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Introduction: Coronavirus disease (COVID-19) has led to significant changes in healthcare systems and its impact on the treatment of cardiovascular conditions, such as ST-elevation myocardial infarction (STEMI), is unknown in countries where the healthcare systems were not saturated, as was the case in Portugal. As such, we aimed to assess the effect on STEMI admissions and outcomes in Portuguese centers., Methods: We conducted a single-center, observational, retrospective study including all patients admitted to our hospital due to STEMI between the date of the first SARS-CoV-2 case diagnosed in Portugal and the end of the state of emergency (March and April 2020). Patient characteristics and outcomes were assessed and compared with the same period of 2019., Results: A total of 104 STEMI patients were assessed, 55 in 2019 and 49 in 2020 (-11%). There were no significant differences between groups regarding age (62±12 vs. 65±14 years, p=0.308), gender (84.8% vs. 77.6% males, p=0.295) or comorbidities. In the 2020 group, there was a significant decrease in the proportion of patients transported to the hospital in pre-hospital emergency medical transportation (38.2% vs. 20.4%, p=0.038), an increase in system delay (49 [30-110.25] vs. 140 [90-180] minutes, p=0.019), a higher Killip-Kimball class, with a decrease in class I (74.5% vs. 51%) and an increase in class III (1.8% vs. 8.2%) and IV (5.5% vs. 18.4%) (p=0.038), a greater incidence of vasoactive support (3.7% vs. 26.5%, p=0.001), invasive mechanic ventilation usage (3.6% vs. 14.3%, p=0.056), and an increase in severe left ventricular dysfunction at hospital discharge (3.6% vs. 16.3%, p=0.03). In-hospital mortality was 14.3% in the 2020 group and 7.3% in the 2019 group p=0.200)., Conclusion: Despite a lack of significant variation in the absolute number of STEMI admissions, there was an increase in STEMI clinical severity and significantly worse outcomes during the SARS-CoV-2 pandemic. An increase in system delay, impaired pre-hospital care and patient fear of in-hospital infection can partially justify these results and should be the target of future actions in further waves of the pandemic., (Copyright © 2021 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. All rights reserved.)

Purpose: Our goal was to assess usefulness of Pattern Matching Filter (PMF) software combined with the PentaRay catheter for complex premature ventricular contraction (PVC) ablation., Methods: A prospective observational study of consecutive patients referred for complex PVC ablation at our tertiary center from January to September 2018. Patients underwent ablation using a pre-specified mapping strategy with the PMF and the PentaRay catheter (PVCs with ≥97% correlation with the template morphology were collected). Procedural endpoints and acute and 12-months success rates were assessed and compared to a retrospective cohort of patients who also underwent a complex PVC ablation with standard activation mapping performed with a Thermocool SmartTouch catheter., Results: During the nine-month enrollment period, seven patients fulfilled our inclusion criteria, while there were four patients in the control group. Patients treated with the PMF and PentaRay had a fivefold number of points acquired (507±213 vs. 90±62) and a halved procedure time (67±42 vs.130±54 min), required a shorter radiofrequency ablation time (294±112 vs.706±613 sec) and had a higher overall success rate (100% vs.75%) when compared to the standard approach. No major complications occurred in either group., Conclusions: In this first study assessing the combined use of the PentaRay catheter and the PMF for complex PVCs ablation, we