INTRODUCTION:The left atrioventricular coupling index (LACI) has emerged as a potential prognostic marker in several clinical settings. This study evaluated the prognostic value of cardiac magnetic resonance (CMR)-derived LACI in patients with heart failure (HF) and reduced left ventricular ejection fraction (LVEF). METHODS:Patients from the multicentre DERIVATE registry with LVEF <50% who underwent CMR were included. LACI was calculated as the ratio between left atrial and left ventricular end-diastolic volumes. Univariable and multivariable Cox regression models estimated hazard ratios (HR) with 95% confidence intervals (CI) for predicting all-cause mortality (ACM), ACM or HF, and HF alone (competing-risk analysis). Time-dependent receiver operating characteristic analysis identified optimal cut-offs for 3-year outcomes. RESULTS:A total of 2170 patients were included (mean age 59.8 ± 13.9 years; 24.7% women; mean LVEF 31.6 ± 11.3%). Median follow-up was 1016 days (580-1609). Median LACI was 19.4% (13.3-28.8). During follow-up, ACM occurred in 191 patients (8.8%), ACM or HF in 565 (26.0%), and HF in 442 (20.4%). After adjustment for clinical and CMR parameters, including LVEF and late gadolinium enhancement (LGE), each 5% increase in LACI was associated with higher risk of ACM (HR 1.06, 95% CI 1.01-1.11; P = .016), ACM or HF (HR 1.09, 95% CI 1.06-1.12; P < .001), and HF (HR 1.09, 95% CI 1.05-1.12; P < .001). The optimal cut-off for ACM was LACI ≥21% (AUC 0.617, 95% CI 0.561-0.673), identifying patients at higher risk of ACM, ACM or HF, and HF (log-rank P < .001 for all). CONCLUSION:CMR-derived LACI independently predicts ACM and HF in patients with reduced LVEF and provides incremental prognostic value beyond LVEF and LGE. A cut-off of ≥21% identifies higher-risk patients and may support clinical risk stratification.

AIMS:Implantable cardioverter-defibrillator (ICD) therapy is the most effective prophylactic strategy of sudden cardiac death (SCD) in patients with ischemic cardiomyopathy (ICM). The aim of current analysis is to evaluate the prognostic impact of late gadolinium enhancement-papillary muscles (LGE-PMs) at cardiovascular magnetic resonance (CMR) and specifically its capability to re-stratify the arrhythmic risk on top to the DERIVATE-ICM Risk Score previously published. METHODS:Eighty-hundred-thirty-nine patients (mean age 65 ± 11 years; males:721[86%]) with ICM and TTE-LVEF <50% were enrolled from the DERIVATE-ICM registry (CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy- Ischemic Cardiomyopathy). Major adverse arrhythmic cardiac events (MAACE) were the primary endpoints. RESULTS:During a median follow-up of 1054 days, MAACE occurred in 86 (9.7%). DERIVATE-ICM Risk Score quartiles Q2-Q3 (HR:2.124 [95% CI:1.084-4.162]; p = 0.028), Q4 (HR: 3.865 [95% CI: 1.875-7.970]; p < 0.001) and the involvement of isolated posteromedial (P)PM (HR:1.985 [95% CI:1.073-3.673]; p = 0.029) were independent predictors of MAACE. The Kaplan-Meier survival curves showed a higher event-free rate in absence of LGE-PPM in patients categorized in the DERIVATE-ICM Risk Score quartiles Q2-Q3 (p = 0.018). Finally, adding LGE-PPM involvement on top of the model included TTE LVEF<35% plus DERIVATE-ICM Risk Score quartiles Q2-Q3 provided a significant improvement of prognostic stratification (p = 0.044). CONCLUSION:This study suggests that, in a wide population of ICM patients, LGE-PPM is independently associated with the occurrence of MAACE. In the intermediate quartiles of the DERIVATE-ICM Risk Score, the absence of LGE-PPM, when added to the Score, may contribute to downward re-stratification of arrhythmic risk. CLINICAL TRIAL REGISTRATION:RCT#NCT03352648.

