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Background: A small but significant reduction in left ventricular (LV) mass after 18 months of migalastat treatment has been reported in Fabry disease (FD). This study aimed to assess the effect of migalastat on FD cardiac involvement, combining LV morphology and tissue characterisation by cardiac magnetic resonance (CMR) with cardiopulmonary exercise testing (CPET). Methods: Sixteen treatment-naïve patients with FD (4 women, 46.4±16.2 years) with cardiac involvement (reduced T1 values on CMR and/or LV hypertrophy) underwent ECG, echocardiogram, troponin T and NT-proBNP (N-Terminal prohormone of Brain Natriuretic Peptide) assay, CMR with T1 mapping, and CPET before and after 18 months of migalastat. Results: No change in LV mass was detected at 18 months compared to baseline (95.2 g/m2 (66.0-184.0) vs 99.0 g/m2 (69.0-121.0), p=0.55). Overall, there was an increase in septal T1 of borderline significance (870.0 ms (848-882) vs 860.0 ms (833.0-875.0), p=0.056). Functional capacity showed an increase in oxygen consumption (VO2) at anaerobic threshold (15.50 mL/kg/min (13.70-21.50) vs 14.50 mL/kg/min (11.70-18.95), p=0.02), and a trend towards an increase in percent predicted peak VO2 (72.0 (63.0-80.0) vs 69.0 (53.0-77.0), p=0.056) was observed. The subset of patients who showed an increase in T1 value and a reduction in LV mass (n=7, 1 female, age 40.5 (28.6-76.0)) was younger and at an earlier disease stage compared to the others, and also exhibited greater improvement in exercise tolerance. Conclusion: In treatment-naïve FD patients with cardiac involvement, 18-month treatment with migalastat stabilised LV mass and was associated with a trend towards an improvement in exercise tolerance. A tendency to T1 increase was detected by CMR. The subset of patients who had significant benefits from the treatment showed an earlier cardiac disease compared to the others. Trial registration number: NCT03838237.

The hypertrophic cardiomyopathy phenotype encompasses a heterogeneous spectrum of genetic and acquired diseases characterized by the presence of left ventricular hypertrophy in the absence of abnormal cardiac loading conditions. This "umbrella diagnosis" includes the "classic" hypertrophic cardiomyopathy (HCM), due to sarcomere protein gene mutations, and its phenocopies caused by intra- or extracellular deposits, such as Fabry disease (FD) and cardiac amyloidosis (CA). All these conditions share a wide phenotypic variability which results from the combination of genetic and environmental factors and whose pathogenic mediators are poorly understood so far. Accumulating evidence suggests that inflammation plays a critical role in a broad spectrum of cardiovascular conditions, including cardiomyopathies. Indeed, inflammation can trigger molecular pathways which contribute to cardiomyocyte hypertrophy and dysfunction, extracellular matrix accumulation, and microvascular dysfunction. Growing evidence suggests that systemic inflammation is a possible key pathophysiologic process potentially involved in the pathogenesis of cardiac disease progression, influencing the severity of the phenotype and clinical outcome, including heart failure. In this review, we summarize current knowledge regarding the prevalence, clinical significance, and potential therapeutic implications of inflammation in HCM and two of its most important phenocopies, FD and CA.

Introduction: Left ventricular (LV) hypertrophy is the main feature of cardiac involvement in Anderson-Fabry disease (FD), but the right ventricle (RV) is also frequently affected. Previous studies failed to demonstrate an independent association between conventional parameters of RV performance and outcomes in FD. Nevertheless, if RV free wall strain (RV-FWS), assessed by 2D speckle tracking analysis, may provide a better prognostication is currently unknown. Methods: We retrospectively evaluated the association between RV-FWS and the occurrence of cardiovascular events in a cohort of 56 patients with FD. The study endpoint comprises cardiovascular mortality, severe heart failure symptoms, new-onset atrial fibrillation and major arrhythmias requiring device implantation. Results: Reduced RV-FWS, defined by values lower than 23%, was found in 25 (45%) patients. During a median follow-up of 47 months, 16 (29%) patients met the study endpoint. A ROC-curve analysis confirmed the threshold of reduced RV-FWS (<23%) as the best cut-off for predicting cardiovascular events, but with a lower power compared to left-sided parameters. On univariable Cox regression analysis, RV-FWS, expressed as continuous variable, was significantly associated with the study endpoint (HR: 0.795, 95% CI: 0.710–0.889, p < 0.001). However, RV-FWS did not retain a significant association with outcomes, after adjustment for LV global longitudinal strain or indexed left atrial volume (p = 0.340 and p = 0.289 respectively). Conclusions: RV-FWS was not independently associated with the occurrence of cardiovascular events in FD, confirming previous observations that prognosis is mainly driven by the severity of LV cardiomyopathy.

