Chronic Total Occlusion of the Left Main and Severe Aortic Stenosis.

Concomitant aortic stenosis and coronary artery disease in the elderly population is frequent and the proper therapeutic approach is a matter of debate. We present 2 challenging cases of patients affected by severe aortic stenosis and chronic total occlusion of the left main, demonstrating the safety and feasibility of transcatheter aortic valve replacement, performed both with balloon-expandable and self-expanding valves.

Pre-TAVI imaging: an Italian survey by the CT PRotocol optimization (CT-PRO) group.

Purpose: The purpose of this survey was to evaluate the current state-of-art of pre-TAVI imaging in a large radiological professional community.

Methods: Between December 2022 and January 2023 all members of the Italian Society of Medical and Interventional Radiology (SIRM) were invited by the CT PRotocol Optimization group (CT-PRO group) to complete an online 24-item questionnaire about pre-TAVI imaging.

Results: 557 SIRM members participated in the survey.

CT or Invasive Coronary Angiography in Stable Chest Pain.

Background: In the diagnosis of obstructive coronary artery disease (CAD), computed tomography (CT) is an accurate, noninvasive alternative to invasive coronary angiography (ICA). However, the comparative effectiveness of CT and ICA in the management of CAD to reduce the frequency of major adverse cardiovascular events is uncertain.

Methods: We conducted a pragmatic, randomized trial comparing CT with ICA as initial diagnostic imaging strategies for guiding the treatment of patients with stable chest pain who had an intermediate pretest probability of obstructive CAD and were referred for ICA at one of 26 European centers.

A very long-term observation of a family with dilated cardiomyopathy and overlapping phenotype from lamin A/C mutation.

Aims: We aim to describe one of the longest longitudinal follow-ups reported so far (>22 years), concerning a whole family affected by a missense lamin A/C mutation (Arg60Gly), which manifested as an overlapping phenotype with cardiac and extracardiac involvement over time.

Methods: Starting from the family history, two generations of that family were prospectively observed, from 1997 until 2020. At baseline, four individuals with dilated cardiomyopathy and cardiac conduction defects showed the same mutation.

Mitro-aortic fibrosa pseudoaneurysm and concomitant aortic stenosis: How to kill two birds with a stone.

Pseudoaneurysm of mitral-aortic intervalvular fibrosa (P-MAIVF) is a rare acquired malformation of the mitral-aortic intervalvular area. It appears as a pulsatile cavity in the mitral-aortic junction communicating with the left ventricular outflow tract. P-MAIVF has been reported as a complication of aortic and mitral valve surgery, infective endocarditis, and thoracic trauma.

Right ventricular function assessed by cardiac magnetic resonance predicts the response to resynchronization therapy.

Background And Aim: Cardiac resynchronization therapy (CRT) reduces mortality and morbidity in chronic heart failure symptomatic patients with broad QRS who are already undergoing optimal medical treatment. However, approximately one-third of implanted patients do not show any benefit from this treatment. Right ventricle (RV) dysfunction leads to a worse outcome in patients with heart failure, but its role in predicting the response to CRT has shown conflicting results.

Efficacy and safety of deferiprone for the treatment of superficial siderosis: results from a long-term observational study.

Background: Superficial siderosis (SS) of the central nervous system is a rare and heterogeneous condition due to deposition of hemosiderin on the surface of the brain and spinal cord. The usually progressive clinical course is characterized by a combination of hearing loss, cerebellar ataxia, and myelopathy. There is no known treatment for SS, but the iron chelator deferiprone (DFP) has been proposed as a potentially useful treatment.

Clinical Spectrum, Therapeutic Options, and Outcome of Advanced Heart Failure in Hypertrophic Cardiomyopathy.

Background: The clinical course of patients with hypertrophic cardiomyopathy and advanced heart failure (HF) subtended by progressive left ventricular dysfunction has received limited attention. Our aim was to assess the outcome of HF and impact of treatment options including the implantable cardioverter-defibrillator and heart transplantation (HT) in patients with hypertrophic cardiomyopathy evaluated at 2 Italian referral centers >3 decades.

Methods And Results: All-cause mortality and a combined end point including death, HT, or appropriate implantable cardioverter-defibrillator shock were assessed in 71 consecutive patients with HF not related to outflow obstruction (7% of the entire hypertrophic cardiomyopathy cohort) followed up for 6.

Calibration of myocardial T2 and T1 against iron concentration.

Background: The assessment of myocardial iron using T2* cardiovascular magnetic resonance (CMR) has been validated and calibrated, and is in clinical use. However, there is very limited data assessing the relaxation parameters T1 and T2 for measurement of human myocardial iron.

Methods: Twelve hearts were examined from transfusion-dependent patients: 11 with end-stage heart failure, either following death (n=7) or cardiac transplantation (n=4), and 1 heart from a patient who died from a stroke with no cardiac iron loading.

Efficacy and safety of deferiprone for the treatment of pantothenate kinase-associated neurodegeneration (PKAN) and neurodegeneration with brain iron accumulation (NBIA): results from a four years follow-up.

