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Neurohormonal blockade (NB)/modulation is the combination of two renin-angiotensin-aldosterone system inhibitors (RAASi) with a beta blocker. It is the core therapy for heart failure with reduced ejection fraction (HFrEF). While improving long term prognosis, it also induces hyperkalemia (serum KWe reviewed the medical records of HFrEF patients attending a HF clinic at a tertiary Portuguese hospital during 2018 (n=240). The number of patients not tolerating maximal neurohormonal blockade/modulation due to hyperkalemia was determined. The incidence and characteristics of hyperkalemia episodes were also assessed.Only six patients (3%) achieved maximal doses of neurohormonal blockade/modulation. Hyperkalemia was the limiting factor in 48 (20%) patients. A total of 185 hyperkalemia episodes occurred in 100 (42%) patients. Forty-five (24%) episodes were moderate or severe (serum KIn daily clinical practice, hyperkalemia is frequent and limits neurohormonal blockade/modulation by leading to the withholding or reducing of the intensity of RAAS inhibition. Considering the negative prognostic impact associated with sub-optimal neurohormonal blockade/modulation, addressing hyperkalemia is an important issue when treating HFrEF patients.

BACKGROUND: Heart failure (HF) is a growing public health problem. Sacubitril/valsartan is now recommended to be used in persistently symptomatic patients with left ventricular ejection fraction (LVEF)

Objective Heart rate variability (HRV), an index of the autonomic cardiac activity, is decreased in patients with epilepsy, and a low HRV is associated with a higher risk of sudden death. Generalized tonic-clonic seizures are one of the most consistent risk factors for SUDEP, but the influence (and relative risk) of each type of seizure on cardiac function is still unknown. Our objective was to assess the impact of the type of seizure (focal to bilateral tonic-clonic seizure – FBTCS – versus non-FBTCS) on periictal HRV, in a group of patients with refractory epilepsy and both types of seizures. Methods We performed a 48-hour Holter recording on 121 patients consecutively admitted to our Epilepsy Monitoring Unit. We only included patients with both FBTCS and non-FBTCS on the Holter recording and selected the first seizure of each type to analyze. To evaluate HRV parameters (AVNN, SDNN, RMSSD, pNN20, LF, HF, and LF/HF), we chose 5-min epochs pre- and postictally. Results We included 14 patients, with a median age of 36 (min–max, 16–55) years and 64% were female. Thirty-six percent had cardiovascular risk factors, but no previously known cardiac disease. In the preictal period, there were no statistically significant differences in HRV parameters, between FBTCS and non-FBTCS. In the postictal period, AVNN, RMSSD, pNN20, LF, and HF were significantly lower, and LF/HF and HR were significantly higher in FBTCS. From preictal to postictal periods, FBTCS elicited a statistically significant rise in HR and LF/HF, and a statistically significant fall in AVNN, RMSSD, pNN20, and HF. Non-FBTCS only caused statistically significant changes in HR (decrease) and AVNN (increase). Significance/conclusion This work emphasizes the greater effect of FBTCS in autonomic cardiac function in patients with refractory epilepsy, compared to other types of seizures, with a significant reduction in vagal tonus, which may be associated with an increased risk of SUDEP.

Background. Sudden cardiac death (SCD) risk stratification in dilated cardiomyopathy (DCM) has been based on left ventricular ejection fraction (LVEF), even though SCD may occur with LVEF > 35%. Family history of unexplained SCD, especially in the young, raises concern about potential inheritable risk factors. It remains largely unknown how genetic tests can be integrated into clinical practice, particularly in the selection of implantable cardioverter defibrillator (ICD) candidates. We aimed to assess the diagnostic yield of genetic testing in DCM patients with a class I recommendation for ICD implantation, based on current guidelines. Methods. We included ambulatory stable adult patients with idiopathic or familial DCM with previously implanted ICD. Molecular analysis included 15 genes (LMNA, MYH7, MYBPC3, TNNT2, ACTC1, TPM1, CSRP3, TCAP, SGCD, PLN, MYL2, MYL3, TNNI3, TAZ, and LDB3) using next-generation sequencing. Results. We evaluated 21 patients, 12 (57%) males and 9 (43%) with familial DCM, including 3 (14%) with a family history of premature unexplained SCD. Mean age at DCM diagnosis was 40 ± 2 years, and mean age at ICD implantation was 50 ± 12 years. LVEF was 27 ± 9%, and LV end-diastolic diameter was 65 ± 7 mm. Genetic variants were found in six (29%) patients, occurring in 5 genes: TPM1, TNNT2, MYH7, PLN, and MYBPC3. The majority were classified as variants of uncertain significance. Family history of SCD was present in both patients with PLN variants. Conclusion. In patients with DCM and ICD, genetic variants could be identified in a significant proportion of patients in several genes, highlighting the potential role of genetics in DCM SCD risk stratification.

