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Sr. Editor: La administración de tratamiento anticoagulante solo ha demostrado ser eficaz contra la embolia pulmonar (EP) aguda de riesgo bajo y de bajo a intermedio1. No obstante, la EP de riesgo intermedio-alto y alto está asociada a un riesgo considerablemente alto de colapso circulatorio a corto plazo, muerte o hipertensión pulmonar tromboembólica crónica de entre el 3 y el 10% cuando se trata solo con anticoagulación1. Aunque la fibrinólisis sistémica reduce este riesgo en un 50%, este tratamiento aumenta significativamente el riesgo de sangrado mayor, tal y como describió el ensayo PEITHO2, lo cual ha limitado el uso de la fibrinólisis sistémica a pacientes de alto riesgo, tal y como recomienda la guía vigente3. Esta limitación ha generado un interés creciente en las terapias dirigidas por catéter (TDC) en pacientes con EP aguda de alto riesgo y una contraindicación o fracaso de la fibrinolisis sistémica, así como en pacientes de riesgo intermedio-alto que desarrollan deterioro hemodinámico a pesar de la anticoagulación3. Las TDC permiten una resolución más rápida de los defectos de perfusión y una mejora hemodinámica sin los efectos hemorrágicos sistémicos asociados a la trombolisis sistémica. A pesar del mayor uso de estas TDC, la evidencia clínica sobre sus beneficios sigue...

To the Editor, Anticoagulation alone has proven efficacy for the treatment of low- and low-to-intermediate- -risk acute pulmonary embolism (PE) patients.1 Nonetheless, intermediate-high and high-risk PE are associated with a considerable risk of short-term circulatory collapse, death or chronic thromboembolic pulmonary hypertension, ranging from 3% to 10%, when treated with anticoagulation alone.1 Although systemic fibrinolysis decreases this risk by 50%, this treatment significantly increases the risk of major bleeding, as seen in PEITHO trial (Fibrinolysis for patients with intermediate-risk pulmonary embolism),2 which has limited the use of systemic fibrinolysis to high-risk patients, as recommended in the current guidelines.3 This limitation has led to growing interest in catheter-directed therapies (CDT) for patients with high-risk acute PE and a contraindication or failure of systemic fibrinolysis and patients with intermediate-high risk who develop worsening hemodynamics despite anticoagulation.3 CDT allow faster resolution of perfusion defects and hemodynamic improvement without the systemic hemorrhagic effects of systemic thrombolysis. Despite an increasing use of CDT, the clinical evidence of its benefits remains scarce, as there are no adequately powered randomized controlled trials and current studies have been limited to immediate hemodynamic improvement or imaging surrogate markers.4-6 This study aimed to assess the safety, feasibility, and mid-term effects of CDT. Between 2020...

ABSTRACT Introduction and objectives: Transfemoral access is globally accepted as the preferential access route for transcatheter aortic valve implantation (TAVI). However, in up to 15% of the patients, this access is considered inadequate. Considering the various alternatives available, the fully percutaneous access routes have been chosen preferentially. This analysis aims to compare outcomes and complications of 3 alternative access routes for transfemoral, trans-subclavian and transcaval TAVI. Methods: Retrospective analysis of patients referred for TAVI using transfemoral, trans-subclavian, and transcaval accesses in a single tertiary center from 2008 through 2021. The primary endpoints were 30-day and 1-year all-cause mortality rates. The secondary endpoints were technical success, residual moderate-to-severe paravalvular leak, major vascular complication, 30-day stroke, 30-day Valve Academic Research Consortium-2 (VARC-2) major bleeding, and 30-day acute kidney injury (AKIN criteria 2 or 3). Results: A total of 642 TAVIs were performed (601 transfemoral, 24 trans-subclavian, and 10 transcaval). A total of 7 patients treated via transapical access were excluded. As expected, baseline comorbidities like left ventricular dysfunction, coronary artery disease, atrial fibrillation, chronic kidney disease, and previous stroke were more frequent in the non-femoral groups. The 1-year and 30-day all-cause mortality rates were higher in the non-transfemoral population (HR, 2.88 and HR, 3.53, respectively). The rates of 30-day stroke and acute kidney injury (AKIN 2 or 3) were also significantly lower in transfemoral patients, but similar between trans-subclavian and transcaval patients. The rates of 30-day major bleeding showed a statistically significant tendency towards lower rates in the transfemoral group. The rates of technical success, major vascular complications, and residual moderate or severe perivalvular leak were similar among the 3 groups. Conclusions: After careful selection, transfemoral access is the preferential access route for TAVI procedures. In intermediate surgical risk patients with severe symptomatic aortic stenosis, non-transfemoral TAVI approaches have poorer outcomes. The worse outcomes of percutaneous alternative access routes are partially associated with worse baseline characteristics.

RESUMEN Introducción y objetivos: El acceso transfemoral (aTF) es el de elección para el implante percutáneo de válvula aórtica (TAVI). Sin embargo, hasta en el 15% de los pacientes este acceso es inadecuado. De las alternativas disponibles, el acceso totalmente percutáneo es el preferido. El objetivo del estudio es comparar los resultados clínicos de los pacientes tratados por aTF frente a los de aquellos con acceso transubclavio (aTS) o transcava (aTCv). Métodos: Análisis retrospectivo de los pacientes tratados con TAVI (2008-2021) en un centro terciario mediante aTF, aTS y aTCv. El objetivo primario fue la mortalidad por cualquier causa a 30 días y 1 año. Los objetivos secundarios fueron el éxito técnico, la regurgitación perivalvular moderada o grave, las complicaciones vasculares mayores, el accidente vascular cerebral, el sangrado mayor y el daño renal agudo a 30 días (Valve Academic Research Consortium II [VARC-2]). Resultados: Se realizaron 642 TAVI (601 por aTF, 24 por aTS y 10 por aTCv). Fueron excluidos 7 pacientes tratados por vía transapical. En los pacientes con acceso alternativo fue más frecuente la comorbilidad, incluyendo disfunción ventricular izquierda, enfermedad coronaria, fibrilación auricular, enfermedad renal e ictus previo. La mortalidad a 1 año y a 30 días fue también más alta en este grupo de pacientes (hazard ratio [HR] = 2,88 y 3,53, respectivamente). A 30 días, las tasas de ictus y daño renal (grado 2 o 3) fueron significativamente más bajas para el aTF, y similares en los pacientes con aTS y aTCv. Hubo también una tendencia a menor sangrado en el grupo de aTF. El éxito técnico, las complicaciones vasculares mayores y la regurgitación perivalvular moderada o grave fueron similares en los tres grupos. Conclusiones: Tras una adecuada selección, el aTF es el preferido para el TAVI. En pacientes de riesgo intermedio con estenosis aórtica grave sintomática, un acceso no transfemoral tiene peores resultados. Los inferiores resultados del uso de vías percutáneas alternativas están parcialmente relacionados con las peores características clínicas basales.