BACKGROUND:the pathophysiological mechanisms underlying spontaneous coronary artery dissection (SCAD) remain incompletely understood. Inflammation may play a pivotal role by promoting vascular susceptibility to SCAD. This study aimed to evaluate pericoronary adipose tissue (PCAT) attenuation, a recognized imaging marker of vascular inflammation, in patients with SCAD. METHODS:patients with SCAD who underwent coronary computed tomography angiography (CCTA) within 48 h of the index event and with an identifiable trigger were included. Patients were classified according to the trigger preceding the event (emotional vs. physical). PCAT attenuation was measured in culprit and non-culprit vessels in all patients. RESULTS:A total of 25 SCAD patients were included (mean age 55 ± 11 years, 80.0% female). Emotional triggers were reported in 17 patients (68.0%), while 8 (32.0%) experienced a physical trigger. Type 2 dissections were more common in the emotional trigger group (64.7% vs. 25.0%, p = 0.040). Patients with emotional triggers exhibited higher PCAT attenuation compared with those with physical triggers in the SCAD-related vessel (-62.35 ± 6.46 HU vs. -70.86 ± 8.45 HU; p = 0.028) and in non-culprit vessels (-61.39 ± 7.24 HU vs. -71.16 ± 5.28 HU; p = 0.001). CONCLUSIONS:patients with SCAD demonstrated elevated PCAT attenuation, particularly in those with emotional triggers, in both culprit and non-culprit vessels. These findings suggest that vascular inflammation may represent a predisposing factor for SCAD and a target for preventive and therapeutic strategies.

AIMS:Selection of the patients for implantable cardioverter defibrillator primary prevention therapy in non-ischaemic cardiomyopathy (NICM) needs to be improved. To evaluate the additional prognostic value of a new cardiac magnetic resonance (CMR) score based on late gadolinium enhancement (LGE) pattern distribution (DERIVATE Risk Score 2.0) when compared with previously published DERIVATE Risk Score 1.0, which is based solely on quantitative parameters, in a cohort of NICM patients enrolled in the DERIVATE registry. METHODS AND RESULTS:One thousand three hundred and eighty-four NICM patients with chronic heart failure and left ventricular ejection fraction (LVEF) < 50% were evaluated for primary sudden cardiac death prevention therapy. Major adverse arrhythmic cardiac events (MAACEs) were the primary endpoint. During a median follow-up of 959 days, MAACE occurred in 128 (9.2%) patients. In the multivariate analyses, male gender [hazard ratio (HR): 1.605 (95% confidence interval, CI: 1.051-2.451); P = 0.028], LVEF per point % [HR: 0.977 (95% CI: 0.961-0.993); P = 0.005] and presence and location of midwall LGE [weighted HR: 1.066 (95% CI: 1.045-1.086), P < 0.001] were independent predictors of MAACE. A multi-parametric CMR-weighted predictive-derived score (DERIVATE Risk Score 2.0) provided a higher additional prognostic value vs. transthoracic echocardiography-LVEF cut-off of 35% when compared with the previous published DERIVATE Risk Score 1.0 with a net reclassification improvement of 54.52% (95% CI: 36.52-72.52%; P < 0.001). These findings were confirmed in the validation cohort. CONCLUSION:The presence of midwall LGE, but also the location of scar, confers an added and independent MAACE risk to a large NICM population influencing the choice of treatment.

AIMS:Accurate risk stratification for patients with non-dilated left ventricular cardiomyopathy (NDLVC) remains challenging due to lack of dedicated clinical trials. This post hoc analysis aims to delineate the arrhythmic risk and assess the incremental value of cardiac magnetic resonance (CMR) imaging in the CarDiac magnEtic Resonance for prophylactic Implantable-cardioVerter defibrillAtor ThErapy (DERIVATE) study cohort meeting the NDLVC diagnostic criteria. METHODS AND RESULTS:Patients with NDLVC from the DERIVATE registry were identified in the absence of left ventricular (LV) dilatation and in the presence of non-ischaemic LV scarring ('fibrotic NDLVC') or isolated LV systolic dysfunction (LV ejection fraction < 50%) without fibrosis ('hypokinetic NDLVC'). The primary endpoint was all-cause mortality. Major adverse arrhythmic cardiac events (MAACE) were the secondary endpoint and included sudden cardiac death (SCD) and aborted SCD. One hundred and ninety-seven NDLVC patients were identified from the cohort of the DERIVATE study (mean age: 59 ± 14 years; male: 135). Over a median follow-up of 2.7 years, 15 (8%) patients died and 8 (4%) experienced MAACE. Patients with 'hypokinetic' NDLVC had significantly lower rates of MAACE than non-ischaemic dilated cardiomyopathy (NIDCM) (P = 0.001), while patients with 'fibrotic' NDLVC had same rate of both primary (P = 0.48) and secondary endpoints (P = 0.616) compared with NIDCM patients. Multivariable analysis identified late gadolinium enhancement (LGE) with midwall distribution as an independent predictor of MAACE in NDLVC patients (hazard ratio 6.7, 95% confidence interval: 1.33-33.67; P = 0.021). CONCLUSION:NDLVC patients exhibit a heterogeneous risk profile for arrhythmic events. The presence of midwall LGE, similarly to NIDCM, is a significant predictor of MAACE, highlighting the importance of CMR imaging for risk stratification.

