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160 survivors of acute myocardial infarction (AMI) were evaluated to assess the clinical significance of supraventricular tachyarrhythmias (SVTA) occurring at discharge from the hospital after the acute event. the variables considered for the study were estimated before hospital discharge; arrhythmias were quantified with a 24 h Hotter ECG monitoring system. SVTA occurred in 88 patients (55%). Single or repetitive supraventricular premature beats were found in 65 (41%), paroxysmal atrial orjunctional tachycardias in 20 (12%), bouts of atrial flutter or fibrillation in 3 (2%). Bivariate statistical analysis showed no relationship between sex, previous cardiovascular history, type, and location of AMI and SVTA occurrence. A close positive relationship was found between age, left atrial dimension (LAD), cardio-thoracic ratio (CTR) and SVTA occurrence; an inverse relationship was found for left ventricular ejection fraction (LVEF). The presence of SVTA appeared significantly related to age above 55 years, to LAD greater than 40 mm, to LVEF less than 45%, to serum creatine kinase peak levels over 1400 U l– and to CTR over 0.49. Multivariate statistical analysis showed that five variables are important in discriminating patients suffering from SVTA: age, LAD, LVEF, left ventricular fractional shortening, and CTR. SVTA occurring at discharge from hospital after AMI are indicative of impaired left ventricular pump function. [ABSTRACT FROM PUBLISHER] more...

To assess the usefulness of a step by step evaluation of exercise left ventricular ejection fraction (LVEF), 219 consecutive patients with recent uncomplicated myocardial infarction and 30 normal subjects underwent a symptom-limited cycloergometer test followed by exercise radionuclide ventriculography (ExRNV). LVEF was monitored throughout the whole test. 49 patients underwent coronary arteriography for clinical reasons. 5 patterns of exercise LVEF could be observed: (a) progressive increase: 55 patients (25%) and 27 normal subjects (90%); (b) progressive decrease: 37 patients (17%); (c) initial increase followed by significant decrease: 54 patients (25%); (d) lack of initial modification and terminal decrease: 35 patients (16%); (e) no modification: 38 patients (17%) and 3 normal subjects (10%). Grouping the patients in this fashion allowed us to increase the specificity of ExRNV from 70% to 100%, without loss of sensitivity (95%). As for the patients in subgroup C, 32/54 showed unequivocal ECG ischaemic changes, occurring simultaneously with LVEF decrease; in 33/54 LVEF dropped during the last workload; in 25/54 the last stage LVEF was equal to or higher than the basal LVEF. The statistical analysis showed that ischaemic ECG changes (P<0.0001), exercise-induced wall-motion abnormalities (P<0.0001), and the presence of multivessel coronary artery disease (P<0.0001) were significantly more frequent in patients showing patterns (b)–(d), which should be considered as ischaemic. Our method allowed the unequivocal identification of ischaemic patterns in LVEF during exercise, which might be missed if only its basal and final values are considered. [ABSTRACT FROM PUBLISHER] more...

Objective: The incidence of metabolic dysfunction-associated steatotic liver disease (MASLD) is rapidly ramping up due to the spread of obesity, which is characterized by expanded and dysfunctional visceral adipose tissue (VAT). Previous studies have investigated the hepatic transcriptome across MASLD, whereas few studies have focused on VAT., Methods: We performed RNA sequencing in 167 hepatic samples from patients with obesity and in a subset of 79 matched VAT samples. Circulating cathepsin D (CTSD), a lysosomal protease, was measured by ELISA, whereas the autophagy-lysosomal pathway was assessed by Western blot in hepatic and VAT samples (n = 20)., Results: Inflammation, extracellular matrix remodeling, and mitochondrial dysfunction were upregulated in severe MASLD in both tissues, whereas autophagy and oxidative phosphorylation were reduced. Tissue comparative analysis revealed 13 deregulated genes, including CTSD, which showed the most robust diagnostic accuracy in discriminating mild and severe MASLD. CTSD expression correlated with circulating protein, whose increase was further validated in 432 histologically characterized MASLD patients, showing a high accuracy in foreseeing severe liver injury. In addition, the assessment of serum CTSD increased the performance of fibrosis 4 in diagnosing advanced disease., Conclusions: By comparing the hepatic and VAT transcriptome during MASLD, we refined the concept by which CTSD may represent a potential biomarker of severe disease., (© 2024 The Authors. Obesity published by Wiley Periodicals LLC on behalf of The Obesity Society.) more...

