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Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5–300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46–1.00) for the first round and 0.55 (0.44–1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47–1.00)). Criterion validity showed a 78 (73–84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research. © The Author(s) 2021.

Summary Andrologists, cardiologists and diabetologists (and general practitioners) have the great opportunity to collaborate and find shared clinical workup for the benefit of a large number of men. Several evidence established a link between erectile dysfunction (ED), cardiovascular disease (CVD), diabetes, and metabolic syndrome. Not only these conditions share many risk factors and pathophysiological mechanisms but also an emerging paradigm indicates that ED is, in fact, an independent marker of cardiovascular disease risk, CV events and CV mortality. However, there is no consensus on the best cardiologic investigation in men with ED with no known CVD and, on the contrary, on what is the clinical and prognostic role of detecting ED during cardiovascular investigation and CVD risk assessment. Only vasculogenic ED, which represents the most common type of organic ED, indeed represents a harbinger of CVD, especially for younger patients, and might be diagnosed by dynamic penile color doppler ultrasonography, which represents a real cardiovascular imaging technique that give evidence on the presence of systemic endothelial dysfunction and atherosclerosis. Furthermore, assessment of glucose and lipid metabolism is warranted as first step workup in all ED patients, and diabetologists should ask their patients for erectile function, address ED patients to andrologists, and consider vasculogenic ED in the context of the cardiovascular and metabolic workup and in the context of diabetic complications. Sexual symptoms (and testosterone levels) should sound as harbinger for cardiovascular and metabolic investigation and cardiologists and diabetologists have the opportunity to have a symptom (erectile dysfunction) and a vascular test (penile color doppler) that help them in better management of patients, their comorbidities and complications.

Background: Coronary microvascular dysfunction (CMD) leads to a worse prognosis in heart transplantation (HT) patients. Coronary flow velocity reserve (CFVR) estimates the physiologic impact of allograft disease on the coronary circulation. Purpose: Our aim was to determine the prognostic role of CFVR and its companion (CFVRC) on long-term survival of HT patients with a follow-up of 28 years. Methods: 134 HT patients, surviving at least 5 years after HT, with normal systolic ventricular function and no evidence of angiographic allograft vasculopathy or symptoms/signs of rejection were included. The enrolled population underwent echocardiographic evaluation of microvascular function by the assessment of both the ratio of hyperemic to rest diastolic peak velocity (DPVh and DPVr). These measurements yield CFVR and its associated companion, defined as CFVRC=v{(DPVr)²+(DPVh)²}, as well as basal and hyperemic coronary microvascular resistance (BMR and HMR). A CFVR=2.5 was considered abnormal; the median value of DPVh (75 cm/s) and CFVRC (80 cm/s) were utilized to dichotomize the population. Results: Based on CFVR and DPVh, HT patients can be assigned to four groups: group 1 (n=32), discordant with preserved CFVR (3.1±0.4); group 2 (n=60), concordant with preserved CFVR (3.4±0.5); group 3 (n=31), concordant with impaired CFVR (1.8±0.3) and group 4 (n=11), discordant with impaired CFVR (2.0±0.2). Survival for each patient group is presented in the Figure (panel A). Specifically, survival was similar in group 1 when compared to group 3 (p=0.8), but significantly lower when compared to group 2 (p=0.03). Therefore, a normal CFVR (>2.5) may not be able to predict the unfavourable long-term outcome. CFVR in fact is an incomplete dimensionless ratio; if the paired velocities are low with high BMR and HMR (group 1), the use of CFVR alone may miss some events, that are yet captured by CFVRC. Differences between survivors and no survivors are presented in the Table. At multivariable survival analysis, CMD, DPVh