This review article - which is devoted to a wide range of physicians, e.g., pathologists, clinicians, radiologists, and general practitioners - is an up-to-date clinico-pathological description of cardiac tumours. Although rare, cardiac tumours are key components in oncology practice since both their early diagnosis and appropriate management denote urgent needs. Primary cardiac tumours (PCTs) are categorized in recent WHO classifications as well as in other scientific contributions. Their incidence is around 0.02 % while their prevalence is between 0.001 % and 0.03 %. Among PCTs, benign lesions account for 85 % of cases, while malignant neoplasms for 15 %. Compared to PCTs, secondary cardiac tumours are 20-30-fold more common. The most frequent PCTs in adulthood are papillary fibroelastoma and cardiac myxoma, while, in childhood, cardiac rhabdomyoma. Heart metastases may occur through direct extension, or, indirectly, via bloodstream, lymphatics or intracavitary diffusion. Thoracic cancers (e.g. from pleura, lung, breast) are the most frequent cause of cardiac metastasis followed by oesophageal and haematological malignancies. Symptoms of PCTs (e.g. arrhythmias, dyspnoea, chest discomfort, syncope) are usually the result of both their regional involvement and location. Non-invasive imaging techniques (e.g. echocardiography, MRI, CT) and biopsy are key means in delineating tumour characteristics, size, and adjacent structure involvement. Most PCTs require surgery, which is recommended to prevent life-threatening complications. While many benign cardiac neoplasms may be completely resected, the treatment of choice for malignant PCTs and metastases is a combination of surgery, radio- and/or chemotherapy, as well as new alternative treatments, which may prolong survival in a small patient subset.

INTRODUCTION Chronic cough (CC) presents a widespread and intricate challenge for diagnosis and treatment, revealing a significant lack of information on medical strategies. This study aims to gather insights from different practitioners in Southern Italy regarding their views and methodologies towards CC. METHODS The investigation engaged 102 medical practitioners from Southern Italy, encompassing 23 general practitioners, 30 pulmonologists, 25 allergologists, and 24 otolaryngologists. It examined their understanding, attitudes, and approaches towards managing CC, with an emphasis on diagnostic processes, treatment preferences, and adherence to guidelines. RESULTS The findings highlight distinct variations in the management of chronic cough among different medical specialists. Notably, otolaryngologists and pulmonologists tend to categorize chronic cough as lasting for more extended periods than allergists and general practitioners. Pulmonologists, in particular, are more prone to suggest chest X-rays for chronic cough patients, reflecting their focus on lung-related conditions. In terms of the primary causes of CC, allergists and pulmonologists often attribute it to asthma, general practitioners to gastro-esophageal reflux disease, and otolaryngologists to upper airways cough syndrome. Pulmonologists are also observed to have the most comprehensive awareness of the various conditions associated with coughing, which is likely due to their specialization in respiratory health. CONCLUSIONS The research underscores the necessity for enhanced education and standardized procedures among healthcare providers in Southern Italy for managing CC, highlighting the importance of interdisciplinary cooperation and customized treatment plans to improve the outcomes for patients with CC and guide future therapeutic strategies.

