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Endomyocardial biopsies for rejection monitoring after heart transplantation are generally performed through the right internal jugular vein. We aimed to assess the feasibility and safety of using the left internal jugular vein approach as a valid alternative to a femoral vein for endomyocardial biopsies whenever thrombosis of the right internal jugular vein precludes insertion of a bioptome. We have reviewed our experience with heart transplantation in the last 2 decades to identify patients in whom surveillance endomyocardial biopsy was performed through the left internal jugular vein. We herein describe the step-by-step procedure and report the preliminary results. From May 1, 2000 to January 31, 2024, 561 orthotopic heart transplants have been performed in our unit. In 49 patients (8.7%), the right internal jugular vein access was found to be unsuitable or occluded at eco-Doppler evaluation; in 15 of them (30%) a total of 206 endomyocardial biopsies have been performed, using the left internal jugular vein, without complications. Our experience demonstrates that the left internal jugular vein is a valid alternative approach for endomyocardial biopsies when the right internal jugular vein is not available. This technique is feasible, safe and reproducible and allows adequate rejection monitoring after heart transplantation., (Copyright © 2024 Italian Federation of Cardiology - I.F.C. All rights reserved.)

Right heart failure (RHF) following implantation of a left ventricular assist device (LVAD) is a common and potentially serious condition with a wide spectrum of clinical presentations with an unfavourable effect on patient outcomes. Clinical scores that predict the occurrence of right ventricular (RV) failure have included multiple clinical, biochemical, imaging and haemodynamic parameters. However, unless the right ventricle is overtly dysfunctional with end-organ involvement, prediction of RHF post-LVAD implantation is, in most cases, difficult and inaccurate. For these reasons optimization of RV function in every patient is a reasonable practice aiming at preparing the right ventricle for a new and challenging haemodynamic environment after LVAD implantation. To this end, the institution of diuretics, inotropes and even temporary mechanical circulatory support may improve RV function, thereby preparing it for a better adaptation post-LVAD implantation. Furthermore, meticulous management of patients during the perioperative and immediate postoperative period should facilitate identification of RV failure refractory to medication. When RHF occurs late during chronic LVAD support, this is associated with worse long-term outcomes. Careful monitoring of RV function and characterization of the origination deficit should therefore continue throughout the patient's entire follow-up. Despite the useful information provided by the echocardiogram with respect to RV function, right heart catheterization frequently offers additional support for the assessment and optimization of RV function in LVAD-supported patients. In any patient candidate for LVAD therapy, evaluation and treatment of RV function and failure should be assessed in a multidimensional and multidisciplinary manner., (© 2024 European Society of Cardiology.)

It has been well assessed that women have been widely under-represented in cardiovascular clinical trials. Moreover, a significant discrepancy in pharmacological and interventional strategies has been reported. Therefore, poor outcomes and more significant mortality have been shown in many diseases. Pharmacokinetic and pharmacodynamic differences in drug metabolism have also been described so that effectiveness could be different according to sex. However, awareness about the gender gap remains too scarce. Consequently, gender-specific guidelines are lacking, and the need for a sex-specific approach has become more evident in the last few years. This paper aims to evaluate different therapeutic approaches to managing the most common women's diseases., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Metabolic Syndrome (MetS), a multifactorial condition that increases the risk of cardio-vascular events, is frequent in Heart-transplant (HTx) candidates and worsens with immunosuppressive therapy. The aim of the study was to analyze the impact of MetS on long-term outcome of HTx patients. Since 2007, 349 HTx patients were enrolled. MetS was diagnosed if patients met revised NCEP-ATP III criteria before HTx, at 1, 5 and 10 years of follow-up. MetS was present in 35% of patients pre-HTx and 47% at 1 year follow-up. Five-year survival in patients with both pre-HTx (65% vs. 78%, p < 0.01) and 1 year follow-up MetS (78% vs 89%, p < 0.01) was worst. At the univariate analysis, risk factors for mortality were pre-HTx MetS (HR 1.86, p < 0.01), hypertension (HR 2.46, p < 0.01), hypertriglyceridemia (HR 1.50, p=0.03), chronic renal failure (HR 2.95, p < 0.01), MetS and diabetes at 1 year follow-up (HR 2.00, p < 0.01; HR 2.02, p < 0.01, respectively). MetS at 1 year follow-up determined a higher risk to develop Coronary allograft vasculopathy at 5 and 10 year follow-up (25% vs 14% and 44% vs 25%, p < 0.01). MetS is an important risk factor for both mortality and morbidity post-HTx, suggesting the need for a strict monitoring of metabolic disorders with a careful nutritional follow-up in HTx patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Sponga, Vendramin, Ferrara, Marinoni, Valdi, Di Nora, Nalli, Benedetti, Piani, Lechiancole, Parpinel, Bortolotti and Livi.)

Heart failure with preserved ejection fraction (HFpEF) is characterized by a notable heterogeneity in both phenotypic and pathophysiological features, with a growing incidence due to the increase in median age and comorbidities such as obesity, arterial hypertension, and cardiometabolic disease. In recent decades, the development of new pharmacological and non-pharmacological options has significantly impacted outcomes, improving clinical status and reducing mortality. Moreover, a more personalized and accurate therapeutic management has been demonstrated to enhance the quality of life, diminish hospitalizations, and improve overall survival. Therefore, assessing the peculiarities of patients with HFpEF is crucial in order to obtain a better understanding of this disorder. Importantly, comorbidities have been shown to influence symptoms and prognosis, and, consequently, they should be carefully addressed. In this sense, it is mandatory to join forces with a multidisciplinary team in order to achieve high-quality care. However, HFpEF remains largely under-recognized and under-treated in clinical practice, and the diagnostic and therapeutic management of these patients remains challenging. The aim of this paper is to articulate a pragmatic approach for patients with HFpEF focusing on the etiology, diagnosis, and treatment of HFpEF.