Background Cardioembolism induced by atrial fibrillation (AF) is responsible for up to 33% of all ischemic strokes. 24-hour Holter monitoring in stroke and transient ischemic attack (TIA) patients is used as a routine investigation to search for occult paroxysmal atrial fibrillation (PAF), which may have crucial prognostic impact. Excessive supraventricular ectopic activity (ESVEA) is also a stroke risk factor, probably related to the risk of developing AF. Purpose To observe the incidence of AF at a long-term follow-up and to evaluate the clinical, electrocardiographic and echocardiographic predictors of new onset AF in stroke patients. Methods Patients in sinus rhythm who performed Holter between October 2009 and October 2011 in the setting of post stroke or TIA were included; patients with previous AF were excluded. These patients were followed for 8 to 10 years. Clinical, electrocardiographic and echocardiographic data were collected. ESVEA was defined by ≥500 premature atrial contractions per 24 hours or any sustained supraventricular tachycardia episode. Results 104 patients were included, 54% were male, with a mean age of 63.8±14.7 years at the time of the event. In relation to cardiovascular risk factors, 59% had hypertension, 47% dyslipidemia, 14% diabetes, 44% were smokers or previous smokers; 67% of patients were high consumers of alcohol. 79.8% had a stroke and 21.2% a TIA. 24-hour Holter monitoring revealed ESVEA in 13.5% of patients and PAF in 1.9%. All patients with PAF had a previous stroke and were older than 55. At a follow-up of 8–10 years, new onset AF was detected in 11.5%; these patients had similar mortality comparing to those in sustained sinus rhythm (21.2% vs 16.7%, p=0.724). Alcohol intake, an established risk factor for development of AF, was associated with a non-significant increase of AF (17.3% vs 11.5%) while the presence of cardiovascular risk factors was not associated with AF development. We found a statistically significant difference between patients with and without ESVEA concerning to new onset of AF (35.7% vs 8.0%, p=0.010). ESVEA seems to be related with a higher mortality at a long follow-up, although this difference wasn't statistically significant (35.7% vs 18.2%, p=0.132). Concerning to echocardiographic parameters, patients whit left atrium enlargement showed a higher incidence of AF at follow-up (14.7% vs 7.9%), and the presence of mitral regurgitation were not related with new onset of AF. Patients' age was also not related with new onset of AF during follow-up. Conclusion Atrial fibrillation is considered the main cause of stroke. Our study showed that ESVEA is a strong predictor of new onset AF and highlights that Holter monitoring could be an important tool not only to diagnose AF but also to identify patients in risk of develop AF. Diagnostic of new AF during long-term follow up didn't correlate with higher mortality. Funding Acknowledgement Type of funding source: None

Introduction: Papillary fibroelastoma (PFE) is a rare primary benign tumor of cardiac origin that predominantly affects the cardiac valves. Although most patients are asymptomatic, serious complications may result given their propensity for embolization. Advances in imaging technology have enabled earlier detection and more accurate characterization of these tumors. We report a case series, describing clinical presentation, treatment and outcome. Methods: Institutional records of a tertiary center between 1997 and 2015 were reviewed for all patients diagnosed with PFE treated surgically and confirmed histologically. Demographic and clinical characteristics, echocardiography findings and treatment modalities were analyzed and recurrence at follow-up was studied. Results: A total of 26 patients (69% male), aged 54±18 years, had a PFE. Clinically, PFE presented with neurologic deficits in eight cases and was asymptomatic in 65.4%. The mitral valve surface was the predominant tumor location (53.8%), followed by the aortic valve (34.6%). Tumor size ranged between 3 mm and 22 mm, 26.9% had a pedicle and 42.4% were mobile. All patients were treated successfully by complete resection, isolated in 88.5% and with valve repair in three cases. No other cardiac procedure was performed concomitantly and there were no major postoperative complications. Median follow-up was 61±49 months and no tumor recurrence or embolic events were documented. Conclusions: Fibroelastomas are generally small, single and detected by chance during routine imaging exams. Complete surgical resection of the tumor has an excellent prognosis and appears to be a good strategy. Resumo: Introdução: Os fibroelastomas papilares (FP) são tumores benignos primários de origem cardíaca, que afetam predominantemente as válvulas. Embora a maioria dos doentes seja assintomática, podem ocorrer complicações graves por causa de sua propensão para embolizar. A melhoria das técnicas de imagem moderna permitiu a deteção precoce e melhor caracterização desses tumores. É nosso objetivo apresentar a casuística do Serviço, descrever a apresentação clínica, o tratamento e os resultados cirúrgicos. Métodos: Foram revistos os dados institucionais entre 1997 e 2015, de todos os doentes com o diagnóstico de FP tratados cirurgicamente, com confirmação histológica. Foram analisadas as características demográficas, clínicas, ecocardiográficas e cirúrgicas. A recorrência durante o seguimento foi também objeto do estudo. Resultados: Foram identificados 26 doentes (69% homens), de 54±18 anos, com FP. O FP apresentou-se com deficit neurológico em oito doentes, nos restantes casos o diagnóstico foi casual. A superfície valvular mitral foi a localização mais frequente (53,8%), seguida da válvula aórtica (34,6%). A dimensão do tumor variou de 3 a 22 mm, 26,9% eram pediculados e a mobilidade estava presente em 42,4% dos casos. Todos os doentes foram tratados com resseção completa, isolada em 88,5% e com reparação valvular em três casos. Nenhum outro procedimento cardíaco foi feito concomitantemente e não se registaram complicações cardíacas major no pós-operatório. O período mediano de seguimento foi de 61±49 meses, não foi documentada recorrência do tumor nem de eventos embólicos. Conclusões: Os fibroelastomas são geralmente pequenos, isolados e mais frequentemente detetados incidentalmente em exames de rotina. A recessão cirúrgica completa do tumor tem um excelente prognóstico e parece ser uma boa estratégia. Keywords: Cardiac tumor, Fibroelastoma, Cardiovascular event, Palavras-chave: Tumor cardíaco, Fibroelastoma, Evento cardiovascular