AIMS:While factors associated with adverse events are well elucidated in setting of isolated left ventricular dysfunction, clinical and imaging-based prognosticators of adverse outcomes are lacking in context of biventricular dysfunction. The purpose of this study was to establish role of clinical variables in prognosis of biventricular heart failure (HF), as assessed by cardiac magnetic resonance imaging. METHODS:Study cohort consisted of 840 patients enrolled in DERIVATE registry with coexisting CMR-derived right ventricular (RV) and left ventricular (LV) dysfunction, as defined by RV and LV ejection fractions ≤45 % and ≤ 50 %, respectively. The primary objective was to identify factors associated with adverse long-term outcomes, defined as composite of all-cause death and HF hospitalizations (DHFH). Kaplan-Meir curves were plotted for survival analysis. Cox proportional hazard models were constructed to estimate adjusted hazard ratios (aHRs) and associated 95 % confidence intervals for clinical variables and their correlation with adverse events. RESULTS:Mean age was 61.0 years; 83.1 % were male, 26.6 % had diabetes mellitus (DM), and 45.9 % had non-ischemic cardiomyopathy. At median follow-up of 2 years, DHFH occurred in 32.5 % of the cohort. Kaplan-Meir analysis showed higher rate of DHFH in patients with DM (35.2 % vs. 22.6 %, p < 0.001). Multivariate Cox regression analysis showed that DM was independently associated with DHFH (aHR 1.61 [95 % CI: 1.15-2.25]; p = 0.003). Importantly, ACE-inhibitor/ARB usage in patients with DM was associated with significant reduction in DHFH (aHR 0.53 [95 % CI: 0.31-0.90]; p = 0.02). CONCLUSION:In patients with biventricular HF, DM was a strong predictor of DHFH, with ACE-inhibitor/ARB usage having cardioprotective effect.

AIMS:The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients. METHODS AND RESULTS:In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117-1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211-3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231-3.690, P = 0.007; HR: 3.161, 95% CI: 1.750-5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084-2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort. CONCLUSION:In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation.

Background: Patients with Amyotrophic Lateral Sclerosis (ALS) undergo respiratory quarterly months controls to check respiratory muscle function, strenght and gas exchange. Arterial blood gas analysis is the gold standard for gas exchange follow-up, however it is an invasive test. End-tidal CO2 (EtCO2) is a non-invasive available tests to check the CO2 blood partial pressure. Aim: The present study aimed to correlate the reliability of ETCO2 vs PaCo2 in ALS patients undergoing routine out-patient controls. Methods: Over six months period between June 2022 and January 2023, all consecutive ALS patients referring to the outpatients clinic for quarterly follow-up were enrolled and after written consent collection they undergo ETCO2 and PaCo2 tests before functional and muscular strength tests. Results were collected. Results Sixty-6 patients were enrolled M/F (38/28), Bulbar/Spinal (6/60), Mean age (63±12). Twenty-3 out of 66 (25%) patients where already on noninvasive ventilation (NIV), the remaining never ventilated. Mean Vital Capacity was 65±13, mean peak cough flow 205±93, mean Snip 47±23 l/min. The comparison between Etco2 (33,13±5) vs mean PaCo2 (37,3±5) showed aa statistically significance P <0.001 and good linear correlation (r= 0.55). Conclutions This study demonstrated in patients with ALS a good positive linear correlation between ETCO2 and PaCo2. Therefore, Etco2 demonstrated to be a reliable non-invasive test to check CO2 blood partial pressure during quaternary months follow-up.

Background: Inflammation plays a key role in atrial fibrillation (AF). Epicardial adipose tissue around the atrial wall can influence atrial morpho-functional properties. The aim of this study was to assess whether an increased quantity and/or density of adipose tissue located around the left atrium (Fat-LA) are related to AF, independently from atrial size. Methods: eighty patients who underwent AF ablation and 80 patients without history of AF were selected. The Fat-LA mass was quantified as tissue within −190 to −30 Hounsfield Units (HU) on cardiac computed tomography angiograms (CCTA), and the mean adipose tissue attenuation was assessed. Results: Adipose tissue mass was higher in patients with AF (5.42 ± 2.94 mL) versus non-AF (4.16 ± 2.55 mL, p = 0.007), but relative fat quantity did not differ after adjusting for atrial size. Mean fat density was significantly higher in AF (−69.15 HU) versus non-AF (−76.82 HU, p < 0.0001) participants. In the logistic regression models, only the addition of mean Fat-LA attenuation led to a significant improvement of the model’s chi-square (from 22.89 of the clinical model to 31.69 of the clinical and adipose tissue attenuation model, p < 0.01) and discrimination (AUC from 0.775 to 0.829). Conclusions: Fat-LA volume is significantly greater only in absolute terms in patients with AF, but this difference does not hold after adjusting for the larger LA of AF subjects. On the contrary, a higher Fat-LA density was associated with AF, independently from LA size, providing incremental value over other variables that are associated with AF.