Visceral adipose tissue (VAT) contributes to metabolic dysfunction-associated steatotic liver disease (MASLD), releasing lipogenic substrates and cytokines which promote inflammation. Metabolic healthy obese individuals (MHO) may shift towardsunhealthy ones (MUHO) who develop MASLD, although the mechanisms are still unexplained. Therefore, we aimed to identify dysfunctional pathways and transcriptomic signatures shared by liver and VAT and to outline novel obesity-related biomarkers which feature MASLD in MUHO subjects, at higher risk of progressive liver disease and extrahepatic comorbidities. We performed RNA-sequencing in 167 hepatic samples and in a subset of 79 matched VAT, stratified in MHO and MUHO. A validation analysis was performed in hepatic samples and primary adipocytes from 12 bariatric patients, by qRT-PCR and western blot. We identified a transcriptomic signature that discriminate MUHO vs MHO, including 498 deregulated genes in liver and 189 in VAT. According to pathway and network analyses, oxidative phosphorylation resulted the only significantly downregulated pathway in both tissues in MUHO subjects. Next, we highlighted 5 genes commonly deregulated in liver and VAT, encompassing C6, IGF1, OXA1L, NDUFB11 and KLHL5 and we built a tissue-related score by integrating their expressions. Accordingly to RNAseq data, serum levels of C6 and IGF1, which are the only secreted proteins among those included in the gene signature were downregulated in MUHO vs MHO. Finally, the expression pattern of this 5-genes was confirmed in hepatic and VAT samples. We firstly identified the liver and VAT transcriptional phenotype of MUHO and a gene signature associated with the presence of MASLD in these at risk individuals., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.) more...

Clinical management of adult patients with congenital heart disease (GUCH) is a difficult task for multiple reasons, which include their own pathology and clinical history complexity, diagnostic complexity and organization of care. GUCH specialists are present in very small numbers and are concentrated in few centers, thus generating considerable transfer problems for patients. During the COVID-19 pandemic, telemedicine has become the standard of care, ensuring health assistance continuity, and implementing communication channels between patients and health professionals. We suggest to stratify GUCH patients into three groups, which correspond to different levels of risk (low, moderate and high, respectively) to develop complications over time, using a GUCH-specific multiparametric complexity score; so, each patient pathway will be defined according to the specific group, with indication of site, timing and type of clinical and instrumental evaluations, including virtual visits and consults. In conclusion, practical tools are provided for the implementation of updated care pathways for GUCH patients, who finally are inserted in a new model of care in which even if in-person visit still represents the crucial moment of each patient care pathway, on the other hand, telemedicine incorporation could contribute to improving and making even more complete and effective GUCH patient care. more...

Background: In adult patients with d-transposition of the great arteries after atrial switch operation, dysfunction of the systemic right ventricle (RV) is a well-known complication. Echocardiographic variables may provide adequate estimation of subpulmonary RV function, but their applicability to the subaortic RV is not straightforward. We evaluate the concordance between tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging-derived ejection fraction of the RV (MRI-RVEF) in these patients., Methods: Patients were recruited from those evaluated at the adult congenital clinic of our department between 2010 and 2012. All patients who had an echocardiographic assessment within 6 months of their MRI examination were selected. Patients clinically unstable, not in sinus rhythm, with a prosthetic systemic atrioventricular valve, permanent pacemaker, or more than moderate systemic atrioventricular valve regurgitation were excluded., Results: Eighteen Mustard-operated patients aged 22 ± 3.7 years were studied. The mean values of TAPSE and RVEF were 13.22 ± 1.7 mm and 49.7 ± 6%, respectively. TAPSE and RVEF were normal in 1 (5.5%) and 10 (55.5%) patients, respectively. Seventeen (94.4%) patients showed reduced TAPSE (12.9 ± 1.3 mm): RVEF was reduced in eight (47%) of these subjects, and normal in nine (53%). In patients with normal RVEF, both the MRI-RV end-diastolic and the MRI-RV end-systolic volumes were significantly lower than in patients with reduced RVEF. There were no other statistically significant differences between these patients. No correlation was found between TAPSE and both the MRI-RV end-diastolic and the end-systolic volumes. Globally, agreement between TAPSE and RVEF was slight (K = 0.09 ± 0.089)., Conclusions: Our results indicate that in these patients TAPSE is not a useful measure of RV function., (© 2013 Wiley Periodicals, Inc.) more...