demonstrate how this approach can improve the level of detail, accuracy and reliability of the activation map, while reducing the number of radiofrequency applications and procedural time. Further studies are warranted to confirm whether this approach can lead to improved outcomes., (Copyright © 2020 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction and Aims: Catheter ablation has been shown to improve left ventricular (LV) ejection fraction (LVEF) in patients with atrial fibrillation (AF) and heart failure (HF). Our aim was to assess the impact of AF ablation on the outcome of patients with HF and LV systolic dysfunction., Methods: We performed a retrospective observational cohort study of all patients with HF and LVEF <50% and with no apparent cause for systolic dysfunction other than AF who underwent catheter ablation in a tertiary referral center between July 2016 and November 2018. The primary endpoint was a ≥5% improvement in LVEF. Secondary endpoints included improvement in New York Heart Association (NYHA) class and reduction in LV end-diastolic diameter (LVEDD) and left atrial diameter (LAD)., Results: Of 153 patients who underwent AF ablation in this period, 22 (77% male, median age 61 [IQR 54-64] years) fulfilled the inclusion criteria. Median follow-up was 11.1 months (IQR 6.1-19.0). After ablation, median LVEF increased from 40% (IQR 33-41) to 58% (IQR 55-62) (p<0.01), mean NYHA class improved from 2.35±0.49 to 1.3±0.47 (p<0.001), and median LAD and LVEDD decreased from 48.0 (IQR 43.5-51.5) mm to 44 (IQR 40-49) mm (p<0.01) and from 61.0 (IQR 54.0-64.8) mm to 55.0 (52.2-58.0) mm (p<0.01), respectively., Conclusion: In patients with HF and LV systolic dysfunction, AF ablation is associated not only with improved functional status but also with favorable structural remodeling, including improvement in LVEF and decreases in LAD and LVEDD., (Copyright © 2021 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: This study aimed to evaluate the performance of non-vitamin K antagonist oral anticoagulation (NOAC) in patients with previous stroke and non-valvular atrial fibrillation (AF) compared with left atrial appendage occlusion (LAAO) in primary and secondary stroke prevention settings., Methods: This was a prospective, single-center, non-randomized cohort study of 302 consecutive patients with non-valvular AF and at high risk for stroke. Two treatment strategies were compared: LAAO (n=91) and long-term treatment with NOAC (n=149). The primary outcome was the composite endpoint of death, stroke and major bleeding. Propensity score and cause-of-death analyses were performed to compare outcomes., Results: In a mean follow-up of 13 months, there were 30 deaths (LAAO 8.8% vs. NOAC 14.8%), five strokes (LAAO 1.1% vs. NOAC 2.7%) and six major bleeds (LAAO 1.1% vs. NOAC 3.4%). There was a non-significant trend for a lower incidence of the primary endpoint in the LAAO group (11.0% vs. 20.9%; HR 0.42, 95% CI 0.17-1.05, p=0.064). Considering only secondary prevention LAAO patients (34.1% of the LAAO group), there was also a non-significant lower incidence of the primary endpoint (LAAO 6.5% vs. 20.9%; HR 0.30, 95% CI 0.07-1.39, p=0.12). While about a fifth of LAAO patients stopped antiplatelet treatment six months after device implantation due to recurrent minor bleeding, no adverse cardiovascular event or major bleeding occurred in this subset of patients., Conclusion: In this registry-based study, LAAO was a reasonable alternative to NOAC for the prevention of a composite endpoint of all-cause mortality, stroke and major bleeding in patients at high risk for stroke., (Copyright © 2021 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. All rights reserved.)