Introduction and objectives: Patients with hypertrophic obstructive cardiomyopathy (HOCM) that remain symptomatic despite optimized medical therapy often undergo alcohol septal ablation (ASA). One of the most frequent complications is complete heart block (CHB), requiring a permanent pacemaker (PPM) in variable rates of up to 20% of patients. The long-term impact of PPM implantation in these patients remains unclear. This study aimed to evaluate the long-term clinical outcomes in patients who implant PPM after ASA. Methods: Patients who underwent ASA at a tertiary center were consecutively and prospectively enrolled. Patients with previous PPM or implantable cardio-defibrillator were excluded from this analysis. Patients with and without PPM implantation after ASA were compared based on their baseline characteristics, procedure data and three-year primary endpoint of composite of all-cause mortality and hospitalization and secondary endpoint of composite of all-cause mortality and cardiac cause hospitalization. Results: Between 2009 and 2019, 109 patients underwent ASA, 97 of whom were included in this analysis (68% female, mean age 65.2 years old). 16 patients (16.5%) required PPM implantation for CHB. In these patients, no vascular access, pacemaker pocket or pulmonary parenchyma complications were noted. The baseline characteristics of comorbidities, symptoms, echocardiographic and electrocardiographic findings were identical in the two groups, with higher mean age (70.6±10.0 years vs. 64.1±11.9 years) and lower beta-blocker therapy rate (56% vs. 84%) in the PPM group. Procedure-related data showed higher creatine kinase (CK) peaks in the PPM group (1692 U/L vs. 1243 U/L), with no significant difference in the alcohol dose. At three years after ASA procedure, there were no differences in the primary and secondary endpoints between the two groups. Conclusions: Permanent pacemaker after ASA induced CHB do not affect long term prognosis in hypertrophic obstructive cardiomyopathy patients. Resumo: Introdução e objetivos: Doentes com miocardiopatia hipertrófica obstrutiva (MCHO) sintomáticos apesar de terapêutica médica otimizada são frequentemente submetidos a ablação septal alcoólica (ASA). O bloqueio atrioventricular (BAV) completo é uma complicação frequente, requerendo implantação de pacemaker definitivo em taxas variáveis que podem ir até 20% dos doentes. O impacto a longo prazo da implantação de pacemaker nestes doentes permanente mal-esclarecido. Esta análise pretende avaliar os outcomes clínicos a longo prazo nestes doentes. Métodos: Doentes consecutivos submetidos a ASA num centro terciário foram incluídos e seguidos prospetivamente. Pacientes com pacemaker ou cardiodesfibrilhador prévio foram excluídos desta análise. Os doentes com e sem implantação de pacemaker após a ASA foram caracterizados e comparados relativamente aos endpoints primário (composto de mortalidade e hospitalização por todas as causas) e secundário (composto de mortalidade por todas as causas e hospitalização de causa cardíaca) a três anos. Resultados: Entre 2009 e 2019, 109 pacientes foram submetidos a ASA e 97 foram incluídos nesta análise (68% sexo feminino, idade media 65,2 anos). Implantaram pacemaker 16 doentes (16,5%) após a ASA por BAV completo. Não se registaram complicações vasculares, da loca ou parenquimatosas pulmonares. As características basais foram semelhantes entre os grupos, com maior idade média (70,6 ± 10,0 anos versus 64,1 ± 11,9 anos) e menor taxa de terapêutica beta-bloqueante (56% versus 84%) no grupo de pacemaker. Dados do periprocedimento revelaram maior pico de creatina cinase (CK) no grupo de pacemaker, sem diferença na dose de álcool usado. Aos três anos, os endpoints primário e secundário não mostraram diferenças entre os dois grupos. Conclusão: A implantação de pacemaker definitivo por BAV completo induzido por ablação septal alcoólica não afeta o prognóstico de pacientes com MCHO.

Sr. Editor: El fracaso del tratamiento es una gran preocupación tras el implante de stents en procedimientos full metal jacket (FMJ), que se definen como stents consecutivos, solapados o no, con longitudes ≥ 60 mm. Estas intervenciones suelen ser necesarias para el tratamiento de lesiones coronarias en tándem o extensas1. En la actualidad, se han aprobado varias marcas de stents, cada una con características distintas y un rendimiento diferente. No obstante, la práctica médica del mundo real no se limita a usar una única marca y emplea muchas distintas. No obstante, hay pocos datos sobre el perfil de seguridad y eficacia de estas diferentes estrategias durante las intervenciones coronarias percutáneas (ICP). Por eso, nuestro objetivo fue comparar los resultados clínicos obtenidos con una única marca de stent frente a varias marcas tras una ICP con FMJ con éxito. Se identificó retrospectivamente a 592 pacientes (3%) tratados con un procedimiento FMJ, de una base de datos dedicada de 23.021 ICP consecutivas realizadas entre enero de 2002 y diciembre de 2018 en una sala de hemodinámica de alto volumen. Se obtuvo el consentimiento informado por escrito de todos los pacientes. La selección de los stents empleados se dejó a criterio del operador. Se excluyó...

To the Editor, Treatment failure is a major concern after full metal jacket (FMJ) stenting procedures, defined as overlapping stent length ≥ 60 mm. These procedures are often required to treat tandem or extensive coronary lesions.1 Several brands of stents are currently approved, each displaying different characteristics and performance. However, real-world practice is not restricted to the use of a brand exclusive strategy and may involve a combination of different brands. Limited data exist on the relative safety and efficacy of these different strategies during percutaneous coronary intervention (PCI). Therefore, our aim was to compare clinical outcomes after the use of a single stent brand vs multiple stent brands following successful FMJ PCI. From a dedicated database of 23 021 consecutive PCI procedures performed between January 2002 and December 2018 at a high-volume coronary intervention laboratory, we retrospectively identified 592 patients (3%) who underwent FMJ procedures. Written informed consent was obtained from all patients. Stent selection was left to the operator’s discretion. We excluded patients with unsuccessful procedures and those lost to follow-up from the analysis. Demographic, clinical, angiographic, and procedural variables were evaluated. The primary endpoint consisted of major adverse cardiac events (MACE), which included all-cause death, myocardial infarction (MI), and...

Introduction. Since the advent and development of transcatheter aortic valve replacement (TAVR) in the contemporary era, balloon aortic valvuloplasty (BAV) has seen renewed interest. We aimed to compare 30-day and 1-year all-cause mortality between patients submitted to BAV as a bridging therapy before definite TAVR and patients submitted directly to TAVR. Methods. This was an observational, retrospective study of patients who underwent TAVR between 2009 and 2022 in a tertiary center. Patients with severe aortic stenosis (SAS) who underwent TAVR without prior BAV (woBAV group) and patients who were performed TAVR with prior BAV (wBAV group) as a bridging therapy were included. Primary endpoint was all-cause mortality at 30 days and 1 year after TAVR between wBAV and woBAV groups. Results. 800 patients were included, of which 767 were in woBAV group and 33 were in wBAV group. 30-day all-cause mortality rate was 21% in wBAV group compared to 4.4% in woBAV (unadjusted hazard ratio [HR], 5.19; 95% confidence interval [CI], 2.3–11.7, p

Introduction: The COVID-19 pandemic has imposed an unprecedented burden on healthcare systems worldwide, changing the profile of interventional cardiology activity. Objectives: To quantify and compare the number of percutaneous coronary interventions (PCIs) performed for acute and chronic coronary syndromes during the first COVID-19 outbreak with the corresponding period in previous years. Methods: Data on PCI from the prospective multicenter Portuguese Registry on Interventional Cardiology (RNCI) were used to analyze changes in PCI for ST-elevation myocardial infarction (STEMI), non-ST-elevation acute coronary syndromes (NSTE-ACS) and chronic coronary syndromes (CCS). The number of PCIs performed during the initial period of the COVID-19 outbreak in Portugal, from March 1 to May 2, 2020, was compared with the mean frequency of PCIs performed during the corresponding period in the previous three years (2017–2019). Results: The total number of PCIs procedures was significantly decreased during the initial COVID-19 outbreak in Portugal (−36%, p