Background: The impact of Obstructive Sleep Apnea Syndrome (OSAS) in terms of mortality, morbidity and quality of life has been well established. Phenotyping OSAS patients has been recognized to be essential in order to make the best therapeutic choice. A particular subset of OSAS patients shows nocturnal respiratory failure (NRF), defined by a nighttime oxygen saturation <90% in more than 30% of the total sleep time (TST90). Aim: To describe phenotype of patients with OSAS and NRF, tracing predictive factors for NRF. Methods: We enrolled 116 patients with OSAS, 67 of which also had NFR. We compared clinical, anthropometric and laboratory data in patients with OSAS vs OSAS+NRF. Concerning statistical analysis, Spearman correlations, multiple linear regression and logistic regression were performed. Results: Patients with OSAS and NRF were more frequently female, had a higher BMI, lower daytime PaO2, higher AHI and a lower number of sleep hours per night. COPD was more diagnosed in the group of patients with NRF. Positive correlations were found between TST90 and, BMI, COPD, arterial hypertension, type 2 diabetes mellitus and AHI. Sex and PaO2 were negative correlated with TST90. After performing a multiple linear regression, COPD, arterial hypertension and AHI remained related with TST90. Finally, using multiple logistic regression, a significant association was found between incidence of NRF and neck circumference, asthenia, memory loss, PaO2, OHS, COPD, diabetes, arterial hypertension and thyroid diseases. Conclusions: Our study describes the phenotype of OSAS and NRF patient, finding clinical predictors associated with NRF.

Background: The association between severe hypovitaminosis D (<10ng/ml) and the illness course and deaths related to respiratory failure Coronavirus disease 19 (COVID-19) infection has been already described in the literature. The sun exposure is known to be responsible to increase Vit D circulating level. Aims: To compare the severity of COVID-19 disease and death's rate with Vit D levels among all patients admitted in a RICU in Bari during the first and second wave of infection before and after sun exposure during summer. Methods: Retrospective, observational single centre study of a total of 83 patients admitted to the COVID RICU in Bari between 1.03.2020-30.04.2020 and 1.09.2020-15.11.2020. Patients with moderate to severe Vitamin D deficiency (<20ng/dl) (Group 1; n = 30) were compared with those with Vitamin D> = 20ng/dl (Group 2; n = 83) Results: A Kaplan Meyer survival curve showed that patients in group1 had significantly reduced survival (Log Rank Mantel Cox 6,836; p = 0.009) compared to group 2. Univariate Cox analysis confirmed that Vitamin D values <20 are a risk factor for mortality (HR 6.370); P = 0.022). Multivariate model of COX Vitamin D values <20 increased the predictive capacity with HR of 30.129 (p = 0.008) compared to the HR of 6.370 of the univariate analysis. Conclusions: In this study, in comparison to previous findings, patients with moderate to severe Vitamin D deficiency (<20ng/dl) have been found at higher risk for severe COVID19 respiratory infection and mortality.

