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Ablation of premature ventricular contractions (PVCs), relies mostly on a detailed activation mapping. This can be impossible to achieve in case of paucity or even absence of PVCs during the procedure. Pacemapping as an alternative has many limitations. We present a case of a patient with very frequent symptomatic PVCs, that on the day of the procedure had total absence of PVCs. We performed successful ablation based exclusively on electrocardiographic imaging confirmed by substrate mapping.

Cardiomyopathies (CM) are a heterogeneous group of muscle heart diseases, divided into 3 main categories (dilated, hypertrophic, and restrictive). In addition to these subgroups, athlete's heart and hypertensive cardiopathy are both the result of heart adaptation to increased loading conditions, making it possible to include them in the CM group. Right heart involvement is clear in some CM as arrhythmogenic CM, carcinoid syndrome, and endomyocardial fibrosis, whereas in others, like hypertrophic or dilated CM, it is known that the right heart has a prognostic impact but less clear is its pathogenic role.

Subaortic septal myectomy is usually performed to mitigate obstruction in patients with the obstructive form of hypertrophic cardiomyopathy (HCM) or in those with congenital subaortic stenosis. Moreover, it is combined with aortic valve replacement in patients with severe aortic valve stenosis (SAS) and asymmetrical septal hypertrophy causing concomitant left ventricular outflow tract obstruction. When both conditions coexist, it is conceptually difficult to identify a cardiomyopathy beyond an adaptive myocardial hypertrophy, strictly related to pressure overload. Myectomy histopathology might be useful to enlighten the cause of the obstruction and establish the diagnosis.The aim was to describe the pathological findings of surgical septal myectomy specimens obtained from a group of patients with diverse clinical diagnosis, including HCM, severe aortic stenosis, and asymmetrical septal hypertrophy.This was a retrospective study of 56 patients undergoing septal myectomy along a 10-year period at a tertiary cardiac surgical center. Clinical, interventional, and anatomopathological findings between patients with and without a preoperative diagnosis of HCM were analyzed and compared.Mean age at intervention was 67.5±20.5 years; 37 (66.1%) were female Preoperative diagnosis of sarcomeric obstructive HCM was assumed in 23 (41.1%) patients. All the other patients (58.9%) were referred for surgery with preoperative diagnosis of asymmetric septal hypertrophy, mainly in the context of severe aortic stenosis (24 patients). Twenty-seven (48.2%) patients had a greater than 30 mmHg intraventricular gradient at rest. Patients with presumed HCM were significantly younger (56.5±15.8 vs. 70.2±13.3 years, P.001), had higher prevalence of significant intraventricular obstruction at rest [20 (87.0%) vs. 8 (34.8%), P.001], and more frequently had moderate or severe mitral regurgitation [9 (39.1%) vs. 5(15.1%), P=.043]. All patients with aortic valve stenosis underwent both aortic valve replacement and septal myectomy. Twelve (52.1%) of the patients with obstructive HCM had isolated septal myectomy, while in the remaining 11, the procedure was combined with intervention on the mitral valve. Histopathological final diagnosis was of nonspecific reactive myocardial hypertrophy in all but 4 (92.2%) patients. In those, 2 (3.6%) had the final diagnosis of HCM and 2 (3.6%) the diagnosis of congenital subaortic membranous stenosis with reactive myocardial hypertrophy. Different grades of subendocardial fibroelastosis and myocardial fibrosis, mainly interstitial, were present [27 (48.2%) and 18 (32%) patients, respectively]. When microscopic data were compared between patients with or without a preoperative clinical diagnosis of HCM, no significant differences were found.In patients submitted to surgical septal myectomy, histology was mostly indistinctive among different clinical entities. Since different myocardial hypertrophy etiologies may share similar pathological expression, there is a need for detailed clinical assessment when trying to define the best strategy for clinical management.

