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INTRODUCTION AND OBJECTIVES: Our aim was to describe the characteristics and outcomes of heart transplants in Spain. METHODS: We analyzed trends in recipient and donor characteristics, recipient-donor interaction, surgical procedures, immunosuppression, and outcomes of patients included in the Spanish heart transplant registry from 2014 to 2023. Changes in survival were analyzed using the Kaplan-Meier method. RESULTS: In 2023, 325 cardiac transplants were performed (4.5% more than in the previous year), with a total of 2987 procedures from 2014 to 2023. There was a trend toward performing more transplants in women (29.2%), with etiologies other than cardiomyopathy (32.6%), and with better pretransplant status (less hepatic [12.5%], renal [glomerular filtration rate, 81.5mL/min/1.73 m2], and respiratory [8.7%] involvement). In 2023, the number of urgent transplants increased (44% of the total), especially those performed after circulatory support with extracorporeal membrane oxygenation (36% of total assistance), and transplants performed with donation after circulatory death (17.9%). Survival improved in the triennium from 2020 to 2022 compared with 2014 to 2016 (83.0% at 1 year from 2020-2022 vs 79.0% from 2014-2016). CONCLUSIONS: The number of transplants performed in Spain showed an upward trend, with recipients with better clinical status and an increasing use of donation after circulatory death. Survival improved in the last triennium.
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INTRODUCTION AND OBJECTIVES: The multiparametric implantable cardioverter-defibrillator HeartLogic index has proven to be a sensitive and timely predictor of impending heart failure (HF) decompensation. We evaluated the impact of a standardized follow-up protocol implemented by nursing staff and based on remote management of alerts. METHODS: The algorithm was activated in HF patients at 19 Spanish centers. Transmitted data were analyzed remotely, and patients were contacted by telephone if alerts were issued. Clinical actions were implemented remotely or through outpatient visits. The primary endpoint consisted of HF hospitalizations or death. Secondary endpoints were HF outpatient visits. We compared the 12-month periods before and after the adoption of the protocol. RESULTS: We analyzed 392 patients (aged 69±10 years, 76% male, 50% ischemic cardiomyopathy) with implantable cardioverter-defibrillators (20%) or cardiac resynchronization therapy defibrillators (80%). The primary endpoint occurred 151 times in 86 (22%) patients during the 12 months before the adoption of the protocol, and 69 times in 45 (11%) patients (P<.001) during the 12 months after its adoption. The mean number of hospitalizations per patient was 0.39±0.89 pre- and 0.18±0.57 postadoption (P<.001). There were 185 outpatient visits for HF in 96 (24%) patients before adoption and 64 in 48 (12%) patients after adoption (P<.001). The mean number of visits per patient was 0.47±1.11 pre- and 0.16±0.51 postadoption (P<.001). CONCLUSIONS: A standardized follow-up protocol based on remote management of HeartLogic alerts enabled effective remote management of HF patients. After its adoption, we observed a significant reduction in HF hospitalizations and outpatient visits.
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BACKGROUND AND METHODS: Heart transplant recipients (HTr) have a higher probability of suffer from severe coronavirus disease-2019 (COVID-19) in comparison to general population, but their risk has changed over the course of the pandemic in relation to various factors. We conducted a prospective study including all HTr at risk of COVID-19 in a tertiary center between February 2020 and October 2022. The aim was to analyze how the prognosis (incidence of pneumonia and mortality) of COVID-19 in HTr has evolved over time, contextualizing variants, vaccination, and other treatments. RESULTS: Of 308 HTr included, 124 got the infection (39.2%). COVID and non-COVID HTr had similar baseline characteristics. COVID-19 patients with pneumonia had a poorer prognosis than those with less severe presentations, with a higher rate of hospitalization (93.3 vs. 14.1%, p < .001) and death (41.0 vs. 1.2%, p < .001). Multivariate analysis identified age ≥60 years (odds ratio [OR] 3.65, 95% confidence interval [CI] 1.16-11.49, p = .027), and chronic kidney disease ≥3a (OR 4.95, 95% CI 1.39-17.54, p = .014) as predictors of pneumonia. Two-dose vaccination (OR 0.20, CI 95% 0.05-0.72, p = .02) and early remdesivir administration (OR 0.17, CI 0.03-0.90, p = .037) were protective factors. Over the course of the pandemic considering three periods in the follow-up (prevaccination February-December 2020, postvaccination January-December 2021, and post early remdesivir indication January-October 2022), we observed a reduction in pneumonia incidence from 62% to 19% (p < .001); and mortality (from 23% to 4%, p < .001). CONCLUSIONS: The prognosis of COVID-19 in HTr has improved over time, likely due to vaccination and early administration of remdesivir.
