The Diagnostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy: Novel ECG Signs.

BACKGROUND: Electrocardiographic (ECG) findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) are limited to small case series. OBJECTIVES: This study aimed to analyze the ECG characteristics of ALVC patients and to correlate ECG with cardiac magnetic resonance and genotype data. METHODS: We reviewed data of 54 consecutive ALVC patients (32 men, age 39 ± 15 years) and compared them with 84 healthy controls with normal cardiac magnetic resonance. RESULTS: T-wave inversion was often noted (57.4%), particularly in the inferior and lateral leads. Low QRS voltages in limb leads were observed in 22.2% of patients. The following novel ECG findings were identified: left posterior fascicular block (LPFB) (20.4%), pathological Q waves (33.3%), and a prominent R-wave in V1 with a R/S ratio ≥0.5 (24.1%). The QRS voltages were lower in ALVC compared with controls, particularly in lead I and II. At receiver-operating characteristic analysis, the sum of the R-wave in I to II ≤8 mm (AUC: 0.909; P < 0.0001) and S-wave in V1 plus R-wave in V6 ≤12 mm (AUC: 0.784; P < 0.0001) effectively discriminated ALVC patients from controls. It is noteworthy that 4 of the 8 patients with an apparently normal ECG were recognized by these new signs. Transmural late gadolinium enhancement was associated to LPFB, a R/S ratio ≥0.5 in V1, and inferolateral T-wave inversion, and a ringlike pattern correlated to fragmented QRS, SV1+RV6 ≤12 mm, low QRS voltage, and desmoplakin alterations. CONCLUSIONS: Pathological Q waves, LPFB, and a prominent R-wave in V1 were common ECG signs in ALVC. An R-wave sum in I to II ≤8 mm and SV1+RV6 ≤12 mm were specific findings for ALVC phenotypes compared with controls.

Cardiac magnetic resonance-guided cardiac ablation: a case series of an early experience.

Radiofrequency (RF) catheter ablation has become a widely used therapeutic approach. However, long-term results in terms of arrhythmia recurrence are still suboptimal. Cardiac magnetic resonance (CMR) could offer a valuable tool to overcome this limitation, with the possibility of targeting the arrhythmic substrate and evaluating the location, depth, and possible gaps of RF lesions. Moreover, real-time CMR-guided procedures offer a radiation-free approach with an evaluation of anatomical structures, substrates, RF lesions, and possible complications during a single procedure. The first steps in the field have been made with cavotricuspid isthmus ablation, showing similar procedural duration and success rate to standard fluoroscopy-guided procedures, while allowing visualization of anatomic structures and RF lesions. These promising results open the path for further studies in the context of more complex arrhythmias, like atrial fibrillation and ventricular tachycardias. Of note, setting up an interventional CMR (iCMR) centre requires safety and technical standards, mostly related to the need for CMR-compatible equipment and medical staff's educational training. For the cardiac imagers, it is fundamental to provide correct CMR sequences for catheter tracking and guide RF delivery. At the same time, the electrophysiologist needs a rapid interpretation of CMR images during the procedures. The aim of this paper is first to review the logistic and technical aspects of setting up an iCMR suite. Then, we will describe the experience in iCMR-guided flutter ablations of two European centres, Policlinico Casilino in Rome, Italy, and Haga Teaching Hospital in The Hague, the Netherlands.

The electrocardiogram in non-ischaemic-dilated cardiomyopathy.

This article summarizes the main electrocardiogram (ECG) findings in dilated cardiomyopathy (DCM) patients. Recent reports are described in the great 'pot' of DCM peculiar ECG patterns that are typical of specific forms of DCM. Patients with late gadolinium enhancement on CMR, who are at greatest arrhythmic risk, have also distinctive ECG features. Future studies in large DCM populations should evaluate the diagnostic and prognostic value of the ECG.

Cardiac magnetic resonance in Fabry disease.