Objective: Global longitudinal strain (GLS) predicts major adverse events in ST-segment elevation myocardial infarction (STEMI) and aortic stenosis (AS). Different cut-off values and different end-points have been proposed for prognostic stratification. We aimed to verify whether a single GLS cut-off value can be used to identify increased risk of all-cause death in STEMI and AS.Patients and methods: One-hundred- seventeen successfully treated first STEMI (age 63.8 +/- 12.5 yrs, 70% men) and 64 AS (age 80.3 +/- 6.9 yrs, 44% men) patients, undergoing echocardiography before discharge and before AS treatment, respectively, were retrospectively analyzed. GLS was analyzed, together with pulmonary artery systolic pressure (PASP), Killip class and Genereux stage. End-point was all-cause death at 6-month follow-up.Results: All-cause death occurred in 4 (3.4%) STEMI and 5 (7.8%) AS patients (p=ns). AS patients who died had GLS similar to died STEMI patients (9.7 +/- 2.1 vs. 11.3 +/- 1.7, p=ns). GLS cut-off <= 12% predicted death with 89% sensitivity and 70% specificity (AUC 0.84, p=0.001): STEMI and AS patients with GLS <= 12% had worse survival than STEMI and AS patients with GLS >12% (log-rank p=0.001). At multivariate Cox regression analysis, lower GLS values independently predicted death (HR 0.667, 95% CI 0.451-0.986, p=0.042), and the prediction model was improved when GLS was added to old age, significant comorbidities, PASP and Killip/Genereux stage (chi 2 6.691 vs. 1.364, p=0.010).Conclusions: Died patients with STEMI and AS show similar values of GLS. A unique cut-off value of GLS can reliably be used to stratify the risk of all-cause death at 6-month follow-up in both two clinical settings.

Echocardiography is the most common diagnostic tool to screen for Fabry cardiomyopathy as it is fast, non-invasive, low-cost, widely available, easily applicable and reproducible. Echocardiography is the first-line investigation, being useful in all the stages of the disease: (1) in gene-positive patients, to unveil signs of early cardiac involvement and allowing timely treatment; (2) in patients with overt cardiomyopathy to estimate the severity of cardiac involvement, the possible related complications, and the effect of treatment. Recently, advanced echocardiographic techniques, such as speckle tracking analysis, are offering new insights in the assessment of Fabry disease patients and in the differential diagnosis of cardiomyopathies with hypertrophic phenotype. The aim of this review is to provide a comprehensive overview on the cardiac structural and functional abnormalities described in Fabry disease by means of echocardiography.

In the past few years, the wide application of cardiac magnetic resonance (CMR) significantly changed the approach to the study of cardiac involvement in Fabry Disease (FD). The possibility to perform non-invasive tissue characterization, including new sequences such as T1/T2 mapping, offered a powerful tool for differential diagnosis with other forms of left ventricular hypertrophy. In patients with confirmed diagnosis of FD, CMR is the most sensitive non-invasive technique for early detection of cardiac involvement and it provides new insight into the evolution of cardiac damage, including gender-specific features. Finally, CMR multiparametric detection of subtle changes in cardiac morphology, function and tissue composition is potentially useful for monitoring the efficacy of specific treatment over time. This paper aims to provide a comprehensive review of current knowledge regarding the application of CMR in FD cardiac involvement and its clinical implication.