Anderson-Fabry disease (AFD) is a lysosome storage disorder resulting from an X-linked inheritance of a mutation in the galactosidase A (GLA) gene encoding for the enzyme alpha-galactosidase A (α-GAL A). This mutation results in a deficiency or absence of α-GAL A activity, with a progressive intracellular deposition of glycosphingolipids leading to organ dysfunction and failure. Cardiac damage starts early in life, often occurring sub-clinically before overt cardiac symptoms. Left ventricular hypertrophy represents a common cardiac manifestation, albeit conduction system impairment, arrhythmias, and valvular abnormalities may also characterize AFD. Even in consideration of pleiotropic manifestation, diagnosis is often challenging. Thus, knowledge of cardiac and extracardiac diagnostic "red flags" is needed to guide a timely diagnosis. Indeed, considering its systemic involvement, a multidisciplinary approach may be helpful in discerning AFD-related cardiac disease. Beyond clinical pearls, a practical approach to assist clinicians in diagnosing AFD includes optimal management of biochemical tests, genetic tests, and cardiac biopsy. We extensively reviewed the current literature on AFD cardiomyopathy, focusing on cardiac "red flags" that may represent key diagnostic tools to establish a timely diagnosis. Furthermore, clinical findings to identify patients at higher risk of sudden death are also highlighted.

Chronic coronary syndrome (CCS), which encompasses a broad spectrum of clinical presentations of coronary artery disease (CAD), is the leading cause of morbidity and mortality worldwide. Recent guidelines for the management of CCS emphasize the dynamic nature of the CAD process, replacing the term "stable" with "chronic", as this disease is never truly "stable". Despite significant advances in the treatment of CAD, patients with CCS remain at an elevated risk of major cardiovascular events (MACE) due to the so-called residual cardiovascular risk. Several pathogenetic pathways (thrombotic, inflammatory, metabolic, and procedural) may distinctly contribute to the residual risk in individual patients and represent a potential target for newer preventive treatments. Identifying the level and type of residual cardiovascular risk is essential for selecting the most appropriate diagnostic tests and follow-up procedures. In addition, new management strategies and healthcare models could further support available treatments and lead to important prognostic benefits. This review aims to provide an overview of the diagnostic and therapeutic challenges in the management of patients with CCS and to promote more effective multidisciplinary care.

It has been consistently demonstrated that circulating lipids and particularly low-density lipoprotein cholesterol (LDL-C) play a significant role in the development of coronary artery disease (CAD). Several trials have been focused on the reduction of LDL-C values in order to interfere with atherothrombotic progression. Importantly, for patients who experience acute coronary syndrome (ACS), there is a 20% likelihood of cardiovascular (CV) event recurrence within the two years following the index event. Moreover, the mortality within five years remains considerable, ranging between 19 and 22%. According to the latest guidelines, one of the main goals to achieve in ACS is an early improvement of the lipid profile. The evidence-based lipid pharmacological strategy after ACS has recently been enhanced. Although novel lipid-lowering drugs have different targets, the result is always the overexpression of LDL receptors (LDL-R), increased uptake of LDL-C, and lower LDL-C plasmatic levels. Statins, ezetimibe, and PCSK9 inhibitors have been shown to be safe and effective in the post-ACS setting, providing a consistent decrease in ischemic event recurrence. However, these drugs remain largely underprescribed, and the consistent discrepancy between real-world data and guideline recommendations in terms of achieved LDL-C levels represents a leading issue in secondary prevention. Although the cost-effectiveness of these new therapeutic advancements has been clearly demonstrated, many concerns about the cost of some newer agents continue to limit their use, affecting the outcome of patients who experienced ACS. In spite of the fact that according to the current recommendations, a stepwise lipid-lowering approach should be adopted, several more recent data suggest a "strike early and strike strong" strategy, based on the immediate use of statins and, eventually, a dual lipid-lowering therapy, reducing as much as possible the changes in lipid-lowering drugs after ACS. This review aims to discuss the possible lipid-lowering strategies in post-ACS and to identify those patients who might benefit most from more powerful treatments and up-to-date management.