Introduction and objectives: Coronary microvascular dysfunction (CMD) is one of the most important pathophysiological features in hypertrophic cardiomyopathy (HCM). The index of microcirculatory resistance (IMR) is an invasive method to assess the coronary microcirculation. The aim was to assess CMD in patients with HCM by IMR. Methods: Adult patients with HCM without epicardial coronary artery disease underwent cardiac catheterization for the assessment of CMD by IMR (normal cut-off value ≤22.0) and coronary flow reserve (CFR) (normal cut-off value ≥2). Cardiovascular magnetic resonance (CMR) was performed to assess the ischemic burden by perfusion imaging during regadenoson-induced hyperemia, and the extent of myocardial fibrosis was assessed by late gadolinium enhancement (LGE), native T1 mapping and extracellular volume (ECV). Results: Fourteen patients were enrolled with a mean age of 62.8±6.2years, 8 (57.1%) males, of whom 9 (64.3%) had obstructive HCM. Using IMR, CMD was detected in 4 (29%) patients. Among four patients with an IMR>22.0, all had non-obstructive HCM and two had angina. CFR22.0, perfusion defects were found in two of the three patients who underwent stress CMR. Increased ECV (>28%) was documented in two of the patients with IMR>22 and in three of the patients with IMR≤22.0. LGE was >15% in 2 of the patients with IMR>22 and in 4 with IMR≤22.0. Conclusions: IMR assessment in HCM is feasible and safe. Patients with abnormal IMR seemed to have more significant tissue abnormalities on CMR. Resumo: Introdução e objetivos: A disfunção microvascular coronária (DMC) constitui uma das mais importantes alterações fisiopatológicas na miocardiopatia hipertrófica (MCH). O índice de resistência microcirculatória (IRM) é um método invasivo para avaliação da microcirculação coronária. O objetivo é avaliar a DMC nos doentes com MCH utilizando o IRM. Métodos: Doentes adultos com MCH, sem doença coronária epicárdica, submetidos a cateterismo cardíaco para avaliação de DMC por IRM (valor normal≤22,0) e reserva de fluxo coronário (RFC) (valor normal≥2). Ressonância magnética cardiovascular (RMC) foi realizada para avaliação de isquémia por sequência de perfusão durante hiperemia induzida por regadenoson; a extensão de fibrose foi avaliada por realce tardio (RT), T1 mapping nativo e volume extracelular (VEC). Resultados: Foram incluídos 14 doentes com média de 62,8±6,2 anos, 8 (57,1%) do género masculino, dos quais 9 (64,3%) tinham MCH obstrutiva. Utilizando IRM, DMC foi detetada em 4 (29%) doentes. Dos 4 doentes com IRM>22,0, todos tinham MCH não obstrutiva e 2 tinham angina. RFC22, foram encontrados defeitos de perfusão em 2 dos 3 doentes que realizaram RMC. VEC aumentado (>28%) foi documentado em 2 doentes com IRM>22 e em 3 com IRM≤22,0. RT>15% foi documentado em 2 doentes com IRM>22 e em 4 com IRM≤22,0. Conclusão: A avaliação do IRM na MCH é segura e exequível. IRM tem potencial para se tornar uma técnica útil na avaliação da microcirculação dos doentes com MCH.

Background: Some studies suggest that patients with low flow low gradient (LF-LG) aortic stenosis (AS) may achieve worse results after undergoing transcatheter aortic valve implantation (TAVI). Purpose: To compare outcomes between LF-LG AS and high gradient (HG) AS patients submitted to TAVI. Methods: Inclusion of 480 consecutive patients who underwent TAVI between 2008 and 2020 at a single tertiary center. Patients were divided into high gradient AS and LF-LG AS; and baseline characteristics and outcomes after the procedure were collected and compared between groups. Results: Patients with LF-LG AS had worse baseline characteristics, with higher Society of Thoracic Surgeons score (p=0.008), New Euroscore II (p

Background: Microvascular dysfunction is an often overlooked feature of hypertrophic cardiomyopathy (HCM). Our aim was to assess the association between microvascular dysfunction, wall thickness, tissue characteristics and myocardial deformation in HCM patients, by analyzing individual myocardial segments. Methods: Prospective assessment including cardiac magnetic resonance to assess wall thickness, T1 and T2 mapping, extracellular volume, late gadolinium enhancement (LGE) and stress perfusion. Results were stratified according to the 16 American Heart Association segments. Results: Seventy-five patients were recruited (1200 segments), 63% male, mean age 54.6±14.8 years, maximal wall thickness of 20.22±4.6 mm. Among the 424 segments (35%) with perfusion defects, 24% had defects only in the endocardial layer and 12% in both endocardial and epicardial layers. Perfusion defects were more often detected in hypertrophied segments (64%). Among the 660 segments with normal wall thickness, 19% presented perfusion defects. Independently of wall thickness, segments with perfusion defects had a higher T1 (β-estimate 30.28, p

Background Two‐dimensional speckle tracking echocardiography has been shown to correlate with microvascular dysfunction, a hallmark of hypertrophic cardiomyopathy (HCM). We hypothesized that there is an association between myocardial work and left ventricular ischemia, with incremental value to global longitudinal strain, in patients with HCM. Methods and Results We performed a prospective assessment of patients with HCM, undergoing 2‐dimensional speckle tracking echocardiography and stress perfusion cardiac magnetic resonance. Results were stratified according to obstructive or nonobstructive HCM and the presence of significant replacement fibrosis (late gadolinium enhancement ≥15% of left ventricular mass). Seventy‐five patients with HCM (63% men, age 55±15 years) were evaluated, 28% with obstructive HCM (mean gradient 89±60 mm Hg). Perfusion defects were found in 90.7%, involving 22.5±16.9% of left ventricular mass, and 38.7% had late gadolinium enhancement ≥15%. In a multivariable analysis, a lower global work index (r=−0.519, β‐estimate −10.822; P=0.001), lower global work efficiency (r=−0.379, β‐estimate −0.123; P=0.041), and impaired global constructive work (r=−0.532, β‐estimate −13.788; P−15.5%) had a sensitivity of 64% and a specificity of 57%. The association between myocardial work and perfusion defects was significant independently of late gadolinium enhancement ≥15% and obstructive HCM. Conclusions Impaired myocardial work was significantly correlated with the extent of ischemia in cardiac magnetic resonance, independently of the degree of left ventricular hypertrophy or fibrosis, with a higher predictive power than global longitudinal strain.

In the midst of the coronavirus disease-2019 (COVID-19) pandemic, an 84-year-old female patient was admitted due to non-exertional syncope preceded by retrosternal pain. She had experienced a prolonged episode of oppressive chest pain 6 days before her presentation, but due to the concern of contracting COVID-19, she did not present for medical care. Upon admission to the emergency department, the patient was in circulatory shock, with her physical examination being remarkable for the presence of a holosystolic murmur. Admission electrocardiogram revealed an inferior ST-segment elevation with Q waves with extension to the posterior wall, consistent with subacute infarct in the right coronary artery (RCA) territory, and the patient was transferred for primary percutaneous coronary intervention. Upon arrival to the catheterization laboratory, a summary transthoracic echocardiogram was performed, which revealed inferior wall and infero-septal akinesia with an 18 mm ventricular septal rupture. Coronary angiography documented occlusion of the proximal segment of a dominant RCA. Due to a high perioperative risk, the patient underwent successful retrograde percutaneous closure with a 24 mm MemoPart™ device, with mild to moderate residual shunt. Despite an immediate clinical improvement, the patient died 12 hours after the procedure due to refractory cardiogenic shock.