Aims: There are limited data about the intraprocedural haemodynamic study performed immediately before and after transcatheter aortic valve implantation (TAVI) in patients with severe aortic stenosis (AS). We aimed to evaluate the acute haemodynamic impact of TAVI in patients with severe AS and to investigate invasive and non-invasive parameters predicting all-cause mortality. Methods and results: A total of 245 consecutive AS patients (43% male, mean age 80.3 ± 7.3 years) undergoing TAVI were enrolled. Intraprocedural left heart catheterization (LHC) and echocardiogram before and after TAVI were performed. The clinical endpoint was the death for any cause. LHC after TAVI revealed significant changes in aortic and left ventricular (LV) pressures, including indexes of intrinsic myocardial contractility and diastolic function such as positive dP/dT (1128.9 ± 398.7 vs. 806.3 ± 247.2 mmHg/s, P ˂ 0.001) and negative dP/dT (1310.7 ± 431.1 vs. 1075.1 ± 440.8 mmHg/s, P ˂ 0.001). Post-TAVI echo showed a significant reduction in LV end-diastolic (P = 0.036) and end-systolic (P ˂ 0.001) diameters, improvement in LV ejection fraction (from 55 ± 12% to 57.2 ± 10.5%, P ˂ 0.001), and pulmonary artery systolic pressure (42.1 ± 14.2 vs. 33.1 ± 10.7 mmHg, P < 0.001). After a mean follow-up time interval of 24 months, 47 patients died. Post-TAVI significant aortic regurgitation at echocardiography was the only independent predictor of mortality (hazard ratio 5.592, confidence interval 1.932–16.184, P = 0.002). Conclusions: Left heart catheterization performed immediately before and after prosthesis release offers a unique insight in the assessment of LV adaptation to severe AS and the impact of TAVI on LV, catching changes in indexes of intrinsic contractility and myocardial relaxation. Aortic regurg

Objective: The objective of this study is to compare hemodynamic and echocardiographic findings between valve-in-valve (VIV) and native-valve (NV) patients submitted to transcatheter aortic valve implantation (TAVI) due to pure aortic regurgitation (AR). Background: Patients with severe AR are surgically treated with variable postinterventional left-ventricular (LV) “reverse remodeling.” TAVI might be considered in selected AR patients. Methods: Twenty-eight patients with pure severe AR caused by either degenerated bioprosthesis or NV disease were successfully treated by TAVI at our institution. LV catheterization before and after TAVI and echocardiography before, after (24–72 h), and at follow-up (3–12 months) were performed. Results: Baseline clinical, hemodynamic, and echocardiographic characteristics were comparable between the two study groups, except for a younger age, higher proto-diastolic LV pressure, and higher LV end-systolic diameter in the NV group. At catheterization, an immediate hemodynamic impact of TAVI in both groups was noticed, with a trend toward better postprocedural residual regurgitation index and significantly lower LV dP/dT values (666.0 ± 177.9 vs. 883.5 ± 259.7 mmHg/s, p = 0.04) in VIV. At echocardiography, both NV and VIV patients showed favorable (early and sustained) post-TAVI echocardiographically detectable reverse remodeling. VIV patients also showed more pronounced early reduction in indexed LV end-diastolic volume (68.1 ± 27.4 vs. 86.5 ± 28.9 ml/m2 in VIV, p < 0.001 and 81.0 ± 29.0 vs. 95.2 ± 37.8 ml/m2 in NV, p = 0.043). Conclusions: Successful TAVI induces a striking hemodynamic impact with major structural (reverse remodeling) consequences in patients with pure AR caused by both bioprosthesis degeneration or NV disease. In the immediate postrelease phase, VIV patients might exhibit a more pronounced early LV contractile and structural benefit.