Background: The Italian Society of Echography and Cardiovascular Imaging (SIECVI) conducted a national survey to understand the volumes of activity, modalities and stressors used during stress echocardiography (SE) in Italy., Methods: We analyzed echocardiography laboratory activities over a month (November 2022). Data were retrieved through an electronic survey based on a structured questionnaire, uploaded on the SIECVI website., Results: Data were obtained from 228 echocardiographic laboratories, and SE examinations were performed in 179 centers (80.6%): 87 centers (47.5%) were in the northern regions of Italy, 33 centers (18.4%) were in the central regions, and 61 (34.1%) in the southern regions. We annotated a total of 4057 SE. We divided the SE centers into three groups, according to the numbers of SE performed: <10 SE (low-volume activity, 40 centers), between 10 and 39 SE (moderate volume activity, 102 centers) and ≥40 SE (high volume activity, 37 centers). Dipyridamole was used in 139 centers (77.6%); exercise in 120 centers (67.0%); dobutamine in 153 centers (85.4%); pacing in 37 centers (21.1%); and adenosine in 7 centers (4.0%). We found a significant difference between the stressors used and volume of activity of the centers, with a progressive increase in the prevalence of number of stressors from low to high volume activity ( P = 0.033). The traditional evaluation of regional wall motion of the left ventricle was performed in all centers, with combined assessment of coronary flow velocity reserve (CFVR) in 90 centers (50.3%): there was a significant difference in the centers with different volume of SE activity: the incidence of analysis of CFVR was significantly higher in high volume centers compared to low - moderate - volume (32.5%, 41.0% and 73.0%, respectively, P < 0.001). The lung ultrasound (LUS) was assessed in 67 centers (37.4%). Furthermore for LUS, we found a significant difference in the centers with different volume of SE activity: significantly higher in high volume centers compared to low - moderate - volume (25.0%, 35.3% and 56.8%, respectively, P < 0.001)., Conclusions: This nationwide survey demonstrated that SE was significantly widespread and practiced throughout Italy. In addition to the traditional indication to coronary artery disease based on regional wall motion analysis, other indications are emerging with an increase in the use of LUS and CFVR, especially in high-volume centers., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Journal of Cardiovascular Echography.)

Intracranial hemorrhage (ICH) is considered a potentially severe complication of oral anticoagulants (OACs) and antiplatelet therapy (APT). Patients with atrial fibrillation (AF) who survived ICH present both an increased ischemic and bleeding risk. Due to its lethality, initiating or reinitiating OACs in ICH survivors with AF is challenging. Since ICH recurrence may be life-threatening, patients who experience an ICH are often not treated with OACs, and thus remain at a higher risk of thromboembolic events. It is worthy of mention that subjects with a recent ICH and AF have been scarcely enrolled in randomized controlled trials (RCTs) on ischemic stroke risk management in AF. Nevertheless, in observational studies, stroke incidence and mortality of patients with AF who survived ICH had been shown to be significantly reduced among those treated with OACs. However, the risk of hemorrhagic events, including recurrent ICH, was not necessarily increased, especially in patients with post-traumatic ICH. The optimal timing of anticoagulation initiation or restarting after an ICH in AF patients is also largely debated. Finally, the left atrial appendage occlusion option should be evaluated in AF patients with a very high risk of recurrent ICH. Overall, an interdisciplinary unit consisting of cardiologists, neurologists, neuroradiologists, neurosurgeons, patients, and their families should be involved in management decisions. According to available evidence, this review outlines the most appropriate anticoagulation strategies after an ICH that should be adopted to treat this neglected subset of patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Lucà, Colivicchi, Oliva, Abrignani, Caretta, Di Fusco, Giubilato, Cornara, Di Nora, Pozzi, Di Matteo, Pilleri, Rao, Parlavecchio, Ceravolo, Benedetto, Rossini, Calvanese, Gelsomino, Riccio and Gulizia.)

Aims: Transthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to cardiac transplantation (CT). Despite case series demonstrating comparable outcomes with CT between patients with ATTR-CM and non-amyloid cardiomyopathies, ATTR-CM is considered to be a contraindication to CT in some centers, partly due to a perceived risk of amyloid recurrence in the allograft. We report long-term outcomes of CT in ATTR-CM at two tertiary centers., Materials and Methods and Results: We retrospectively evaluated ATTR-CM patients across two tertiary centers who underwent transplantation between 1990 and 2020. Pre-transplantation characteristics were determined and outcomes were compared with a cohort of non-transplanted ATTR-CM patients. Fourteen (12 male, 2 female) patients with ATTR-CM underwent CT including 11 with wild-type ATTR-CM and 3 with variant ATTR-CM (ATTRv). Median age at CT was 62 years and median follow up post-CT was 66 months. One, three, and five-year survival was 100, 92, and 90%, respectively and the longest surviving patient was Censored > 19 years post CT. No patients had recurrence of amyloid in the cardiac allograft. Four patients died, including one with ATTRv-CM from complications of leptomeningeal amyloidosis. Survival among the cohort of patients who underwent CT was significantly prolonged compared to UK patients with ATTR-CM generally ( p < 0.001) including those diagnosed under age 65 years ( p = 0.008) or with early stage cardiomyopathy ( p < 0.001)., Conclusion: CT is well-tolerated, restores functional capacity and improves prognosis in ATTR-CM. The risk of amyloid recurrence in the cardiac allograft appears to be low., Competing Interests: DH receives speaker fees from Akcea Therapeutics Ltd., and Alnylam UK. SeL receives support fees from Abiomed. MF has received consulting income from Intellia, Novo-Nordisk, Pfizer, Eidos, Prothena, Akcea, Alnylam, Caleum, Alexion, Jennsen and her institution has received clinical trial fundings from Pfizer, Eidos, and Alnylam. JuG receives consulting fees from Alnyman, Ionis, Eidos, Intellia, Pfizer, and ATTRalus. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Razvi, Porcari, Di Nora, Patel, Ioannou, Rauf, Masi, Law, Chacko, Rezk, Ravichandran, Gilbertson, Rowczenio, Blakeney, Kaza, Hutt, Lachmann, Wechalekar, Moody, Lim, Chue, Whelan, Venneri, Martinez-Naharro, Merlo, Sinagra, Livi, Hawkins, Fontana and Gillmore.)

