Coronary Intravascular Lithotripsy Effectiveness and Safety in a Real-World Cohort.

Background: Coronary artery calcification is a predictor of adverse outcomes after percutaneous coronary intervention (PCI). Intravascular lithotripsy (IVL) is a promising tool for the treatment of calcified lesions. The aim of this study was to assess the effectiveness and safety of IVL. Methods: A single-center observational study of PCI procedure, with assessment of the outcomes of patients undergoing PCI using IVL, was performed. Angiographic procedural success was used as the primary effectiveness endpoint. The primary safety endpoint was defined as a composite of cardiac death, myocardial infarction and target vessel revascularization within 30 days. Results: A total of 111 patients were included. Indications for PCI spanned the spectrum of chronic (53.2%) and acute coronary syndromes (43%). Lesion preparation before IVL was performed with non-compliant (42%), cutting or OPN (14.4%) balloons and with atherectomy techniques in 11% of procedures. Intravascular imaging was used in 21.6% of procedures. The primary effectiveness endpoint was achieved in 100% and the primary safety endpoint in 3.6% of procedures. Peri-procedural complications were minimal and successfully resolved. Conclusions: IVL was an effective and safe technique for the treatment of calcified coronary lesions. These findings contribute to the growing body of evidence supporting the use of IVL in the management of these challenging scenarios.

Transcatheter interventions for left-sided valvular heart disease complicated by cardiogenic shock: a consensus statement from the European Association of Percutaneous Cardiovascular Interventions (EAPCI) in collaboration with the Association for Acute Cardiovascular Care (ACVC) and the ESC Working Group on Cardiovascular Surgery.

Valvular heart disease (VHD) is one of the most frequent causes of heart failure (HF) and is associated with poor prognosis, particularly among patients with conservative management. The development and improvement of catheter-based VHD interventions have broadened the indications for transcatheter valve interventions from inoperable/high-risk patients to younger/lower-risk patients. Cardiogenic shock (CS) associated with severe VHD is a clinical condition with a very high risk of mortality for which surgical treatment is often deemed a prohibitive risk. Transcatheter valve interventions might be a promising alternative in this setting given that they are less invasive. However, supportive scientific evidence is scarce and often limited to small case series. Current guidelines on VHD do not contain specific recommendations on how to manage patients with both VHD and CS. The purpose of this clinical consensus statement, developed by a group of international experts invited by the European Association of Percutaneous Cardiovascular Interventions (EAPCI) Scientific Documents and Initiatives Committee, is to perform a review of the available scientific evidence on the management of CS associated with left-sided VHD and to provide a rationale and practical approach for the application of transcatheter valve interventions in this specific clinical setting.

Management of coronary artery disease in patients undergoing transcatheter aortic valve implantation. A clinical consensus statement from the European Association of Percutaneous Cardiovascular Interventions in collaboration with the ESC Working Group on Cardiovascular Surgery.

Significant coronary artery disease (CAD) is a frequent finding in patients with severe aortic stenosis undergoing transcatheter aortic valve implantation (TAVI), and the management of these two conditions becomes of particular importance with the extension of the procedure to younger and lower-risk patients. Yet, the preprocedural diagnostic evaluation and the indications for treatment of significant CAD in TAVI candidates remain a matter of debate. In this clinical consensus statement, a group of experts from the European Association of Percutaneous Cardiovascular Interventions (EAPCI) in collaboration with the European Society of Cardiology (ESC) Working Group on Cardiovascular Surgery aims to review the available evidence on the topic and proposes a rationale for the diagnostic evaluation and indications for percutaneous revascularisation of CAD in patients with severe aortic stenosis undergoing transcatheter treatment. Moreover, it also focuses on commissural alignment of transcatheter heart valves and coronary re-access after TAVI and redo-TAVI.

Development of deep learning segmentation models for coronary X-ray angiography: Quality assessment by a new global segmentation score and comparison with human performance.

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.

Predictors of coronary artery disease in cardiac arrest survivors: coronary angiography for everyone? A single-center retrospective analysis. / Preditores de doença arterial coronária em sobreviventes à parada cardíaca: angiografia coronária para todos? Uma análise retrospectiva em centro único.

