultados: Responderam ao questionário online 63 atletas, 15 mulheres e 48 homens, sendo que 71,4% tinham entre 18 e 30 anos de idade. Estes eram nadadores, futebolistas e praticantes de atletismo; 85,7% possuíam experiência superior a três anos na modalidade e 57,1% competiam em nível internacional. Sobre as principais TRPE, 92,1% tinham conhecimento, 58,7% conheciam mais de quatro, 96,8% usavam com frequência ao menos uma TRPE e 65,1% utilizavam a mais de três anos. O conhecimento de TRPE está associado à idade (p = 0,001), escolaridade (p = 0,001), tempo de prática (p = 0,001), horas de treino diário (0,001) e nível competitivo (p = 0,03). Quanto ao uso da técnica de contraste, 36,5% dos respondentes já a haviam utilizado e, destes, 34,9% a consideram efetiva. Conclusão: Os atletas que utilizaram a técnica de contraste relataram boa percepção de recuperação.

Acute coronay syndrome in a patient with severe hemophilia A: Dificult decisions.

Hemophilia A is an inherited coagulation disease characterized by factor VIII (FVIII) deficiency and is associated with high hemorrhagic risk, especially in its severe forms. As the average life expectancy of patients with hemophilia has increased, so has the prevalence of acute coronary events. There is however limited experience in dealing with them. The strategy of acting on acute coronary events in patients with hemophilia, as demonstrated in the present case, is a real challenge, not only due to the need for antiplatelet therapy (which is essential in the prevention of stent thrombosis, but increases hemorrhagic risk), but also due to the lack of specific recommendations related to the most adequate and safe replacement therapy in these situations. The authors describe the case of a 48-year-old man with unstable angina and a previous diagnosis of severe hemophilia A who underwent percutaneous coronary intervention under FVIII therapy without hemorrhagic complications.

Heart and brain interactions in heart failure: Cognition, depression, anxiety, and related outcomes.

BACKGROUND: Cognitive impairment, anxiety and depression are common in heart failure (HF) patients and its evolution is not fully understood. OBJECTIVES: To assess the cognitive status of HF patients over time, its relation to anxiety and depression, and its prognostic impact. METHODS: Prospective, longitudinal, single center study including patients enrolled in a structured program for follow-up after hospital admission for HF decompensation. Cognitive function, anxiety/depression state, HF-related quality of life (QoL) were assessed before discharge and during follow-up (between 6th and 12th month) using Montreal Cognitive Assessment (MoCA), Hospital Anxiety and Depression Scale (HADS) and Kansas City Cardiomyopathy Questionnaire, respectively. HF related outcomes were all cause readmissions, HF readmissions and the composite endpoint of all-cause readmissions or death. RESULTS: 43 patients included (67±11.3 years, 69% male); followed-up for 8.2±2.1 months. 25.6% had an abnormal MoCA score that remained stable during follow-up (22.6±4.2 vs. 22.2±5.5; p=NS). MoCA score <22 at discharge conferred a sixfold greater risk of HF readmission [HR=6.42 (1.26-32.61); p=0.025], also predicting all-cause readmissions [HR=4.00 (1.15-13.95); p=0.03] and death or all-cause readmissions [HR=4.63 (1.37-15.67); p=0.014]. Patients with higher MoCA score showed a greater ability to deal with their disease (p=0.038). At discharge, 14% and 18.6% had an abnormal HADS score for depression and anxiety, respectively, which remained stable during follow-up and was not related to MoCA. CONCLUSIONS: Cognitive function, anxiety and depressive status remain stable in HF patients despite optimized HF therapy. Cognitive status shall be routinely screened to adopt attitudes that improve management as it has an impact on HF-related QoL and prognosis.

Non-invasive telemonitoring improves outcomes in heart failure with reduced ejection fraction: a study in high-risk patients.

AIMS: Non-invasive telemonitoring (TM) in patients with heart failure (HF) and reduced left ventricular ejection fraction (HFrEF) may be useful in the early diagnosis of HF decompensation, allowing therapeutic optimization and avoiding re-hospitalization. We describe a TM programme in this population and evaluate its effectiveness during a 12 month period. METHODS AND RESULTS: We conducted a single-centre study of patients discharged from hospital after decompensated HF, allocated into three groups: prospective TM programme, prospective HF protocol follow-up programme (PFP) with no TM facilities, and retrospective propensity-matched usual care (UC). TM effectiveness was assessed by all-cause hospitalizations and mortality; HF-related hospitalization (HFH), days lost to unplanned hospital admissions/death, functional capacity and quality of life (New York Heart Association, Kansas City Cardiomyopathy Questionnaire, 6 min walk test, and plasma N-terminal pro-brain natriuretic peptide) were also evaluated. A total of 125 patients were included [65.9 ± 11.9 years, 32% female, left ventricular ejection fraction 27% (21-32)]. TM was similar to PFP regarding effectiveness; TM reduced all-cause hospitalization and mortality (HR 0.27; 95% CI 0.11-0.71; P < 0.01) and HFH (HR 0.29; 95% CI 0.10-0.89; P < 0.05) as compared with UC. TM reduced the average number of days lost due to unplanned hospital admissions or all-cause death as compared with PFP (5.6 vs. 12.4 days, P < 0.05) and UC (5.6 vs. 48.8 days, P < 0.01). Impact on quality of life was similar between TM and PFP (P = 0.36). CONCLUSIONS: In patients with HFrEF and recent HF hospitalization, non-invasive TM reduced 12 month all-cause hospitalization/mortality and HFH as compared with usual care. TM also reduced the number of days lost due to unplanned hospital admission/death as compared with either an optimized protocol-based follow-up programme or usual care.