Background: The Italian Society of Echocardiography and Cardiovascular Imaging (SIECVI) conducted a national survey to understand better how different echocardiographic modalities are used and accessed in Italy., Methods: We analyzed echocardiography laboratory activities over a month (November 2022). Data were retrieved via an electronic survey based on a structured questionnaire, uploaded on the SIECVI website., Results: Data were obtained from 228 echocardiographic laboratories: 112 centers (49%) in the northern, 43 centers (19%) in the central, and 73 (32%) in the southern regions. During the month of observation, we collected 101,050 transthoracic echocardiography (TTE) examinations performed in all centers. As concern other modalities there were performed 5497 transesophageal echocardiography (TEE) examinations in 161/228 centers (71%); 4057 stress echocardiography (SE) examinations in 179/228 centers (79%); and examinations with ultrasound contrast agents (UCAs) in 151/228 centers (66%). We did not find significant regional variations between the different modalities. The usage of picture archiving and communication system (PACS) was significantly higher in the northern (84%) versus central (49%) and southern (45%) centers ( P < 0.001). Lung ultrasound (LUS) was performed in 154 centers (66%), without difference between cardiology and noncardiology centers. The evaluation of left ventricular (LV) ejection fraction was evaluated mainly using the qualitative method in 223 centers (94%), occasionally with the Simpson method in 193 centers (85%), and with selective use of the three-dimensional (3D) method in only 23 centers (10%). 3D TTE was present in 137 centers (70%), and 3D TEE in all centers where TEE was done (71%). The assessment of LV diastolic function was done routinely in 80% of the centers. Right ventricular function was evaluated using tricuspid annular plane systolic excursion in all centers, using tricuspid valve annular systolic velocity by tissue Doppler imaging in 53% of the centers, and using fractional area change in 33% of the centers. When we divided into cardiology (179, 78%) and noncardiology (49, 22%) centers, we found significant differences in the SE (93% vs. 26%, P < 0.001), TEE (85% vs. 18%), UCA (67% vs. 43%, P < 0001), and STE (87% vs. 20%, P < 0.001). The incidence of LUS evaluation was similar between the cardiology and noncardiology centers (69% vs. 61%, P = NS)., Conclusions: This nationwide survey demonstrated that digital infrastructures and advanced echocardiography modalities, such as 3D and STE, are widely available in Italy with a notable diffuse uptake of LUS in the core TTE examination, a suboptimal diffusion of PACS recording, and conservative use of UCA, 3D, and strain. There are significant differences between northern and central-southern regions and echocardiographic laboratories that pertain to the cardiac unit. This inhomogeneous distribution of technology represents one of the main issues that must be solved to standardize the practice of echocardiography., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Journal of Cardiovascular Echography.)

The combination of oral anticoagulants (OAC) and dual antiplatelet therapy (DAPT) is the mainstay for the treatment of patients with atrial fibrillation (AF) presenting with acute coronary syndrome (ACS) and/or undergoing PCI. However, this treatment leads to a significant increase in risk of bleeding. In most cases, according to the most recent guidelines, triple antithrombotic therapy (TAT) consisting of OAC and DAPT, typically aspirin and clopidogrel, should be limited to one week after ACS and/or PCI (default strategy). On the other hand, in patients with a high ischemic risk (i.e., stent thrombosis) and without increased risk of bleeding, TAT should be continued for up to one month. Direct oral anticoagulants (DOAC) in triple or dual antithrombotic therapy (OAC and P2Y12 inhibitor) should be favored over vitamin K antagonists (VKA) because of their favorable risk/benefit profile. The choice of the duration of TAT (one week or one month) depends on a case-by-case evaluation of a whole series of hemorrhagic or ischemic risk factors for each patient. Likewise, the specific DOAC treatment should be selected according to the clinical characteristics of each patient. We propose a series of paradigmatic clinical cases to illustrate the decision-making work-up in clinical practice.

Studying cardiac masses is one of the most challenging tasks for cardiac imagers. The aim of this review article is to focus on the modern imaging of cardiac masses proceeding through the most frequent ones. Cardiac benign masses such as myxoma, cardiac papillary fibroelastoma, rhabdomyoma, lipoma, and hemangioma are browsed considering the usefulness of most common cardiovascular imaging tools, such as ultrasound techniques, cardiac computed tomography, cardiac magnetic resonance, and in the diagnostic process. In the same way, the most frequent malignant cardiac masses, such as angiosarcoma and metastases, are highlighted. Then, the article browses through nontumoral masses such as cysts, mitral caseous degenerative formations, thrombi, and vegetations, highlighting the differential diagnosis between them. In addition, the article helps in recognizing anatomic normal variants that should not be misdiagnosed as pathological entities., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Journal of Cardiovascular Echography.)

Background and Aim of the Study: In patients with acute Type A aortic dissection (A-AAD) whether repair should be limited to ascending aorta/hemiarch replacement or extended to include the aortic arch is still debated. We have analyzed our experience to compare outcomes of patients with A-AAD treated with these 2 different surgical strategies., Methods: From 2006 to 2020, a total of 213 patients have undergone repair of A-AAD at our Center; in 163 of them ascending aorta/hemiarch replacement (Group 1) and in 75 ascending aorta and arch replacement (Group 2) were performed. The primary endpoint was early survival and secondary endpoints late survival, freedom from late complications and reoperations. Patients were compared according to era of operation: 2006 to 2013 (Era 1) and 2014 to 2020 (Era 2)., Results: Overall hospital mortality was 12% and 5% in Group 1 and 2; mortality remained stable in Era 1 and 2 for Group 1 (15%), while it decreased from 8% to 1% in Group 2 patients ( p = 0.24). Actuarial survival at 5 and 10 years is 72 ± 4% and 49 ± 5% in Group 1 and 77 ± 6% and 66 ± 9% in Group 2 ( p = 0.073). Actuarial freedom from reoperation in the entire series is 94 ± 2% and 92 ± 3% at 5 and 10 years. Freedom from reoperation at 5 and 10 years is 92 ± 2% and 89 ± 3% in Group 1 and 98 ± 1% at all intervals in Group 2 ( p = 0.068)., Conclusions: An aggressive approach to A-AAD provides superior long-term results without increasing mortality. Furthermore, arch replacement during A-AAD repair represents a more stable solution with lower incidence of late aortic-related complications. Immediate aortic arch replacement should be considered in the treatment of A-AAD especially in experienced centers.