Objective Patients with epilepsy, mainly drug-resistant, have reduced heart rate variability (HRV), linked to an increased risk of sudden death in various other diseases. In this context, it could play a role in SUDEP. Generalized convulsive seizures (GCS) are one of the most consensual risk factors for SUDEP. Our objective was to assess the influence of GCS in HRV parameters in patients with drug-resistant epilepsy. Methods We prospectively evaluated 121 patients with refractory epilepsy admitted to our Epilepsy Monitoring Unit. All patients underwent a 48-hour Holter recording. Only patients with GCS were included (n = 23), and we selected the first as the index seizure. We evaluated HRV (AVNN, SDNN, RMSSD, pNN50, LF, HF, and LF/HF) in 5-min epochs (diurnal and nocturnal baselines; preictal – 5 min before the seizure; ictal; postictal – 5 min after the seizure; and late postictal – >5 h after the seizure). These data were also compared with normative values from a healthy population (controlling for age and gender). Results We included 23 patients, with a median age of 36 (min-max, 16–55) years and 65% were female. Thirty percent had cardiovascular risk factors, but no previously known cardiac disease. HRV parameters AVNN, RMSSD, pNN50, and HF were significantly lower in the diurnal than in the nocturnal baseline, whereas the opposite occurred with LF/HF and HR. Diurnal baseline parameters were inferior to the normative population values (which includes only diurnal values). We found significant differences in HRV parameters between the analyzed periods, especially during the postictal period. All parameters but LF/HF suffered a reduction in that period. LF/HF increased in that period but did not reach statistical significance. Visually, there was a tendency for a global reduction in our patients’ HRV parameters, namely AVNN, RMSSD, and pNN50, in each period, comparing with those from a normative healthy population. No significant differences were found in HRV between diurnal and nocturnal seizures, between temporal lobe and extra-temporal-lobe seizures, between seizures with and without postictal generalized EEG suppression, or between seizures of patients with and without cardiovascular risk factors. Significance/conclusion Our work reinforces the evidence of autonomic cardiac dysfunction in patients with refractory epilepsy, at baseline and mainly in the postictal phase of a GCS. Those changes may have a role in some SUDEP cases. By identifying patients with worse autonomic cardiac function, HRV could fill the gap of a lacking SUDEP risk biomarker.

Introduction: Biomarkers in dilated cardiomyopathy (DCM) reflect various pathobiological processes, including neurohormonal activation, oxidative stress, matrix remodeling, myocyte injury and myocyte stretch. We assessed the role of biomarkers in clinical and echocardiographic parameters and in left ventricular (LV) reverse remodeling (LVRR). Methods: In this prospective study of 50 DCM patients (28 men, aged 59±10 years) with LV ejection fraction (LVEF) 10 U in LVEF after optimal medical therapy. Results: Baseline LVEF was 25.4±9.8% and LV end-diastolic diameter (LVEDD)/body surface area (BSA) was 34.2±4.5 mm/m2. LVRR occurred in 34% of patients within 17.6±15.6 months. No correlation was found between B-type natriuretic peptide (BNP), 25-hydroxyvitamin D (25(OH)D), CA-125, high-sensitivity C-reactive protein (hs-CRP), lipoprotein(a) [Lp(a)], noradrenaline, adrenaline, renin or aldosterone and LVRR. Patients in NYHA class III or IV, with pulmonary congestion or ankle edema, had higher CA-125, cystatin C, BNP and hs-CRP levels (p10 U da LVEF, após a terapêutica médica otimizada. Resultados: A LVEF basal foi de 25,4±9,8% e o diâmetro do VE (LVD)/BSA de 34,2±4,5 mm/m2. A LVRR ocorreu em 34%, em 17,6±15,6 meses. Não houve correlação entre BNP, 25-OH-vit D, CA 125, hsCRP, Lp(a), noradrenalina, adrenalina, renina, aldosterona e LVRR. Doentes em classe NYHA (III-IV), com congestão pulmonar ou edema periférico apresentaram níveis mais elevados de CA 125, cistatina C, BNP e hsCRP (p

Introduction: Since 2011, the European guidelines have included a specific low-density lipoprotein cholesterol (LDL-C) target

Background Heart Rate Variability (HRV) is an increasing area of interest in patients with epilepsy. The effects of epilepsy on the autonomic control of the heart are not completely understood and that autonomic dysfunction has been implicated in some cases of Sudden Unexpected Death in Epilepsy (SUDEP). Objective To study the influence of generalized tonic-clonic seizures (GTCS) on HRV of patients with focal refractory epilepsy. Method We prospectively evaluated (January 2015 to July 2018) 121 patients admitted to our institution's Epilepsy Monitoring Unit. All patients performed a 48-hour Holter recording. Patients who had GTCS during the recording were included and we selected the first GTCS as the index seizure. HRV (AVNN, SDNN, RMSSD, pNN50, and LF/HF) was evaluated by analyzing 5-min-ECG epochs during inter-ictal and post-ictal periods: baseline, pre-ictal (5 min before the GTCS seizure), post-ictal (5 min after the seizure), and late post-ictal (>5 hours after the seizure). We compared HRV data from these patients with normative values for a healthy population (controlling age and gender). The study was approved by our Institution Ethics Committee and all patients gave informed consent. Results Twenty three patients were included (mean age: 38.61±11.58; 70% Female). Thirty percent presented cardiovascular risk factors without known cardiac disease. We found significant differences between the analyzed periods for all but one (LF/HF) HRV metrics (using Friedman test, p Conclusion Our work shows reduced HRV after a GTCS in patients with focal resistant epilepsy, both in inter-ictal and post-ictal periods, when compared to normative healthy population values. These results might reflect long term structural changes in autonomic centers. The HRV changes were significant particularly during the post-ictal period, and should prompt further investigation, giving this period is critical for SUDEP.

Heart failure (HF) is a chronic syndrome associated with poor prognosis and high healthcare costs, mainly due to frequent hospitalizations. These are preceded by changes in several physiological parameters, creating an opportunity for early detection and treatment of hemodynamic unbalance, and hospitalizations avoidance. Invasive telemonitoring systems have proven efficacy on this detection. Conversely, non-invasive systems need further evidence, mainly due to poor technological adherence.

