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Introduction and Objectives: Although automatic artificial intelligence (AI) coronary angiography (CAG) segmentation is arguably the first step toward future clinical application, it is underexplored. We aimed to (1) develop AI models for CAG segmentation and (2) assess the results using similarity scores and a set of criteria defined by expert physicians., Methods: Patients undergoing CAG were randomly selected in a retrospective study at a single center. Per incidence, an ideal frame was segmented, forming a baseline human dataset (BH), used for training a baseline AI model (BAI). Enhanced human segmentation (EH) was created by combining the best of both. An enhanced AI model (EAI) was trained using the EH. Results were assessed by experts using 11 weighted criteria, combined into a Global Segmentation Score (GSS: 0-100 points). Generalized Dice Score (GDS) and Dice Similarity Coefficient (DSC) were also used for AI models assessment., Results: 1664 processed images were generated. GSS for BH, EH, BAI and EAI were 96.9+/-5.7; 98.9+/-3.1; 86.1+/-10.1 and 90+/-7.6, respectively (95% confidence interval, p<0.001 for both paired and global differences). The GDS for the BAI and EAI was 0.9234±0.0361 and 0.9348±0.0284, respectively. The DSC for the coronary tree was 0.8904±0.0464 and 0.9134±0.0410 for the BAI and EAI, respectively. The EAI outperformed the BAI in all coronary segmentation tasks, but performed less well in some catheter segmentation tasks., Conclusions: We successfully developed AI models capable of CAG segmentation, with good performance as assessed by all scores., (Copyright © 2022 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction and Objectives: The aim of this study was to assess the impact of a conservative strategy in non-ST-segment elevation myocardial infarction in patients in the Portuguese Registry of Acute Coronary Syndromes., Methods: The 3780 patients included in the study over a three-year period were divided into three groups: group 1, patients treated by a conservative strategy during hospitalization; group 2, patients who underwent coronary angiography without percutaneous coronary intervention (PCI); and group 3, patients who underwent PCI. Clinical and procedural data and in-hospital complications were compared. The primary endpoint was defined as in-hospital or one-year mortality and the secondary endpoint as the presence of at least one of the following in-hospital complications: major bleeding according to the GUSTO criteria, need for blood transfusion, invasive ventilation, heart failure or reinfarction., Results: Of the patients analyzed, 16.5% were treated by a conservative strategy. Patients in this group were older, more often women, and had more high-risk factors. A conservative strategy was associated with a higher rate of the primary endpoint - in-hospital mortality (10.6% vs. 1.1% vs. 0.6% in groups 1, 2 and 3, respectively, p<0.001, odds ratio (OR) 6.974, 95% confidence interval [CI]: 2.775-17.527) and one-year mortality (26.1% vs. 6.8% vs. 4.1%, p<0.001, hazard ratio (HR) 2.925, 95% CI: 1.433 -5.974) - and of the secondary endpoint - 37.2% vs. 18.9% vs. 14.6%, p<0.001; OR 1.471 95% CI: 1.043 -2.076., Conclusions: In this patient population, a conservative strategy is an independent predictor of in-hospital mortality, in-hospital complications and one-year mortality., (Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Arrhythmogenic right ventricular cardiomyopathy, also known as arrhythmogenic right ventricular dysplasia, is a condition in which myocardium is replaced by fibrous or fibrofatty tissue, predominantly in the right ventricle. It is clinically characterized by potentially lethal ventricular arrhythmias, and is a leading cause of sudden cardiac death. Its prevalence is not known exactly but is estimated at approximately 1:5000 in the adult population. Diagnosis can be on the basis of structural and functional alterations of the right ventricle, electrocardiographic abnormalities (including depolarization and repolarization alterations and ventricular arrhythmias) and family history. Diagnostic criteria facilitate the recognition and interpretation of non-specific clinical features of this disease. The authors present a case in which the diagnosis of arrhythmogenic right ventricular cardiomyopathy was prompted by the suspicion of right ventricular disease on transthoracic echocardiography. This was confirmed by detection of epsilon waves on analysis of the ECG, which generally go unnoticed but in this case were the key to the diagnosis. Their presence was also shown by non-conventional ECG techniques such as modified Fontaine ECG. The course of the disease culminated in the occurrence of ventricular tachycardia, which prompted placement of an implantable cardioverter-defibrillator., (Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