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COVID-19 , Transplante de Coração , Humanos , Pessoa de Meia-Idade , COVID-19/epidemiologia , COVID-19/prevenção & controle , Pandemias , SARS-CoV-2 , Estudos Prospectivos , Transplante de Coração/efeitos adversos , TransplantadosRESUMO
Chronic obstructive pulmonary disease (COPD) and cardiovascular disease (CVD) frequently coexist, increasing the prevalence of both entities and impacting on symptoms and prognosis. CVD should be suspected in patients with COPD who have high/very high risk scores on validated scales, frequent exacerbations, precordial pain, disproportionate dyspnea, or palpitations. They should be referred to cardiology if they have palpitations of unknown cause or angina pain. COPD should be suspected in patients with CVD if they have recurrent bronchitis, cough and expectoration, or disproportionate dyspnea. They should be referred to a pulmonologist if they have rhonchi or wheezing, air trapping, emphysema, or signs of chronic bronchitis. Treatment of COPD in cardiovascular patients should include long-acting muscarinic receptor antagonists (LAMA) or long-acting beta-agonists (LABA) in low-risk or high-risk non-exacerbators, and LAMA/LABA/inhaled corticosteroids in exacerbators who are not controlled with bronchodilators. Cardioselective beta-blockers should be favored in patients with CVD, the long-term need for amiodarone should be assessed, and antiplatelet drugs should be maintained if indicated.
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Doenças Cardiovasculares , Doença Pulmonar Obstrutiva Crônica , Humanos , Doenças Cardiovasculares/complicações , Administração por Inalação , Antagonistas Muscarínicos/uso terapêutico , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Quimioterapia Combinada , Corticosteroides/uso terapêutico , Dispneia , Dor/tratamento farmacológico , Agonistas de Receptores Adrenérgicos beta 2/uso terapêutico , Broncodilatadores/uso terapêuticoRESUMO
Heart transplant (HT) remains the best therapeutic option for patients with advanced heart failure (HF). The allocation criteria aim to guarantee equitable access to HT and prioritize patients with a worse clinical status. To review the HT allocation criteria, the Heart Failure Association of the Spanish Society of Cardiology (HFA-SEC), the Spanish Society of Cardiovascular and Endovascular Surgery (SECCE) and the National Transplant Organization (ONT), organized a consensus conference involving adult and pediatric cardiologists, adult and pediatric cardiac surgeons, transplant coordinators from all over Spain, and physicians and nurses from the ONT. The aims of the consensus conference were as follows: a) to analyze the organization and management of patients with advanced HF and cardiogenic shock in Spain; b) to critically review heart allocation and priority criteria in other transplant organizations; c) to analyze the outcomes of patients listed and transplanted before and after the modification of the heart allocation criteria in 2017; and d) to propose new heart allocation criteria in Spain after an analysis of the available evidence and multidisciplinary discussion. In this article, by the HFA-SEC, SECCE and the ONT we present the results of the analysis performed in the consensus conference and the rationale for the new heart allocation criteria in Spain.
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Insuficiência Cardíaca , Transplante de Coração , Adulto , Humanos , Criança , Espanha/epidemiologia , Insuficiência Cardíaca/cirurgia , Consenso , Choque CardiogênicoRESUMO
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.
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Insuficiência Cardíaca , Transplante de Coração , Piridazinas , Humanos , Simendana/uso terapêutico , Cardiotônicos/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/cirurgia , Hidrazonas/uso terapêutico , Piridazinas/uso terapêuticoRESUMO
INTRODUCTION AND OBJECTIVES: The Spanish heart transplant registry updates its data annually. The current update presents the data for the year 2022. METHODS: We describe the main clinical characteristics, treatments received, and survival outcomes including procedures performed in 2022, along with their trends since 2013. RESULTS: In 2022, 311 cardiac transplants were performed, representing a 3.0% increase compared with 2021. Compared with previous years, no significant changes in demographic and clinical characteristics were observed in 2022, confirming the trends identified in the last decade. These trends indicate a decrease in urgent procedures and the use of circulatory support, particularly ventricular assist devices. In the last decade, survival rates at 1 and 3 years were 81.4% and 73.4% respectively, with a slight, nonsignificant improvement. CONCLUSIONS: In the last decade, there has been a stabilization in the characteristics of heart transplant procedures and their outcomes. This trial was registered at ClinicalTrial.gov (Identifier: NCT03015311).