Fabry disease (FD) is a rare X-linked inherited lysosomal storage disorder caused by deficient a-galactosidase A activity that leads to an accumulation of glycolipids, mainly globotriaosylceramide (Gb3) and globotriaosylsphingosine, in affected tissues, including the heart. Cardiovascular involvement usually manifests as left ventricular hypertrophy (LVH), myocardial fibrosis, heart failure, and arrhythmias, which limit the quality of life and represent the most common causes of death. Following the introduction of enzyme replacement therapy, early diagnosis and treatment have become essential in slowing down the disease progression and preventing major cardiac complications. Recent advances in the understanding of FD pathophysiology suggest that in addition to Gb3 accumulation, other mechanisms contribute to the development of cardiac damage. FD cardiomyopathy is characterized by an earlier stage of glycosphingolipid accumulation and a later one of hypertrophy. Morphological and functional aspects are not specific in the echocardiographic evaluation of Anderson-Fabry disease. Cardiac magnetic resonance with tissue characterization capability is an accurate technique for the differential diagnosis of LVH. Progress in imaging techniques has improved the diagnosis and staging of FD-related cardiac disease: a decreased myocardial T1 value is specific of FD. Late gadolinium enhancement is typical of the later stage of cardiac involvement but as in other cardiomyopathy is also valuable to predict the outcome and cardiac response to therapy.

MR guided cardiac ablation: how to build an interventional magnetic resonance suite and what's the role of the imaging.

Magnetic resonance (MR) represents a new interesting imaging approach for guiding electrophysiology (EP)-based ablation procedures of atrial flutter and typical atrial fibrillation. This new approach permits to reach good results if compared with conventional EP ablation. Tissue characterization by MR permits to detect cardiac anatomy and pathological substrate like myocardial scars well visualized with late gadolinium enhancement (LGE) sequences. Intra-procedural imaging is useful to real-time follow the catheter during the ablation procedure and at the same time to visualize cardiac anatomy in addition to understanding if the ablation is correctly performed using oedema sequences. Performing cardiac ablations inside an MR room permits to reduce radiation exposure and occupational illnesses.

Electrocardiogram in arrhytmogenic cardiomyopathy.

Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and subsequently revised in 2010 and in 2020 by an international task force. According to the last consensus of 2020, ACM is defined as a heart muscle disease affecting right ventricle, left ventricle or both, whose principal pathologic feature is fibrofatty myocardial replacement that impairs systolic ventricular function and predisposes to lethal ventricular arrhythmias. ECG findings not only could help to early recognize affected patients but also could identify the ones with maximum risk of ventricular arrhythmias and sudden cardiac death.

Predictors of Left Ventricular Scar Using Cardiac Magnetic Resonance in Athletes With Apparently Idiopathic Ventricular Arrhythmias.

Background In athletes with ventricular arrhythmias (VA) and otherwise unremarkable clinical findings, cardiac magnetic resonance (CMR) may reveal concealed pathological substrates. The aim of this multicenter study was to evaluate which VA characteristics predicted CMR abnormalities. Methods and Results We enrolled 251 consecutive competitive athletes (74% males, median age 25 [17-39] years) who underwent CMR for evaluation of VA. We included athletes with >100 premature ventricular beats/24 h or ≥1 repetitive VA (couplets, triplets, or nonsustained ventricular tachycardia) on 12-lead 24-hour ambulatory ECG monitoring and negative family history, ECG, and echocardiogram. Features of VA that were evaluated included number, morphology, repetitivity, and response to exercise testing. Left-ventricular late gadolinium-enhancement was documented by CMR in 28 (11%) athletes, mostly (n=25) with a subepicardial/midmyocardial stria pattern. On 24-hour ECG monitoring, premature ventricular beats with multiple morphologies or with right-bundle-branch-block and intermediate/superior axis configuration were documented in 25 (89%) athletes with versus 58 (26%) without late gadolinium-enhancement (P<0.001). More than 3300 premature ventricular beats were recorded in 4 (14%) athletes with versus 117 (53%) without positive CMR (P<0.001). At exercise testing, nonsustained ventricular tachycardia occurred at peak of exercise in 8 (29%) athletes with late gadolinium-enhancement (polymorphic in 6/8, 75%) versus 17 athletes (8%) without late gadolinium-enhancement (P=0.002), (P<0.0001). At multivariable analysis, all 3 parameters independently correlated with CMR abnormalities. Conclusions In athletes with apparently idiopathic VA, simple characteristics such as number and morphology of premature ventricular beats on 12-lead 24-hour ambulatory ECG monitoring and response to exercise testing predicted the presence of concealed myocardial abnormalities on CMR. These findings may help cost-effective CMR prescription.

Dietary supplementation of Saanen goats with dried licorice root modifies chemical and textural properties of dairy products.