Current guidelines recommend the use of the Modified Diamond-Forrester (MDF) method to assess the pre-test likelihood of obstructive coronary artery disease (CAD). We aimed to compare the performance of the MDF method with two contemporary algorithms derived from multicenter trials that additionally incorporate cardiovascular risk factors: the calculator-based 'CAD Consortium 2' method, and the integer-based CONFIRM score.We assessed 1069 consecutive patients without known CAD undergoing coronary CT angiography (CCTA) for stable chest pain. Obstructive CAD was defined as the presence of coronary stenosis ≥50% on 64-slice dual-source CT. The three methods were assessed for calibration, discrimination, net reclassification, and changes in proposed downstream testing based upon calculated pre-test likelihoods.The observed prevalence of obstructive CAD was 13.8% (n=147). Overestimations of the likelihood of obstructive CAD were 140.1%, 9.8%, and 18.8%, respectively, for the MDF, CAD Consortium 2 and CONFIRM methods. The CAD Consortium 2 showed greater discriminative power than the MDF method, with a C-statistic of 0.73 vs. 0.70 (p0.001), while the CONFIRM score did not (C-statistic 0.71, p=0.492). Reclassification of pre-test likelihood using the 'CAD Consortium 2' or CONFIRM scores resulted in a net reclassification improvement of 0.19 and 0.18, respectively, which would change the diagnostic strategy in approximately half of the patients.Newer risk factor-encompassing models allow for a more precise estimation of pre-test probabilities of obstructive CAD than the guideline-recommended MDF method. Adoption of these scores may improve disease prediction and change the diagnostic pathway in a significant proportion of patients.

Objective: To evaluate the performance of traditional cardiovascular (CV) risk factors in identifying a higher than expected coronary atherosclerotic burden. Methods: We assessed 2069 patients undergoing coronary CT angiography, with assessment of calcium score (CS), for suspected coronary artery disease. A higher than expected atherosclerotic burden was defined as CS >75th percentile (CS >P75) according to age and gender-adjusted monograms. The ability of traditional CV risk factors to predict a CS >P75 was assessed in a customized logistic regression model (“Clinical Score”) and by the calculation of SCORE (Systemic Coronary Risk Evaluation). The population attributable risk (PAR) of risk factors for CS >P75 was calculated. Results: The median CS was 3.0 (IQR 0.0–98.0); 362 patients had CS >P75. The median SCORE was 3.0 (IQR 1.0–4.0). With the exception of hypertension, all traditional CV risk factors were independent predictors of CS >P75: diabetes, dyslipidemia, smoking and family history (OR 1.3–2.2, p≤0.026). The areas under the ROC curves for CS >P75 were 0.64 for the Clinical Score (95% CI 0.61–0.67, pP75 were in the two lower quartiles of the Clinical Score. Altogether, the traditional risk factors explain 56% of the prevalence of CS >P75 (adjusted PAR 0.56). Conclusion: Despite the association of CV risk factors with a higher than expected atherosclerotic burden, they appear to explain only half of its prevalence. Even when integrated in scores, the predictive power of these risk factors was modest, exposing the limitations of risk stratification based solely on demographic and clinical risk factors. Resumo: Objetivo: O objetivo deste trabalho foi avaliar o desempenho dos fatores de risco cardiovascular (CV) clássicos na identificação de carga aterosclerótica superior ao esperado. Métodos: Avaliámos 2069 doentes (dts) que realizaram AngioTC cardíaca e ScCa para exclusão de doença coronária. Definiu-se carga aterosclerótica superior ao esperado um ScCa acima do percentil 75 (ScCa>p75) de acordo com nomogramas ajustados para o sexo e idade. A capacidade dos fatores de risco clássicos preverem ScCa>p75 avaliou-se num modelo de regressão logística customizado (score clínico) e pelo SCORE. Avaliou-se o Population Attributable Risk (PAR) dos fatores de risco para ScCa>p75. Resultados: A mediana de ScCa foi 3,0 [IIQ 0,0-98,0]; 362 dts com ScCa>p75. A mediana do HeartScore foi 3,0 [IIQ 1,0-4,0]. Exceto a hipertensão arterial, todos os fatores de risco foram preditores independentes de CaSc>p75: diabetes mellitus, dislipidemia, tabagismo e história familiar (OR 1,3-2,2, p≤0,026). As áreas abaixo da curva ROC para SaCa>p75 foram 0,64 para score clínico (IC95% 0,61-0,67; pp75 encontravam-se nos dois quartis de score clínico mais baixos. No seu conjunto, os fatores de risco clássicos explicam 56% da prevalência de ScCa>p75 (PAR ajustado 0,56). Conclusão: Apesar de os fatores de risco CV se associarem a uma carga aterosclerótica superior ao esperado, justificam pouco mais de metade da sua prevalência. O poder preditor destes fatores de risco é modesto, mesmo integrados em scores, revelando as limitações da estratificação de risco baseada apenas em dados demográficos e fatores de risco clínicos. Keywords: Atherosclerotic burden, Risk factor, Attributable risk, Calcium score, CT angiography, Palavras-chave: Carga Aterosclerótica, Fator de risco, Score de cálcio, AngioTC