The authors present a rare case of hypertrophic cardiomyopathy associated with left ventricular noncompaction cardiomyopathy and coronary artery-left ventricular fistulae in a 42-year-old woman presenting with non-ST-elevation myocardial infarction. Coronary angiography, transthoracic echocardiography and cardiac magnetic resonance revealed the structural abnormalities of the left ventricle and the coronary tree., (Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

The authors present two cases of purulent pericarditis secondary to pneumococcus pneumonia, a rare entity in the antibiotic era, one of them in an apparently healthy person. A systematized diagnostic approach to moderate pericardial effusion is presented, together with a review of purulent pericarditis. The presence of pericardial effusion with persistent fever with or without known etiology, particularly in the immunocompromised but also in the apparently healthy patient, should always raise the possibility of purulent pericarditis., (Copyright © 2011 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Left ventricular noncompaction (LVNC) is now recognized as a distinct form of cardiomyopathy with a clinical presentation and natural history of its own. Common manifestations of LVNC include heart failure, ventricular arrhythmias and embolic events, but serious atrioventricular conduction disturbances are rarely reported in the literature. The authors describe the case of a 40-year-old woman who went to the emergency department due to syncope. The ECG revealed left bundle branch block (LBBB) and 2:1 atrioventricular block (AVB) and the patient was admitted for pacemaker implantation. During hospitalization she developed torsade de pointes and complete AVB with increased QTc. The echocardiogram showed images compatible with LVNC. This case provides additional evidence that LVNC may be complicated by 2:1 (or complete) AVB, intraventricular conduction disturbances (LBBB) and repolarization abnormalities (long QT). This combination of electrocardiographic changes has rarely been reported in the literature. We describe a series of affected patients, focusing on electrocardiographic characteristics., (Copyright © 2011 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Introduction: Pulmonary angiography by computed tomography (CT) is the method of choice for the detection of acute pulmonary embolism (PE). Studies have shown that the severity of PE can be estimated by clot burden scores., Objective: To evaluate the correlation between an angiographic clot burden score (Qanadli score - QS) and parameters of right ventricular dysfunction (RVD) in patients admitted for PE., Methods: We performed a retrospective study of 107 patients (60% female) admitted to an intensive care unit for PE (intermediate/high risk) between January 1, 2007 and September 30, 2011. Images from 16-slice multidetector CT angiography were reviewed in 102 patients and the QS calculated. Based on a cut-off of 18 points established by ROC curve analysis, two groups were formed (A<18 points vs. B ≥18 points) and the clinical, laboratory, ECG, echocardiographic and CT angiography parameters were compared. The statistical analysis was performed using SPSS., Results: The overall mean age was 61.4 years. With regard to symptoms at admission, there was a greater prevalence in group B of fatigue, chest pain and syncope (p=0.017), with higher Geneva and Wells scores and shock index. In terms of ECG parameters, heart rate and percentage of right bundle branch block, T-wave inversion (V(1)-V(3)) and S(1)Q(3)T(3) pattern (p=0.034) were higher in group B, as was the ECG score (p=0.009). Laboratory tests revealed that group B had higher troponin and d-dimers, with lower creatinine clearance by the MDRD formula (p=0.020) and PO(2)/FiO(2) ratio. Echocardiography showed higher pulmonary artery systolic pressure in group B, and CT angiography revealed larger right ventricular (RV) diameters and higher RV/LV ratio (p=0.002), and greater superior vena cava, azygos vein and coronary sinus diameters in this group. Pulmonary artery (PA) diameter and the PA/aorta ratio were similar. Interventricular septal bowing and reflux of contrast into the inferior vena cava (p=0.001) were greater in group B, and QS>18 was an independent predictor of RVD (RV/LV ratio>1) (OR: 10.85; p<0.001) (area under the curve on ROC analysis: 0.79; p<0.001). The percentage of patients receiving fibrinolytic treatment was higher in group B (p=0.045), and in-hospital mortality was similar in both groups (overall 4.9%)., Conclusions: QS >18 points proved to be an independent predictor of RVD in PE, and correlated linearly with variables associated with higher morbidity and mortality., (Copyright © 2012 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

We describe the case of a 37-year-old pregnant woman who presented at 29 weeks of gestation with syncope and shortness of breath caused by pulmonary embolism. Due to persistent hypotension thrombolytic therapy with tenecteplase was administered and the clinical and hemodynamic response was excellent, with no maternal or fetal hemorrhagic complications. The clinical presentation of pulmonary embolism is sometimes camouflaged by the physiological changes that occur in pregnancy and diagnosis is often delayed by reluctance to expose the fetus to ionizing radiation. Systemic thrombolysis is considered a high-risk treatment in pregnancy and very few women have received it. However the complication rates of thrombolytic therapy are acceptable in the light of the underlying disease.