Introduction and Objectives: Dilated cardiomyopathy (DCM) is a myocardial disease that can progress to a terminal stage, requiring heart transplantation. In this work we aim to contribute to knowledge of genetic variants in adult patients undergoing heart transplantation due to end-stage DCM, reporting the results obtained in our single-center tertiary hospital series using target next-generation sequencing (NGS). Methods and Results: Genetic variants were screened in 15 genes, preselected based on variants previously identified in DCM patients. Thirteen unrelated patients were included, nine (69%) male, mean age at diagnosis 33±13 years, eight (62%) with familial DCM. Nine genetic variants were identified in six (46%) patients: five in LMNA, two in LBD3, one in TNNT2 and one in TCAP. These variants were new in most patients. The majority were classified as of uncertain significance. Two patients were double and triple heterozygotes in the LBD3 and LMNA genes, respectively. Conclusion: Our results highlight the potential of NGS in the genetic characterization of DCM patients. LMNA is one of the most frequently mutated genes and should be included in all target gene assessments of end-stage DCM patients until more data are available. Resumo: Introdução e objetivos: A miocardiopatia dilatada é uma doença miocárdica que pode evoluir para um estádio terminal, requerendo transplante cardíaco. Neste trabalho, pretendemos contribuir para o conhecimento das variantes genéticas presentes em pacientes adultos submetidos a transplante cardíaco, descrevendo os resultados obtidos utilizando técnicas de sequenciação de ADN de nova geração. Métodos e resultados: Variantes genéticas foram pesquisadas em 15 genes pré-selecionados com base em variantes previamente identificadas em pacientes com miocardiopatia dilatada. Foram incluídos 13 pacientes não relacionados, nove (69%) do sexo masculino, com idade média na altura do diagnóstico de 33±13 anos, oito (62%) com doença familiar. Foram identificadas nove variantes genéticas em seis (46%) pacientes: LMNA-5, LBD3-2, TNNT2-1 e TCAP- 1. A maioria das variantes genéticas foi classificada como de significado incerto. Dois pacientes eram heterozigotos duplos e triplos nos genes LBD3 e LMNA, respetivamente. Conclusões: Os nossos resultados reforçam o potencial das novas tecnologias de sequenciação na caracterização genética de doentes com miocardiopatia dilatada. Até que mais dados estejam disponíveis, o gene LMNA é um dos mais frequentemente envolvidos e deverá ser incluído na avaliação de pacientes com miocardiopatia dilatada em fase terminal. Keywords: Dilated cardiomyopathy, Heart transplantation, Next-generation sequencing, Genetic variants, Palavras-chave: Miocardiopatia dilatada, Transplante cardíaco, Sequenciação de nova geração, Variantes genéticas

Resumo: O crescente uso de antraciclinas, aliado ao aumento da sobrevida dos doentes oncológicos, motiva a necessidade de monitorizar os efeitos tóxicos destes fármacos. Para que a sua cardiotoxicidade possa ser detetada, prevenida ou atenuada, torna‐se essencial que todos os doentes sejam, do ponto de vista cardiovascular, submetidos a uma rigorosa avaliação inicial e a um estreito acompanhamento. Diversos ensaios clínicos comprovaram o efeito cardioprotetor produzido por medidas não farmacológicas como o exercício físico, a adoção de um estilo de vida saudável, o controlo de fatores de risco e o tratamento de comorbilidades; foi também verificado um efeito cardioprotetor com estratégias farmacológicas como o uso de bloqueadores‐beta, inibidores da enzima de conversão da angiotensina, antagonistas do recetor da angiotensina, estatinas, dexrazoxane ou derivados lipossomais. No entanto, atualmente não existe qualquer diretriz científica que oriente as estratégias de prevenção nestes doentes. Com esta revisão propomo‐nos abordar o estado da arte relativo à avaliação, monitorização e, principalmente, à prevenção da cardiotoxicidade provocada pelas antraciclinas. Abstract: The increasing use of anthracyclines, together with the longer survival of cancer patients, means the toxic effects of these drugs need to be monitored. In order to detect, prevent or mitigate anthracycline‐induced cardiomyopathy, it is essential that all patients undergo a rigorous initial cardiovascular assessment, followed by close monitoring. Several clinical trials have shown the cardioprotective effect of non‐pharmacological measures such as exercise, healthy lifestyles, control of risk factors and treatment of comorbidities; a cardioprotective effect has also been observed with pharmacological measures such as beta‐blockers, angiotensin‐converting enzyme inhibitors, angiotensin receptor antagonists, statins, dexrazoxane and liposomal formulations. However, there are currently no guidelines for managing prevention in these patients. In this review the authors discuss the state of the art of the assessment, monitoring, and, above all, the prevention of anthracycline‐induced cardiotoxicity. Palavras‐chave: Antraciclinas, Cardiotoxicidade, Prevenção, Quimioterapia, Keywords: Anthracyclines, Cardiotoxicity, Prevention, Chemotherapy

Introduction: Cardiac remodeling is manifested as changes in size, shape and function of the heart. We studied the prevalence, prognosis and predictors of left ventricular reverse remodeling (LVRR) in idiopathic dilated cardiomyopathy (IDCM) after optimized medical therapy. Methods: A total of 113 IDCM patients were followed for 7.1±5.6 years. LVRR was defined as an increase of 10 units in ejection fraction (EF) and decrease in left ventricular diastolic diameter (LVDD), in the absence of resynchronization therapy. Results: Baseline EF was 27±8% and LVDD index was 37.1±6.3 mm/m2. LVRR occurred in 34.5% within 22.6 months. Final EF was 47.5±10.1%, LVDD index was 30.2±3.9 mm/m2. LVRR was associated with better NYHA class (I–II) and lower BNP (p