The prevalence, complexity, clinical importance, heterogeneity and unpredictability of inherited cardiovascular diseases make the development of inherited cardiovascular disease centers an inevitability, with the ultimate goal of reducing the morbidity and mortality associated with these conditions. An inherited cardiovascular disease center may be seen as a subunit of a cardiology department, with health professionals specializing in these types of disorders, organized to provide excellence in all related areas, including diagnosis, treatment, follow-up, prevention, risk stratification and prognosis. Among its objectives are the development of action protocols and the creation of databases that enable patients to be included in national and international research networks. To achieve these objectives these centers should include functional units of clinical and basic sciences, research, training and education, acting in harmony in a holistic approach to patients and their families. As most experience on inherited cardiovascular diseases is based on hypertrophic cardiomyopathy and on “hypertrophic cardiomyopathy centers”, these centers represent an excellent opportunity to learn how to set up inherited cardiovascular disease centers. European centers will differ from country to country, reflecting the heterogeneity of national health systems, but will share a common core, presented in this document. Though we are aware that this ambitious project is not at all easy and may be difficult to implement in its entirety – in fact we consider it a major step – our position is that all the efforts to achieve it are worthwhile, considering that the main goal will always be the well-being of those affected by these particular disorders. Resumo: A prevalência, complexidade, importância clínica, heterogeneidade e imprevisibilidade das doenças cardíacas heredofamiliares torna a criação de Unidades de Doenças Cardíacas Hereditárias uma inevitabilidade, cujo objectivo final é reduzir a morbilidade e a mortalidade relacionadas com estas patologias. Uma Unidade de Doenças Cardíacas Hereditárias é uma subunidade de um departamento de Cardiologia constituída por profissionais de saúde especializados neste tipo de doenças, organizado de forma a proporcionar a excelência em todos as suas áreas, incluindo diagnóstico, tratamento, seguimento, prevenção, estratificação de risco e determinação de prognóstico. Alguns dos seus objectivos são o desenvolvimento de protocolos de actuação e a criação de bases de dados que permitam a inclusão de doentes em registos e redes de investigação nacionais e internacionais. Para atingir estes objectivos estes centros devem integrar Unidades Funcionais (clínica, ciências básicas, investigação, treino e educação), actuando harmonicamente numa abordagem holística de doentes e suas famílias. Como grande parte do conhecimento referente às doenças cardíacas hereditárias se baseia na miocardiopatia hipertrófica e na experiência adquirida com os “centros de miocardiopatia hipertrófica”, estes representam um excelente modelo para aprender como criar e desenvolver as Unidades de Doenças Cardíacas Hereditárias. A nível Europeu, as características destas Unidades serão diferentes de um país para outro, reflectindo a heterogeneidade dos diferentes sistemas e serviços nacionais de saúde, compartilhando no entanto um core comum, apresentado neste documento. Embora conscientes de que este projecto é ambicioso e de que a sua concretização como um todo não será uma tarefa fácil, consideramos a aplicação e divulgação deste conceito a nível nacional e Europeu um passo de grande importância. Assim, a nossa posição é que todos os esforços para atingir esta meta valem a pena, considerando que o principal objectivo será sempre o bem-estar dos doentes afectados por estas patologias. Keywords: Inherited cardiovascular diseases, Inherited cardiovascular disease centers, Hypertrophic cardiomyopathy, Hypertrophic cardiomyopathy centers, Palavras-chave: Doenças cardíacas hereditárias, Unidades de doenças cardíacas hereditárias, Miocardiopatia hipertrófica, Centros de miocardiopatia hipertrófica

Background The aim of this study was to assess the usefulness of a new miniaturized echocardiographic system (MS) to perform bedside echocardiography in initial outpatient cardiology consultations, in addition to physical examination. Methods One hundred eighty-nine patients referred for initial cardiology outpatient consultations at two tertiary hospitals in two countries were studied. Each patient was submitted to physical examination followed by MS assessment. Scanning time, the number of examinations with abnormal results after physical examination and the MS, and the information obtained by physical examination alone and followed by the MS (in terms of its importance in reaching a diagnosis, in the necessity of performing routine echocardiography, and in the decision to release the patient from the outpatient clinic) were assessed. Results The scanning time with the MS was 180 ± 86 seconds. Its use after physical examination led to diagnoses in 141 patients (74.6%) and to an additional 37 patients (19.6%) being released from the outpatient clinic. After physical examination followed by MS assessment, only 64 patients (33.9%) were sent to the echocardiography lab. The MS modified the decision of whether to send a patient to the echocardiography lab, with referral determined by the MS in 27 patients (14.3%) and no referral determined by the MS in 58 patients (30.7%). Conclusions The new MS caused a negligible increase in the duration of consultations. It showed additive clinical value over physical examination, increasing the number of diagnoses, reducing the use of unnecessary routine echocardiography, increasing the number of adequate echocardiographic studies, and determining a large number of releases from the outpatient clinic.