OBJECTIVE: To identify predictors of coronary artery disease in survivors of cardiac arrest, to define the best timing for coronary angiography and to establish the relationship between coronary artery disease and mortality. METHODS: This was a single-center retrospective study including consecutive patients who underwent coronary angiography after cardiac arrest. RESULTS: A total of 117 patients (63 ± 13 years, 77% men) were included. Most cardiac arrest incidents occurred with shockable rhythms (70.1%), and the median duration until the return of spontaneous circulation was 10 minutes. Significant coronary artery disease was found in 68.4% of patients, of whom 75% underwent percutaneous coronary intervention. ST-segment elevation (OR 6.5, 95%CI 2.2 - 19.6; p = 0.001), the presence of wall motion abnormalities (OR 22.0, 95%CI 5.7 - 84.6; p < 0.001), an left ventricular ejection fraction ≤ 40% (OR 6.2, 95%CI 1.8 - 21.8; p = 0.005) and elevated high sensitivity troponin T (OR 3.04, 95%CI 1.3 - 6.9; p = 0.008) were predictors of coronary artery disease; the latter had poor accuracy (area under the curve 0.64; p = 0.004), with an optimal cutoff of 170ng/L. Only ST-segment elevation and the presence of wall motion abnormalities were independent predictors of coronary artery disease. The duration of cardiac arrest (OR 1.015, 95%CI 1.0 - 1.05; p = 0.048) was an independent predictor of death, and shockable rhythm (OR 0.4, 95%CI 0.4 - 0.9; p = 0.031) was an independent predictor of survival. The presence of coronary artery disease and the performance of percutaneous coronary intervention had no impact on survival; it was not possible to establish the best cutoff for coronary angiography timing. CONCLUSION: In patients with cardiac arrest, ST-segment elevation, wall motion abnormalities, left ventricular dysfunction and elevated high sensitivity troponin T were predictive of coronary artery disease. Neither coronary artery disease nor percutaneous coronary intervention significantly impacted survival.

OBJETIVO: Identificar os preditores de doença arterial coronária em sobreviventes à parada cardíaca, visando definir o melhor momento para realização de angiografia coronária e estabelecer o relacionamento entre doença arterial coronária e mortalidade. MÉTODOS: Este foi um estudo retrospectivo em centro único, que incluiu os pacientes consecutivamente submetidos à angiografia coronária após uma parada cardíaca. RESULTADOS: Incluímos 117 pacientes (63 ± 13 anos, 77% homens). A maioria dos incidentes de parada cardíaca ocorreu com ritmos chocáveis (70,1%), e o tempo mediano até retorno da circulação espontânea foi de 10 minutos. Identificou-se doença arterial coronária em 68,4% dos pacientes, dentre os quais 75% foram submetidos à intervenção coronária percutânea. Elevação do segmento ST (RC de 6,5; IC95% 2,2 - 19,6; p = 0,001), presença de alterações da contratilidade segmentar (RC de 22,0; IC95% 5,7 - 84,6; p < 0,001), fração de ejeção ventricular esquerda ≤ 40% (RC de 6,2; IC95% 1,8 - 21,8; p = 0,005) e níveis elevados de troponina T de alta sensibilidade (RC de 3,04; IC95% 1,3 - 6,9; p = 0,008) foram preditores de doença arterial coronária; esse último teve baixa precisão (área sob a curva de 0,64; p = 0,004), tendo o nível de 170ng/L como ponto ideal de corte. Apenas elevação do segmento ST e presença de alterações da contratilidade segmentar foram preditores independentes de doença arterial coronária. A duração da parada cardíaca (RC de 1,015; IC95% 1,0 - 1,05; p = 0,048) foi um preditor independente de óbito, e ritmo chocável (RC de 0,4; IC95% 0,4 - 0,9; p = 0,031) foi um preditor independente de sobrevivência. A presença de doença arterial coronária e a realização de intervenção coronária percutânea não tiveram impacto na sobrevivência; não foi possível estabelecer o melhor ponto de corte para o momento da angiografia coronária. CONCLUSÃO: Em pacientes com parada cardíaca, elevação do segmento ST, alterações da contratilidade segmentar, disfunção ventricular esquerda e níveis elevados de troponina T de alta sensibilidade foram preditivos de doença arterial coronária. Nem doença arterial coronária nem a intervenção coronária percutânea tiveram impacto significante na sobrevivência.