A 73-year-old man presented with acute coronary syndrome and 3-vessel coronary disease and underwent carotid ultrasonography before surgical revascularization. His medical history included right carotid stenting and iliofemoral bypass. He had no substantial blood pressure differences between arms, and no obvious subclavian or carotid bruits.A pulsed-wave Doppler ultrasonogram revealed a “bunny” waveform pattern of the left vertebral artery. This pattern, characterized by a systolic peak, a midsystolic dip, and a more blunted systolic peak (Fig. 1), suggested left subclavian stenosis.1 Computed tomographic angiograms showed irregular plaque, constituting 60% to 70% stenosis of the left subclavian artery and extending toward the origin of the left vertebral artery (Figs. 2 and 3).To avoid the effect of upstream subclavian stenosis and possible coronary subclavian steal after revascularization,2 we prepared a left internal mammary artery bypass to the left anterior descending coronary artery as a free graft.The bunny waveform resembles a rabbit's profile. It is thought to be caused by post-stenotic pressure decrease resulting from high-velocity turbulent flow through the stenosis during systole. The degree of midsystolic velocity decrease, which is associated with the severity of subclavian stenosis, indicates a “pre-steal” state that precedes systolic flow reversal seen in more advanced disease.The usefulness of carotid ultrasonography before surgical revascularization is debated.3 Current guidelines rate the need for cerebrovascular duplex examination before cardiac surgery as “uncertain” in all clinical situations, particularly in asymptomatic patients before coronary artery bypass grafting.4 More research is needed in this area.Recognizing the vertebral Doppler bunny waveform pattern in our patient led to the diagnosis of clinically unsuspected subclavian stenosis and to an important change in our surgical approach to revascularization.

Abstract Funding Acknowledgements Nothing to declare OnBehalf None Background Peri-coronary fat attenuation index (pFAI) has emerged as a clinical marker of coronary inflammation, which is measurable from standard coronary CT angiography (CCTA). It compares well with gold-standard methods for the assessment of coronary inflammation and can predict future cardiovascular events. pFAI could prove invaluable to differentiate an inflammatory from noninflammatory coronary artery status, helping unravel the mechanisms subtending an event classified as myocardial infarction with nonobstructive coronary arteries (MINOCA) or Tako-Tsubo syndrome (TTs). Methods and Results Patients admitted with MINOCA and TTs diagnosis between 2011 and 2018, who had both CCTA and CMR performed during or shortly after the acute phase, were selected and pFAI measured in their index CCTA. pFAI was also measured in a control subjects who had CCTA for atypical chest pain work-up, no obstructive coronary artery disease found in their CCTA and no cardiac events at a minimum 2-year follow-up. In the n = 106 MINOCA/TTs patients selected, mean pFAI averaged for the 3 coronary arteries was -68.37 ± 8.29 vs -78.03 ± 6.20 in the n = 106 controls (p < 0.0001) and the statistical difference was confirmed also when comparing mean pFAI in each single coronary artery between MINOCA/TTs and controls (p < 0.0001). Non-obstructive coronary plaques at CCTA, and high-risk plaques in particular, were also more frequently found (p < 0.01) in the MINOCA/TTs group compared with controls. Conclusions In MINOCA and TTs patients, CCTA is not only able to detect otherwise angiographically invisible atherosclerotic plaques, but its diagnostic yield can be further expanded using the simple off-line measurement of pFAI for the characterization of peri-coronary fat tissue. In MINOCA/TTs mean pFAI clearly demonstrates higher values in comparison with controls, a finding which has been previously associated with coronary artery inflammation. We speculate that this newly-available diagnostic tool in the future may help select patients for new therapies, for example therapies targeting coronary inflammation. I was able to build a table. Abstract 1176 Figure.

ObjectiveIgG4‐related disease (IgG4‐RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor‐like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4‐RD.MethodsReview of all cases at three tertiary‐referral centers since disease description in 2003.ResultsEleven patients were identified at a median age at presentation of 58 years (IQR, 38–65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4‐positive plasma cells per high‐powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy.ConclusionsIgG4‐RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients.Level of Evidence4 Laryngoscope, 130:2574–2580, 2020

Two articles this week focus on Erdheim-Chester disease (ECD), a rare histiocytosis that mainly affects adults. Clonal somatic mutations primarily involving proteins in the BRAF and MPAK pathways have established ECD as a myeloid neoplasm, with targeted therapies now available for patients. In the first paper, an international panel presents new consensus recommendations for evaluation and treatment of ECD. In the second paper, Pegoraro and colleagues present long-term outcomes of patients with ECD treated with sirolimus, with responses in patients both with and without BRAF mutations.

"Usefulness of echocardiographic McConnell’s sign in the computerized-medicine-era." Acta Cardiologica, 74(4), pp. 365–366