Introduction: Diagnosis of Brugada syndrome (BS) currently requires documentation of a characteristic repolarization pattern (type 1 Brugada ECG). Mutations in the SCN5A gene, which codes for sodium channel Na(v) 1.5, are found in 38% of familial cases of BS. Sodium current dysfunction negatively affects the cardiac fast response action potential, particularly in atrial and ventricular myocytes and in the fast-conducting Purkinje system., Objectives: To detect carriers of SCN5A mutations without using the characteristic repolarization pattern (type 1 Brugada ECG)., Methods: Of a total of 141 members of three different families including 55 carriers of two nonsense SCN5A mutations causing BS, all those aged over 16 (113 individuals, 42 carriers) were studied. The PR interval (PR) and QT dispersion (QTd) between leads V1 and V3 were measured on conventional ECG. Using signal-averaged ECG the total duration of the filtered QRS complex (fQRS), the root-mean-square (RMS40) and the low-amplitude signal (LAS) were measured. The following procedures were developed to detect carriers/To detect carriers the following procedures were developed: (1) a screening test (ScreenTest) with PS (PR+fQRS) > or = 250 (250ms is 80% of the theoretical maximum in healthy individuals); and (2) a diagnostic test (DiagTest) for the simultaneous fulfillment of four conditions: PS > or = 250 and QTd > or = 10 and LAS > 26 and RMS40 < or = 29 (the latter two cut-offs are approximately 70% of the theoretical maximum in healthy carriers)., Results: Significant differences in PR, QTd, QRSf, RMS40 and LAS were found between carriers and non-carriers. The SCN5A gene was associated with all these variables, the strongest association being with PR. Both tests were applied to 63 family members (38 carriers). The ScreenTest was positive in 38 of 38 carriers, with eight false positives in 27 non-carriers (sensitivity [SE] = 100% and specificity [SP] = 66.67%). From ROC curve analysis a cut-off of PS = 252.5 shows SE = 100% and SP = 76% and a cut-off of PS = 260 shows SE=94.7% and SP = 84%. The DiagTest was positive in 36 of 38 carriers, with three false positives: SE = 94.74% and SP = 88.89%. From ROC curve analysis a multivariate logistic model identifies a cut-off with SE = 92% and SP = 92%. In the same group the SE and SP of the characteristic spontaneous repolarization pattern (type 1 Brugada ECG) to detect carriers were 52.4% and 97.2%, respectively, and the difference between the SE of the DiagTest and of the typical repolarization pattern is statistically significant., Conclusions: The ScreenTest and DiagTest are more effective tools than the characteristic repolarization pattern to discriminate between carriers and non-carriers of these two nonsense SCN5A mutations. We suggest their use in first-degree relatives of Brugada patients when the results of genetic testing are not available, in a score of disease probability in individuals with idiopathic Brugada ECG, and in patients with arrhythmias or other Brugada-related symptoms presenting type 2 or type 3 Brugada ECG.

Systolic anterior motion (SAM) is a postoperative complication of mitral valve repair, with an incidence of 5 to 10%. Early recognition of the signs and symptoms of SAM is essential for the management of these patients. This article focuses on the pathophysiology and dynamics of SAM and the treatment strategies described in the literature. The authors present a case study and echocardiographic images illustrating the clinical relevance of the mechanism involved, in order to clarify whether surgical reintervention is necessary.

Fabry disease is caused by intracellular accumulation of glycosphingolipids in various tissues, secondary to mutations in the GLA gene (Xq22). Classically described as affecting hemizygous males with no residual alpha-galactosidase A activity, it is now known to affect both sexes, with later and less severe manifestations in females. The manifestations of this disease are systemic: neurological, cutaneous (angiokeratomas), renal, cardiovascular (left ventricular hypertrophy, valve thickening or rhythm disturbances), cochlear-vestibular, and cerebrovascular. In the absence of treatment there is progressive damage to vital organs with renal failure, stroke, heart failure or rhythm perturbations, leading to severe impairment of quality of life as well as reduced life expectancy. We describe the case of a female patient with a history of cryptogenic ischemic stroke at the age of 38 years and chronic renal failure with proteinuria, who presented to the emergency room with atrial fibrillation. The echocardiogram revealed concentric left ventricular hypertrophy, diastolic dysfunction and decreased longitudinal strain in the basal septum. In the context of a screening protocol, she was diagnosed with Fabry disease and a previously undescribed mutation was identified., (Copyright © 2011 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