In the past decades, a number of impressive scientific and technologic discoveries in cardiovascular medicine have opened up a wide range of diagnostic and therapeutic possibilities beyond what was thought to be possible a few years ago. Among the different cardiologic subspecialties, noninvasive cardiac imaging, particularly echocardiography, is an excellent example of the impact of these advances that influence prevalence, diagnosis, and therapy of heart diseases as well as the type of medical practice and the clinical approach to the cardiac patient. 1 Today, and in the next decades, echocardiography is and will continue to be afirst-line method in the approach topatients with heart disease, the‘‘workhorse’’of cardiac diagnosis, withan increasing utilization rate (being at least equal to electrocardiography and chest radiography). When taking into account the intrinsic advantages of this technique (accessible, innocuous, low cost, objective, quantitative, reproducible),echocardiographyplaysanessentialroleinthe diagnosis, management, and outcome of cardiac patients by providing useful information in ‘‘everything’’ that the clinician needs. Accordingly, in the future, more and more patients will need echocardiography to guide clinical decision making. One of the most striking advances in echocardiography over the years has been the progressive miniaturization of echocardiography machines, which led to the development of the newest generation of hand-held imaging devices, the pocket-size scanners. The small dimensions of these scanners (similar in size to smartphones) and the high-quality images that they provide allows us to carry them in our white coat pocket and to use them routinely at the bedside, making point-of-care ultrasonography a reality. However, the development of these powerful devices has not always been peaceful, and several controversial issues, such as diagnostic accuracy, clinical scenarios where they can be used, potential users and their level of technical competence, workflow integration, costeffectiveness, storage,andreimbursement,havebeenidentifiedandextensively discussed in the past years. In our times of limited resources and of an increasing need for speed in the health care system, these cheaper and user-friendly miniaturized ultrasound scanners are appealing. Today, defining the exact role of this technology is more demanding and urgent than ever, and much work still needs to be done to define with precision its role in the delivery of cardiac care. Aortic stenosis is the most common valvular heart disease in the elderly and is associated with high rates of morbidity and mortality. It is also a leading cause of valvular surgery and of percutaneous valvular replacement, with an important economic burden. The diagnosis ofits severityisclassicallybasedonsymptomsandtypicalphysicalsigns,usually confirmedbyechocardiography,thatprovidesdetailedandaccurateanatomic and hemodynamic data on the severity of the disease. Inthis issue ofthe Journal,Abeetal. 2 describe ascoring system (exclusively based on two-dimensional imaging of aortic cusp motion) for grading the severity of aortic stenosis by using one of these pocket scannersandcomparetheresultswith(a)physicalexaminationfindingsperformed by experts and (b) results from high-end echocardiography machines. They concluded that physical examination by experts was quite accurate in distinguishing moderate-to-severe aortic stenosis from mild-to-negligible stenosis. Moreover, compared with findings on physical examination, the scoring system showed even better agreement with valve orifice area determined by using the continuity equation. The investigators also were able to identify a cutoff score that indicated patients with a high likelihood of ‘‘significant’’ stenosis, in whom a high-end echocardiographic examination was required for amore-thoroughevaluation,andanothercutoffscorethatreliablyidentified patients who did not have significant aortic stenosis (in whom a comprehensive echocardiographic evaluation was not a high priority need). Theinvestigators suggest that point-of-care scanning does not replace a state-of-the-art assessment in patients with aortic stenosis but instead that this simple and rapid approach can be helpful in triaging patients and in determining which one needs to go to the head of the line for a full echocardiography study, and, perhaps for other diagnostic evaluations as well as a surgical or percutaneous replacement referral, and which patient does not need this type of expedited evaluation. In this article, 2 major important topics, the determination of aortic stenosis severity and the use of small portable echocardiography scanners at the ‘‘point of care,’’ deserve a thoughtful reflection.