The number and complexity of percutaneous interventions for the treatment of structural heart disease has increased in clinical practice in parallel with the development of new imaging technologies, in order to render these interventions safer and more accurate. Complementary imaging modalities are commonly used, but they require additional mental reconstruction and effort by the interventional team. The concept of fusion imaging, where two different modalities are fused in real time and on a single monitor, aims to solve these limitations. This is an important tool to guide percutaneous interventions, enabling a good visualization of catheters, guidewires and devices employed, with enhanced spatial resolution and anatomical definition. It also allows the marking of anatomical reference points of interest for the procedure. Some studies show decreased procedural time and total radiation dose with fusion imaging; however, there is a need to obtain data with more robust scientific methodology to assess the impact of this technology in clinical practice. The aim of this review is to describe the concept and basic principles of fusion imaging, its main clinical applications and some considerations about the promising future of this imaging technology., (Copyright © 2020 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction and Aims: Cardiac allograft vasculopathy (CAV) is one of the most significant complications after orthotopic heart transplantation. We aimed to investigate the incidence and predictors of CAV in a large cohort of orthotopic heart transplantation patients., Methods: We conducted a retrospective analysis on a prospective cohort of 233 patients who underwent transplantation between November 2003 and May 2014. Baseline clinical data and invasive coronary angiograms (n=712) performed as part of the follow-up program were analyzed by two independent investigators., Results: We included 157 male and 45 female patients with a median age of 66 years. A third of patients had previous ischemic heart disease, 30% peripheral arterial disease, 37% hypertension and 47% dyslipidemia, and 17% were smokers. Acute moderate or severe rejection occurred in 42 patients during the first year. Over a median follow-up of 2920 days, 18% were diagnosed with CAV, with an incidence of 2.91 cases per 100 person-years. Predictors of CAV were previous ischemic heart disease (HR 2.32, 95% CI 1.21-4.45, p=0.01), carotid artery disease (HR 2.44, 95% CI 1.27-4.71, p<0.01), and donor age (HR 1.04, 95% CI 1.00-1.07, p=0.01)., Conclusion: In a single-center cohort of orthotopic heart transplantation patients, predictors of CAV were previous ischemic heart disease, carotid artery disease and donor age., (Copyright © 2020 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Spontaneous coronary artery dissection is a rare cause of acute coronary syndrome that typically occurs in relatively young patients without classical cardiovascular risk factors for coronary artery disease. The etiology appears to be multifactorial and optimal management is not clearly established, so the treatment strategy is often selected based on clinical presentation and coronary anatomy. We present two cases of spontaneous coronary artery dissection with different initial approaches, highlighting the importance of a case-by-case assessment., (Copyright © 2019 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by ventricular dilatation and impaired systolic function. Familial forms account for 30-50% of cases. Autosomal dominant inheritance is the predominant pattern of transmission. Causal genetic variants have been identified in several genes and molecular diagnosis has implications for genetic counseling and risk stratification., Objective: We aimed to estimate the frequency of genetic variants and the molecular basis of DCM in Portugal., Methods: We performed a multicenter study of unrelated patients, recruited between 2013 and 2014. Variants in 15 genes were screened using PCR with direct sequencing (next-generation sequencing with at least 30-fold coverage combined with Sanger sequencing)., Results: A total of 107 patients were included, 64 (60%) men, mean age at diagnosis 38±13 years, with 48 (45%) familial cases. In total, 31 rare variants in eight genes (mainly in MYBPC3, TNNT2 and LMNA) were identified, in 28 patients (26%). Only four variants had been previously described in association with DCM, 11 with hypertrophic cardiomyopathy, and nine variants were novel. Four variants were likely pathogenic and the remainder were of uncertain significance. We found no major differences in the main clinical and imaging characteristics between patients with or without rare variants and patients with likely pathogenic variants., Conclusions: Our results reflect the complexity and diversity of DCM genetics. For better interpretation of the pathogenicity of the variants found and their causative roles in DCM, molecular cascade screening of families is imperative. Further insight into genotype-phenotype correlations and risk stratification is desirable., (Copyright © 2019 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Percutaneous closure of the left atrial appendage (LAA) is a promising therapy in patients with atrial fibrillation with high risk for stroke and contraindication for oral anticoagulation (OAC). Intracardiac