Introduction: Paravalvular leak (PVL) is a common serious complication associated with prosthetic valve implantation. Objective: The aim of this study was to report our single-center experience in a retrospective review and to analyze possible predictors of success. Methods: We performed 33 percutaneous PVL closures in 26 patients (54% female, mean age 65±13 years). All mitral prostheses were studied previously with 3D transesophageal echocardiography (TEE), and aortic prostheses with 2D/3D TEE. 3D TEE and fluoroscopy were used for the assessment, planning, and guidance of the interventions. Twelve patients also underwent computed tomography angiography for better characterization of anatomic details. Results: Eighteen patients (69.2%) were admitted due to heart failure (New York Heart Association [NYHA] III or IV, seven (26.9%) because of heart failure and hemolysis, and one (3.8%) due to hemolysis only. Regarding the leaks, 46.2% were in aortic and 53.8% in mitral prostheses, 88.5% in mechanical and 7.7% in biological prostheses, and 3.8% in transcatheter aortic valve implants. All the aortic patients had severe aortic regurgitation. Furthermore, all mitral patients but one had moderate to severe or severe mitral regurgitation. Closure was successful in 17 patients (65.4%), partially successful in four (15.4%) and unsuccessful in five (19.2%). After the procedure, 69% were in NYHA I-II. Hemolysis worsened in three patients despite successful closure; all required further valvular surgery and two died. Regarding angiographic and echocardiographic procedural success, we analyzed age, gender, type of prosthesis (mechanical or biological), location (aortic or mitral), clinical data, maximum leak diameter, anatomic regurgitant orifice, leak location (anterior, posterior, inferior and lateral for mitral leaks and left, right and non-coronary sinus for aortic leaks), and number of devices (plugs) used for closure. No parameters presented a significant relationship with success excepting previous hemolysis. There was a relationship between clinical improvement and reduction of PVL (p=0.0001). In follow-up, cardiac-related events (new hospital admissions, cardiac valvular surgery, need for transfusion) were more frequent in patients with partially successful or unsuccessful closure (p=0.012). There was a relationship between cardiac-related events and death (p=0.029). Conclusion: Percutaneous PVL closure has emerged as an alternative treatment for PVL. Predictors of procedural success are difficult to establish. Survival is related to reduction of regurgitation and improvement in NYHA functional class. Resumo: Introdução: Os leaks perivalvulares são uma complicação potencialmente grave após cirurgia protésica, com uma taxa de mortalidade ainda importante, sobretudo em doentes submetidos a reoperações sucessivas. Objetivo: Avaliar retrospetivamente a nossa experiência e resultados e encontrar possíveis fatores preditores de sucesso. Métodos: Revimos 33 procedimentos consecutivos de encerramento de leak perivalvular efetuados em 26 doentes (dts) (54% dos dts eram de sexo feminino, idade média 65 ± 13 anos). Todos os casos de leaks em posição mitral foram previamente estudados com ecocardiograma transesofágico 3 D (ETE), os leaks em posição aórtica foram avaliados por ETE 2D e nalguns casos 3D. Utilizaram-se a ecocardiografia transesofágica (2D/3D) e a fluoroscopia para a avaliação, planeamento, orientação e libertação dos dispositivos de encerramento; 12 dts efetuaram previamente angioTAC para melhor caracterização e definição anatómica do leak. Resultados: Clinicamente 18 dts tinham queixas de insuficiência cardíaca (New York Heart Association [NYHA] III/IV (69,2%), 7 dts tinham insuficiência cardíaca e hemólise (26,9%) e um doente apenas emólise (3,8%); 46,2% dos leaks eram aórticos e 53,8% mitrais, a maioria, 88,5%, em próteses mecânicas, 7,7% em próteses biológicas e 3,8% (um dt) em prótese percutânea aórtica (VAP). Todos os dts de leak aórtico tinham insuficiência cardíaca por regurgitação grave, os dts com leak mitral tinham insuficiência cardíaca, alguns, hemólise e um tinha apenas hemólise. O grau de regurgitação mitral era moderado a grave ou grave, com excepção do doente só com hemólise.O encerramento foi considerado de sucesso em 17 dts (65,4%), sucesso parcial em 4 (15,4%) e insucesso em 5 (19,2%). Após o procedimento, 69% melhoraram, com melhoria das queixas de ICC (NYHA I-II), 3 dts tiveram um agravamento da hemólise apesar do encerramento ser considerado de sucesso, tendo necessitado de cirurgia, complicada em dois casos (morte).Na tentativa de encontrar fatores preditores de sucesso de encerramento do leak, analisámos dados clínicos (idade, sexo, tipo e localização da prótese, manifestações clinicas), dados ecocardiográficos (diâmetro máximo do leak, área do orifício anatómico, localização do leak, classificando-o em anterior, posterior, inferior e lateral para as próteses mitrais e para as próteses aórticas de acordo com o seio coronário adjacente em esquerdo, direito e não coronário) e ainda o número de dispositivos de encerramento utilizados (plugs). Nenhum dos parâmetros avaliados teve correlação com o sucesso do encerramento, com excepção para a hemólise. Verificou-se relação entre melhoria clínica e redução do leak (p = 0,0001). Os dts com sucesso parcial/insucesso tiveram mais eventos cardíacos (p = 0,012), a ocorrência de mais eventos cardíacos (internamento hospitalar, nova cirurgia cardíaca, transfusões) se relacionou com pior prognóstico e morte (p = 0,029). Conclusão: O encerramento percutâneo de leaks protésicos pode ser uma opção ao tratamento convencional cirúrgico. Os fatores preditores de sucesso do encerramento são difíceis de estabelecer. A sobrevida destes dts relacionou-se com redução da regurgitação e melhoria clínica (NYHA).

Aims: The aim of the study is to investigate the association between the degree of ischemia due to coronary microvascular dysfunction (CMD) and the left ventricular (LV) tissue characteristics, systolic performance, and clinical manifestations in hypertrophic cardiomyopathy (HCM).Methods and Results: This prospective study enrolled 75 patients with HCM without obstructive epicardial coronary artery disease. Each patient underwent cardiovascular magnetic resonance (CMR) including parametric mapping, perfusion imaging during regadenoson-induced hyperemia, late gadolinium enhancement (LGE) and three-dimensional longitudinal, circumferential, and radial strains analysis. Electrocardiogram, 24-h Holter recording, and cardiopulmonary exercise testing (CPET) were performed to assess arrhythmias and functional capacity. In total, 47 (63%) patients were men with the mean age of 54.6 (14.8) years, 51 (68%) patients had non-obstructive HCM, maximum wall thickness (MWT) was 20.2 (4.6) mm, LV ejection fraction (LVEF) was 71.6 (8.3%), and ischemic burden was 22.5 (16.9%) of LV. Greater MWT was associated with the severity of ischemia (β-estimate:1.353, 95% CI:0.182; 2.523, p = 0.024). Ischemic burden was strongly associated with higher values of native T1 (β-estimate:9.018, 95% CI:4.721; 13.315, p < 0.001). The association between ischemia and LGE was significant in following subgroup analyses: MWT 15–20 mm (β-estimate:1.941, 95% CI:0.738; 3.143, p = 0.002), non-obstructive HCM (β-estimate:1.471, 95% CI:0.258; 2.683, p = 0.019), women (β-estimate:1.957, 95% CI:0.423; 3.492, p = 0.015) and age 15% (AUC 0.766, sensitivity 0.724, specificity 0.659). Ischemia was also associated with atrial fibrillation or flutter (AF/AFL) (OR-estimate:1.481, 95% CI:1.020; 2.152, p = 0.039), but no association was seen for non-sustained ventricular tachycardia. Ischemia was associated with shorter time to anaerobic threshold (β-estimate: −0.442, 95% CI: −0.860; −0.023, p = 0.039).Conclusion: In HCM, ischemia associates with morphological markers of severity of disease, fibrosis, arrhythmia, and functional capacity.

Resumo: Introdução: A ecografia intravascular coronária tem ganhado importância nos laboratórios de hemodinâmica pela evidência de impacto prognóstico positivo para os doentes. Este trabalho tem como objetivo caraterizar a utilização de ecografia intravascular em intervenções coronárias percutâneas em Portugal. Métodos: Estudo observacional retrospetivo com base no Registo Nacional de Cardiologia de Intervenção da Sociedade Portuguesa de Cardiologia. De 2002 a 2016 caraterizou‐se o perfil clínico e angiográfico dos doentes submetidos a intervenção coronária percutânea, a percentagem de utilização de ecografia intravascular e as artérias coronárias avaliadas. Resultados: Foram incluídas 118 706 intervenções coronárias percutâneas, com utilização de ecografia intravascular em 2266 (1,9%). A evolução temporal caraterizou‐se por ausência em 2002 e uma utilização maioritariamente crescente de 2003 (0,1%) a 2016 (2,4%). O grupo de doentes com utilização de ecografia intravascular coronária tinha idade semelhante ao grupo de doentes sem utilização de ecografia intravascular coronária, com uma menor prevalência de doentes do sexo masculino e uma maior prevalência de fatores de risco cardiovasculares (hipertensão arterial, hipercolesterolemia, diabetes), enfarte agudo do miocárdio prévio, intervenção coronária percutânea prévia, doença coronária multivaso, lesões coronárias do tipo C ou em bifurcação e reestenose intra‐stent. A ecografia intravascular foi utilizada em 54,8% das intervenções coronárias percutâneas eletivas e em 19,15% das intervenções coronárias percutâneas do tronco comum. Conclusão: A ecografia intravascular coronária teve uma utilização crescente em Portugal desde 2003, ocorre preferencialmente em intervenções coronárias percutâneas eletivas, de doentes com maior risco cardiovascular, com lesões coronárias mais complexas e lesões do tronco comum. Abstract: Introduction: Coronary intravascular ultrasound (IVUS) is increasingly important in catheterization laboratories due to its positive prognostic impact. This study aims to characterize the use of IVUS in percutaneous coronary intervention (PCI) in Portugal. Methods: A retrospective observational study was performed based on the Portuguese Registry on Interventional Cardiology of the Portuguese Society of Cardiology. The clinical and angiographic profiles of patients who underwent PCI between 2002 and 2016, the percentage of IVUS use, and the coronary arteries assessed were characterized. Results: A total of 118 706 PCIs were included, in which IVUS was used in 2266 (1.9%). Over time, use of IVUS changed from none in 2002 to generally increasing use from 2003 (0.1%) to 2016 (2.4%). The age of patients in whom coronary IVUS was used was similar to that of patients in whom IVUS was not used, but in the former group there were fewer male patients, and a higher prevalence of cardiovascular risk factors (hypertension, hypercholesterolemia and diabetes), previous myocardial infarction, previous PCI, multivessel coronary disease, C‐type or bifurcated coronary lesions, and in‐stent restenosis. IVUS was used in 54.8% of elective PCIs and in 19.15% of PCIs of the left main coronary artery. Conclusion: Coronary IVUS has been increasingly used in Portugal since 2003. It is used preferentially in elective PCIs, and in patients with higher cardiovascular risk, with more complex coronary lesions and lesions of the left main coronary artery. Palavras‐chave: Ecografia intravascular, Intervenção coronária percutânea, Doença coronária, Cardiologia, Keywords: Intravascular ultrasound, Percutaneous coronary intervention, Coronary disease, Cardiology