Background: Although anthracycline cardiotoxicity is clearly related to the cumulative dose administered, subclinical cardiac dysfunction has been reported across a wide range of treatment regimens, and its clinical significance is still unclear. Purpose of this study is to investigate by exercise echocardiography for subclinical cardiac dysfunction in survivors of pediatric cancer treated with low-moderate anthracycline doses, and to evaluate whether it may alter the response of the cardiovascular system to dynamic exercise., Procedure: Post-exercise left ventricular end-systolic wall stress (ESS), left ventricular posterior wall dimension and percent thickening at end systole, and cardiopulmonary exercise test-derived indexes of cardiac function were examined in 55 apparently healthy patients (mean age 13.5 ± 2.9 years, median anthracycline cumulative dose 240 mg/m(2)) and in 63 controls., Results: Subclinical cardiac dysfunction was identified in 17 patients (30%) presenting reduced left ventricular posterior wall dimension or percent thickening, or increased values of left ventricular ESS as compared to controls (group A), while the remaining patients formed group B. Reduced oxygen consumption at peak exercise in both groups of patients was the only cardiopulmonary exercise test variable resulting significantly different between patients and controls: no differences were found among the groups of patients., Conclusions: Our results confirm that even patients treated with a median anthracycline dose of 240 mg/m(2) (range 100-490) are at considerable risk of exhibiting subclinical cardiac dysfunction that, however, does not seem to alter the physiologic response of the cardiovascular system to dynamic exercise. more...

Objectives: To evaluate whether the stenting of aortic coarctation enhance the risk of exercise-induced hypertension (EIH)., Background: There is the theoretical concern that aortic stents may cause increased aortic wall impedance and therefore systolic hypertension during exercise., Methods: Blood pressure and the Doppler derived peak and mean systolic pressure gradient (PSG and MSG) across the distal aorta at the peak of exercise were evaluated in young patients (mean age 14 +/- 3 years) with aortic coarctation successfully treated with surgery or with stent implantation at least 1 year before the test. Only patients who reached the 85% maximal predicted heart rate or whose exercise test was interrupted because of severe hypertension, and in whom significant aortic narrowings were excluded by a MRI or a CT scan performed in the six months preceding the exercise test were included in the study., Results: Seventeen patients formed the surgery-group, while 15 patients the stent-group. Patients in surgery-group were younger at coarctation repair and with a longer follow-up than those in stent-group. No difference was present regarding age, body surface area, gender, and presence, and degree of mildly hypoplastic aortic segments between the two groups as well as between patients with or without EIH. EIH was found in 35% of surgery-group patients and in 33% of stent-group patients. PSG and MSG were similar in the patients with or without EIH., Conclusions: EIH can be found in a high number of young patients successfully treated for aortic coarctation but at intermediate follow-up stent implantation does not seem to enhance the risk of EIH. more...

Hypertension at rest or during effort is not uncommon in patients with aortic coarctation (CoA), even those with a successful repair or mild degree of obstruction. Anatomic factors and functional abnormalities have been proposed as causes of this finding. Recently, aortic arch geometry was reported in association with hypertension at rest in patients with successful CoA repair. Forty-one patients (age 15.7 +/- 4.6 years) without significant obstruction at rest (mean systolic Doppler gradient at rest < or =25 mm Hg) were selected for the study. All patients underwent a maximal cardiopulmonary exercise test and magnetic resonance imaging of the aorta. Aortic arch shape was defined on global geometry as normal, gothic, and crenel. Percentage of anatomic narrowing (AN) was also calculated. Twenty-four patients (58%) showed exercise-induced hypertension (EIH). Regarding the shape of the aortic arch, normal geometry was present in 17 patients (41%), 9 (21%) had gothic geometry, and 15 (36%) had crenel geometry. There were no differences among the 3 geometries in regard to the incidence of EIH (70.6% in normal, 55.6% in gothic, and 46.7% in crenel) or AN (36.9% in normal, 33.5% in gothic, and 36.6% in crenel). In conclusion, our results fail to show a correlation between a specific aortic arch shape and the incidence of EIH and significant AN in patients with native or residual CoA or repeat CoA. Therefore, at present, the role of aortic arch geometry in identifying patients at risk of EIH is still uncertain. more...

We describe a case of a child operated on for an anomalous origin of the left coronary artery from the pulmonary artery and proximal hypoplasia of the anomalous coronary, in whom residual inducible myocardial ischaemia was detected by means of transesophageal atrial pacing combined with transthoracic echocardiography. more...