The aim of the present study was to evaluate the effect of dietary integration with dried licorice root on the chemical-nutritional qualities of goat milk and cheeses. The study was conducted for 60 d, during which 30 Saanen goats were divided into 2 groups: a control group (CG) that received a standard diet and an experimental group (LG+) whose diet was supplemented with licorice. At the end of the study, milk samples were collected to determine chemical-nutritional compositions and fatty acid (FA) profiles. Cheeses produced with CG and LG+ bulk milk were analyzed for chemical-physical parameters after 3 (T3) and 30 (T30) d of ripening. A different FA profile and a significant increase in protein and casein were observed in LG+ milk samples compared with CG milk. Regarding cheeses, an increase of proteins and fat was found in LG+ cheeses, which also were harder, more elastic, and more gummy than the CG samples after both 3 and 30 d of ripening. A different protein profile was detected in the 2 groups without significant variations in casein fractions (αS2-casein and ß-casein) during ripening. Moreover, greater oxidative stability was found in LG+ cheeses at both T3 and T30. Different families of volatile compounds were detected in T30 cheeses obtained from both groups. A significant reduction of octanoic acid and an increase in nonanal and ketones were found in LG+ T3 cheeses, whereas the LG+ T30 cheeses were characterized by a significant decrease of hexanoic acid an increase of 3-methyl-1-butanol and acetoin. We concluded that it is possible to assert that dietary integration with dried licorice root modified chemical and technological properties of goat cheeses, reducing lipid oxidation during ripening and inducing changes in texture that could improve consumer acceptability, although further studies are needed from this point of view.

Sacubitril/valsartan effect on left ventricular remodeling: the case of a super-responder.

Sacubitril/valsartan has been shown to improve clinical outcomes in patients with heart failure and reduced ejection fraction (HFrEF), but its effects on left ventricular (LV) systolic function and reverse remodeling parameters remain to be established. We hereby describe the case of a 41 year old man with HFrEF and severe reduction of left ventricular ejection fraction (LVEF). The patient was first treated with triple HF therapy (beta-blocker, angiotensin converting enzyme inhibitor and mineralocorticoid antagonist), but after three months he was still symptomatic and with an LVEF firmly low. In consideration of poor response to therapy, we switched angiotensin converting enzyme inhibitor to sacubitril/valsartan to the maximum tolerated dose (49/51 mg bid) with a marked improvement in LV systolic function and reduction in LV volumes at follow-up. In light of the almost normalized LVEF the patient was also removed from the list for the implantation of a cardiac resynchronization therapy defibrillator. In conclusion, our case showed a strong beneficial effect of sacubitril/valsartan on reverse remodeling and LVEF beyond the benefits of concomitant optimal medical therapy. This result is particular noteworthy because it was obtained although the patient wasn't able to reach the full dose of the drug. Physicians should always consider this drug effect when more demanding therapeutic strategies are needed for their HF patients.

Multimodality imaging evaluation of a primary cardiac lymphoma.

Primary cardiac lymphoma is a rare form of non-Hodgkin lymphoma that involves the heart with extension to pericardium and great vessels. Prognosis is poor in the absence of a prompt diagnosis and adequate therapy. Differential diagnosis includes malignant neoplasms such as angiosarcoma or metastatic carcinoma and melanoma. Clinical manifestations may be heterogeneous. Multimodality imaging work-up represents the best method for tumor detection and evaluation of its size and extension: echocardiography, computed tomography, magnetic resonance imaging, and nuclear imaging are the best imaging tools. Definitive diagnosis is achieved with cytological and histological evaluation. We report the case of a 76-year-old woman admitted to our emergency department with symptoms of congestive heart failure. Multimodality imaging work-up showed a mediastinal bulky tumor involving heart and pericardium. Pathology revealed a large B-cell primary cardiac lymphoma.

Pulse-dose radiofrequency can reduce chronic pain in trapezio-metacarpal osteoarthritis: A mini-invasive therapeutic approach.

AIM: To assess the efficiency of pulse-dose radiofrequency (PDRF) in the management of chronic pain in patients with trapezio-metacarpal osteoarthritis (OA). METHODS: Seventy-five patients with trapezio-metacarpal OA were treated with the PDRF between October 2011 and September 2013. A 22-gauge cannula with a 5-cm length was introduced with percutaneous access in the lateral region of the affected trapezio-metacarpal joint. The PDRF procedure was performed with 1200 pulses at 45 V and 20 msec duration, followed by a 480 msec silent phase. The follow-up visits were performed at 1 month, 3 and 6 months after the PDRF procedure. All patients underwent a second treatment between 7 and 9 months after the first PDRF procedure with a new follow-up scheme at 1, 3, 6 and 9 months. RESULTS: Mean visual analogue scale (VAS) scores before the procedures was 8.5 ± 1.1. A great reduction in pain intensity was reported at 3 months after the PDRF procedure (mean VAS scores 3.1 ± 0.9, P < 0.05). Pain intensity began to increase back to pre-procedural values after 4 months (mean VAS scores at 6 months was 7.9 ± 1.3, P < 0.05). After the second PDRF treatment the mean VAS scores decreased to 3.3 ± 0.8 at 3 months, but increased to 8.1 ± 1.6 at 9 months. (P < 0.05). No complications after the procedures were observed. CONCLUSIONS: The PDRF may be a safe, repeatable and effective short-term pain management technique in patients with trapezio-metacarpal OA. Larger, randomized controlled studies are indicated to better clarify the efficacy and utility of the PDRF.