echocardiography (ICE) may make this percutaneous procedure feasible in patients in whom transesophageal echocardiography (TEE) is inadvisable. Our aim was to assess the efficacy and safety of LAA closure and the feasibility of ICE compared to TEE to guide the procedure., Methods: In this cohort study of patients who underwent LAA closure between May 2010 and January 2017, clinical and imaging assessment was performed before and after the procedure., Results: In 82 patients (mean age 74±8 years, 64.4% male) the contraindications for OAC were severe bleeding or anemia (65%), high bleeding risk (14%), labile INR (16%), or recurrent embolic events (5%). The procedural success rate was 96.3%. The procedure was guided by TEE or ICE, and no statistically significant differences were observed between the two techniques. During follow-up, one patient had an ischemic stroke at 12 months, two had bleeding complications at six months, and there were four non-cardiovascular deaths. Embolic and bleeding events were less frequent than expected from the observed CHA 2 DS 2 VASc (0.6% vs. 6.3%; p<0.001) and HAS-BLED (1.2% vs. 4.1%; p<0.001) risk scores., Conclusions: In this population percutaneous LAA closure was shown to be safe and effective given the lower frequency of events than estimated by the CHA 2 DS 2 VASc and HAS-BLED scores. The clinical and imaging results of procedures guided by ICE in the left atrium were not inferior to those guided by TEE., (Copyright © 2018 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: In adults, unexplained left ventricular hypertrophy is usually due to sarcomeric hypertrophic cardiomyopathy (HCM). Fabry disease (FD) is rare but may mimic sarcomeric HCM, and has an adverse prognosis in the absence of specific treatment. We aimed to assess cardiologists' awareness of FD based on data from the Portuguese Registry of Hypertrophic Cardiomyopathy., Methods: A total of 811 index patients, aged 55 ± 16 years, 486 (59.9%) male, were included. Three groups were characterized: A - 128 patients, 74 (57.8%) male, with pathogenic or likely pathogenic mutation(s) in sarcomeric genes; B - 234 patients, 146 (62.4%) male, with negative genetic testing; and C - 449 patients, 266 (59.2%) male, no genetic testing performed. The groups were compared in terms of whether FD was excluded in the registry. Potential red flags for FD were also analyzed and compared between groups., Results: Patients in group A were younger and more frequently had familial HCM (A - 53.9% vs. B - 20.1% vs. C - 18.3%; p <0.001). FD was recorded as excluded in 217 (26.8%), similar in all groups; GLA gene testing was performed in only 50/217 patients (A - 48.6%, B - 25.7%, p = 0.019; C - 13.4%, p = 0.036 for B vs. C), mostly in women (p <0.001) in groups B and C. Alpha-galactosidase A (α-Gal A) activity was assessed in 39/217 (18%) patients, with no difference between groups, but more often in men (p = 0.005). Among patients with potential red flags for FD, only 46.7% underwent specific tests (GLA gene testing and/or α-Gal A activity). When GLA genotyping was performed no mutations were identified., Conclusions: There is a need to improve cardiologists' alertness for the identification of FD among the Portuguese HCM population., (Copyright © 2018 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Exercise-induced cardiac remodeling is frequent in athletes. This adaptation is structurally manifested by an increase in cardiac dimensions and mass. Soldiers are also subject to intense physical exercise, although with different characteristics., Objective: To compare exercise-induced cardiac remodeling in competitive athletes and in soldiers on a special forces training course., Methods: We studied 17 soldiers (all male and Caucasian, mean age 21±3 years) who completed a special forces course and 17 basketball players (47.3% male, 64.7% Caucasian, mean age 21±3 years). Assessment included a transthoracic echocardiogram and analysis of myocardial mechanics. This assessment was performed at the beginning and end of the military course and the sports season, respectively., Results: Cardiac remodeling was observed in both groups. The soldiers presented a predominantly eccentric pattern, with increased left ventricular (LV) size (49.7±3.2 vs. 52.8±3.4 mm; p<0.01), increased LV mass (93.1±7.7 vs. 100.2±11.4 g/m2; p<0.01) and decreased relative wall thickness (0.40±0.1 vs. 0.36±0.1; p=0.05). The basketball players showed a concentric pattern, with decreased LV size (52.0±4.7 vs. 50.4±4.7 mm; p=0.05), and increased relative wall thickness (0.33±0.1 vs. 0.36±0.1; p=0.05). Although there was no significant difference in LV myocardial strain in the groups separately, when compared there was a significant decrease (-20.2±1.6% vs. -19.4±2.1%; p=0.03)., Conclusion: Cardiac remodeling was frequent, with an eccentric pattern in soldiers and a concentric pattern in basketball players. Myocardial deformation may represent a physiological adaptation to physical exercise., (Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal., Methods: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected., Results: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year., Conclusions: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease., (Copyright © 2017 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)