Background and objective: We reviewed a single-institution experience to verify the impact of surgery during different time intervals on early and late results in the treatment of patients with type A acute aortic dissection (A-AAD). Materials and Methods : From 2004 to 2021, a total of 258 patients underwent repair of A-AAD; patients were equally distributed among three periods: 2004-2010 (Era 1, n = 90), 2011-2016 (Era 2, n = 87), and 2017-2021 (Era 3, n = 81). The primary end-point was to assess whether through the years changes in indications, surgical strategies and techniques and increasing experience have influenced early and late outcomes of A-AAD repair. Results : Axillary artery cannulation was almost routinely used in Eras 2 (86%) and 3 (91%) while one femoral artery was mainly cannulated in Era 1 (91%) ( p < 0.01). Retrograde cerebral perfusion was predominantly used in Era 1 (60%) while antegrade cerebral perfusion was preferred in Eras 2 (94%,) and 3 (100%); ( p < 0.01). There was a significant increase of arch replacement procedures from Era 1 (11%) to Eras 2 (33%) and 3 (48%) ( p < 0.01). A frozen elephant trunk was mainly performed in Era 3. Hospital mortality was 13% in Era 1, 11% in Era 2, and 4% in Era 3 ( p = 0.07). Actuarial survival at 3 years is 74%, in Era 1, 78% in Era 2, and 89% in Era 3 ( p = 0.05). Conclusions : With increasing experience and a more aggressive approach, including total arch replacement, repair of A-AAD can be performed with low operative mortality in many patients. Patient care and treatment by a specific team organization allows a faster diagnosis and referral for surgery allowing to further improve early and late outcomes.

Aims: We aimed to describe how treatment of patients in cardiogenic shock bridged to heart transplantation with mechanical circulatory support, using either biventricular assist devices (BVADs) or extracorporeal life support (ECLS), has evolved in the last 20 years in our centre., Methods: Since 1998, 72 patients with refractory heart failure and indication for heart transplantation have received mechanical circulatory support: 40 had an ECLS system and 32 a BVAD., Results: Early mortality was similar (17.5 vs. 9.4%, P = 0.25) regardless of the type of support. After a median support time of 8 (1-27) vs. 34 (0-385) days (P < 0.01), 70 vs. 65.6% (P = 0.69) of patients underwent transplantation in the two groups. Prior to transplantation, BVAD patients were more stable with lower need for mechanical ventilation (9 vs. 57%, P < 0.01) and dialysis (0 vs. 38%, P < 0.01). Thirty-day mortality after transplantation was similar (18 vs. 14%, P = 0.53). Patients with extracorporeal support had higher rates of renal (P = 0.02) and respiratory failure (P = 0.03), infections (P = 0.02), atrial fibrillation (P = 0.03) and longer ICU stay (P = 0.01). Late survival was similar, while 5-year freedom from coronary vasculopathy was higher in those with BVAD (P = 0.03)., Conclusion: Although ECLS provides faster and simpler assistance compared with BVADs, no differences in early and medium-term outcomes in the last 20 years were observed in patients with cardiogenic shock. However, BVADs provided longer duration of support, better multiorgan recovery allowing more adequate graft selection, resulting in a lower rate of posttransplant complications., (Copyright © 2020 Italian Federation of Cardiology - I.F.C. All rights reserved.)