Young athletes are considered the healthiest group in society. Although rare, there are still reports of sudden death or cardiac arrest on the playing fields. Clinical evaluation is of paramount importance for the identification of possible pathological states that confer increased risk of these events. Interpretation of the electrocardiogram of young athletes can help identify changes associated with heart disease that might preclude the participation in sports. In this context, it is essential to recognize the electrocardiographic patterns that represent the structural and electrical remodeling resulting from continued adaptation to exercise, and which thus do not increase the risk of adverse events during exercise. The European Society of Cardiology (ESC) and the American Heart Association (AHA) have issued consensus documents summarizing which electrocardiographic abnormalities should be considered ‘physiological’, resulting from adaptation to exercise (‘athlete's heart’), and which should be considered pathological and thus require further study. However, the two societies have different approaches with respect to the electrocardiographic screening of athletes. This paper provides a brief review of current evidence regarding the electrocardiographic findings considered normal and abnormal in athletes, and presents the arguments of the ESC and AHA for electrocardiographic screening in this population. Resumo: Os jovens atletas são considerados como o grupo mais saudável da sociedade. Apesar de raros, surgem ainda relatos de morte súbita ou paragem cardiorrespiratória nos campos de jogo. A avaliação clínica reveste-se de primordial importância para a identificação de eventuais estados patológicos que confiram risco acrescido destes eventos. A interpretação do eletrocardiograma destes jovens atletas pode permitir identificar alterações associadas a patologia cardíaca que condicione ou não a prática do exercício físico. Neste contexto, torna-se essencial reconhecer os padrões eletrocardiográficos que representam a remodelagem estrutural e elétrica resultantes da adaptação ao exercício continuado, e que por sua vez não acarretam risco acrescido de eventos adversos durante a prática de exercício. As Sociedades Europeia e Americana de Cardiologia emitiram documentos de consenso que procuram resumir quais as alterações eletrocardiográficas consideradas como «fisiológicas» e resultantes da adaptação ao exercício (coração de atleta) e quais as alterações consideradas como patológicas e que portanto implicam o estudo subsequente dos casos em que são identificadas. Estas sociedades apresentam, no entanto uma abordagem diferente no que se refere ao rastreio eletrocardiográfico de atletas. Este artigo fornece uma breve revisão da evidência atual referente aos achados eletrocardiográficos considerados como normais e anormais em atletas, assim como apresenta as argumentações das Sociedades Europeia e Americana de Cardiologia referentes ao rastreio eletrocardiográfico nesta população. Keywords: Athlete's heart, Electrocardiographic screening, Sudden death, Palavras-chave: Coração de atleta, Rastreio de ECG, Morte súbita

Cardiac rhythm disturbances are common in sleep apnea patients. The authors report on a 74-year-old female patient with atrial fibrillation, with a background history of arterial hypertension, obesity, and obstructive sleep apnea. Holter monitoring was performed, on 5 different occasions. Pauses were seen in every Holter monitoring, the number of which varied between 10 and 72. All pauses occurred during the sleeping period and adjacent hours (23:00–8:59 hours) with a single exception. In this patient, we can speculate that parasympathetic may predominate over sympathetic activity during sleep.

The electrocardiogram (ECG) is a diagnostic test commonly used in daily Primary Care practice. General Practitioners (GP) often feel unsure about their interpretation of ECGs, so they engage external services to provide it. To evaluate accuracy of ECG readings done by GPs by comparison with those done by a cardiologist as the gold standard. We studied 195 ECGs collected consecutively during first semester of 2010 in an urban Health Centre of Portugal. Each ECG was read by each physician and inter-observer agreement was evaluated. After coding by Novacode, sensitivity and specificity of GP’s readings were calculated. Inter-observer agreement between GP readings was “good” with an intraclass correlation coefficient of 0.727 (CI 95%: 0.670–0.779). When compared with gold standard, GP achieved a “good” agreement with an intraclass correlation coefficient of 0.712 (CI 95%: 0.659–0.762). The overall accuracy of GP for detecting abnormalities was 81.0% (95%CI: 75.7–85.6%), with a sensitivity of 84.8% (95%CI: 77.3–90.6%) and a specificity of 77.5% (95%CI: 69.7–84.2%). For normal tests, accuracy was 79.9% (95%CI: 74.7–84.3). In the most prevalent classes of abnormalities, accuracy was higher than 90%. GP showed good skills in reading ECGs in their practice of Primary Care. Better attention should be given to ischemic abnormalities present on ECGs. Key message: General Practitioners demonstrate good skills for reading the ECGs of patients on a primary care centre when compared to the gold standard defined by a cardiologist reading.

Objective: To assess the prognostic significance of iron deficiency (ID) in a chronic heart failure (CHF) outpatient population. Methods and Results: We prospectively evaluated 127 patients with stable CHF and left ventricular ejection fraction ≤45%. Clinical and analytical data as well as information regarding the occurrence of the composite endpoint of overall mortality and nonfatal cardiovascular events were assessed. Among the 127 patients enrolled [81% men, median age: 62 years (25th-75th percentile: 53-68)], 46 (36%) patients had ID. Women, patients with higher plasma brain natriuretic peptide levels (>400 pg/ml) and with right ventricular systolic dysfunction presented ID more frequently (p < 0.05 for all). At 225 ± 139 days of follow-up, the composite endpoint occurred in 15 (12%) patients. It was more frequent in ID (24 vs. 5%, p = 0.001) and anemic patients (25 vs. 8%, p = 0.014). In a Cox regression analysis, ID was associated with a higher likelihood of composite endpoint occurrence (HR 5.00, 95% CI 1.59-15.78, p = 0.006). In a multivariable analysis adjusted for clinical variables, including the presence of anemia, ID remained a significant predictor of the composite endpoint (HR 5.38, 95% CI 1.54-18.87, p = 0.009). Conclusion: In a CHF outpatient population, ID carried a higher risk of unfavorable outcome, irrespectively of the presence of anemia.

Familial dilated cardiomyopathy (FDCM) is characterized by genetic heterogeneity, incomplete age-dependent penetrance, and a multifactorial pathogenesis ([1][1]). Diagnosis is still dependent on clinical criteria and familial investigation ([2][2]). On the other hand, several abnormalities have been

The authors present a case report of successful treatment of pulmonary artery perforation by endovascular stent graft implantation. Resumo: Os autores descrevem um caso de sucesso do tratamento da rotura da artéria pulmonar com a implantação de um stent endovascular. Keywords: Swan-Ganz catheterization, Hemoptysis, Palavras-chave: Cateterização Swan-Ganz, Hemoptises

