Change in prevalence of ATTR variants in Italy - Results from a National Survey.

Introduction: Hereditary transthyretin amyloidosis (ATTRv) is a rare, heterogenous, inherited disorder caused by over 130 gene mutations. Its prevalence was estimated to 4.33/million in 2020 in Italy.

[Management of cardiac amyloidosis in Italy: a national survey of the Italian Cardiac Amyloidosis Network (RIAC)].

Background: The national survey of the SIC-ANMCO Amyloidosis Centers was designed to create a geographical mapping of the centers that identify and follow patients with cardiac amyloidosis in Italy and to describe their diagnostic capabilities and multidisciplinary organization.

Methods: The survey was administered to 212 centers in Italy. Among them, 153 centers responded (72%), 31/35 (89%) of which were academic medical centers and 122/177 (69%) hospitals.

Air bubbles around the heart: a rare case report of pneumohydropericardium in heart transplantation.

Background: Pneumohydropericardium is a rare but significant condition characterized by the presence of both air and fluid in the pericardial space. It is often associated with trauma, invasive procedures, or thoracic pathologies, and its occurrence following heart transplantation is particularly unusual. Early recognition is essential to prevent complications such as cardiac tamponade, although conservative management can be effective in cases without haemodynamic compromise.

Cardiac amyloidosis red flags: What all the cardiologist have to know.

Cardiac amyloidosis is becoming increasingly important among cardiologist and an early diagnosis is very important. Amyloidosis is a systemic disease and many cardiac and extracardiac elements (red flags) should raise the suspicion of the disease. Electrocardiographic and imaging techniques (such as echocardiography, cardiac magnetic resonance and scintigraphy) are useful tools to make a diagnosis together with the presence of orthopedic issues, peripheral neuropathy or plasma cell dyscrasia.

Estimation of Right Atrial Pressure by Ultrasound-Assessed Jugular Vein Distensibility in Patients With Heart Failure.

Background: Clinical evaluation of central venous pressure is difficult, depends on experience, and is often inaccurate in patients with chronic advanced heart failure. We assessed the ultrasound-assessed internal jugular vein (JV) distensibility by ultrasound as a noninvasive tool to identify patients with normal right atrial pressure (RAP ≤7 mm Hg) in this population.

Methods: We measured JV distensibility as the Valsalva-to-rest ratio of the vein diameter in a calibration cohort (N=100) and a validation cohort (N=101) of consecutive patients with chronic heart failure with reduced ejection fraction who underwent pulmonary artery catheterization for advanced heart failure therapies workup.

Ultrasound-Assisted, Catheter-Directed Thrombolysis for Acute Intermediate/High-Risk Pulmonary Embolism: Design of the Multicenter USAT IH-PE Registry and Preliminary Results.

Catheter-based revascularization procedures were developed as an alternative to systemic thrombolysis for patients with intermediate-high- and high-risk pulmonary embolisms. USAT IH-PE is a retrospective and prospective multicenter registry of such patients treated with ultrasound-facilitated, catheter-directed thrombolysis, whose preliminary results are presented in this study. The primary endpoint was the incidence of pulmonary hypertension (PH) at follow-up.

Case Report: A rare homozygous patient affected by systemic amyloidosis with a prominent heart involvement.

Hereditary transthyretin amyloidosis is a severe, adult-onset autosomal dominant inherited systemic disease predominantly affecting the peripheral and autonomic nervous system, heart, kidney, and the eyes. We present a case of a Caucasian 65-year-old man with cardiac amyloidosis and the homozygous mutation Val142Ile (classically, Val122Ile) in the transthyretin gene. We provide a genotype-phenotype correlation regarding the genetic status of both heterozygous and homozygous individuals and their clinical conditions at the time of genetic testing.

Biochemical but not imaging parameters are predictive of outcome in septic shock: a pilot study.

Background: Septic shock is a severe form of sepsis marked by hypotension with an ominous outcome despite the introduction of modern intensive care. The aim of the present study is to obtain a panel with biomarkers, echocardiographic and vascular parameters to better risk stratify patients and identify those at higher risk of ominous outcome.

Methods: Between May 2013 and April 2016, 35 consecutive patients admitted at the Intensive Care Unit (ICU) of ASST Great Metropolitan Hospital Niguarda with the diagnosis of severe sepsis or septic shock were enrolled.

Prevalence and Rate of Resolution of Left Atrial Thrombus in Patients with Non-Valvular Atrial Fibrillation: A Two-Center Retrospective Real-World Study.

Background And Aim: Thromboembolic events due to left atrial appendage (LAA) thrombosis are the main complication of non-valvular atrial fibrillation (NVAF). Although anticoagulants are effective in patients with NVAF, a minimal residual thromboembolic risk persists. Little is known about the prevalence of LAA thrombus and the rate of resolution after the recommended period of anticoagulation therapy, including vitamin K antagonists (VKA), heparin, and non-vitamin K antagonist oral anticoagulants (NOACs).

Primary mediastinal large B-cell lymphoma and pregnancy: a challenging clinical scenario.