Objectives: Patients with mechanical circulatory support bridged to a heart transplant (HTx) are at higher risk of postoperative graft dysfunction. In this subset, a mode of graft preservation that shortens graft ischaemia should be beneficial., Methods: The outcomes of 38 patients on mechanical circulatory support (extracorporeal life support, left ventricular assist device and biventricular assist device) who received a HTx between 2015 and 2020 were analysed according to the method of graft preservation: cold storage (CS) group, 24 (63%) or ex vivo perfusion (EVP) group, 14 (37%)., Results: The median age was 57 (range 30-73) vs 64 (35-75) years (P = 0.10); 88% were men (P = 0.28); extracorporeal life support was more frequent in the CS group (54% vs 36%; P = 0.27) versus left ventricular and biventricular assist devices in the EVP group (46% vs 64%; P = 0.27). Clamping time was shorter in the EVP group (P < 0.001) and ischaemic time >4 h was higher in the CS group (P = 0.01). Thirty-day mortality was 13% (0-27%) in the CS group and 0% (P = 0.28) in the EVP group. A significantly lower primary graft failure [7% (0-23%) vs 42% (20-63%); P = 0.03] was observed in the EVP group. Survival at 1 year was 79 ± 8% (63-95%) in the CS group and 84 ± 10% (64-104%) in the EVP group (P = 0.95)., Conclusions: Our results support the use of ex vivo graft perfusion in patients on mechanical circulatory support as a bridge to a HTx. This technique, by shortening graft ischaemic time, seems to improve post-HTx outcomes., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Aims: The prognosis for patients affected by light-chain cardiac amyloidosis and acquired transthyretin-related (TTR) amyloidosis is poor. Heart transplantation (HTx) could improve prognosis also enabling autologous stem cell transplantation (ASCT) in the first group., Methods and Results: A total of 36 patients affected by systemic amyloidosis have been referred to our centre from 2009 to 2019. Of these, nine had cardiac involvement: seven by light-chain amyloidosis and two by acquired TTR amyloidosis. None died while waiting for HTx. A specific internal protocol useful to select candidates and to monitor the organ involvement after HTx was developed. Median age at diagnosis was 54 years and 66% were male. The most common short-term complication after HTx was renal failure (44%), followed by acute cardiac rejection more than 2R (22%). ASCT was performed in six out of seven light-chain cardiac amyloidosis patients, with a median time of 6 months after HTx. Two patients affected by light-chain cardiac amyloidosis died due to amyloidosis relapse: one before undergoing ASCT. After a median follow-up of 31 (7-124) months, 1- and 5-year survival was 88 and 66% in the cardiac light-chain amyloidosis group. Conversely, 1- and 5-year survival was 100% in the acquired TTR amyloidosis group., Conclusion: HTx may represent a valuable option in carefully selected patients. ASCT after HTx is an effective treatment that could decrease amyloidosis relapse in light-chain cardiac amyloidosis patients. A multidisciplinary approach is mandatory to select the best candidates and to obtain the most effective results with a specific surveillance follow-up protocol., (Copyright © 2020 Italian Federation of Cardiology - I.F.C. All rights reserved.)

Patients with heart failure suffered by a complex syndrome, where the filling of the ventricle or ejection of the blood is impaired. In this setting, the exercise capacity decreases for many reasons, one of them being the insufficient oxygen transfer due to reduced cardiac output and anemia. Ventricular assist device has emerged as a durable and safe therapy for patients with end-stage heart failure. The benefits of cardiac rehabilitation in ventricular assist device patients are enormous: the first aim is to progressively reduce the physical and functional impairments of these patients, so that they will be able to resume meaningful daily activities., Competing Interests: Disclosure Nothing., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Cardiac allograft vasculopathy (CAV) is an important cause of late mortality after heart transplantation, which may be influenced by preexisting coronary disease (CAD) in the donor heart., Methods: The aim of this study was to verify whether CAD in the donor heart had any influence on survival, cardiac-related adverse events (CRAEs), and coronary disease progression after transplantation. Donor coronary angiography performed in 289 hearts showed absence of CAD in 232 (no-CAD group) and moderate (≤50%) stenoses (CAD group) in 57. The 2 groups were compared for survival, freedom from CRAEs, and development of grade ≥ 2 CAV after transplantation., Results: Of 30-day mortality and postoperative complication rate was similar as mean follow-up (76 ± 56 and 75 ± 55 months) for no-CAD and CAD (P = 0.8). Ten-year actuarial survival was 58 ± 4% and 62 ± 7% for no-CAD and CAD (P = 0.4). Ten-year freedom from grade ≥ 2 CAV and from CRAEs was 81 ± 4% and 66 ± 5% vs 75 ± 8% and 67 ± 9% in no-CAD and CAD (P = 0.9 and 0.9, respectively)., Conclusions: Donor hearts with moderate CAD did not affect survival, freedom from CRAEs and did not accelerate development of high-grade CAV after transplantation supporting the use of such grafts to expand the donor pool. Routine use of coronary angiography in donor selection appears justified., (© 2020 Steunstichting ESOT. Published by John Wiley & Sons Ltd.)

To evaluate the impact of donor-recipient age matching on clinical outcomes after heart transplantation, a total of 509 patients (January 1990-December 2018, mean follow-up 111 ± 80 months) were stratified into 4 groups (young-R/young-D, young-R/old-D, old-R/young-D, old-R/old-D) according to the recipient (young-R < 60, old-R ≥ 60 years) and the donor (young-D < 50, old-D ≥ 50 years) age. No difference was found among 30-day mortality (P = .11) and postoperative complications between groups. Both unadjusted and adjusted survival was significantly higher for group young-R/young-D than that of other groups, in which survival was similar [adjusted HR for mortality of 2.0(1.2-3.4), 2.1(1.4-3.8) and 2.5(1.6-4.1) for groups old-R/young-D, young-R/old-D, old-R/old-D, respectively]. Compared to other groups, the incidence of grade ≥ 2 CAV was significantly lower in old-R/young-D group [adjusted HR 0.4(0.2-0.7)]. Among young recipients, the rate of acute grade ≥ 2 rejection episodes was higher in those receiving an old donor graft (P = .04). Old recipient groups were more affected by neoplasms and severe renal failure than young recipient groups (P < .01). Employment of hearts from donors ≥50 years of age adversely affects survival in recipients <60 years of age but does not influence outcomes in older recipients. Also, donor and recipient ages seem to have opposite effects on incidence of rejections and CAV of high grade., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Bicuspid pulmonary valve is a rare echocardiographic finding, particularly if not associated with other congenital heart diseases. We report the incidental case of a severe giant pulmonary arterial aneurysm associated to bicuspid pulmonary valve in an asymptomatic 79-year-old patient. Multimodality cardiac imaging was important for the correct diagnosis and to exclude any other potential complication. ( Level of Difficulty: Beginner. )., (© 2020 The Authors.)