Calcium plays a key role in heart muscle contraction and relaxation. Hypocalcemic heart failure is a rare and potentially reversible disturbance, which reflects this intrinsic relationship.The authors present the case of a 35-year-old woman who developed acute heart failure during the early postoperative period following total thyroidectomy. The echocardiogram showed severe global left ventricular dysfunction. Laboratory tests showed severe hypocalcemia and new-onset hypoparathyroidism. Cardiac catheterization showed angiographically normal coronary arteries. After clinical, hemodynamic and metabolic stabilization, a repeat echocardiogram revealed recovery of left ventricular function. Subsequently, cardiac magnetic resonance imaging was performed, which also showed no alterations. The patient was discharged asymptomatic, medicated with calcium carbonate, calcitriol and levothyroxine.This case highlights the importance of considering hypocalcemia as a cause of reversible myocardial dysfunction. Resumo: O cálcio assume um papel fundamental na contração e relaxamento do músculo cardíaco. A insuficiência cardíaca hipocalcémica é um distúrbio raro e potencialmente reversível, que traduz esta relação intrínseca.Os autores apresentam o caso de uma mulher de 35 anos que desenvolveu um quadro de insuficiência cardíaca aguda, durante o pós-operatório precoce de tiroidectomia total. O ecocardiograma mostrou disfunção global grave do ventrículo esquerdo. Analiticamente apresentava hipocalcemia grave e hipoparatiroidismo de novo. O cateterismo cardíaco mostrou artérias coronárias angiograficamente normais. Após estabilização clínica, hemodinâmica e metabólica, repetiu ecocardiograma que revelou recuperação da função ventricular esquerda. Posteriormente, realizou ressonância magnética cardíaca que também não mostrou alterações. A doente teve alta assintomática, medicada com carbonato de cálcio, calcitriol e levotiroxina. Este caso salienta a importância de considerar a hipocalcemia como uma causa reversível de disfunção miocárdica. Keywords: Acute heart failure, Hypocalcemia, Hypoparathyroidism, Palavras-chave: Insuficiência cardíaca aguda, Hipocalcemia, Hipoparatiroidismo

Resumo: O cálcio assume um papel fundamental na contração e relaxamento do músculo cardíaco. A insuficiência cardíaca hipocalcémica é um distúrbio raro e potencialmente reversível, que traduz esta relação intrínseca.Os autores apresentam o caso de uma mulher de 35 anos que desenvolveu um quadro de insuficiência cardíaca aguda, durante o pós-operatório precoce de tiroidectomia total. O ecocardiograma mostrou disfunção global grave do ventrículo esquerdo. Analiticamente apresentava hipocalcemia grave e hipoparatiroidismo de novo. O cateterismo cardíaco mostrou artérias coronárias angiograficamente normais. Após estabilização clínica, hemodinâmica e metabólica, repetiu ecocardiograma que revelou recuperação da função ventricular esquerda. Posteriormente, realizou ressonância magnética cardíaca que também não mostrou alterações. A doente teve alta assintomática, medicada com carbonato de cálcio, calcitriol e levotiroxina. Este caso salienta a importância de considerar a hipocalcemia como uma causa reversível de disfunção miocárdica. Abstract: Calcium plays a key role in heart muscle contraction and relaxation. Hypocalcemic heart failure is a rare and potentially reversible disturbance, which reflects this intrinsic relationship.The authors present the case of a 35-year-old woman who developed acute heart failure during the early postoperative period following total thyroidectomy. The echocardiogram showed severe global left ventricular dysfunction. Laboratory tests showed severe hypocalcemia and new-onset hypoparathyroidism. Cardiac catheterization showed angiographically normal coronary arteries. After clinical, hemodynamic and metabolic stabilization, a repeat echocardiogram revealed recovery of left ventricular function. Subsequently, cardiac magnetic resonance imaging was performed, which also showed no alterations. The patient was discharged asymptomatic, medicated with calcium carbonate, calcitriol and levothyroxine. This case highlights the importance of considering hypocalcemia as a cause of reversible myocardial dysfunction. Palavras-chave: Insuficiência cardíaca aguda, Hipocalcemia, Hipoparatiroidismo, Keywords: Acute heart failure, Hypocalcemia, Hypoparathyroidism

Resumo: Os autores apresentam um caso clínico de um homem de 68 anos com clínica de insuficiência cardíaca predominantemente direita, no contexto de estenose aórtica grave associada a hipertensão pulmonar. Concomitantemente, foi diagnosticada anemia que, após estudo endoscópico, se concluiu ser secundária a angiodisplasias intestinais, tendo sido feito o diagnóstico de síndrome de Heyde.Após cirurgia de substituição valvular houve resolução do quadro, com melhoria dos sintomas de insuficiência cardíaca prévia e estabilização dos valores de hemoglobina.Com este caso pretende‐se mostrar a necessidade do conhecimento de associações menos frequentes na estenose aórtica grave para uma atuação terapêutica imediata e adequada. Abstract: The authors present the case of a 68‐year‐old man with predominantly right heart failure in the context of severe aortic stenosis associated with pulmonary hypertension. Anemia was diagnosed which, after endoscopic study, was considered to be secondary to angiodysplasia and a diagnosis of Heyde syndrome was made.After valve replacement surgery the patient's heart failure improved and hemoglobin levels stabilized.We present this case to show the need to recognize less common associations of severe aortic stenosis, in order to provide immediate and appropriate treatment. Palavras‐chave: Estenose aórtica grave, Hemorragia gastrointestinal, Angiodisplasias, Síndrome de Heyde, Hipertensão pulmonar, Keywords: Severe aortic stenosis, Gastrointestinal bleeding, Angiodysplasia, Heyde syndrome, Pulmonary hypertension