Because myocyte dysfunction and disarray are early abnormalities in hypertrophic cardiomyopathy (HC), we tested if Doppler myocardial imaging (DMI) could identify systolic and diastolic dysfunction in mutation carriers (MC) (genotype positive patients without hypertrophy, defined as phenotype negative after conventional screening tests). In a single family with a missense mutation in the myosin binding protein C gene (Arg 502 Gln) we identified 5 MCs; these subjects were asymptomatic and had normal physical examination, normal electrocardiogram, treadmill stress test, ambulatory Holter electrocardiogram, and normal conventional M-mode, 2-dimensional, and Doppler echocardiography. In each patient we performed a DMI study and measured the peak velocities of the systolic (S), rapid filling (E), and atrial contraction (A) waves in the 4 sides of the mitral annulus, in 8 left ventricular segments (apical views), in the tricuspid annulus, and in 2 right ventricular segments. These data were compared with those from 10 normal volunteers matched for sex, age, and body surface. Compared with the normal volunteers, the MCs had lower left ventricular systolic velocities and higher right ventricular systolic velocities; lower diastolic rapid filling velocities; higher or similar atrial contraction velocities; reduced E/A; lower percentage of annular sides and segments with E/A >1 and lower average number of sides and/or segments with E/A >1 per patient; similar right ventricular rapid filling velocities; and similar or higher atrial contraction wave velocities. Thus, DMI detects important left and right ventricular annular and regional myocardial contraction and relaxation abnormalities independently of the presence of hypertrophy, in HC. These results show that DMI is more sensitive than conventional echocardiography and establishes a new and highly accurate method for the noninvasive screening of MCs of the disease.

RESUMO: A presença de Hipertensão Pulmonar e de Cor Pulmonale é um factor de mau prognóstico no contexto da Doença Pulmonar Crónica Obstrutiva.A Ecocardiografia modo M. bidimensional e Doppler é um método não invasivo que permite avaliar as alterações morfológicas e funcionais do coração direito e estimar a Pressão Sistólica na Artéria Pulmonar.No entanto, condicionantes vários como a hiperinsuflação pulmonar, as alterações da parede torácica e a obesidade frequentemente presentes em doentes com patologia respiratória crónica, podem impedir a obtenção de exame com boa qualidade téscnica.O objectivo deste trabalbo é avaliar a exeqwoilidade deste método nos doentes com Doença Pulmonar Crónica Obstrutiva e correlacionar os dados obtidos por Ecocardiografia com os aspectos clinicos sugestivos de falência cardíaca direita.Foram estudados 35 doentes, 29 do sexo masculino e 6 do sexo feminino, com idade média de 66,2 anos, com Doença Pulmonar Crónica Obstrutiva seguidos na Consults de Oxigenioterapia de Longa Duração do Serviço de Pneumologia do Hospital de Santa Maria.Cada doente realizou um Ecocardiograma modo M, bidimensional e Doppler para determinação da espessura da parede livre do ventrículo direito (VD), do diâmetro da cavidade ventricular direita e da Pressão Sistólica na Artéria Pulmonar (PAPs) utilizando as vias paraesternal esquerda, apical e subcostal.Em 80% dos doentes estudados obtiveram-se registos satisfatórios que permitiram determinar a espessura da parede livre do VD, avaliar o diâmetro desta cavidade e estimar a PAPs.Foram registados critérios de Hipertrofia Ventricular Direita em 75% dos registos validados (espessura média da parede livre do VD - 7,1±1,3 mm) e critérios de dilatação