High-resolution computed tomography and magnetic resonance imaging protocols in the diagnosis of fibrotic interstitial lung disease: overview for "non-radiologists".

Fibrotic interstitial lung diseases (ILDs) include a number of pulmonary disorders characterized by infiltration of inflammatory cells in lung parenchyma and fibrosis resulting in decreased lung compliance. Idiopathic pulmonary fibrosis (IPF) represents the most common ILD. ILDs can be divided in two anatomo-pathological and radiographic patterns: usual interstitial pneumonitis (UIP) and non-specific interstitial pneumonitis (NSIP). The different radiological features of UIP and NSIP are discussed. The American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association guidelines for the diagnosis and management of IPF have identified several characteristic high-resolution computed tomography (HRCT) features of UIP. However, even if these guidelines recommend to avoid surgical lung biopsy in case of confident UIP diagnosis on HRCT, they present some limitations, the most important of which is represented by interobserver agreement. Magnetic resonance imaging (MRI) can be considered as a radiation-free alternative to HRCT for several lung diseases. However, the clinical value of MRI for IPF diagnosis remains to be proven. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 300-306).

Bone Marrow Lipid Profiles from Peripheral Skeleton as Potential Biomarkers for Osteoporosis: A 1H-MR Spectroscopy Study.

RATIONALE AND OBJECTIVES: To characterize the lipidic profile of bone marrow in the calcaneus and femoral neck of healthy, osteopenic, and osteoporotic women, by using magnetic resonance spectroscopy (MRS) at 3T. The final goal was to identify specific metabolites with the potential ability to discriminate between healthy, osteopenic, and osteoporotic subjects. MATERIALS AND METHODS: Sixty-two and thirty three postmenopausal women recruited to investigate calcaneus and femoral neck, respectively, underwent a bone mineral density (BMD) measurement to be classified as healthy subjects (n = 22), osteopenic (n = 45), or osteoporotic (n = 28) patients. MRS spectra were used to quantify and compare bone marrow fat resonances between the three BMD groups. Between-group differences were tested using a Welch analysis of variance. Multiple comparisons were made with the Games-Howell correction. Relationships between pairs of variables were assessed with linear correlation analysis. Reproducibility analysis was performed for all the lipid resonances in both sites. RESULTS: The reproducibility was satisfactory. In femoral neck, methylene (L13), glycerol (L41, L43), and total lipid resonances were significantly lower in healthy as compared to osteoporotic subjects. On the other hand, in calcaneus, L13/glycerol significantly discriminated between osteopenic and osteoporotic subjects whereas L13/(unsaturated lipid) discriminated between healthy and osteopenic group. However, the reproducibility of both unsaturated lipid and glycerol resonances were less optimal. CONCLUSIONS: MRS of bone marrow lipid profiles from peripheral skeletal sites may be a promising tool for screening of large population to identify individuals with or at risk for developing osteoporosis. Moreover, it provides information about the metabolic changes occurring in bone marrow with the development of osteoporosis, which are skeletal site dependent.

Structured multidisciplinary discussion of HRCT scans for IPF/UIP diagnosis may result in indefinite outcomes.

Recent ATS/ERS/JRS/ALAT guidelines for the diagnosis and management of Idiopathic Pulmonary Fibrosis (IPF) have defined key features and specific high-resolution computerized tomography (HRCT) patterns for the diagnosis of UIP. The aim is the sorting of patients with suspected IPF into three subgroups, confident, possible or inconsistent with UIP patterns, after a multidisciplinary discussion (MDD). Specialists in respiratory diseases, radiologists and pathologists should reach IPF diagnosis based on either patients' clinical, radiological and laboratory data, either submitting patients to surgical biopsy. After ATS/ERS/JRS/ALAT recommendations have been applied, it has been identified a subgroup of patients showing uniform apical-basal distribution of honeycombing and reticular abnormalities that could not be categorized as confident, or possible nor inconsistent with UIP. These patients were subsequently diagnosed with IPF after MDD and lung biopsy. Inclusion of this pattern in the recommendation for IPF diagnosis would be worth considering.