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Cardiologia , Insuficiência Cardíaca , Transplante de Coração , Humanos , Sociedades Médicas , Transplante de Coração/métodos , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/cirurgia , Sistema de Registros , Espanha/epidemiologiaRESUMO
INTRODUCTION AND OBJECTIVES: Hypertension is highly common in heart failure (HF). However, there is limited information on its prevalence, circadian variation, and relationship with the various HF phenotypes. The objective of this study was to describe the prevalence of hypertension and its patterns in HF. METHODS: This was a cross-sectional observational study of patients with optimized stable chronic HF. The patients underwent blood pressure (BP) measurement in the office and 24-hour ambulatory monitoring. We estimated the prevalence of hypertension, and its diurnal (controlled, uncontrolled, white coat, and masked) and nocturnal (dipper, nondipper, and reverse dipper) patterns. We also analyzed the factors associated with the different patterns and HF phenotypes. RESULTS: From 2017 to 2021, 266 patients were included in the study (mean age, 72±12 years, 67% male, 46% with reduced ejection fraction). Hypertension was present in 83%: controlled in 68%, uncontrolled in 10%, white coat in 10%, and masked in 11%. Among patients with high office BP, 51% had white coat hypertension. Among those with normal office BP, 14% had masked hypertension. The prevalence of dipper, nondipper, and reverse dipper patterns was 31%, 43%, and 26%, respectively. Systolic BP was lower in HF with reduced ejection fraction than in HF with preserved ejection fraction (P <.001). CONCLUSIONS: Ambulatory BP monitoring in HF identified white coat hypertension in more than half of patients with high office BP and masked hypertension in a relevant percentage of patients. The distribution of daytime patterns was similar to that of the population without HF in the literature, but most of the study patients had a pathological nocturnal pattern.
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Insuficiência Cardíaca , Hipertensão , Hipertensão Mascarada , Hipertensão do Jaleco Branco , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Hipertensão do Jaleco Branco/diagnóstico , Hipertensão do Jaleco Branco/epidemiologia , Hipertensão do Jaleco Branco/complicações , Monitorização Ambulatorial da Pressão Arterial , Hipertensão Mascarada/diagnóstico , Hipertensão Mascarada/epidemiologia , Hipertensão Mascarada/complicações , Prevalência , Estudos Transversais , Hipertensão/diagnóstico , Hipertensão/epidemiologia , Hipertensão/complicações , Pressão Sanguínea/fisiologia , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/complicações , Ritmo Circadiano/fisiologiaRESUMO
INTRODUCTION AND OBJECTIVES: The Index for Mortality Prediction After Cardiac Transplantation (IMPACT) score was derived and validated as a predictor of mortality after heart transplantation (HT). The primary objective of this work is to externally validate the IMPACT score in a contemporary Spanish cohort. METHODS: Spanish Heart Transplant Registry data were used to identify adult (>16 years) HT patients between January 2000 and December 2015. Retransplantation, multiorgan transplantation and patients in whom at least one of the variables required to calculate the IMPACT score was missing were excluded from the analysis (N = 2810). RESULTS: Median value of the IMPACT score was five points (IQR: 3, 8). Overall, 1-year survival rate was 79.1%. Kaplan-Meier 1-year survival rates by IMPACT score categories (0-2, 3-5, 6-9, 10-14, ≥15) were 84.4%, 81.5%, 79.3%, 77.3%, and 58.5%, respectively (Log-Rank test: p < .001). Performance analysis showed a good calibration (Hosmer-Lemeshow chi-square for 1 year was 7.56; p = .47) and poor discrimination ability (AUC-ROC .59) of the IMPACT score as a predictive model. CONCLUSIONS: In a contemporary Spanish cohort, the IMPACT score failed to accurately predict the risk of death after HT.