Preditores de doença arterial coronária em sobreviventes à parada cardíaca: angiografia coronária para todos? Uma análise retrospectiva em centro único / Predictors of coronary artery disease in cardiac arrest survivors: coronary angiography for everyone? A single-center retrospective analysis

RESUMO Objetivo: Identificar os preditores de doença arterial coronária em sobreviventes à parada cardíaca, visando definir o melhor momento para realização de angiografia coronária e estabelecer o relacionamento entre doença arterial coronária e mortalidade. Métodos: Este foi um estudo retrospectivo em centro único, que incluiu os pacientes consecutivamente submetidos à angiografia coronária após uma parada cardíaca. Resultados: Incluímos 117 pacientes (63 ± 13 anos, 77% homens). A maioria dos incidentes de parada cardíaca ocorreu com ritmos chocáveis (70,1%), e o tempo mediano até retorno da circulação espontânea foi de 10 minutos. Identificou-se doença arterial coronária em 68,4% dos pacientes, dentre os quais 75% foram submetidos à intervenção coronária percutânea. Elevação do segmento ST (RC de 6,5; IC95% 2,2 - 19,6; p = 0,001), presença de alterações da contratilidade segmentar (RC de 22,0; IC95% 5,7 - 84,6; p < 0,001), fração de ejeção ventricular esquerda ≤ 40% (RC de 6,2; IC95% 1,8 - 21,8; p = 0,005) e níveis elevados de troponina T de alta sensibilidade (RC de 3,04; IC95% 1,3 - 6,9; p = 0,008) foram preditores de doença arterial coronária; esse último teve baixa precisão (área sob a curva de 0,64; p = 0,004), tendo o nível de 170ng/L como ponto ideal de corte. Apenas elevação do segmento ST e presença de alterações da contratilidade segmentar foram preditores independentes de doença arterial coronária. A duração da parada cardíaca (RC de 1,015; IC95% 1,0 - 1,05; p = 0,048) foi um preditor independente de óbito, e ritmo chocável (RC de 0,4; IC95% 0,4 - 0,9; p = 0,031) foi um preditor independente de sobrevivência. A presença de doença arterial coronária e a realização de intervenção coronária percutânea não tiveram impacto na sobrevivência; não foi possível estabelecer o melhor ponto de corte para o momento da angiografia coronária. Conclusão: Em pacientes com parada cardíaca, elevação do segmento ST, alterações da contratilidade segmentar, disfunção ventricular esquerda e níveis elevados de troponina T de alta sensibilidade foram preditivos de doença arterial coronária. Nem doença arterial coronária nem a intervenção coronária percutânea tiveram impacto significante na sobrevivência.

ABSTRACT Objective: To identify predictors of coronary artery disease in survivors of cardiac arrest, to define the best timing for coronary angiography and to establish the relationship between coronary artery disease and mortality. Methods: This was a single-center retrospective study including consecutive patients who underwent coronary angiography after cardiac arrest. Results: A total of 117 patients (63 ± 13 years, 77% men) were included. Most cardiac arrest incidents occurred with shockable rhythms (70.1%), and the median duration until the return of spontaneous circulation was 10 minutes. Significant coronary artery disease was found in 68.4% of patients, of whom 75% underwent percutaneous coronary intervention. ST-segment elevation (OR 6.5, 95%CI 2.2 - 19.6; p = 0.001), the presence of wall motion abnormalities (OR 22.0, 95%CI 5.7 - 84.6; p < 0.001), an left ventricular ejection fraction ≤ 40% (OR 6.2, 95%CI 1.8 - 21.8; p = 0.005) and elevated high sensitivity troponin T (OR 3.04, 95%CI 1.3 - 6.9; p = 0.008) were predictors of coronary artery disease; the latter had poor accuracy (area under the curve 0.64; p = 0.004), with an optimal cutoff of 170ng/L. Only ST-segment elevation and the presence of wall motion abnormalities were independent predictors of coronary artery disease. The duration of cardiac arrest (OR 1.015, 95%CI 1.0 - 1.05; p = 0.048) was an independent predictor of death, and shockable rhythm (OR 0.4, 95%CI 0.4 - 0.9; p = 0.031) was an independent predictor of survival. The presence of coronary artery disease and the performance of percutaneous coronary intervention had no impact on survival; it was not possible to establish the best cutoff for coronary angiography timing. Conclusion: In patients with cardiac arrest, ST-segment elevation, wall motion abnormalities, left ventricular dysfunction and elevated high sensitivity troponin T were predictive of coronary artery disease. Neither coronary artery disease nor percutaneous coronary intervention significantly impacted survival.