Introduction: In obstructive hypertrophic cardiomyopathy (HCM), alcohol septal ablation (ASA) can lead to gradient reduction and symptom improvement. We aimed to assess the efficacy and safety of ASA in a long-term outcome study. Methods: We analyzed patients who underwent ASA over a seven-year period in a tertiary center. The primary echocardiographic endpoint was >50% reduction in left ventricular outflow tract (LVOT) gradient within a year of the procedure. The primary clinical endpoints were improvement in functional capacity and a combined endpoint of cardiac death and rehospitalization for cardiac cause. The follow-up period was 4.17±2.13 years. Results: A total of 80 patients, mean age 63.9±12.3 years, 30.0% male, were analyzed. Baseline LVOT gradient was 96.3±34.6 mmHg and interventricular septal thickness was 21.6±3.1 mm. Minor complications were observed in 6.3% and major complications in 2.5%, and 8.8% received a permanent pacemaker.The primary echocardiographic endpoint was achieved by 85.7%. At three-month follow-up, LVOT gradient was 25.8±26.0 mmHg in the successful procedure group, compared to 69.2±35.6 mmHg in the other patients (p=0.001). At six months, LVOT gradient was 27.1±27.4 vs. 58.2±16.6 mmHg (p=0.024). Among 74 patients in NYHA class III/IV before the procedure, 57 (77%) improved to NHYA class I/II. The combined primary clinical endpoint (cardiac death and rehospitalization for cardiac cause) was observed in 27.5% (n=22). In the unsuccessful group, the combined endpoint was observed in 54.5%, compared to only 22.7% in the successful group. Only two patients died of cardiac causes. Conclusion: ASA is a safe procedure with a high success rate. Patients who achieved significant reductions in LVOT gradient suffered less cardiac death and rehospitalization for cardiac cause. Resumo: Introdução: Na miocardiopatia hipertrófica obstrutiva (MCHO), ablação septal alcoólica (ASA) pode levar a redução do gradiente e melhoria sintomática. O objetivo foi avaliar a eficácia e segurança da ASA no desfecho a longo prazo. Métodos: Análise de doentes submetidos a ASA, durante sete anos, num centro terciário. Endpoint primário ecocardiográfico: redução >50% do gradiente na câmara de saída do ventrículo esquerdo (CSVE) durante o primeiro ano após o procedimento. Endpoints primários clínicos: a) melhoria da capacidade funcional; b) endpoint combinado: morte de causa cardíaca+hospitalização de causa cardíaca. Tempo de seguimento 4,17±2,13 anos. Resultados: Oitenta doentes, idade média de 63,9±12,3 anos, 30,0% homens. De base, o gradiente na CSVE era 96,3±34,6 mmHg, espessura do septo interventricular 21,6±3,1 mm. Complicações minor foram verificadas em 6,3%, complicações major em 2,5% e 8,8% receberam um pacemaker definitivo. O endpoint primário ecocardiográfico foi atingido em 85,7%. Aos três meses, o gradiente na CSVE foi de 25,8±26,0 mmHg no grupo com sucesso no procedimento contrastando com 69,2±35,6 mmHg nos restantes doentes (p=0,001). Aos seis meses, os gradientes na CSVE foram 27,1±27,4 versus 58,2±16,6 mmHg (p=0,024).De entre os 74 doentes em classe NYHA III/IV antes do procedimento, 57 (77%) melhoraram para classe NYHA I/II. O endpoint primário combinado (morte de causa cardíaca+hospitalização de causa cardíaca) verificou-se em 27,5% (n=22). No grupo sem sucesso, o endpoint primário composto verificou-se em 54,5%, contrastando com apenas 22,7% no grupo com sucesso. Apenas dois doentes apresentaram morte de causa cardíaca. Conclusão: A ASA é um procedimento seguro com elevada taxa de sucesso. Doentes que atingiram redução significativa do gradiente na CSVE apresentam menor morte de causa cardíaca e hospitalização de causa cardíaca. Keywords: Obstructive hypertrophic cardiomyopathy, Alcohol septal ablation, Left ventricular outflow tract gradient, Palavras chave: Miocardiopatia hipertrófica obstrutiva, Ablação septal alcoólica, Gradiente na câmara de saída do ventrículo esquerdo

Coronary artery fistulas (CAFs) are rare abnormal communications between a normal coronary artery and a cardiac chamber or great vessel, such as the pulmonary artery, bypassing the myocardial capillary network. We report the case of a 17-year-old male with a medical history of pulmonary valve stenosis, who presented with progressive dyspnea and fatigue. Transthoracic Doppler echocardiography showed multiple continuous flows both on the apical interventricular septum and entering the left atrium. A tortuous CAF arising from the left main coronary artery to the left atrium was revealed by coronary angiography. The lesion was successfully closed percutaneously using an off-label Amplatzer™ Duct Occluder II Additional Sizes with a backup support of a modified “mother–child” system. This case highlights the effort of both pediatric and adult cardiology teams to come up with new potential strategies and combined techniques to overcome the difficulties of managing complicated CAFs, such as the use of percutaneous coronary intervention techniques and the selection of the most adequate occlusion devices, even when used off-label.

Purpose Noninvasive ischemia testing (NIST) is recommended for most patients suspected to have stable coronary artery disease (CAD) before invasive coronary angiography (ICA). We sought to assess the diagnostic predictive ability of NIST over clinical risk profiling in a contemporary sample of patients undergoing the currently recommended diagnostic triage strategy. Methods From 2006 to 2011, 2,600 consecutive patients without known CAD undergoing elective ICA in a single tertiary-care center were retrospectively identified and the prevalence of obstructive CAD determined. To understand the incremental value of frequently used clinical parameters in predicting obstructive CAD, receiver operating characteristic curves were plotted for six sequential models starting with Framingham risk score and then progressively adding multiple clinical factors and finally NIST results. Results At ICA 1,268 patients (48.8%) had obstructive CAD. The vast majority (85%) were classified in an intermediate clinical pretest probability of CAD and NIST prior to ICA was used in 86% of the cohort. The most powerful correlate of obstructive CAD was the presence of severe angina (odds ratio (OR) = 9.1; 95% confidence interval (CI) 4.3-19.1). Accordingly, the incorporation of NIST in a sequential model had no significant effect on the predictive ability over that achieved by clinical and symptomatic status model (C-statistic 0.754; 95% CI 0.732-0.776, p = 0.28). Conclusions Less than half the patients with suspect stable obstructive CAD referred to a tertiary-level center for elective ICA had the diagnosis confirmed. In this clinical setting, the results of NIST may not have the power to change the discriminative ability over clinical judgment alone.