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Transplante de Coração , Adulto , Estudos de Coortes , Humanos , Sistema de Registros , Medição de Risco , Taxa de SobrevidaRESUMO
INTRODUCTION AND OBJECTIVES: This report updates the annual data of the Spanish Heart Transplant Registry with the procedures performed in 2021. METHODS: We describe the clinical profile, therapeutic characteristics and outcomes in terms of survival of the procedures performed in 2021. Their temporal trends are updated for the 2012 to 2020 period. RESULTS: In 2021, 302 heart transplants were performed (8.6% increase versus 2020). The tendency in 2021 confirmed that of prior years, with fewer urgent transplants and a preference for the use of ventricular assist devices. The remaining characteristics and survival showed a clear trend toward stability in the last decade. Compared with 2019, the SARS-CoV-2 pandemic (2020 and 2021) did not affect short- or long-term survival. CONCLUSIONS: In 2021, transplant activity returned to prepandemic levels. The SARS-CoV-2 pandemic did not significantly affect transplant outcomes. The main transplant features and outcomes have clearly stabilized in the last decade.
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BACKGROUND: The surgical approach for the treatment of a left ventricular assist device with severe infection may be controversial. MATERIAL & METHODS: We present the case of a patient implanted with a HeartWare™ HVAD as a bridge to transplant and chronic infection of the device by Pseudomonas who underwent a conservative partial treatment of the driveline tunnel and subsequently a heart transplantation and device removal were done. CONCLUSIONS: The two-step simplified approach allowed the patient to be transplanted in a short period of time, with the abdominal wall healed and almost two-thirds of the driveline subcutaneous tunnel sterilized.
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Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Insuficiência Cardíaca/cirurgia , Humanos , Estudos RetrospectivosRESUMO
Telemonitoring through multiple variables measured on cardiac devices has the potential to improve the follow-up of patients with heart failure. The HeartLogic algorithm (Boston Scientific), implemented in some implantable cardiac defibrillators and cardiac resynchronisation therapy, allows monitoring of the nocturnal heart rate, respiratory movements, thoracic impedance, physical activity and the intensity of heart tones, with the aim of predicting major clinical events. Although HeartLogic has demonstrated high sensitivity for the detection of heart failure decompensations, its effects on hospitalisation and mortality in randomised clinical trials has not yet been corroborated. This review details how the HeartLogic algorithm works, compiles available evidence from clinical studies, and discusses its application in daily clinical practice.
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Hyperkalaemia is a growing concern in the treatment of patients with heart failure and reduced ejection fraction (HFrEF) as it limits the use of some prognostic-modifying drugs and has a negative impact on prognosis. The objective of the present study was to estimate the prevalence of hyperkalaemia in outpatients with HFrEF and its impact on achieving optimal medical treatment. For this purpose, a multicentre, prospective, and observational study was carried out on consecutive HFrEF patients who were monitored as outpatients in heart failure (HF) units and who, in the opinion of their doctor, received optimal medical treatment. A total of 565 HFrEF patients were included from 16 specialised HF units. The mean age was 66 ± 12 years, 78% were male, 45% had an ischemic cause, 39% had atrial fibrillation, 43% were diabetic, 42% had a glomerular filtration rate < 60 mL/min/1.7 m2, and the mean left ventricular ejection fraction was 31 ± 7%. Treatment at the study entry included: 76% on diuretics, 13% on ivabradine, 7% on digoxin, 18.9% on angiotensin-conversing enzyme inhibitors (ACEi), 11.3% on angiotensin receptors blockers (ARBs), 63.8% on angiotensin-neprilysin inhibitors (ARNi), 78.5% on mineralocorticoid receptor antagonists (MRAs), and 92.9% on beta-blockers. Potassium levels in the baseline analysis were: ≤5 mEq/L = 80.5%, 5.1−5.4 mEq/L = 13.8%, 5.5−5.9 mEq/L = 4.6%, and ≥6 mEq/L = 1.06%. Hyperkalaemia was the reason for not prescribing or reaching the target dose of an MRAs in 34.8% and 12.5% of patients, respectively. The impact of hyperkalaemia on not prescribing or dropping below the target dose in relation to ACEi, ARBs, and ARNi was significantly less. In conclusion, hyperkalaemia is a frequent problem in the management of patients with HFrEF and a limiting factor in the optimisation of medical treatment.