The CTo-aBCDE score: A new predictor of success in chronic total occlusions.

INTRODUCTION: Patient selection for percutaneous coronary intervention (PCI) in chronic total occlusions (CTOs) is crucial to procedural success. Our aim was to identify independent predictors of success in CTO PCI in order to create an accurate score. METHODS: In a single-center observational registry of CTO PCI, demographic and clinical data and anatomical characteristics of coronary lesions were recorded. Linear and logistic regression analysis were used to identify predictors of success. A score to predict success was created and its accuracy was measured by receiver operating curve analysis. RESULTS: A total of 377 interventions were performed (334 patients, age 68±11 years, 75% male). The success rate was 65% per patient and 60% per procedure. Predictors of success in univariate analysis were absence of active smoking (OR 2.02, 95% CI 1.243-3.29; p=0.005), presence of tapered stump (OR 5.2, 95% CI 2.7-10.2; p<0.001), absence of tortuosity (OR 6.44; 95% CI 3.02-13.75; p<0.001), absence of bifurcation (OR 1.95; 95% CI 1.08-3.51; p=0.026), absence of calcification (OR 3.1; 95% CI 3.10-5.41; p<0.001), LAD as target vessel (OR 1.9, 95% CI 1.0-3.5; p=0.048), and CTO length <20 mm (OR 3.00, 95% CI 1.69-5.3; p<0.001). Only anatomical factors were independent predictors of success, and an anatomical score (0-11 points) with high accuracy (area under the curve 0.831) was subsequently created. A score <3 was associated with low probability of success (15%), 3-8 with intermediate probability (55%), and >8 with high probability (95%). CONCLUSION: In our sample only anatomical characteristics were predictors of success. The creation of a score to predict success, with good accuracy, may enable selection of cases that can be treated by any operator, those in which a dedicated operator will be desirable, and those with an extremely low probability of success, which should be considered individually for conservative management, surgical revascularization or PCI by a team experienced in CTO.

Intraventricular Thrombus Formation and Embolism in Takotsubo Syndrome: Insights From the International Takotsubo Registry.

OBJECTIVE: Takotsubo syndrome (TTS) is characterized by acute left ventricular dysfunction, which can contribute to intraventricular thrombus and embolism. Still, prevalence and clinical impact of thrombus formation and embolic events on outcome of TTS patients remain unclear. This study aimed to investigate clinical features and outcomes of patients with and without intraventricular thrombus or embolism. Additionally, factors associated with thrombus formation or embolism, as well as predictors for mortality, were identified. Approach and Results: TTS patients enrolled in the International Takotsubo Registry at 28 centers in Australia, Europe, and the United States were dichotomized according to the occurrence/absence of intraventricular thrombus or embolism. Patients with intraventricular thrombus or embolism were defined as the ThrombEmb group. Of 1676 TTS patients, 56 (3.3%) patients developed intraventricular thrombus and/or embolism following TTS diagnosis (median time interval, 2.0 days [range, 0-38 days]). Patients in the ThrombEmb group had a different clinical profile including lower left ventricular ejection fraction, higher prevalence of the apical type, elevated levels of troponin and inflammatory markers, and higher prevalence of vascular disease. In a Firth bias-reduced penalized-likelihood logistic regression model apical type, left ventricular ejection fraction ≤30%, previous vascular disease, and a white blood cell count on admission >10×103 cells/µL emerged as independent predictors for thrombus formation or embolism. CONCLUSIONS: Intraventricular thrombus or embolism occur in 3.3% of patients in the acute phase of TTS. A simple risk score including clinical parameters associated with intraventricular thrombus formation or embolism identifies patients at increased risk. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01947621.

Comparative analysis of fractional flow reserve and instantaneous wave-free ratio: Results of a five-year registry. / Análise comparativa do fractional flow reserve (FFR) e do instantaneous wave­free ratio (iFR): resultados de um registo de 5 anos.