Resumo: Introdução: A insuficiência valvular mitral (IM) é a valvulopatia mais comum e começa agora a ser alvo de abordagens percutâneas. O dispositivo MitraClip é virtualmente o único dispositivo com uma experiência considerável, ultrapassando atualmente as 20 000 implantações. Objetivo: Descrever a experiência inicial da terapêutica percutânea da IM com o dispositivo MitraClip. Métodos: Descrevem‐se os primeiros seis casos de MitraClip realizados nesta instituição (idade 58,5 ± 13,1 anos), com IM 4 + de etiologia funcional e insuficiência cardíaca classe III ou IV (n = 3) da New York Heart Association (NYHA), sem indicação cirúrgica ou recusados para cirurgia, com um follow‐up médio 290 ± 145 dias avaliado clinicamente pela sobrevivência, classe funcional, teste da marcha dos seis minutos, estudo ecocardiográfico e determinação do proBNP. Resultados: O sucesso do procedimento (IM ≤ 2 +) foi de 100%, com um tempo de procedimento de 115,8 ± 23,7 mins, sem intercorrências intra‐hospitalares, alta entre o 4.° e 8.° dia e uma melhoria consistente no teste da marcha dos seis minutos (329,8 ± 98,42 versus 385,3 ± 106,95 m) e da classe funcional (três dos pacientes com melhoria de duas classes funcionais NYHA). Na totalidade do follow‐up (290 ± 145 dias) constatam‐se dois óbitos, em dois dos quatro pacientes que tinham sido considerados para transplantação cardíaca. Conclusões: O procedimento de implantação do dispositivo MitraClip é seguro, com uma alta taxa de sucesso imediato nos pacientes com IM funcional. A médio prazo o benefício clínico parece esbater‐se ou anular‐se nos pacientes com insuficiência cardíaca mais terminal, nomeadamente nos pacientes a aguardar ou recusados para transplante. Abstract: Introduction: Mitral regurgitation (MR) is the most common valvular disease and has recently become the target of a number of percutaneous approaches. The MitraClip is virtually the only device for which there is considerable experience, with more than 20 000 procedures performed worldwide. Objective: To describe our initial experience of the percutaneous treatment of MR with the MitraClip device. Methods: We describe the first six MitraClip cases performed in this institution (mean age 58.5±13.1 years), with functional MR grade 4+ and New York Heart Association (NYHA) heart failure class III or IV (n=3), with a mean follow‐up of 290±145 days. Results: Procedural success (MR ≤2+) was 100%. Total procedure time was 115.8±23.7 min, with no in‐hospital adverse events and discharge between the fourth and eighth day, and consistent improvement in the six‐minute walk test (329.8±98.42 vs. 385.33±106.95 m) and in NYHA class (three patients improved by two NYHA classes). During follow‐up there were two deaths, in two of the four patients who had been initially considered for heart transplantation. Conclusion: In patients with functional MR the MitraClip procedure is safe, with both a high implantation and immediate in‐hospital success rate. A longer follow‐up suggests that the clinical benefit decreases or disappears completely in patients with more advanced heart disease, namely those denied transplantation or on the heart transplant waiting list. Palavras‐chave: MitraClip, Insuficiência mitral, Reparação percutânea da válvula mitral, Insuficiência mitral funcional, Keywords: MitraClip, Mitral regurgitation, Percutaneous mitral valve repair, Functional mitral regurgitation

A 39-year-old woman underwent uneventful percutaneous occlusion of an ostium secundum atrial septal defect (ASD) with a 22 mm Ultrasept ASD Occluder®. Transesophageal echocardiography (TEE) performed two years after implantation revealed a de novo residual left-to-right shunt through the correctly implanted device. Three-dimensional transesophageal echocardiography (3D TEE) further clarified this finding by showing a perforation of the device membrane coating. The patient underwent transcatheter closure of the residual shunt with a 20 mm Ultrasept PFO® device. The procedure was guided by fluoroscopy and real-time 3D TEE. At the end of the procedure 3D TEE documented correct device deployment with complete defect coverage and absence of residual shunt. Resumo: Doente de 39 anos, género feminino, foi submetida com sucesso a encerramento percutâneo de comunicação interauricular do tipo ostium secundum com dispositivo Ultrasept Atrial Septal Defect Occluder® de 22 mm. O ecocardiograma transesofágico, realizado dois anos após implantação, revelou shunt esquerdo-direito residual de novo através do dispositivo corretamente implantado. O ecocardiograma transesofágico tridimensional (ETE3D) objetivou uma perfuração do revestimento do dispositivo. A doente foi submetida a encerramento percutâneo do shunt residual com dispositivo UltraSept PFO Occluder® de 20 mm. O procedimento foi guiado por fluoroscopia e ETE3D. No final do procedimento por ETE3D, foi documentado o correto posicionamento do dispositivo, com completa cobertura da perfuração e ausência de shunt residual. Keywords: Atrial septal defect, Residual shunt, Percutaneous intervention, Palavras-chave: Comunicação interauricular, Shunt residual, Intervenção percutânea

Resumo: O índice de resistência da microcirculação (IRM) permite o estudo quantitativo, invasivo e em tempo real do estado da microcirculação coronária. O objetivo principal foi a validação da técnica de determinação do IRM num modelo animal de grande porte. O objetivo secundário foi a comparação de duas doses de papaverina, 5 e 10 mg, para indução de hiperémia máxima e quanto à sua evolução temporal. Foram estudados oito porcos. Avaliou‐se a Pd e o Tmn em condições basais e com hiperémia máxima induzida pela injeção intracoronária de 5 ou 10 mg de papaverina e sucessivamente aos dois, cinco, oito e dez minutos. Para o compromisso da microcirculação foram injetadas microesferas de 40 μm de diâmetro. Em cada animal foram realizadas 14 determinações de IRM. Não se observaram diferenças significativas entre as duas doses de papaverina quanto às repostas da Pd e ao valor de IRM obtido, 11 ± 4,5 U com 5 mg de papaverina e de 10,6 ± 3 U com a dose de 10 mg (p = 0,612). A evolução temporal dos valores de IRM foi semelhante, deixando de haver diferenças relativamente ao valor basal após cinco minutos. Com a injeção das microesferas houve uma elevação significativa do IRM (41 ± 16 U, p = 0,001). O IRM avalia em tempo real a resistência da microcirculação coronária. A administração intracoronária de 5 mg de papaverina é tão eficaz como a de 10 mg para a indução de hiperémia máxima, com o retorno às condições basais a ocorrer cinco minutos após a sua administração. Abstract: Introduction: The index of microcirculatory resistance (IMR) enables/provides quantitative, invasive, and real‐time assessment of coronary microcirculation status. Aims: The primary aim of this study was to validate the assessment of IMR in a large animal model, and the secondary aim was to compare two doses of intracoronary papaverine, 5 and 10 mg, for induction of maximal hyperemia and its evolution over time. Methods: Measurements of IMR were performed in eight pigs. Mean distal pressure (Pd) and mean transit time (Tmn) were measured at rest and at maximal hyperemia induced with intracoronary papaverine, 5 and 10 mg, and after 2, 5, 8 and 10 minutes. Disruption of the microcirculation was achieved by selective injection of 40‐μm microspheres via a microcatheter in the left anterior descending artery. Results: In each animal 14 IMR measurements were made. There were no differences between the two doses of papaverine regarding Pd response and IMR values – 11±4.5 U with 5 mg and 10.6±3 U with 10 mg (p=0.612). The evolution of IMR over time was also similar with the two doses, with significant differences from resting values disappearing after five minutes of intracoronary papaverine administration. IMR increased with disrupted microcirculation in all animals (41±16 U, p=0.001). Conclusions: IMR provides invasive and real‐time assessment of coronary microcirculation. Disruption of the microvascular bed is associated with a significant increase in IMR. A 5‐mg dose of intracoronary papaverine is as effective as a 10‐mg dose in inducing maximal hyperemia. After five minutes of papaverine administration there is no significant difference from resting hemodynamic status. Palavras‐chave: Microcirculação coronária, Índice de resistência da microcirculação, Modelo animal, Keywords: Coronary microcirculation, Index of microcirculation resistance, Animal model