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INTRODUCTION AND OBJECTIVES: HeartLogic is a multiparametric algorithm incorporated into implantable cardioverter-defibrillators (ICD). The associated alerts predict impending heart failure (HF) decompensations. Our objective was to analyze the association between alerts and clinical events and to describe the implementation of a protocol for remote management in a multicenter registry. METHODS: We evaluated study phase 1 (the investigators were blinded to the alert state) and phases 2 and 3 (after HeartLogic activation, managed as per local practice and with a standardized protocol, respectively). RESULTS: We included 288 patients from 15 centers. In phase 1, the median observation period was 10 months and there were 73 alerts (0.72 alerts/patient-y), with 8 hospitalizations and 2 emergency room admissions for HF (0.10 events/patient-y). There were no HF hospitalizations outside the alert period. In the active phases, the median follow-up was 16 (95%CI, 15-22) months and there were 277 alerts (0.89 alerts/patient-y); 33 were associated with HF hospitalizations or HF death (n=6), 46 with minor decompensations, and 78 with other events. The unexplained alert rate was 0.39 alerts/patient-y. Outside the alert state, there was only 1 HF hospitalization and 1 minor HF decompensation. Most alerts (82% in phase 2 and 81% in phase 3; P=.861) were remotely managed. The median NT-proBNP value was higher within than outside the alert state (7378 vs 1210 pg/mL; P <.001). CONCLUSIONS: The HeartLogic index was frequently associated with HF-related events and other clinically relevant situations, with a low rate of unexplained events. A standardized protocol allowed alerts to be safely and remotely detected and appropriate action to be taken on them.
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Desfibriladores Implantáveis , Insuficiência Cardíaca , Algoritmos , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/terapia , Hospitalização , Humanos , Sistema de RegistrosRESUMO
INTRODUCTION AND OBJECTIVES: Intrapatient blood level variability (IPV) of calcineurin inhibitors has been associated with poor outcomes in solid-organ transplant, but data for heart transplant are scarce. Our purpose was to ascertain the clinical impact of IPV in a multi-institutional cohort of heart transplant recipients. METHODS: We retrospectively studied patients aged ≥18 years, with a first heart transplant performed between 2000 and 2014 and surviving≥ 1 year. IPV was assessed by the coefficient of variation of trough levels from posttransplant months 4 to 12. A composite of rejection or mortality/graft loss or rejection and all-cause mortality/graft loss between years 1 to 5 posttransplant were analyzed by Cox regression analysis. RESULTS: The study group consisted of 1581 recipients (median age, 56 years; women, 21%). Cyclosporine immediate-release tacrolimus and prolonged-release tacrolimus were used in 790, 527 and 264 patients, respectively. On multivariable analysis, coefficient of variation> 27.8% showed a nonsignificant trend to association with 5-year rejection-free survival (HR, 1.298; 95%CI, 0.993-1.695; P=.056) and with 5-year mortality (HR, 1.387; 95%CI, 0.979-1.963; P=.065). Association with rejection became significant on analysis of only those patients without rejection episodes during the first year posttransplant (HR, 1.609; 95%CI, 1.129-2.295; P=.011). The tacrolimus-based formulation had less IPV than cyclosporine and better results with less influence of IPV. CONCLUSIONS: IPV of calcineurin inhibitors is only marginally associated with mid-term outcomes after heart transplant, particularly with the tacrolimus-based immunosuppression, although it could play a role in the most stable recipients.
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Inibidores de Calcineurina , Transplante de Coração , Adolescente , Adulto , Inibidores de Calcineurina/uso terapêutico , Feminino , Rejeição de Enxerto/epidemiologia , Rejeição de Enxerto/prevenção & controle , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , TacrolimoRESUMO
INTRODUCTION AND OBJECTIVES: The present report updates the main characteristics and outcomes of heart transplants in Spain to 2020. METHODS: We describe the main features of recipients, donors, surgical procedure, and immunosuppression in 2020. We also analyze the temporal trends of these characteristics and outcomes (survival) for the period 2011 to 2019. RESULTS: In 2020, 278 heart transplants were performed (7.3% decrease vs 2019). The findings in 2020 confirmed previous observations of an increase in pretransplant sternotomy, a slight decrease in urgent transplants carried out with ventricular assist devices, a slight decrease in donor age, an increase in the use of allografts with previous arrest, and a decrease in ischemia time. Survival continued to improve in recent triennia, reaching 82.0% at 1 year in the period 2017 to 2019. CONCLUSIONS: The slight decrease in the number of heart transplants performed in 2020 in Spain, most likely due to the SARS-CoV-2 pandemic, did not change the main characteristics of the procedure. No change was observed in the tendency to improved survival.