Objective To describe a novel case of COVID-19 associated acute necrotizing encephalopathy in a patient with aplastic anemia where there was early brainstem predominant involvement. Methods Evaluation of cause, clinical symptoms, and treatment response. Results A 59-year-old woman with a background of transfusion-dependent aplastic anemia, presented with seizures and reduced level of consciousness, 10 days following the onset of subjective fevers, cough, and headache. Nasopharyngeal swab testing for SARS-CoV-2 was positive and admission CT demonstrated diffuse swelling of the brainstem. She required intubation and mechanical ventilation for airway protection, given her reduced level of consciousness. The patient’s condition deteriorated and MRI on day 6 demonstrated worsening brainstem swelling with symmetrical hemorrhagic lesions in the brainstem, amygdalae, putamina and thalamic nuclei. Appearances were consistent with hemorrhagic acute necrotizing encephalopathy with early brainstem involvement. The patient showed no response to steroid therapy and died on the eighth day of admission. Conclusions COVID-19 may be associated with an acute severe encephalopathy and, in this case, was considered most likely to represent an immune-mediated phenomenon. As the pandemic continues, we anticipate that the spectrum of neurological presentation will broaden. It will be important to delineate the full clinical range of emergent COVID-19 related neurological disease.

Background Although everolimus potentially improves long-term heart transplantation (HTx) outcomes, its early postoperative safety profile had raised concerns and needs optimization. Methods This 6-month, open-label, multicenter randomized trial was designed to compare the cumulative incidence of a primary composite safety endpoint comprising wound healing delays, pericardial effusion, pleural effusion needing drainage, and renal insufficiency events (estimated glomerular filtration rate ≤30/mL/min per 1.73 m2) in de novo HTx recipients receiving immediate everolimus (EVR-I) (≤144 hours post-HTx) or delayed everolimus (EVR-D) (4-6 weeks post-HTx with mycophenolate mofetil as a bridge) with reduced-dose cyclosporine A. Cumulative incidence of biopsy-proven rejection ≥ 2R, rejection with hemodynamic compromise, graft loss, or death was the secondary composite efficacy endpoint. Results Overall, 181 patients were randomized to the EVR-I (n = 89) or EVR-D (n = 92) arms. Incidence of primary safety endpoint was higher for EVR-I than EVR-D arm (44.9% vs 32.6%; P = 0.191), mainly driven by a higher rate of pericardial effusion (33.7% vs 19.6%; P = 0.04); wound healing delays, acute renal insufficiency events, and pleural effusion occurred at similar frequencies in the study arms. Efficacy failure was not significantly different in EVR-I arm versus EVR-D arm (37.1% vs 28.3%; P = 0.191). Three patients in the EVR-I arm and 1 in the EVR-D arm died. Incidence of clinically significant adverse events leading to discontinuation was higher in EVR-I arm versus EVR-D arm (P = 0.02). Conclusions Compared with immediate initiation, delayed everolimus initiation appeared to provide a clinically relevant early safety benefit in de novo HTx recipients, without compromising efficacy., The 6-month, open-label, multicenter randomized trial is designed to compare primary safety endpoints in de novo heart transplantation and delayed everolimus initiation seems to provide a clinically relevant early safety benefit compared to immediate initiation without compromising efficacy. Supplemental digital content is available in the text.

Takotsubo cardiomyopathy is a syndrome characterized by acute regional systolic dysfunction of the left ventricle, frequently related to psycho-physical acute stress, and usually reversible. This rare syndrome involves more often the female sex with the highest frequency of occurrence between the seventh and eighth decade of life. Etiology has not been clarified yet and several hypotheses have been postulated: multiple epicardial coronary artery damage, abnormal coronary microcirculation, catecholamine-mediated cardiac toxicity, and neurogenic stunning. Clinical presentation is not easy to distinguish from an acute coronary syndrome: chest pain at rest or dyspnea, new-onset electrocardiographic changes, characterized by ST-segment elevation or T-wave inversion. Coronary angiography, which should be performed within 48 h of symptom onset in order to be diagnostic, excludes the presence of significant atherosclerotic stenosis or plaque rupture. Ventricular angiography shows the typical regional wall motion abnormalities (apical akinesia and hyperkinesia of the mid-basal segments) that give to the syndrome its name (takotsubo is a traditional Japanese octopus trap or pot). Echocardiography performed in the acute phase also evidences wall motion abnormalities that characteristically regress in the following days. There is no specific treatment for this syndrome, but supportive and symptomatic therapy is usually administered