The sensitivity of DPD scintigraphy to detect transthyretin cardiac amyloidosis in V30M mutation depends on the phenotypic expression of the disease.

Background: There is a growing need for a non-invasive test to detect cardiac involvement in patients with transthyretin-related hereditary amyloidosis (ATTR) caused by V30M mutation. 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy is a promising method, but its accuracy in this particular mutation remains unknown.Methods: A cohort of 179 patients: 92 with early-onset disease (EoD, symptoms <50-years-old), 33 with late-onset disease (LoD) and 54 asymptomatic carriers were prospectively evaluated and underwent DPD scintigraphy, which was compared with the results of echocardiogram, ambulatory blood pressure monitoring, 24 h-Holter, myocardial 123I-metaiodobenzylguanidine imaging and NT-proBNP.Results: Amyloid cardiomyopathy, defined as septal thickness ≥13 mm, was present in 32 patients (17.9%) and was more frequent in those with LoD (OR: 3.68, p = .003). Cardiac DPD uptake was present in 22 individuals (12.3%) and correlated with parameters indicative of cardiac amyloidosis. DPD imaging was strongly influenced by the age of disease onset: among patients with myocardial thickening, cardiac DPD retention was present in 11/15 (73.3%) with LoD, in contrast to only 4/17 (26.7%) with EoD (p = .005). Two patients with myocardial thickening and normal DPD scintigraphy underwent endomyocardial biopsy that confirmed ATTR amyloidosis.Conclusion: DPD scintigraphy presents suboptimal sensitivity to detect cardiac involvement in ATTRV30M, particularly in symptomatic patients with EoD.

Accuracy and utility of a pacemaker respiratory monitoring algorithm for the detection of obstructive sleep apnea in patients with atrial fibrillation.

INTRODUCTION: The usefulness and diagnostic value of new-generation pacemakers (PM) with enhanced monitoring capabilities are not yet clearly established. The aim of this study was to evaluate the diagnostic utility and accuracy of a PM-incorporated respiratory monitoring algorithm and its interaction with atrial fibrillation (AF). METHODS: A single-center prospective study was performed in consecutive patients who underwent PM implantation featuring a respiratory monitoring algorithm. All patients had polysomnography recording. The respiratory disturbance index of the polysomnography and pacemaker (RDI-PM) were recorded on the same night. Occurrence and burden of AF were also recorded. The diagnostic utility of RDI-PM and its interaction with AF were evaluated. RESULTS: A total of 81 patients were included (age 73 ± 11 years). Obstructive sleep apnea syndrome (OSAS) was diagnosed in 62%. RDI-PM had good diagnostic accuracy for OSAS (area under the curve: 0.767 [95% CI: 0.65-0.88]; p < 0.001), with an ideal diagnostic cut-off of 13.3 (sensitivity 78%; specificity 78%) and 90% sensitivity for the diagnosis of moderate-to-severe OSAS. Time to AF first episode and total AF burden were not significantly different between patients with and without OSAS. However, in those whose OSAS diagnosis was based on RDI-PM, there was a significantly greater AF burden in patients with vs without OSAS (cut-off ≥13, 488 vs 83 min, p = 0.05). In patients with AF, the RDI-PM cut-off of 13.3 decreased specificity (57%) vs the general population, but in patients without AF the specificity was 100% and sensitivity 77%. CONCLUSION: OSAS was prevalent in PM patients. RDI-PM diagnosed OSAS accurately, with high sensitivity for the detection of moderate-to-severe OSAS, making it a suitable screening method. AF, however, significantly decreased the specificity of RDI-PM for OSAS diagnosis.

Protocol-based follow-up program for heart failure patients: Impact on prognosis and quality of life.

INTRODUCTION: Heart failure is associated with high rates of readmission and mortality, and there is a need for measures to improve outcomes. This study aims to assess the impact of the implementation of a protocol-based follow-up program for heart failure patients on readmission and mortality rates and quality of life. METHODS: A quasi-experimental study was performed, with a prospective registry of 50 consecutive patients discharged after hospitalization for acute heart failure. The study group was followed by a cardiologist at days 7-10 and the first, third, sixth and 12th month after discharge, with predefined procedures. The control group consisted of patients hospitalized for heart failure prior to implementation of the program and followed on a routine basis. RESULTS: No significant differences were observed between the two groups regarding mean age (67.1±11.2 vs. 65.8±13.4 years, p=0.5), NYHA functional class (p=0.37), or median left ventricular ejection fraction (27% [19.8-35.3] vs. 29% [23.5-40]; p=0.23) at discharge. Mean follow-up after discharge was similar (11±5.3 vs. 10.9±5.5 months, p=0.81). The protocol-based follow-up program was associated with a significant reduction in all-cause readmission (26% vs. 60%, p=0.003), heart failure readmission (16% vs. 36%, p=0.032), and mortality (4% vs. 20%, p=0.044). In the study group there was a significant improvement in all quality of life measures (p<0.001). CONCLUSION: A protocol-based follow-up program for patients with heart failure led to a significant reduction in readmission and mortality rates, and was associated with better quality of life.