Congenital left ventricular diverticulum: Multimodality imaging evaluation and literature review.

Congenital ventricular diverticulum is a rare cardiac malformation. We present the case of a 57-year-old man who underwent cardiac catheterization for suspected unstable angina. No coronary artery disease was diagnosed and a left ventricular diverticulum was incidentally found. Coronary CT and cardiac MRI were performed in order to confirm the diagnosis of a muscular type diverticulum and to exclude a post-ischemic aneurysm.

Idiopathic Pulmonary Fibrosis (IPF) incidence and prevalence in Italy.

BACKGROUND: Studies of Idiopathic Pulmonary Fibrosis (IPF) epidemiology show regional variations of incidence and prevalence; no epidemiological studies have been carried out in Italy. OBJECTIVE: To determine incidence and prevalence rates of IPF in the population of a large Italian region. METHODS: in this cross-sectional study study data were collected on all patients of 18 years of age and older admitted as primary or secondary idiopathic fibrosing alveolitis (ICD9-CM 516.3) to Lazio hospitals, from 1/1/2005 to 31/12/2009, using regional hospital discharge, population and cause of death databases. Reporting accuracy was assessed on a random sample of hospital charts carrying the ICD9-CM 516.3, 516.8, 516.9 and 515 codes, by reviewing radiology and pathology findings to define cases as IPF "confident", "possible" or "inconsistent". RESULTS: Annual prevalence and incidence of IPF were estimated at 25.6 per 100,000 and 7.5 per 100,000 using the ICD9-CM code 516.3 without chart audit while they were estimated at 31.6 per 100,000 and at 9,3 per 100,000 for the IPF "confident" definition after hospital chart audit. CONCLUSION: The data provide a first estimate of IPF incidence in Italy and indicate that incidence and prevalence in southern European regions may be similar to those observed in northern Europe and North America.

Osteoporosis detection by 3T diffusion tensor imaging and MRI spectroscopy in women older than 60 years.

Aim of this study was to evaluate the cancellous bone quality of postmenopausal women (age >60 years) by diffusion tensor imaging (DTI) using mean diffusivity (MD) and fractional anisotropy (FA) in combination with proton magnetic resonance spectroscopy (1H-MRS). 20 postmenopausal women older than 60 years were introduced to dual-energy X-ray absorptiometry (DXA) examination in femoral neck and to an MRI spectroscopy and DTI evaluation at 3T. We observed that fat fraction (FF) can discriminate healthy and osteoporotic patients. Water mean diffusivity (MD) and FA can discriminate the healthy group from osteopenic and osteoporotic group. MD/FF vs FA/FF graph extracted from the femoral neck identifies all healthy individuals, according to DXA results. DTI and spectroscopy protocol performed in the femoral neck could be highly sensitive and specific in identifying healthy subjects.

The Role of ECG-Gated CT in Patients with Bicuspid Aortic Valve Replacement: New Perspectives in Short- and Long-Term Followup.

The aim of our study was to compare the results of the TTE (transthoracic echocardiography) with the results obtained by the ECG-gated 64 slices CT during the followup of patients with bicuspid aortic valve (BAV), after aortic valve replacement; in particular we evaluated the aortic root and the ascending aorta looking for a new algorithm in the followup of these patients. From January 1999 to December 2009 our attention was focused on 67 patients with isolated surgical substitution of aortic valve; after dismissal they were strictly observed. During the period between May and September 2010, these patients underwent their last evaluation, and clinical exams, ECG, TTE, and an ECG-gated-MDCT were performed. At followup TTE results showed an aortic root of 36.7 ± 4 mm and an ascending aorta of 39.6 ± 4.8 mm. ECG- gated CT showed an aortic root of 37.9 ± 5.5 mm and an ascending aorta of 43.1 ± 5.2. The comparison between preoperative and postoperative TTE shows a significant long-term dilatation of the ascending aorta while the aortic root diameter seems to be stable. ECG-gated CT confirms the stability of the aortic root diameter (38.2 ± 5.3 mm versus 37.9 ± 5.5 mm; <0.0001) and the increasing diameter value of the ascending aorta (40.2 ± 3.9 mm versus 43.1 ± 5.2 mm; P = 0.0156). Due to the different findings between CT and TTE studies, ECG-gated CT should no longer be considered as a complementary exam in the followup of patients with BAV, but as a fundamental role since it is a real necessity.