Resistance to enzyme replacement therapy (ERT) is a major therapeutic challenge in Fabry disease (FD). Recent reports attribute to immune-mediated inflammation a main role in promoting disease progression and resistance to ERT. Aim of the study is to report a Gb3-induced auto-reactive panmyocarditis causing inefficacy of ERT and severe electrical instability, which required cardiac transplantation. Examining the explanted heart from a 57-year-old man with FD cardiomyopathy (CM) on 3-year ERT presenting incoming ventricular fibrillation, we documented a severe virus-negative myocarditis extended to cardiomyocytes, intramural coronary vessels, conduction tissue, and subepicardial ganglia. Serology was positive for anti-Gb3, anti-heart, and anti-myosin antibodies. In vitro Gb3 stimulation of patient's peripheral blood mononuclear cells (PBMC) induced high amount production of inflammatory cytokine IL1-β, IL-6, IL-8, and TNF-α. PBMC were stained using the monoclonal antibodies CD3-V500, CD4-V450, CD8-APCcy7, CD45RO-PerCPcy5.5 and CD27-FITC from BD Biosciences and CD56-PC7 from Bekman Coulter. The phenotypic analysis of PBMC showed a lower frequency of CD8 (9.2%) vs. 19.3% and NKT cells (1.6% vs. 2.4%) in Fabry patient respect to healthy donor, suggesting a possible homing to peripheral tissues. A Gb3-induced auto-reactive myocarditis is suggested as a possible cause of FDCM progression and ERT resistance. Immune-mediated inflammation of systemic Fabry cells may coexist and be controlled by implemental immunosuppressive therapy., (© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.)

Purpose of Review: To deeply investigate one type of intracellular cardiac storage disease, the Fabry disease and one example of extracellular cardiac storage disease, the cardiac amyloidosis, with the aim to collect data about cardiac treatment at the end-stage level and the feasibility of heart transplantation (HTx) in this setting of patients., Recent Findings: Some registry describes that Fabry disease and cardiac amyloidosis showed similar characteristics as hypertrophic cardiomyopathies; thus, their correct diagnosis is often missing or time consuming. A multiorgan approach is mandatory to recognize the main systemic findings of these diseases, involving also the kidneys, the brain, the autonomous system and the skin., Summary: The early diagnosis of these diseases is required to start as soon as possible the correct therapy for the patients affected. However, the end-stage heart failure is common and HTx could be offered to selected patients, especially if affected by light chain cardiac amyloidosis, to allow to perform the autologous stem cell transplantation after the cardiac transplant. Considering the Fabry disease patients, the enzyme recombinant therapy is also mandatory after HTx to avoid potential release of the systemic disease on the donor graft.

Danon disease is characterized by hypertrophic cardiomyopathy, skeletal myopathy, and intellectual disability due to deficiency of the lysosome-associated membrane protein-2 (LAMP-2). Although heart transplantation is considered an option for end stage Danon cardiomyopathy, scarce information is available about long term follow up. We report on long term follow up (14.7 years, IQ range 9-21 years) of 4 patients, transplanted for Danon disease cardiomyopathy, showing two LAMP-2 gene variants, the novel c.815T > C and the previously reported c.294G > A. We have also analysed previous published paper on this topic comparing available data from different follow up. Being a skeletal and cardiac muscle disease, with systemic effects, long term results about HTx are indispensable to justify any treatments in this subset of patients., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: There are data showing race-related differences regarding left ventricular (LV) geometry in hypertensive patients. Several authors reported that concentric remodeling is the most common remodeling pattern in hypertensive African-Americans, and this pattern may be related to prognosis. There is little information about the LV remodeling patterns in hypertensive Africans that migrated to Europe, which might have different distributions from those seen in African-Americans. The aim our study was to describe the prevalence of LV remodeling patterns in hypertensive African-Europeans and to compare it with that of hypertensive Caucasians., Methods: This is a descriptive study that included 135 consecutive treated hypertensive African-Europeans and 128 hypertensive Caucasians. Patients were examined by transthoracic echocardiography and categorized into the four classic geometric patterns according to LV mass index (LVMi) and relative wall thickness (RWT)., Results: The mean age and gender distribution were similar in the 2 groups. Caucasians had significantly higher body mass index, LV diastolic and systolic diameters, while African-Europeans had higher RWT and interventricular septum thickness. No differences in LVMi was found. In the African-European group, the prevalence of normal pattern, concentric remodeling, concentric hypertrophy and eccentric hypertrophy were 13%, 36%, 45% and 6% respectively, while in Caucasians they were 21%, 33%, 34% and 12%, respectively. African-Europeans had a higher prevalence of concentric remodeling and hypertrophy compared to Caucasians (81% vs. 67%, p = .005)., Conclusions: This study shows important differences in LV geometry between treated African-European and Caucasian hypertensive patients. Also, African-Europeans may have slightly different characteristics compared to African-Americans., (Copyright © 2019 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

: Heart transplantation is a life-saving therapy for some patients admitted for acute myocarditis. However, controversial exists about the major risk of rejection following heart transplantation in specific types of myocarditis. Because of relatively few data on the post heart transplant outcomes, we report the long-term follow-up of a 39-year-old patient with a previous history of ulcerative colitis, which rapidly worsened heart failure until an emergency heart transplant in 2004.The clinical course was complicated by many episodes of rejection; lastly, after the development of severe cardiac allograft vasculopathy, re-heart transplantation was needed. The main findings of this case are: 1) inflammatory aetiology should always be suspected in patients with concomitant autoimmune disease that developing rapidly progressing heart failure; 2) patients with inflammatory myocardial disease undergoing heart transplantation should also undergo strict immunological surveillance; 3) the option of performing the re-heart transplant in a patient with a so complex management in the first one could be uncertain, but in this case the young age and lack of noncardiac comorbidities were effective to favour the survivor after two immunologically so challenging heart transplantation.