Resumo: Introdução: A ablação septal alcoólica (ASA) é a forma percutânea de tratamento invasivo da cardiomiopatia hipertrófica obstrutiva (CMHO). A propósito da descrição da experiência no nosso centro, procurar-se-á rever as indicações, os aspetos técnicos e práticos e os resultados da ASA, assim como a sua posição atual em comparação com a miectomia cirúrgica. Objetivo: Avaliar os resultados da ASA numa série de doentes consecutivos tratados nos primeiros quatro anos de atividade. Métodos: Estudo retrospetivo de todos os doentes, consecutivos e não selecionados, com CMHO, tratados por ASA, entre janeiro de 2009 e fevereiro de 2013. Resultados: Durante o período de quatro anos foram tratados com ASA 40 doentes. Em três doentes (7,5%) repetiu-se o procedimento. A taxa de sucesso foi de 84%. A taxa de complicações minor foi de 7,5%. Foi necessário implantar pacemaker definitivo por bloqueio auriculoventricular em dois doentes (6%, do subgrupo sem pacemaker prévio). A taxa de complicações major foi de 5%. Não houve mortalidade intra-hospitalar nesta população. Durante o seguimento clínico (22 ± 14 meses) a mortalidade cardiovascular foi de 2,5%. A mortalidade total foi de 5%. Discussão e conclusão: Os resultados apresentados refletem a experiência inicial do tratamento com ASA no nosso centro. O procedimento foi bem-sucedido na maioria dos doentes, sendo a taxa de sucesso semelhante à descrita em outras séries, mas ainda com possibilidade de beneficiar de uma melhor seleção dos doentes. A intervenção também se revelou segura, com uma baixa ocorrência de complicações e sem mortalidade associada. A ASA é uma alternativa percutânea no tratamento invasivo dos doentes com CMHO refratária à terapêutica médica. Abstract: Introduction: We describe our center's initial experience with alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy. The procedure, its indications, results and clinical outcomes will be addressed, as will its current position compared to surgical myectomy. Objective: To assess the results of ASA in all patients treated in the first four years of activity at our center. Methods: We retrospectively studied all consecutive and unselected patients treated by ASA between January 2009 and February 2013. Results: In the first four years of experience 40 patients were treated in our center. In three patients (7.5%) the intervention was repeated. Procedural success was 84%. Minor complications occurred in 7.5%. Two patients received a permanent pacemaker for atrioventricular block (6% of those without previous pacemaker). The major complication rate was 5%. There were no in-hospital deaths; during clinical follow-up (22±14 months) cardiovascular mortality was 2.5% and overall mortality was 5%. Discussion and Conclusion: The results presented reflect the initial experience of our center with ASA. The success rate was high and in line with published results, but with room to improve with better patient selection. ASA was shown to be safe, with a low complication rate and no procedure-related mortality. Our experience confirms ASA as a percutaneous alternative to myectomy for the treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy refractory to medical treatment. Palavras-chave: Cardiomiopatia hipertrófica obstrutiva, Tratamento percutâneo, Ablação septal alcoólica, Keywords: Obstructive hypertrophic cardiomyopathy, Percutaneous treatment, Alcohol septal ablation

Resumo: A doença cardiovascular é uma das principais causas de mortalidade e morbilidade em todo o mundo e, apesar de os avanços na terapêutica médica e intervenção percutânea, o prognóstico de doenças como a cardiopatia isquémica e o pós-enfarte do miocárdio continuam a ser consideravelmente inferiores ao desejado. Essa situação justifica a aposta na investigação de novas ferramentas terapêuticas, como é o caso da terapia com células estaminais. O conhecimento mais vasto das células de linhagem hematopoiética levou à sua utilização numa primeira fase, tanto em investigação pré-clínica como clínica. A existência de alguns resultados controversos e de uma melhoria correspondente às expectativas iniciais abriu a caminho a outros tipos celulares com potencial terapêutico, como é o caso das células precursoras do mesênquima. Estas células têm características imunomoduladoras que lhes conferem um racional de utilidade terapêutica em várias áreas da Cardiologia. Neste artigo, os autores efetuam a revisão do estado da arte relativo à utilização de células precursoras do mesênquima, desde a sua investigação pré-clínica até à sua utilização em ensaios clínicos em doentes com cardiopatia isquémica, insuficiência cardíaca de várias etiologias ou disritmias. É ainda perspetivado o futuro da utilização destas células. Abstract: Cardiovascular disease is among the main causes of mortality and morbidity worldwide. Despite significant advances in medical and interventional therapy, the prognosis of conditions such as ischemic heart disease is still dismal. There is thus a need to investigate new therapeutic tools, one of which is stem cell therapy. Hematopoietic stem cells are the most studied type, and the fact that their biology is relatively well understood has led to their being used in preclinical research and clinical trials. However, the results of some of these studies have been controversial, which has opened the way for studies on other cell types, such as mesenchymal stem cells. These cells have immunomodulatory properties which suggest that they have therapeutic potential in cardiology. In the present article, the authors review the state of the art regarding mesenchymal stem cells, from basic and translational research to their use in clinical trials on ischemic heart disease, heart failure and arrhythmias, and discuss possible future uses. Palavras-chave: Células precursoras do mesênquima, Sistema cardiovascular, Cardiopatia isquémica, Insuficiência cardíaca, Disritmias, Investigação translacional, Keywords: Mesenchymal stem cells, Cardiovascular system, Myocardial ischemia, Heart failure, Arrhythmias, Translational research

Acromegaly is a rare disease, mostly caused by a growth hormone (GH)-secreting benign pituitary tumor, with an increased production of GH and insulin-like growth factor 1 (IGF-1). Cardiovascular complications are common and are associated with cardiomyocyte apoptosis and concentric cardiac hypertrophy. Suppression of GH and IGF-1 appears to improve cardiac function only in the short term, with little or no decrease in left ventricular mass or improvement in cardiac function after prolonged treatment. Alcohol septal ablation (ASA) has emerged as a minimally invasive alternative to septal myectomy, with significant improvement in symptoms, gradients and left ventricular remodeling. In this report, we describe the case of a 73-year-old woman with acromegaly due to a pituitary adenoma diagnosed and treated surgically at the age of 38 but with recurrence and reoperation at the age of 50. She was referred to our cardiology department due to a three-month history of progressively worsening exercise-induced dyspnea and orthopnea under optimal medical therapy. Echocardiography and magnetic resonance imaging revealed severe basal hypertrophy of the interventricular septum (19 mm), dynamic left ventricular outflow tract obstruction with a gradient of 70 mmHg at rest and 120 mmHg with Valsalva maneuver, and systolic anterior movement (SAM). Genetic testing excluded the most frequent forms of familial hypertrophic cardiomyopathy. ASA was performed with injection of 2 cc of alcohol in the first septal branch of the left coronary artery, without complications. At one-year reassessment, significant clinical and echocardiographic improvement was noted, with disappearance of SAM. To our knowledge, there have been no previously reported cases of ASA in hypertrophic cardiomyopathy due to acromegaly. We report a case of successful ASA in acromegalic cardiomyopathy. Resumo: A acromegalia é uma doença rara, causada essencialmente por um tumor benigno da glândula pituitária secretor de hormona de crescimento (HC), provocando uma produção excessiva de HC e de fator de crescimento semelhante à insulina tipo 1 (IGF-1). As complicações cardiovasculares são comuns e têm sido associadas à apoptose de cardiomiócitos e à hipertrofia cardíaca concêntrica. A supressão de HC e de IGF-1 parece melhorar a função cardíaca apenas a curto prazo, com pouco ou nenhum efeito na redução da massa ventricular esquerda ou na melhoria da função cardíaca a longo prazo. A ablação septal alcoólica (ASA) surgiu como uma alternativa minimamente invasiva à miectomia septal com melhoria significativa a nível de sintomas, gradientes e remodelagem ventricular esquerda. Neste caso clínico, descrevemos as imagens de uma mulher de 73 anos de idade, com acromegalia devido a um adenoma da glândula pituitária diagnosticado, e submetida a cirurgia aos 38 anos, mas com recorrência e reoperação aos 50 anos. A doente foi referenciada ao nosso departamento de cardiologia por um quadro com três meses de evolução de dispneia de esforço e ortopneia com agravamento progressivo sob terapêutica médica otimizada. O ecocardiograma e ressonância magnética cardíaca revelaram uma hipertrofia grave da porção basal do septo interventricular (19 mm), com um gradiente obstrutivo dinâmico da câmara de saída do ventrículo esquerdo de 70 mmHg em repouso e de 120 mmHg com a manobra de Valsalva e com movimento sistólico anterior (SAM) da válvula mitral. A avaliação genética excluiu as formas mais frequentes de cardiomiopatia hipertrófica (CMH) familiar. A ASA foi realizada com 2 cc de injeção de álcool na primeira artéria coronária septal, sem complicações. Na reavaliação do primeiro ano, houve uma melhora clínica e ecocardiográfica significativa com o desaparecimento de SAM. Tanto quanto é do nosso conhecimento, não existe na literatura a descrição de casos de ASA em doentes com CMH por acromegalia. Relatamos aqui o caso de uma ASA bem-sucedida numa cardiomiopatia acromegálica. Keywords: Hypertrophic cardiomyopathy, Acromegaly, Alcohol septal ablation, Palavras-chave: Cardiomiopatia hipertrófica, Acromegalia, Ablação septal alcoólica