Few data are available on the prevalence of right ventricle (RV) systolic dysfunction, assessed including RV strain, and RV to pulmonary artery (PA) coupling in patients with aortic stenosis (AS) submitted to TAVI and the early effect of the procedure. We performed standard and speckle tracking echocardiography in 80 patients with severe AS the day before TAVI and within 48 h after TAVI. In all patients we measured TAPSE/PASP (cut-off for RV-PA uncoupling 0.31) and in 60/80 we were able to analyze RV global longitudinal strain (RV-GLS) and RV free wall strain (RV-FWS). RVFAC and TAPSE were impaired in 8.3% while RV-GLS and RV-FWS in 45% and 33.3% respectively before TAVI. TAPSE/PASP < 0.31 was documented in 7/80 patients (8.7%) before TAVI. These subjects differed from patients with TAPSE/PASP ≥ 0.31 for: enlarged left ventricular (LV) end-diastolic and end-systolic volumes (p < 0.001), worst LV ejection fraction (p < 0.001) and RVFAC (p < 0.001), worst RV-GLS and RV-FWS (p = 0.01 and p = 0.03) and bigger right atrium (RA) area (p < 0.001). After TAVI, RV systolic function did not improve while PASP significantly decreased (p = 0.005) driving the improvement of TAPSE/PASP (p = 0.01). Patients with TAPSE/PASP improvement (51%) differed from the others for worst pre-TAVI diastolic function (E/eʹ p = 0.045), RVFAC (p = 0.042), RV-GLS (p = 0.049) and RA area (p = 0.02). RV-GLS unveils RV systolic dysfunction in as much as 45% of patients with AS vs only 8.3% revealed by conventional echocardiography. RV systolic function does not significantly improve early after TAVI while RV-PA coupling does. Patients with lower TAPSE/PASP at baseline have worst LV and RV systolic function as well as larger RA. Patients who improve TAPSE/PASP after TAVI are those with worst diastolic function, RV systolic function and larger RA at baseline.

AIM: To summarize our experience on the implementation of a telemedicine service dedicated to adult congenital heart disease (ACHD) patients during the lockdown for the first wave of Coronavirus disease 2019 (COVID-19). METHODS: This is a prospective study enrolling all ACHD patients who answered a questionnaire dedicated telematic cardiovascular examination. RESULTS: A total of 289 patients were enrolled, 133 (47%) were male, 25 (9%) were affected by a genetic syndrome. The median age was 38 (29-51) years, whereas the median time interval between the last visit and the telematic follow-up was 9.5 (7.5-11.5) months. Overall, 35 patients (12%) reported a worsening of fatigue in daily life activity, 17 (6%) experienced chest pain, 42 (15%) had presyncope and 2 (1%) syncope; in addition, 28 patients (10%) presented peripheral edema and 14 (5%) were orthopneic. A total of 116 (40%) patients reported palpitations and 12 had at least one episode of atrial fibrillation and underwent successful electrical (8) or pharmacological (4) cardioversion. One patient was admitted to the emergency department for uncontrolled arterial hypertension, five for chest pain, and one for heart failure. Two patients presented fever but both had negative COVID-19 nasal swab. CONCLUSION: During the COVID-19 pandemic, the use of telemedicine dramatically increased and here we report a positive experience in ACHD patients. The postpandemic role of telemedicine will depend on permanent regulatory solutions and this early study might encourage a more systematic telematic approach for ACHD patients.

On 11 March 2020, the World Health Organization declared the coronavirus disease 2019 (COVID-19) outbreak to be a ‘pan- demic’. In the following months, the disease spread in Italy to include >2 million infected patients (3.7% of the population), with >80 000 COVID-19-related deaths. While vaccines have been developed, based on the current knowledge of biology of coronavirus infection, COVID-19 is empirically treated with antivirals, immunomodulatory and antimalarial drugs, but most of these approaches do not specifically target the cellular mechanisms involved in viral entry and spreading. It has been demonstrated that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infects human and some animal cell lines through the Angiotensin-converting enzyme 2 (ACE2) receptor, but the endocytic pathway represents an alter- native entry route for SARS-CoV-2 and other several viruses, including Ebola virus and African swine fever virus. The endo- somal protein, Niemann–Pick C1, deficiency in which causes the lysosomal disorder Niemann–Pick disease type C (NPC) , has been identified as a necessary entry receptor for Ebola virus [1, 2]. Similarly, it is well established that SARS-CoV-2 infection requires an acidic endosomal environment [3]. Of note, endo- some luminal pH and further vesicle maturation are controlled by ion channels, pumps and membrane proteins, suggesting that drugs targeting these structures may potentially have broad-spectrum antiviral properties. Indeed, recent studies have described the inhibitory effect of experimental and Food and Drug Administration-approved drugs acting on the endoso- mal compartment against viruses of high relevance for human and animal health, including SARS-CoV-2 and Ebola virus (Figure 1A) [4]. In Fabry disease, the genetically determined deficit of alpha- galactosidase A leads to glycosphingolipids accumulation in kidney, heart, vessels and lungs. Glycosphingolipid storage causes impairment of several lysosomal