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COVID-19 , Cardiologia , Insuficiência Cardíaca , Transplante de Coração , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/cirurgia , Humanos , Sistema de Registros , SARS-CoV-2 , Sociedades Médicas , Espanha/epidemiologiaRESUMO
Despite the important changes in the management of heart transplantation in the recent decades, the use of endomyocardial biopsy is still necessary for the follow-up of these patients. However, this technique has several limitations, the most important being the substantial interobserver variability. In the last years multiple attempts have been made to find non-invasive assays for cardiac allograft surveillance, such as imaging modalities and serum biomarkers. This state-of-the-art review focuses on describing the different serum biomarkers that have been proposed for non-invasive diagnosis of acute rejection and that are paving the way towards precision medicine in the field of heart transplantation.
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Biomarcadores/sangue , Rejeição de Enxerto/sangue , Transplante de Coração/efeitos adversos , Medicina de Precisão/tendências , Doença Aguda , Aloenxertos , Biópsia , Rejeição de Enxerto/diagnóstico , Humanos , Miocárdio/patologiaRESUMO
BACKGROUND: Truncating TTN variants (TTNtv) are the main cause of dilated cardiomyopathy (DCM). The dynamic nature of this entity has previously been described. Based on own empirical observations and previous evidences, this study assessed repolarisation patterns and the possible association with morphological and functional status of TTNtv-DCM patients. METHODS: Electrocardiograms (ECGs) of index patients with TTNtv-DCM and their relatives were included and matched in time with an echocardiogram. All individuals were classified into five phenotype groups: 1) Reduced left ventricular ejection fraction (LVEF <50%); 2) Recovered LVEF: at least 10% increase and LVEF >30% after optimal medical treatment; 3) Borderline phenotype (mildly enlarged ventricle and/or hyper-trabeculation); 4) Genotype positive, phenotype negative; and 5) Non-carriers. All electrocardiograms were evaluated by two blinded observers in qualitative and quantitative terms [T index (mm)=Σ T-wave amplitude (V5, V6, II, aVF)] and these data were compared with demographic and clinical information. The Δ T-index was calculated in those individuals with more than one electrocardiogram. RESULTS: Seventy-eight (78) electrocardiograms were included (46% female, mean age 50 years). T-index and prevalence of an abnormal T-wave had significantly different results among the groups (p<0.0001). Age and haemodynamic factors were shown to be ECG-modifiers, especially in phenotype-negative patients. T-index enabled individuals with reduced LVEF (<2.5) to be identified and to differentiate patients with favourable and unfavourable responses to treatment (Δ T index >3.5 and ≤2, respectively). CONCLUSION: Repolarisation changes enabled characterisation of the spectrum of TTNtv-DCM. The T-index identified potential carriers and patients with the worst profiles of the spectrum of the disease.
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Cardiomiopatia Dilatada , Cardiomiopatia Dilatada/diagnóstico , Conectina , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Volume Sistólico , Função Ventricular EsquerdaRESUMO
Non-compacted myocardiopathy is rare, the prevalence ranging between 0.01-0.26%. in adults. We present the macroscopic, microscopic and electron microscopy findings of cardiac transplant samples from a 36-year-old patient diagnosed with non-compacted myocardiopathy. This condition shows a high genetic and phenotypic heterogeneity, with superposition of different phenotypes and variability in the hereditary patterns. Clinical diagnosis is established by coupling imaging results to clinical characteristics. The clinical manifestations of non-compacted myocardiopathy are variable, including arrhythmic events and variable degrees of cardiac failure, although some patients may be asymptomatic. In certain cases a heart transplant may be necessary. The differential diagnosis should be made with hypertrophic and dilated myocardiopathy. However, only a few reports can be found in the literature that discuss the pathology of this condition.