INTRODUCTION AND OBJECTIVE: Assessment of coronary lesions by the instantaneous wave free ratio (iFR) has generated significant debate. We aimed to assess the diagnostic performance of iFR and its impact on the decision to use fractional flow reserve (FFR) and on procedural characteristics. METHODS: In this single-center registry of patients undergoing functional assessment of coronary lesions, FFR was used as a reference for assessing the diagnostic performance of iFR. An iFR value <0.86 was considered positive and a value >0.93 was considered negative. RESULTS: Functional testing was undertaken of 402 lesions, of which 154 were assessed with both techniques, 222 with FFR only, and 26 with iFR only. Using a cut-off of ≤0.80 for iFR, the area under the curve was 0.73 (95% CI 0.65-0.81), with an optimal value of ≤0.91. FFR was undertaken in 93 out of 94 lesions with an inconclusive iFR and was performed in 69.1% of the remaining iFR-tested lesions. Concordance between iFR and FFR was 87% (chi-square=22.43; p<0.001). Notwithstanding, there were four out of 13 cases (30.7%) of positive iFR with negative FFR and three out of 42 (7.1%) cases of negative iFR and positive FFR. This difference was significant (p=0.026). iFR had no impact on procedure time, fluoroscopy time or radiation dose. CONCLUSION: iFR had a reasonable diagnostic performance. Operators often chose to perform FFR despite conclusive iFR results. iFR and FFR were highly concordant, but a non-negligible proportion of lesions classified as ischemic by iFR were classified as non-ischemic by FFR. iFR had no impact on procedural characteristics.

Acute type A aortic dissection in a patient with paraganglioma. / Dissecção aórtica aguda do tipo A em doente com paraganglioma.

Acute aortic dissection is the most common acute aortic syndrome. It is more prevalent in males and in the elderly, and has a high mortality. Hypertension is the main risk factor. Diagnosis is based on clinical features, laboratory tests and imaging exams. Treatment is usually surgical, although in some cases an endovascular approach is an alternative. Paraganglioma is an uncommon neuroendocrine tumor. Most produce catecholamines, and so usually manifest with hypertensive crisis, palpitations, headache and sweating. This tumor is diagnosed by measurement of plasma or urinary catecholamines and by computed tomography, magnetic resonance imaging and 123I-metaiodobenzylguanidine (MIBG) scintigraphy. Surgery is the only potentially curative treatment.

Combined MitraClip implantation and left atrial appendage occlusion using the Watchman device: A case series from a referral center.

INTRODUCTION: Patients referred for percutaneous transcatheter mitral valve repair using the MitraClip® system frequently have atrial fibrillation, which imposes additional challenges due to the need for oral anticoagulation. Left atrial appendage occlusion is currently regarded as a non-inferior alternative to anticoagulation in patients with non-valvular atrial fibrillation and both high thromboembolic and bleeding risk. Considering that both MitraClip implantation and left atrial appendage occlusion are percutaneous techniques that require transseptal puncture, it is technically attractive to consider their concomitant use. OBJECTIVES: We aim to evaluate the feasibility of a combined approach with MitraClip implantation and left atrial appendage occlusion in a single procedure. METHODS: We report the first case series regarding this issue, discussing the specific advantages, pitfalls and technical aspects of combining these two procedures. RESULTS: Five patients underwent left atrial appendage occlusion with the Watchman® device followed by MitraClip implantation in the same procedure. All patients experienced significant reduction in mitral valve regurgitation of at least two grades, optimal occluder position, no associated complications and significant clinical improvement assessed by NYHA functional class (reduction of at least one functional class, with four patients in class I at one-month follow-up). CONCLUSION: In selected patients rejected for surgical mitral valve repair, with atrial fibrillation and increased risk of bleeding and embolic events, a combined approach with MitraClip implantation and left atrial appendage occlusion in a single procedure is feasible, safe and effective.

Balloon aortic valvuloplasty in the transcatheter aortic valve implantation era: A single-center registry.