Introduction: Multiple Myeloma (MM) is hematological neoplasia originating from plasma cells, which accounts for almost 1% of all oncologic malignancies. The median age of patients at diagnosis is about 65 years old and over. In this age group, cardiovascular (CV) diseases often co-exist, increasing the risk of adverse events related to MM treatment. A comprehensive search on the main educational platforms was performed and high-quality original articles and reviews were included. Areas Covered: Patients affected by MM are at risk for heart failure, uncontrolled systemic hypertension, accelerated ischemic heart disease, arterial/venous thromboembolism, and arrhythmias. These complications may be due to the effects of chemotherapy on the CV system, which may play on preexisting risk factors, and amyloid deposition at cardiac level. Expert Opinion: This review provides an updated overview of the spectrum of CV diseases that may affect MM patients, highlighting possible treatment strategies according to the latest recommendations. Cooperation between onco-hematologist and cardiologist is crucial in managing this population, in particular for adequate risk assessment, early diagnosis of CV complications, and proper treatment.

AIM: To summarize our experience on the implementation of a telemedicine service dedicated to adult congenital heart disease (ACHD) patients during the lockdown for the first wave of Coronavirus disease 2019 (COVID-19). METHODS: This is a prospective study enrolling all ACHD patients who answered a questionnaire dedicated telematic cardiovascular examination. RESULTS: A total of 289 patients were enrolled, 133 (47%) were male, 25 (9%) were affected by a genetic syndrome. The median age was 38 (29-51) years, whereas the median time interval between the last visit and the telematic follow-up was 9.5 (7.5-11.5) months. Overall, 35 patients (12%) reported a worsening of fatigue in daily life activity, 17 (6%) experienced chest pain, 42 (15%) had presyncope and 2 (1%) syncope; in addition, 28 patients (10%) presented peripheral edema and 14 (5%) were orthopneic. A total of 116 (40%) patients reported palpitations and 12 had at least one episode of atrial fibrillation and underwent successful electrical (8) or pharmacological (4) cardioversion. One patient was admitted to the emergency department for uncontrolled arterial hypertension, five for chest pain, and one for heart failure. Two patients presented fever but both had negative COVID-19 nasal swab. CONCLUSION: During the COVID-19 pandemic, the use of telemedicine dramatically increased and here we report a positive experience in ACHD patients. The postpandemic role of telemedicine will depend on permanent regulatory solutions and this early study might encourage a more systematic telematic approach for ACHD patients.

A longliving patient with MPS type I is herein depicted.

We present a 63-year-old man with Hurler–Scheie syndrome,who underwent cardia surgery, being one of the long-lived patients ever reported.

Valvular heart diseases (VHD) are common in the elderly population and several therapeutic options are available to treat them. When selecting the management strategy for VHD patients, to evaluate the operatory risk, using special scores like STS score, EuroSCORE II and other more specific ones is pivotal. However, the risk scores do not consider some aspects, such as the clinical needs of the single patients, the management of local resources and the expertise of the center. The Heart Team, combining the experience of various specialists, can optimize the decision-making process through a better evaluation of the risk/benefit ratio of the therapeutic choices. The multidisciplinary discussion of the most complex cases is recommended by the international guidelines for the management of VHD and it is part of the clinical practice of many centers. So far, both trials and large clinical studies supporting such multidisciplinary management are lacking. Despite these important limitations, the Heart Team can be actually regarded as a promising tool to locally promote best practices for the treatment of VHD patients.