A 65-year-old woman with a dual-chamber pacemaker implanted in 2006 for symptomatic carotid sinus hypersensitivity was incidentally found to have loss of ventricular capture on routine pacemaker interrogation. A chest X-ray raised the suspicion of perforation and migration of the right ventricular lead, confirmed by three-dimensional echocardiogram and CT scan. On the basis of this case, we review myocardial lead perforation, including predisposing factors, pathophysiological mechanisms, diagnostic approach and therapeutic options.

Noncompaction of the ventricular myocardium (NVM) is a rare congenital disease caused by an arrest in normal myocardial embryogenesis, leading to persistence of numerous prominent trabeculations which communicate with the left ventricle. It was first described as a congenital condition affecting children, but several cases have been reported of late presentation, and recent studies suggest it may be underdiagnosed. The main clinical manifestations are congestive heart failure, arrhythmias (supraventricular or ventricular) and systemic embolism. We describe a series of twenty patients, focusing on clinical history, echocardiography and follow-up.

The unilateral absence of one pulmonary artery is a rare congenital abnormality. The authors report a clinical case of a two-year-old boy with no previous medical history who was referred for evaluation after the detection of pulmonary asymmetry on the chest X-ray with a right mediastinal shift. The CT scan and pulmonary perfusion scintigraphy pointed to an absent right pulmonary artery, which was confirmed by right heart catheterization and cardiac magnetic resonance imaging. This is an important pathology because early diagnosis and timely correction can prevent future complications. Since at this time the patient is asymptomatic, the authors opted for careful clinical vigilance.

Aortic valve stenosis can be complicated by gastrointestinal bleeding from angiodysplasia. A deficiency of high molecular weight multimers of von Willebrand factor (vWF) (type 2A von Willebrand disease) provides the link between this association, which is known as Heyde syndrome. Aortic valve replacement corrects the vWF abnormalities with long-term resolution of gastrointestinal bleeding. The authors present a case report and a review of this association.

Left ventricular noncompaction (LVNC) is a rare congenital disease caused by an arrest in normal myocardial embryogenesis, leading to persistence of numerous prominent trabeculations that communicate with the left ventricle. It was first described as a congenital condition affecting children, but several cases have been reported of late presentation. The main clinical manifestations are congestive heart failure, arrhythmias (supraventricular or ventricular) and systemic embolism. We present the case of a 51-year-old patient brought to our emergency department after an episode of symptomatic ventricular flutter requiring electrical cardioversion. Two-dimensional echocardiography with color Doppler suggested the diagnosis and the three-dimensional echocardiogram revealed the deep trabeculations typical of LVNC.

The authors present a family with Brugada syndrome, the largest ever described in Portugal. From a fortuitous electrocardiographic finding in a previously symptomatic young man with a family history of sudden death, 126 possibly affected family members were studied. This article describes the diagnostic approach, risk stratification for sudden death and therapeutic interventions offered to this family, and presents a brief review of Brugada syndrome.

Cardiac involvement is found in approximately 5% of patients with sarcoidosis. The course of the disease is variable, ranging from benign arrhythmias to high-degree heart block or sudden death, and diagnosis is difficult. Endomyocardial biopsy can confirm the diagnosis but the diagnostic yield of the procedure is low because of the uneven distribution of the granulomatous process. Cardiac sarcoidosis should be presumed in patients with known sarcoidosis who develop arrhythmias, conduction disease, heart failure or ECG abnormalities. The authors present a case report and brief review of cardiac sarcoidosis.

Lupus is an inflammatory autoimmune disease that can affect multiple organ systems. As such, it can be diagnosed because of multiorgan or constitutional symptoms, but frequently medical treatment is sought only when there is clinical involvement of a particular organ or system, even if established--but undiagnosed--systemic disease is already present. The authors present the case of a patient admitted to a cardiology unit for acute pericarditis, in whom the diagnosis of systemic lupus erythematosus was made, and highlight the need to consider cardiac involvement in systemic diseases.