Left ventricular pseudoaneurysm is a rare complication of acute myocardial infarction, cardiac surgery, trauma or infection.Since surgical repair is associated with high morbidity and mortality, percutaneous closure has been described as an alternative.In this regard, we present a case in which a symptomatic large left ventricular pseudoaneurysm was treated by percutaneous closure due to the patient's high surgical risk, using a double snare technique.Despite the technical difficulties, this procedure had a good final result followed by clinical success, confirming that this procedure is an effective alternative to surgery in high-risk patients. Resumo: O pseudoaneurisma do ventrículo esquerdo consiste numa complicação rara após enfarte agudo do miocárdio, cirurgia cardíaca, trauma ou infeção.Dado que a sua correção cirúrgica se associa a uma elevada morbi-mortalidade, o encerramento percutâneo tem sido descrito como uma possível alternativa.Neste contexto, apresentamos um caso de um volumoso pseudoaneurisma do ventrículo esquerdo, o qual fora encerrado percutaneamente, utilizando uma técnica de duplo snare, devido ao elevado risco cirúrgico da doente.Apesar de algumas dificuldades técnicas, este procedimento teve um bom resultado final, com sucesso clínico a longo prazo, confirmando que o encerramento percutâneo constitui uma alternativa efetiva à cirurgia, em doentes de alto risco operatório. Keywords: Left ventricle, Pseudoaneurysm, Percutaneous closure, Double snare, Palavras-chave: Ventrículo esquerdo, Pseudoaneurisma, Encerramento percutâneo, Duplo snare

Pseudoaneurysm of the ascending aorta is a rare complication, usually after thoracic surgery or trauma.Since surgical repair is associated with very high morbidity and mortality, percutaneous closure has been described as an alternative.In this regard, we present a case in which a symptomatic large pseudoaneurysm of the ascending aorta was treated percutaneously due to the high surgical risk.Despite the technical difficulties, this procedure had a good final result followed by clinical success. Resumo: O pseudoaneurisma da aorta ascendente consiste numa complicação rara, habitualmente na sequência de cirurgia cardiotorácica ou traumatismo.Dado que a reparação cirúrgica do mesmo se associa a uma elevada morbimortalidade, o encerramento percutâneo tem vindo a ser descrito como uma alternativa viável.Neste contexto, apresentamos um caso caracterizado por um volumoso e sintomático pseudoaneurisma da aorta ascendente, o qual fora submetido a tratamento percutâneo, devido ao elevado risco cirúrgico.Apesar das dificuldades do ponto de vista técnico, este procedimento obteve um bom resultado final, com sucesso em termos clínicos. Keywords: Ascending aorta, Pseudoaneurysm, Percutaneous closure, Palavras-chave: Aorta ascendente, Pseudoaneurisma, Encerramento percutâneo

Canine dirofilariosis is a life-threatening parasitic disease that is increasingly reported worldwide. Once diagnosed the main treatment goals are to improve the animal’s clinical condition and to eliminate all life stages of the parasite with minimal posttreatment side effects. This can be achieved through mechanical, surgical, or chemotherapeutical approaches. Currently, manual extraction is the preferred method to remove adult heartworms due to its diminished invasiveness, reduced damage to the vascular endothelium, and shortened anaesthesia duration. However, it remains an expensive technique that can be highly traumatic. To address this issue, a nontraumatic homemade catheter-guided snare was developed for heartworm removal by adapting and folding a 0.014-inch coronary wire (BMW, Abbott Vascular). Transvenous heartworm extraction was performed on a dog severely infected with adult heartworms by inserting the modified snare into a 6-F Judkins right coronary guiding catheter BMW (Cordis) and advancing it into the right ventricle under fluoroscopic guidance. Fifteen adult specimens of Dirofilaria immitis were successfully extracted from the pulmonary artery and right ventricle without complications. To assure the death of both larvae and adults, postoperative treatment was successfully managed using ivermectin, doxycycline, and melarsomine, with no recurrence after surgery.

Mesenchymal stem/stromal cells have unique properties favorable to their use in clinical practice and have been studied for cardiac repair. However, these cells are larger than coronary microvessels and there is controversy about the risk of embolization and microinfarctions, which could jeopardize the safety and efficacy of intracoronary route for their delivery. The index of microcirculatory resistance (IMR) is an invasive method for quantitatively assessing the coronary microcirculation status.To examine heart microcirculation after intracoronary injection of mesenchymal stem/stromal cells with the index of microcirculatory resistance.Healthy swine were randomized to receive by intracoronary route either 30x106 MSC or the same solution with no cells (1% human albumin/PBS) (placebo). Blinded operators took coronary pressure and flow measurements, prior to intracoronary infusion and at 5 and 30 minutes post-delivery. Coronary flow reserve (CFR) and the IMR were compared between groups.CFR and IMR were done with a variance within the 3 transit time measurements of 6% at rest and 11% at maximal hyperemia. After intracoronary infusion there were no significant differences in CFR. The IMR was significantly higher in MSC-injected animals (at 30 minutes, 14.2U vs. 8.8U, p = 0.02) and intragroup analysis showed a significant increase of 112% from baseline to 30 minutes after cell infusion, although no electrocardiographic changes or clinical deterioration were noted.Overall, this study provides definitive evidence of microcirculatory disruption upon intracoronary administration of mesenchymal stem/stromal cells, in a large animal model closely resembling human cardiac physiology, function and anatomy.

We report the case of a 52-year-old man who presented to our emergency department (ED) after three episodes of syncope in the seven hours before admission. During his stay in the ED he had recurrent ventricular tachycardia (VT) requiring external electrical cardioversion. A 12-lead electrocardiogram (ECG) showed a short QT (SQT) interval (270 ms, QTc 327 ms), with frequent R-on-T extrasystoles triggering sustained polymorphic VT. After exclusion of other precipitating causes, the patient was diagnosed as having SQT syndrome (SQTS) according to the Gollob criteria. To our knowledge, this is the first known documentation of an SQT-caused arrhythmic episode on a 12-lead ECG, as well as the first reported case of SQTS in Portugal. The patient received an implantable cardioverter-defibrillator and was discharged. At a follow-up assessment 14 months later he was symptom-free, interrogation of the device showed no arrhythmic events, and the ECG showed a QT interval of 320 ms (QTc 347 ms). Resumo: Os autores apresentam o caso de um homem com 52 anos que recorreu ao Serviço de Urgência (SU) por três episódios sincopais ocorridos nas últimas sete horas pré-admissão hospitalar. Durante a estadia no SU, objetivaram-se períodos de taquicardia ventricular (TV) recorrente, com necessidade de cardioversão elétrica externa, tendo o doente sido transferido para a unidade de cuidados intensivos cardíacos para monitorização e orientação clínica. O electrocardiograma de 12 derivações (ECG) registado no contexto de um dos episódios revelava um intervalo QT curto (260 ms, QTc 327 ms) em ritmo sinusal, com fenómeno de R-on-T desencadeando TV polimórfica mantida. Após exclusão de causas precipitantes, o doente foi classificado como tendo Síndrome QT Curto (SQTC), de acordo com os critérios de Gollob. Tanto quanto é do nosso conhecimento, esta é a primeira documentação no ECG de um episódio arrítmico causado por SQTC e a primeira descrição de um caso de SQTC em Portugal. Foi submetido a implantação de cardioversor-desfibrilhador implantável (CDI) monocameral e teve alta hospitalar. No seguimento aos 14 meses pós-implantação, o doente negava recorrência da sintomatologia, a interrogação do CDI não mostrou novos episódios arrítmicos, e no ECG persistia um intervalo QT de 320 ms (QTc 347 ms). Keywords: Syncope, Short QT syndrome, Sudden cardiac death, Ventricular arrhythmia, Palavras-chave: Síncope, Síndrome QT curto, Morte súbita cardíaca, Arritmia ventricular