Interruption of the aortic arch in association with aortic atresia is a rare condition. We report the second case in literature in which survival was provided by an aortopulmonary fistulous communication. more...

The case of a one-year-old child in whom a large catheter-related right atrial thrombus of more than a week's duration was dissolved by urokinase is presented. After one week of unsuccessful heparin treatment, urokinase, via a central venous catheter, was added at a dose of 1500 IU/kg/h. Urokinase was subsequently increased by 1000 U/kg/h every day up to a maximum of 4500 IU/kg/h on the basis of thrombus size reduction as assessed by daily cross-sectional echocardiography. One week later the thrombus was almost completely dissolved. No major bleeding occurred. Warfarin was given for the next three months and the international normalized ratio (INR) was maintanied between 2 and 3. At the end of warfarin treatment echocardiography was negative for right atrial thrombosis. This case suggests that local high dose urokinase therapy can be safely and successfully used to dissolve large right atrial thromboses of more than a week's duration. more...

In children, acute myocardial infarction is a rare complication of infective endocarditis. Since it is usually fatal, few data on the patients who have survived are available. We report the case of a child with acute myocardial infarction and residual impairment of the coronary reserve, complicating a mitral endocarditis. more...

Abnormal incorporation of the common pulmonary vein can result in a fibromuscular partition of the left atrium, often appearing as an elongated mass on transthoracic echocardiographic examination. Differentiation from an atrial thrombus or tumour may sometimes be difficult. We report a case of a young patient with a left atrial mass detected by ultrasound study and correctly diagnosed by magnetic resonance imaging as remnant of the common pulmonary vein. more...

A placebo-controlled single-blind study was performed to evaluate the efficacy of oral propafenone on stable potentially malignant ventricular tachyarrhythmias in 13 patients who had suffered a myocardial infarction two months or longer before the trial. All patients exhibited at a 24 hour Holter monitoring a minimum mean frequency of 10 ventricular premature depolarizations (VPDs) per hour and repetitive VPDs. Ventricular tachyarrhythmias characterization was obtained by means of multiple Holter monitorings and exercise stress testings. Propafenone was considered as effective when a well-defined quantitative and qualitative reduction of ventricular tachyarrhythmias was reached. After an initial placebo phase, patients received propafenone 450 mg or 900 mg daily. Acute effectiveness of propafenone was proved in 8 on 13 patients (62%) who showed a significative reduction of VPDs (89%, p less than 0.02) and a suppression of the most complex forms of ventricular tachyarrhythmic events. The efficacy of propafenone was confirmed, three months later, in each patient, side effects were infrequent, minimal and of no clinical consequence. Oral propafenone can be considered as an effective drug for reducing the level of potentially malignant ventricular tachyarrhythmias in patients with previous myocardial infarction. more...

The clinical characteristics of supraventricular tachyarrhythmias (SVTA) and their relation to left ventricular dysfunction were assessed in 208 consecutive patients with recent myocardial infarction. Arrhythmias were quantified on hospital discharge by 24 hour electrocardiographic recording. All the variables were evaluated between the second and the fourth week after infarction. SVTA occurred in 113 (54%) patients: Supraventricular premature beats (SVPB) in 49 (24%), frequent or repetitive SVPB in 37 (18%), atrial or junctional tachycardia in 23 (11%), atrial flutter or fibrillation in 4 (2%). Most of these arrhythmias occurred in the absence of symptoms, and the most complex forms were always selflimiting. No relation was found among the presence of different forms of SVTA and sex, coronary risk factors, previous history of ischemic heart disease, type or site of acute myocardial infarction, NYHA functional class. Age, left atrial dimension (LAD), cardio-thoracic ratio (CTR) and left ventricular ejection fraction (LVEF) at rest differed significantly among three groups of patients: those without SVTA, those with SVPB less than 100 per hour and those with frequent-repetitive SVPB or atrial-junctional tachycardia. The more SVTA complexity, the worse LAD, CTR, LVEF and the higher the age. Multivariate discriminant analysis showed that CTR was directly and LVEF inversely related to the occurrence of SVPB less than 100 per hour, while the presence of frequent-repetitive SVPB or supraventricular tachycardia was closely related to increasing age, LAD, CTR and decreasing LVEF. Patients with atrial fibrillation always showed the worst values of LAD, CTR, LVEF and age. The results of the present study show that different types of SVTA occurring at discharge from hospital after myocardial infarction are clinically benign, but always suggestive of different degrees of left ventricular dysfunction. more...