Background: 2D speckle tracking echocardiography (2DSTE) is superior to standard echocardiography in the assessment of subtle right ventricle (RV) systolic dysfunction. In this study we aimed to: 1) test the hypothesis that 2DSTE may unveil subtle RV systolic dysfunction in patients with Fabry disease; 2) investigate whether the physiologic difference between the 3-segment (RV-FWS) and the 6-segment (RV-GLS) RV strain (∆RV strain) is preserved in Fabry patients. Methods and results: Standard echocardiography and 2DSTE were performed in 49 Fabry patients and 49 age- and sex-matched healthy controls. Fabry patients were divided in two groups according to the presence/absence of left ventricular hypertrophy (LVH+: left ventricular wall thickness > 12 mm, 49% of total Fabry patients). RV systolic function assessed by standard echocardiography was normal in the majority of Fabry patients (92%) while RV-GLS and RV-FWS were impaired in about 40%. RV-GLS and RV-FWS were significantly worse in patients LVH+ vs LVH- and vs controls (RV-GLS: LVH+ vs LVH-: −18.4 ± −4.3% vs −23.8 ± −3.1% p<0.001; LVH+ vs controls: −18.4 ± −4.3% vs −23.9 ± −2.8% p<0.001; RV-FWS: LVH+ vs LVH-: −21.8 ± −5.3% vs −26.7 ± −3.8% p = 0.002, LVH+ vs controls −21.8 ± −5.3% vs −26.8 ± −3.9% p<0.001). No difference was found between LVH- patients and controls in both RV-GLS (p = 0.65) and RV-FWS (p = 0.79). ∆RV strain was similar among the groups. Conclusions: In Fabry cardiomyopathy impaired RV-GLS and RV-FWS is a common finding, while RV strain is preserved in Fabry patients without overt cardiac involvement. The physiologic difference between RV-FWS and RV-GLS is maintained in Fabry patients, regardless of the presence of cardiomyopathy.

Aims: In Fabry cardiomyopathy, left ventricular outflow tract obstruction mimicking hypertrophic cardiomyopathy is a very rare finding, with few cases reported and successfully treated with cardiac surgery. In our population of patients with Fabry disease and severe left ventricular hypertrophy (LVH) at the time of diagnosis, we observed an evolution towards a midventricular obstructive phenotype. Methods and results: We present a case series of three classically affected Fabry male patients with significant diagnostic delay and severe cardiac involvement (maximal wall thickness >20 mm) at first evaluation. All patients developed midventricular obstructive form over time despite prompt initiation and optimal compliance to enzyme replacement therapy. The extension and distribution of the LVH, involving the papillary muscles, was the main mechanism of obstruction, unlike the asymmetric septal basal hypertrophy and the mitral valve abnormalities commonly seen as substrate of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. Conclusions: Fabry cardiomyopathy can evolve over time towards a midventricular obstructive form due to massive LVH in classically affected men with significant diagnostic delay and severe LVH before enzyme replacement therapy initiation. This newly described cardiac phenotype could represent an adverse outcome of the disease.