The authors present the case of a 68-year-old man with predominantly right heart failure in the context of severe aortic stenosis associated with pulmonary hypertension. Anemia was diagnosed which, after endoscopic study, was considered to be secondary to angiodysplasia and a diagnosis of Heyde syndrome was made. After valve replacement surgery the patient's heart failure improved and hemoglobin levels stabilized.We present this case to show the need to recognize less common associations of severe aortic stenosis, in order to provide immediate and appropriate treatment. Resumo: Os autores apresentam um caso clínico de um homem de 68 anos com clínica de insuficiência cardíaca predominantemente direita, no contexto de estenose aórtica grave associada a hipertensão pulmonar. Concomitantemente, foi diagnosticada anemia que, após estudo endoscópico, se concluiu ser secundária a angiodisplasias intestinais, tendo sido feito o diagnóstico de síndrome de Heyde.Após cirurgia de substituição valvular houve resolução do quadro, com melhoria dos sintomas de insuficiência cardíaca prévia e estabilização dos valores de hemoglobina.Com este caso pretende-se mostrar a necessidade do conhecimento de associações menos frequentes na estenose aórtica grave para uma atuação terapêutica imediata e adequada. Keywords: Severe aortic stenosis, Gastrointestinal bleeding, Angiodysplasia, Heyde syndrome, Pulmonary hypertension, Palavras-chave: Estenose aórtica grave, Hemorragia gastrointestinal, Angiodisplasias, Síndrome de Heyde, Hipertensão pulmonar

Post-cardiac injury syndrome (PCIS) is an inflammatory process involving the pericardium secondary to cardiac injury. It can develop after cardiac trauma, cardiac surgery, myocardial infarction, and, rarely, after certain intravascular procedures. We report a rare case of an iatrogenic cardiac rupture followed by PCIS with delayed inflammatory pericardial effusion after pacemaker implantation. A comprehensive literature review on this topic is provided. Resumo: A síndrome pós-lesão cardíaca (SPLC) corresponde a um processo inflamatório envolvendo o pericárdio, secundário à lesão cardíaca. Pode desenvolver-se após traumatismo cardíaco, cirurgia cardíaca, enfarte agudo do miocárdio, e, raramente, após alguns procedimentos intravasculares. Os autores apresentam o caso invulgar de uma rotura cardíaca iatrogénica após implantação de um pacemaker, seguida pelo desenvolvimento de um derrame pericárdico retardado, inflamatório, correspondendo a SPLC. A propósito do referido caso clínico, é efetuada uma revisão compreensiva da literatura acerca desta entidade. Keywords: Post-cardiac injury syndrome, Cardiac rupture, Pacemaker implantation, Palavras-chave: Síndrome pós-lesão cardíaca, Rotura cardíaca, Implantação de pacemaker

We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

However, the clinical manifestations and prognosis of biventricular arrhythmogenic cardiomyopathy not yet been established. We present a case of ARVC with left ventricle (LV) involvement, with no typical symptoms and no relevant medical history.

Familial dilated cardiomyopathy (FDCM) is characterized by clinical and genetic heterogeneity. There are still few survival studies concerning this subgroup of patients.To determine the prognosis of patients with FDCM on optimal medical therapy and attending a heart failure clinic.This is a prospective study including patients with FDCM, defined according to the guidelines of the European Society of Cardiology. Cardiovascular morbidity and all-cause mortality were evaluated.Thirty-six patients, 23 (64%) men, were followed for 3.8 +/- 2.5 years. Age at baseline was 42 +/- 14 years and 67% were in NYHA class II. In 22% heart failure symptoms first occurred after a respiratory infection, and in 6%, after pregnancy/delivery. Most patients were in sinus rhythm (89%) and 33% had left bundle branch block (LBBB). Baseline left ventricular (LV) ejection fraction was 28 +/- 9%, LV end-diastolic diameter was 68 +/- 8 mm and left atrial dimension was 46 +/- 9 mm. Baseline serum sodium was 140 +/- 3 mEq/l. All patients were taking angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin-II receptor blockers (ARBs), 81% beta-blockers and 47% spironolactone. During follow-up, 5 patients died, 4 underwent heart transplantation and one received an implantable cardioverter-defibrillator. Five-year survival was 68%.Five-year survival of our patients with FDCM, under optimal medical therapy, was similar to that of other forms of nonischemic DCM reported in the literature.

The pathological significance of myocardial adrenergic activity in patients with heart failure is well documented. No previous study has assessed the usefulness of I123-metaiodobenzylguanidine (123I-MIBG) cardiac uptake imaging for the evaluation of familial dilated cardiomyopathy (DCM).To evaluate cardiac adrenergic activity, using 123I-MIBG cardiac uptake imaging, in members of a genotyped family with DCM.Clinical evaluation, 12-lead ECG, 2D echocardiogram, heart rate variability analysis by 24h Holter, plasma B-type natriuretic peptide (BNP) measurements and 123I-MIBG cardiac imaging were performed in all participants. Anterior projection planar images and single photon emission computed tomographies of the thorax were obtained 20 min and 4 hours after the intravenous administration of 370 MBq of 123I-MIBG (early and late images). Heart/mediastinal (H/M) ratio and myocardial washout (MW) rate were obtained based on the anterior planar images. In polar maps, segmental uptake of 123I-MIBG was evaluated using a 4-grade visual score: grade 1 - uptake75% of maximum myocardial uptake (MMU); grade 2 - uptake 51-75% of MMU; grade 3 - uptake 26-50% of MMU; grade 4 - uptakeor = 25% of MMU.Eleven adults were included: 4 with DCM, 4 with isolated left ventricular enlargement (LVE), and 3 with normal echocardiogram. Patients with DCM and LVE presented higher MW rates, lower H/M ratios and higher visual score grades than those with normal 2D echocardiograms. One patient with a normal echocardiogram but carrying the disease locus also presented an abnormal MIBG cardiac scintigram.Patients with the phenotypic expression of the disease (DCM and LVE) and even carriers of the DCM gene with normal echocardiograms may present an abnormal MIBG cardiac scintigram, probably reflecting cardiac adrenergic hyperactivity. If confirmed in larger numbers, this method may be useful for the evaluation of DCM families.