A 26-weeks pregnant woman presented with progressively worsening dyspnoea and poor general conditions. Using low-dose radiation multi-imaging techniques and thoracic biopsy a primary mediastinal large B cell was diagnosed. A multidisciplinary approach identified the correct hemodynamic management, the best therapeutic strategy and the timing for delivery.

Prevalence of hypertension mediated organ damage in subjects with high-normal blood pressure without known hypertension as well as cardiovascular and kidney disease.

Purpose of our study was to assess the prevalence of hypertension mediated organ damage (HMOD) in healthy subjects with high-normal Blood Pressure (BP) comparing them with subjects with BP values that are considered normal (<130/85 mmHg) or indicative of hypertension (≥140/90 mmHg). Seven hundred fifty-five otherwise healthy subjects were included. HMOD was evaluated as pulse wave velocity (PWV), left ventricular mass index (LVMI), and carotid intima-media thickness (IMT) and plaque.

Gamma Knife Radiosurgery for Treatment of Brain Metastases during the COVID-19 Outbreak.

Introduction: The WHO declared 2019 severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) a public health emergency of international concern. The National and Regional Health System has been reorganized, and many oncological patients died during this period or had to interrupt their therapies. This study summarizes a single-centre experience, during the COVID-19 period in Italy, in the treatment of brain metastases with Gamma Knife stereotactic radiosurgery (GKRS).

Assessment of right ventricular function in advanced heart failure with nonischemic dilated cardiomyopathy: insights of right ventricular elastance.

Background: The right ventriculoarterial coupling (R-V/A), a measure of right ventricular systolic dysfunction (RVSD) adaptation/maladaptation to chronic overload, and consequent pulmonary hypertension, has been little investigated in nonischemic dilated cardiomyopathy (NIDCM). We examined the correlates of R-V/A and traditional echocardiographic indices of RVSD, over the spectrum of pulmonary hypertension and tertiles of mean pulmonary artery pressures (PAPm).

Methods: In 2016-2017, we studied 81 consecutive patients for heart transplant/advanced heart failure.

Could two-dimensional radial strain be considered as a novel tool to identify pre-clinical hypertrophic cardiomyopathy mutation carriers?

Treatment of overt form of hypertrophic cardiomyopathy (HCM) is often unsuccessful. Efforts are focused on a possible early identification in order to prevent or delaying the development of hypertrophy. Our aim was to find an echocardiographic marker able to distinguish mutation carriers without left ventricular hypertrophy (LVH) from healthy subjects.

Safety of centrifugal left ventricular assist device in patients previously treated with MitraClip system.

Introduction: No data regarding the safety of continuous-flow left ventricular assist device (CF-LVAD) implantation in patients with previous MitraClip have been reported. Thus, it remains unknown whether an initial treatment strategy with MitraClip therapy might complicate future heart failure management in patients who are also considered for CF-LVAD.

Methods: We retrospectively identified 6 patients (median age of 62 years; 2 women) who had been treated with MitraClip, that were eventually implanted with a CF-LVAD (all Heartware HVAD) in 3 hospitals between 2013 and 2018.

Portico Sheathless Transcatheter Aortic Valve Implantation via Distal Axillary Artery.

Transcatheter aortic valve implantation has been designed to treat older patients affected by severe aortic stenosis who are considered high-risk surgical candidates because of multiple comorbidities. The least invasive approach for transcatheter aortic valves implantation should be considered the transfemoral retrograde route, because it is minimally invasive and is feasible with local anesthesia and mild sedation. Despite significant technical improvements in recent years, the transfemoral approach is contraindicated in cases of severe peripheral artery disease.

Annexin A5 in treated hypertensive patients and its association with target organ damage.

Objective: Annexin A5 (AnxA5) has been previously linked to the presence of carotid and cardiac target organ damage (TOD) in the context of heart failure and rheumatologic patients. However, information is scant in the context of hypertension. Aim of our study was to evaluate AnxA5 in treated hypertension patients compared with normotensive controls and to determine whether it is associated with vascular and heart TOD evaluated as arterial stiffness, carotid plaque and left ventricular hypertrophy.

Evolut R Implantation to Treat Severe Pure Aortic Regurgitation in a Patient With Mitral Bioprosthesis.

Transcatheter aortic valves have been designed to treat high-risk surgical candidates affected by severe aortic stenosis, but little is known about the use of transcatheter valves in patients with severe pure aortic regurgitation. We describe the implantation of Medtronic CoreValve Evolut R (Medtronic, Minneapolis, MN) to treat an 82-year-old patient affected by severe pure aortic regurgitation who underwent prior mitral valve replacement with a biological valve protruding into the left ventricular outflow tract.

Long-Term Effects of Radiotherapy on Arterial Stiffness in Breast Cancer Women.

Radiotherapy for breast cancer may expose heart and vessels to late radiation-induced complications. Although recent technical progress in radiation therapy (RT) has been associated with drastic reduction in cardiovascular (CV) mortality, the prolonged life expectancy of patients with cancer requires CV evaluation for many years. The aim of our study was to evaluate local changes in vascular and cardiac function because of previous breast RT.