Objective: To evaluate the long-term effect of Deferiprone (DFP) in reducing brain iron overload and improving neurological manifestations in patients with NBIA.

Methods: 6 NBIA patients (5 with genetically confirmed PKAN), received DFP solution at 15 mg/kg po bid. They were assessed by UPDRS/III and UDRS scales and blinded video rating, performed at baseline and every six months.

Pelvic floor failure: MR imaging evaluation of anatomic and functional abnormalities.

Pelvic floor failure is a common disorder that can seriously jeopardize a woman's quality of life by causing urinary and fecal incontinence, difficult defecation, and pelvic pain. Multiple congenital and acquired risk factors are associated with pelvic floor failure, including altered collagen metabolism, female sex, vaginal delivery, menopause, and advanced age. A complex variety of fascial and muscular lesions that range from stretching, insertion detachment, denervation atrophy, and combinations of pelvic floor relaxation to pelvic organ prolapse may manifest in a single patient.

Italian registry of cardiac magnetic resonance.

Objectives: Forty sites were involved in this multicenter and multivendor registry, which sought to evaluate indications, spectrum of protocols, impact on clinical decision making and safety profile of cardiac magnetic resonance (CMR).

Materials And Methods: Data were prospectively collected on a 6-month period and included 3376 patients (47.2 ± 19 years; range 1-92 years).

Impact of heart magnetic resonance imaging on chelation choices, compliance with treatment and risk of heart disease in patients with thalassaemia major.

This study aimed to verify the impact of heart magnetic resonance imaging on chelation choices and patient compliance in a single-institution cohort as well as its predictive value for heart failure and arrhythmias. Abnormal cardiac T2* values determined changes in treatment in most subjects. Heart T2* was confirmed to be highly predictive over 1 year for heart failure and arrhythmias.

A pilot trial of deferiprone for neurodegeneration with brain iron accumulation.

Deferiprone was shown to reverse iron deposition in Friedreich's ataxia. This multi-center, unblinded, single-arm pilot study evaluated safety and efficacy of deferiprone for reducing cerebral iron accumulation in neurodegeneration with brain iron accumulation. Four patients with genetically-confirmed pantothenate kinase-associated neurodegeneration, and 2 with parkinsonism and focal dystonia, but inconclusive genetic tests, received 15 mg/kg deferiprone bid.

On T2* magnetic resonance and cardiac iron.

Background: Measurement of myocardial iron is key to the clinical management of patients at risk of siderotic cardiomyopathy. The cardiovascular magnetic resonance relaxation parameter R2* (assessed clinically via its reciprocal, T2*) measured in the ventricular septum is used to assess cardiac iron, but iron calibration and distribution data in humans are limited.

Methods And Results: Twelve human hearts were studied from transfusion-dependent patients after either death (heart failure, n=7; stroke, n=1) or transplantation for end-stage heart failure (n=4).

International reproducibility of single breathhold T2* MR for cardiac and liver iron assessment among five thalassemia centers.

Purpose: To examine the reproducibility of the single breathhold T2* technique from different scanners, after installation of standard methodology in five international centers.

Materials And Methods: Up to 10 patients from each center were scanned twice locally for local interstudy reproducibility of heart and liver T2*, and then flown to a central MR facility to be rescanned on a reference scanner for intercenter reproducibility. Interobserver reproducibility for all scans was also assessed.

Myocardial iron overload assessment by T2* magnetic resonance imaging in adult transfusion dependent patients with acquired anemias.

Only limited data are available regarding myocardial iron overload in adult patients with transfusion dependent acquired anemias. To address this topic using MRI T2* we studied 27 consecutive chronic transfusion dependent patients with acquired anemias: (22 myelodysplastic syndrome, 5 primary myelofibrosis). Cardiac MRI T2* values obtained ranged from 5.

Onset of cardiac iron loading in pediatric patients with thalassemia major.

We reviewed cardiac T2* assessments from 77 thalassemia major patients between the ages of 2.5 and 18 years to study optimal timing of cardiac iron screening by magnetic resonance imaging. No patient under 9.

[Bentall operation after previous repair of tetralogy of Fallot].

Pathologies that involve the ascending aorta are described in case reports of patients after complete repair of tetralogy of Fallot. Here, we present a case report. Preoperative tests showed an aortic ratio of > 1.

Effects of combined deferiprone and desferrioxamine iron chelating therapy in beta-thalassemia major end-stage heart failure: a case report.

Despite usual iron chelating therapy based on desferrioxamine, patients affected by beta-thalassemia major (beta-TM) often develop progressive heart failure caused by myocardial iron overload, which is the leading cause of mortality within the third decade of life. Heart transplantation is a limited therapeutic option, as very often these patients have multi-organ iron deposits and infective complications (particularly hepatitis C), secondary to frequent blood transfusions. We report the case of a 26-year-old male affected by beta-TM with end-stage heart failure, who showed a dramatic improvement in symptoms and myocardial function when a new oral iron chelating agent, deferiprone, was added to standard therapy with desferrioxamine.