ventricular direita em 25% dos casos.Em 60% destes doentes conseguiu-se estimar a PAPs a partir do fluxo de Insuficiência Tricúspide. O valor médio da PAPs foi de 60±21,6 mmHg, registando-se critérios de Hipertensão Pulmonar (HTP) em 77% dos casos estimados.Nos doentes em que não foi possível quantificar a PAPs procedeu-se à avaliação do fluxo da câmara de saida do VD. Destes, 66% apresentavam critérios sugestivos de HTP.Dos doentes com critérios ecocardiográficos sugestivos de Hipertrofia ventricular direita 50% apresentavam história clínica de um ou mais episódios prévios de falêncla cardíaca direita.Dos doentes com critérios ecocardiográficos de dilataçãio do VD 83% apresentavam história clínica de um ou mais episódios prévios de falência cardíaca direita.Os dados preliminares parecem assim sugerir que a Ecocardiografia é um método não invasivo útil na avaliaçãio dos doentes com Doença Pulmonar Crónica Obstrutiva permitindo o diagnóstico precoce de Cor Pulmonale e a quantificação da PAPs. ABSTRACT: NON INVASIVE DIAGNOSIS OF PULMONARY HYPERTENSION AND COR PULMONALE IN PATIENTS WITH CHRONIC OBSTRUCTIVE PULMONARY DISEASEPulmonary hypertension and cor pulmonale are indicators of poor prognosis in patients with Chronic Obstructive Pulmonary Disease (COPD).M-mode, bidimensional and Doppler echocardiography is a non invasive technique usefull in the assessment of the morphological and functional characteristics of the right heart and usefull in the evaluation of the systolic pulmonary artery pressure (sPAP).Yet, some characteristics often present in these patients such as pulmonary emphysema, obesity and deformities of the thoracic wall make difficult the obtention of adequate recordings.The aim of this study was to analyse the feasibility of this method in patients with COPD and to compare its sensitivity to clinical methods in the assessment of Pulmonary Hipertension and Cor Pulmonale in these patients.Thirty-five patients with COPD under long therm Oxigeniotherapy were studied by M-mode, bidimensional and Doppler Echocardiography using left paraesternal, apical and subcostal views, in order to assess the thickness of the free right ventricula wall, the dimension of the right ventricle and the Pulmonary systolic pressure.Adequate recordings were obtained in 80% of the patients.75% of the patients bad echocardiographic: criteria of right ventricular hypertrophy (thickness of the right ventricular wall - 7,1±1,3 mm) and 25% bad echocardiograpbic criteria of right ventricular dilatation.In 60% of the patients it was possible to estimate the sPAP through the measurement of the peak velocity of the tricuspid regurgitant jet.The mean value of the sPAP was 60Ã21,6 mmHg. Ecbocardiographic criteria of pulmonary hypertension were recorded in 77% of the patients.When it was not possible to estimate de sPAP we recorded the pulmonic ejection flow. Using this approach 77% of the cases studied had echocardiographic criteria of Pulmonary Hypertension.50% of the patients with echocardiographic criteria of right ventricular hypertrophy had clinical evidence of right heart failure while 83% of the patients with echocardiograpbic criteria of right ventricular dilatation had clinical evidence of right heart failure.The preliminary data sugest that echocardiography is usefull as a non invasive method in the evaluation of patients with COPD allowing the early diagnosis of Cor Pulmonale and the quantification of the sPAP. Palavras-chave: Ecocardiografia, hipertensão pulmonar, cor pulmonale, doença pulmonar crónica obstrutiva, Key-words: Echocardiography, Pulmonary Hypertension, Cor Pulmonale, Chronic Obstructive Pulmonary Disease