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Aims: Right ventricular hypertrophy (RVH) is a common finding in Anderson–Fabry disease (AFD), but the prognostic role of right ventricular (RV) involvement has never been assessed. The aim of our study was to evaluate the prognostic significance of RVH and RV systolic function in AFD. Methods and results: Forty-five AFD patients (56% male patients) with extensive baseline evaluation, including assessment of RVH and RV systolic function, were followed-up for an average of 51.2 ± 11.4 months. RV systolic function was assessed by standard and tissue Doppler echocardiography. Cardiovascular events were defined as new-onset atrial fibrillation (AF), sustained ventricular arrhythmias, heart failure, or pacemaker/implantable cardioverter defibrillator implantation; renal events were defined as progression to dialysis and/or renal transplantation or significant worsening of glomerular filtration rate; and cerebrovascular events were defined as transient ischaemic attack or stroke. Fourteen patients (31.1%) presented RVH, while RV systolic function was normal in all cases. During the follow-up period, 13 patients (28.8%, 11 male) experienced 18 major events, including two deaths. Cardiovascular events occurred in eight patients (17.7%). The most common event was pacemaker/implantable cardioverter defibrillator implantation (six patients, 13.3%), followed by AF (three cases, 6.6%). Only one case of worsening New York Heart Association class (from II to III and IV) was observed. Ischaemic stroke occurred in three cases (6.6%). Renal events were recorded in three patients (6.6%). At univariate analysis, several variables were associated with the occurrence of events, including RVH (HR: 7.09, 95% CI: 2.17 to 23.14, P = 0.001) and indexes of RV systolic function (tricuspid annular plane systolic excursion HR: 0.77, 95% CI: 0.62 to 0.96, P = 0.02; and RV tissue Doppler systolic velocity HR: 0.76, 95% CI: 0.61 to 0.93, P = 0.01). At mul

Successful Transcatheter Treatment of Left Pulmonary Artery to Left Atrium Communication Diagnosed in Adulthood

OBJECTIVE: We investigated whether echocardiography may help identify, among patients admitted with a suspect of non-ST-segment elevation acute myocardial infarction (NSTEMI), those with athero-thrombotic coronary artery disease (CAD).PATIENTS AND METHODS: We studied consecutive patients admitted with a clinical suspect of first NSTEMI. Echocardiography was assessed within 24 hours from admission. Patients were divided into two groups. according to the results of coronary angiography: 1) patients with obstructive stenosis (>= 50%) and/or images of thrombosis in one or more coronary arteries (CAD group); 2) patients with no evidence of obstructive coronary arteries (NOCAD group).RESULTS: Of 101 patients enrolled in the study, 53 (52.5%) showed obstructive CAD and 48 (47.5%) NOCAD. At echocardiographic examination, regional wall motion abnormalities were found in 52.8% of patients in the CAD group and 43.7% in the NOCAD group (p=0.43). Left ventricle ejection fraction was 56.4 +/- 6.8 vs. 54.7 +/- 9.8% (p=0.30) and wall motion score index was 1.16 +/- 0.26 vs. 1.21 +/- 0.32 (p=0.39) in the two groups, respectively. A multivariable logistic regression independent predictors of obstructive CAD included age, male gender, typical angina. diabetes and hypertension.CONCLUSIONS: Our data showed that. in patients with acute chest pain and increased serum troponin T concentration, routine standard echocardiography does not significantly improve the diagnostic accuracy for the presence of obstructive CAD.

Severe silent myocardial ischemia during echocardiographic examination in a patient during cardiac screening for a kidney transplant intervention

Over the past few decades, lot of evidences have shown atherosclerosis as a chronic progressive disease with an exquisite inflammatory feature. More recently, the role of innate immune response in the onset and progression of coronary artery disease (CAD) and an adaptive immunity imbalance, mostly involving T cell sub-sets, have been documented. Therefore, like in many other inflammatory and autoimmune disorders, an altered innate-adaptive immunity crosstalk could represent the key of the inflammatory burden leading to atherosclerotic plaque formation and progression and to the breakdown of plaque stability. In this review, we will address the role of inflammasome in innate immunity and in the imbalance of adaptive immunity. We will discuss how this altered immune crosstalk is related to CAD onset and progression. We will also discuss how unravelling the key molecular mechanisms is of paramount importance in the development of therapeutic tools to delay the chronic progression and prevent the acute destabilization of atherosclerotic plaque.

A 20-year-old boy was admitted to our Hospital for recurrent pleural effusion, effort breathless and initial signs of right heart failure. He had primary diagnosis, in childhood, of secundum ASD and had undergone, at the age of 16 years, ASD repair with patch. A MRI showed anomalous connection of two pulmonary veins in highest superior vena cava. We performed a modified Warden procedure with a prosthetic conduit. We report the advantages of this technique in reintervention in adult congenital patient. Learning objective To give information about the possible surgical technique in adult congenital patient with anomalous pulmonary veins connection who had previously undergone surgical procedure.