Serum uric acid (UA) has been shown to be a predictor of cardiovascular (CV) morbidity and mortality, and it may play a role in the pathogenesis of CV disease affecting vascular structure and function. However, there is limited evidence of its specific association with carotid artery stiffness and structure. The aim of our study was to evaluate whether UA is associated with early signs of atherosclerosis, namely local carotid arterial stiffness and intima-media thickening. We evaluated 698 consecutive asymptomatic patients, referred to the Cardiovascular Department for risk factors evaluation and treatment. All patients underwent carotid artery ultrasonography with measurement of common carotid intima-media thickness (IMT) and echo-tracking carotid artery stiffness index Beta. Patients with hyperuricemia (defined as serum uric acid ≥7 mg/dL in men and ≥6 mg/dL in women) had higher IMT (0.97±0.22 vs 0.91±0.18, p<0.001) and stiffness index Beta (8.3±3.2 vs 7.5±2.7, p=0.005). UA levels correlated with both IMT (r=0.225; p<0.001) and stiffness index Beta (r=0.154; p<0.001); the correlations were statistically significant in males and females. In a multivariate model which included age, arterial pressure, serum glucose and LDL-cholesterol, serum UA emerged as an independent explanatory variable of IMT and stiffness index Beta. Carotid IMT and local arterial stiffness are related to UA independently of established CV risk factors; UA may play a role in the early development of atherosclerosis.

Background: The ejection fraction/velocity ratio (EFVR) is a simple function-corrected index of aortic stenosis severity with a good correlation with aortic valve area measured using the Gorlin formula at cardiac catheterization. It is calculated by dividing left ventricular ejection fraction (LVEF) to 4 × (peak jet velocity) 2 ., Objective: Our aim was to evaluate the value of EFVR in predicting adverse events in patients with asymptomatic aortic stenosis., Methods: We analyzed the clinical and echocardiographic data of 216 asymptomatic patients with at least moderate aortic stenosis (AVA ≤ 1.5 cm 2 ). The primary end-point was cardiovascular death or aortic valve replacement., Results: There were 119 (55%) men and mean age was 68 ± 10 years. The mean follow-up time was 4.2 ± 1.6 years (median 4.3 years). During follow-up, the composite end-point of death or aortic valve replacement was reached in 105 patients (49%). Using multivariate Cox regression analysis, EFVR and valvulo-arterial impedance emerged as independent variables associated with outcome (P < 0.001 and P = 0.001, respectively). In the subgroup of patients with severe aortic stenosis (AVA < 1 cm 2 ), EFVR ≤ 0.9 was associated with an increased hazard ratio for the composite end-point of mortality and aortic valve replacement (HR 2.14, 95% CI: 1.15-4.0, P = 0.017), even after adjusting for aortic valve area., Conclusions: In patients with asymptomatic moderate to severe aortic stenosis, EFVR is useful for risk stratification. Our results suggest that incorporating EFVR in the evaluation of patients with asymptomatic aortic stenosis might help identify those who are most likely to benefit from early elective aortic replacement., (© 2018 Wiley Periodicals, Inc.)

Aim: To assess adverse outcomes attributable to non-cardiac co-morbidities and to compare their effects by left ventricular ejection fraction (LVEF) group [LVEF <50% (heart failure with reduced ejection fraction, HFrEF), LVEF ≥50% (heart failure with preserved ejection fraction, HFpEF)] in a contemporary, unselected chronic heart failure population., Methods and Results: This community-based cohort enrolled patients from October 2009 to December 2013. Adjusted hazard ratio (HR) and the population attributable fraction (PAF) were used to compare the contribution of 15 non-cardiac co-morbidities to adverse outcome. Overall, 2314 patients (mean age 77 ±10 years, 57% men) were recruited [n = 941 (41%) HFrEF, n = 1373 (59%) HFpEF]. Non-cardiac co-morbidity rates were similarly high, except for obesity and hypertension which were more prevalent in HFpEF. At a median follow-up of 31 (interquartile range 16-41) months, 472 (20%) patients died. Adjusted mortality rates were not significantly different between the HFrEF and HFpEF groups. After adjustment, an increasing number of non-cardiac co-morbidities was associated with a higher risk for all-cause mortality [HR 1.25; 95% confidence interval (CI) 1.10-1.26; P < 0.001], all-cause hospitalization (HR 1.17; 95% CI 1.12-1.23; P < 0.001), heart failure hospitalization (HR 1.28; 95% CI 1.19-1.38; P < 0.001), non-cardiovascular hospitalization (HR 1.16; 95% CI 1.11-1.22; P < 0.001). The co-morbidities contributing to high PAF were: anaemia, chronic kidney disease, chronic obstructive pulmonary disease, diabetes mellitus, and peripheral artery disease. These findings were similar for HFrEF and HFpEF. Interaction analysis yielded similar results., Conclusions: In a contemporary community population with chronic heart failure, non-cardiac co-morbidities confer a similar contribution to outcomes in HFrEF and HFpEF. These observations suggest that quality improvement initiatives aimed at optimizing co-morbidities may be similarly effective in HFrEF and HFpEF., (© 2018 The Authors. European Journal of Heart Failure © 2018 European Society of Cardiology.)