INTRODUCTION: Percutaneous balloon aortic valvuloplasty (BAV) has been limited by the risk of complications and restenosis. However, growing use of transcatheter aortic valve implantation (TAVI) has revived interest in this technique. We analyzed the current indications for BAV and outcomes in a single center. METHODS: Acute results and long-term outcomes were analyzed in a retrospective single-center registry of patients undergoing BAV between January 2013 and January 2016. RESULTS: Twenty-three patients underwent BAV, 56.5% male, mean age 78±7 years. Indications were severe aortic stenosis and decompensated heart failure (n=5), urgent non-cardiac surgery (n=8), or bridge to definitive treatment (n=10). Peak invasive gradient decreased from a median of 54.0±19.0 mmHg to 28.5±13.8 mmHg (p=0.002). Complications included one ischemic stroke, one lower limb ischemia and one femoral pseudoaneurysm requiring surgery. During a mean follow-up of 11±10 months, eight patients underwent TAVI and two underwent surgical aortic valve replacement. Thirteen patients died, nine of non-cardiovascular causes. On Kaplan-Meier analysis mortality was significantly lower among patients undergoing definitive treatment (20.0% vs. 84.6% at two-year follow-up; p=0.005). CONCLUSION: BAV should be considered for selected patients with temporary contraindications to definitive therapy or as palliative therapy.

Circulating miR-122-5p/miR-133b Ratio Is a Specific Early Prognostic Biomarker in Acute Myocardial Infarction.

BACKGROUND: MicroRNAs (miRNAs) are key players in cardiovascular development and disease. However, not only miRNAs of a cardiac origin have a critical role in heart function. Recent studies have demonstrated that miR-122-5p, a hepatic miRNA, increases in the bloodstream during ischemic cardiogenic shock and it is upregulated in the infarcted myocardium. The aim of the present study was to determine the potential of circulating miR-122-5p as a biomarker for early prognostic stratification of ST-segment elevation acute myocardial infarction (STEMI) patients. METHODS AND RESULTS: One hundred and forty-two consecutive STEMI patients treated with primary angioplasty were included in the study. Serum levels of miR-1-3p, -122-5p, -133a-3p, -133b, -208b-3p and -499a-5p were measured at the time of cardiac catheterization by quantitative polymerase chain reaction and related to in-hospital and long-term outcome. During a follow up of 20.8 months, 9 patients died, 6 had recurrence of myocardial infarction, and 26 patients suffered an adverse cardiovascular event. Event-free survival was significantly worse in patients with a higher miR-122-5p/133b ratio (3rd tertile distribution, above 1.42 Log(10)), having almost a 9-fold higher risk of death or myocardial infarction and a 4-fold higher risk of adverse cardiovascular events. CONCLUSIONS: This study showed that the miR-122-5p/133b ratio is a new prognostic biomarker for the early identification of STEMI patients at a higher risk of developing major adverse events after undergoing primary percutaneous coronary intervention. (Circ J 2016; 80: 2183-2191).

Adult-onset Still's disease and cardiac tamponade: a rare association.

Adult-onset Still's disease is a rare disorder with potentially severe clinical features, including cardiac involvement. This systemic inflammatory disease of unknown origin should be considered in the differential diagnosis of pericarditis, with or without pericardial effusion. Cardiac tamponade is a very rare sequela that requires an invasive approach, such as percutaneous or surgical pericardial drainage, in addition to the usual conservative therapy. The authors describe a case of adult-onset Still's disease rendered more difficult by pericarditis and cardiac tamponade, and they briefly review the literature on this entity.

Non-compaction cardiomyopathy: prevalence, prognosis, pathoetiology, genetics, and risk of cardioembolism.

Left ventricular non-compaction (LVNC) is thought to arise from arrest of the normal process of trabecular remodeling or "compaction" that takes place during embryonic life and is characterized by the presence of a two-layered ventricular wall, with a compact epicardial layer and a non-compacted endocardial layer. It is an uncommon condition that can occur isolated or in association with other disorders, including congenital heart anomalies and mitochondrial or musculoskeletal disorders. Both familial and sporadic forms are recognized, and several responsible genes have been identified, although only a minority of patients can be successfully genotyped. The diagnosis is usually made by echocardiography, but cardiac magnetic resonance imaging has been used increasingly. Management is mainly empirical and directed at the major clinical manifestations: heart failure, arrhythmias, and systemic embolic events. This article will review the major features of LVNC and present new trends in the diagnosis and management of this intriguing condition.