The relation between exercise left ventricular ejection fraction and blood pressure (BP) responses after an acute myocardial infarction (AMI) was investigated. Twenty-eight to 37 days after an uncomplicated AMI, 224 consecutive patients underwent exercise radionuclide angiography in the 40 degrees semisupine position. In 180 patients (group A, 80%), BP increased more than 5 mm Hg every stage; in 44 patients, BP responses were abnormal; in 33 (group B, 15%), BP did not increase during 2 stages; in 11 (group C, 5%), it decreased more than 5 mm Hg after an initial increase. Ejection fraction did not differ significantly among the 3 groups at rest (51 +/- 13 in group A, 50 +/- 18 in group B, 47 +/- 13 in group C [difference not significant]) or at peak exercise (51 +/- 16% in group A, 46 +/- 19% in group B, and 43 +/- 16% in group C, [difference not significant]). Exercise-induced left ventricular failure or hemodynamic decompensation occurred in 22 patients. In these patients, ejection fraction at rest was 44 +/- 19% and decreased to 35 +/- 16% (p less than 0.05) with exercise. Only 9 of these patients (41%) had abnormal BP responses, with the other 13 (59%) showing a normal BP responses. The The 35 patients with abnormal BP responses in the absence of hemodynamic decompensation were asymptomatic, terminating exercise because of fatigue. The ejection fraction at rest and during exercise in these patients was similar to that in patients with normal BP responses.(ABSTRACT TRUNCATED AT 250 WORDS) more...

160 survivors of acute myocardial infarction (AMI) were evaluated to assess the clinical significance of supraventricular tachyarrhythmias (SVTA) occurring at discharge from the hospital after the acute event. All the variables considered for the study were estimated before hospital discharge; arrhythmias were quantified with a 24 h Holter ECG monitoring system. SVTA occurred in 88 patients (55%). Single or repetitive supraventricular premature beats were found in 65 (41%), paroxysmal atrial or junctional tachycardias in 20 (12%), bouts of atrial flutter or fibrillation in 3 (2%). Bivariate statistical analysis showed no relationship between sex, previous cardiovascular history, type, and location of AMI and SVTA occurrence. A close positive relationship was found between age, left atrial dimension (LAD), cardio-thoracic ratio (CTR) and SVTA occurrence; an inverse relationship was found for left ventricular ejection fraction (LVEF). The presence of SVTA appeared significantly related to age above 55 years, to LAD greater than 40 mm, to LVEF less than 45%, to serum creatine kinase peak levels over 1400 U l-1 and to CTR over 0.49. Multivariate statistical analysis showed that five variables are important in discriminating patients suffering from SVTA: age, LAD, LVEF, left ventricular fractional shortening, and CTR. SVTA occurring at discharge from hospital after AMI are indicative of impaired left ventricular pump function. more...

Unlabelled: The correct definition of left ventricular ejection fraction (LVEF) normal response to exercise is still debated. The lack of unanimous agreement firstly depends on the different normality criteria adopted in literature. In order to make ligh, we carefully reviewed several papers on this matter, and performed exercise radionuclide angiography (RNA), by multiple gated blood pool, in 2 different populations. I group: 39 normal subjects, selected on the basis of normal clinical examinations, ECG, X-ray film, exercise test, at rest LVEF greater than 50%: 20 males, mean age 43 +/- 13%. II group: 22 patients, abnormal from the clinical point of view, but elsewhere included in control groups: 13 males, mean age 54 +/- 9%. 14 of them refer only atypical chest pain, in 5 the sole abnormal finding is an exercise-induced ST depression greater than 1 mm, in 2 a left bundle branch block at rest, 1 patient suffers from X syndrome. Symptom limited exercise RNA was carried out by adopting a semi supine (40 degrees) cycloergometer, with a 25 watt initial workload and 25 watt subsequent increases every 3 minutes; count acquisition lasted 2 minutes, from the end of the 1st to the end of the 3rd, during each stage of the test., Results: I group: constant LVEF increment during exercise in all subjects: mean LVEF at rest was 65 +/- 8%, at maximum workload 80 +/- 8%: mean increase was 15 +/- 7%.(ABSTRACT TRUNCATED AT 250 WORDS) more...