Cardiac transplantation is the gold standard therapy for patients below 60 years presenting with severe heart failure (HF) despite maximal medical therapy, who have no other surgical option and no contraindications to this procedure. We evaluated our experience with this important form of heart failure therapy. Between February 1987 and December 2002, 32 patients, aged 37 +/- 16 years, 19 males, with ejection fraction of 18 +/- 7%, underwent heart transplantation in our center. Seven (22%) patients were in NYHA class IV with hemodynamic support. Seventeen (53%) patients had idiopathic dilated cardiomyopathy (DCM), 7 (22%) had ischemic DCM, 3 (9%) had valvular DCM and the remainder had other causes of left ventricular dysfunction. Overall survival rate was 68% at first year post-transplantation, 59% at 5 years and 59% at 10 years. One year after cardiac transplantation, 95% of patients were in NYHA class I and the rest were in NYHA class II. Among the 13 patients who died, in five (18%) death occurred during the first month: the most frequent cause was hemodynamic failure. Causes of late death were: allograft vasculopathy (n = 3), allograft rejection (n = 1), infection (n = 1), sudden death (n = 1), hemodynamic failure (n = 1) and bradyarrhythmia (n = 1). Among the patients followed for more than one year, only three died. Early complications were: infection (8 episodes, 7 of respiratory location), right heart failure (3 patients), pericardial effusion (5 patients) and others (7 patients). Late complications were: a) allograft rejection: 17 (53%) patients, 72 episodes (10 ISHLT grade 3, 6 of whom were treated with intravenous corticotherapy, 8 grade 2 and 54 grade 1); b) infections: 19 (59%) patients; 35 episodes, 25 requiring hospitalization: 10 (28%) involving the respiratory tract, 6 (17%) the oropharynx, 5 (14%) the urinary tract, 4 (11%) the skin and 10 (28%) of undetermined location; c) chronic allograft rejection: 6 (19%) patients; d) arterial hypertension: 14 (45%) patients; d) renal failure: 5 (16%) patients; e) diabetes: 2 (6%) patients; f) cancer: 2 (6%) patients. Patients with severe heart failure and a very poor prognosis who underwent cardiac transplantation in our hospital showed marked improvement in functional capacity and quality of life and had an overall survival similar to the results of international heart transplantation registries. Complications during follow-up were similar to those usually described in the literature.

The autonomic nervous system (ANS), a complex and self-organized entity, plays a crucial role in cardiovascular regulation. In this review, the authors describe some concepts of anatomo-physiology and cellular/molecular biology of the ANS and of its two major components, the parasympathetic and sympathetic system, both in the normal individual and in the patient with heart failure. Adrenergic and cholinergic neurotransmission, neurotransmitters, adrenergic and cholinergic receptors and intracellular signal transduction are discussed. Some aspects of sympathetic-parasympathetic interaction at the cellular level are described as well as the opposite role of two cyclic nucleotides, cyclic adenosine and guanosine monophosphate. Major emphasis is placed on the beta-adrenergic system, the combination of the beta receptor, G protein complex and adenylyl cyclase. The activation of neurohumoral compensatory mechanisms in heart failure is discussed as well as the increasingly recognized importance of sympathetic overactivation and parasympathetic tonus reducting in this setting. Cellular mechanisms of adrenergic stimulation in heart failure and its consequences are presented, with special focus on down-regulation of beta-1 adrenergic receptors; the authors analyze the steps of phosphorlation and oncoupling, sequestration, internalization and lysosomal degradation, the main result of which is reduced response to catecholamines.

Dissertação de Mestrado em Ciências do Desporto, especialização em Gestão Desportiva, apresentada à Faculdade de Ciências do Desporto e de Educação Física da Universidade do Porto

To evaluate the medium term results of surgical closure of ventricular septal defect (VSD) performed during the first year of life, using echocardiography (echo).We studied prospectively 29 patients aged from 17 to 68 months (mean = 37) who underwent surgical closure of perimembranous VSD during the first year of life. The postoperative follow-up time ranged from 6 to 60 months (mean = 26). Fifteen age-matched normal children were used as a control group in evaluating the left ventricular (LV) systolic function. The echo study included: 1) the assessment of LV systolic function using the shortening fraction, ejection fraction, pre-ejection to ejection time ratio (PET/ET), aortic flow acceleration time, acceleration to ejection time ratio, mean aortic flow acceleration; 2) detection and quantification of residual VSD as well as tricuspid and or aortic regurgitation; 3) determination of right ventricular systolic pressure (RVSP). The RVSP was evaluated from the maximum flow velocity from a residual VSD or tricuspid regurgitation, using the simplified Bernoulli equation.The LV systolic function parameters from patients versus (vs) normals showed a significantly different shortening fraction (34 +/- 5 vs 39 +/- 4; p = 0.005) and PET/ET ratio (0.34 +/- 0.04 vs 0.31 +/- 0.03; p = 0.02). None of the other studied parameters was significantly different from normal. Five (17%) patients had a small residual VSD. Two (7%) patients had mild aortic regurgitation. Tricuspid regurgitation was detected in 23 (79%) patients being trivial in 20 and mild to moderate in 3. The RVSP was quantified in 22 (76%) patients, ranging from 30 to 45 mmHg (mean +/- SD = 36 +/- 4). None of the remaining 7 patients showed changes in the end-systolic interventricular septal configuration.Our study shows that surgical closure of perimembranous VSD performed during the first year of life was possible without significant mobility. Furthermore, the echo allowed a complete and noninvasive cardiac evaluation of the anatomic, functional and hemodynamic status of this group of patients, thus limiting the need for postoperative control cardiac catheterization.

Diltiazem is a calcium channel blocker whose effects on left ventricular function (LVF) are controversial. We studied 12 patients with ischemic heart disease (IHD) before starting and 15 and 30 days after having initiated Diltiazem 60 mg t.i.d. LVF was accessed by means of the normalized indexes of the calibrated apexcardiogram: nS for systolic LVF evaluation and nA for diastolic LVF evaluation. Recent works have shown that these indexes have a good correlation with invasive parameters of LVF. We verified that nS did not change and nA decreased significantly (p0.05) after Diltiazem. We preliminary concluded that Diltiazem has no deleterious effect on LV systolic function and improves LV diastolic function, by decreasing nA, a parameter which correlates well with LV end diastolic pressure.