Clinical presentations leading to arrhythmogenic left ventricular cardiomyopathy.

Objectives: To describe a cohort of patients with arrhythmogenic left ventricular cardiomyopathy (ALVC), focusing on the spectrum of the clinical presentations.

Methods: Patients were retrospectively evaluated between January 2012 and June 2020. Diagnosis was based on (1) ≥3 contiguous segments with subepicardial/midwall late gadolinium enhancement in the left ventricle (LV) at cardiac magnetic resonance a likely pathogenic/pathogenic arrhythmogenic cardiomyopathy (AC) associated genetic mutation familial history of AC red flags for ALVC (ie, negative T waves in V4-6/aVL, low voltages in limb leads, right bundle branch block like ventricular tachycardia) or (2) pathology examination of explanted hearts or autoptic cases suffering sudden cardiac death (SCD).

Impact on clinical outcomes of right ventricular response to percutaneous correction of secondary mitral regurgitation.

Aims: In patients with heart failure and reduced ejection fraction (HFrEF) and secondary mitral regurgitation (SMR), impaired right ventricular function (RVF) may negatively influence the prognosis. Percutaneous mitral valve repair (pMVR) can promote the recovery of RVF. We sought to characterize the response of the right ventricle to pMVR in HFrEF with SMR and to assess the association between improved RVF after pMVR and outcomes.

Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy.

Objectives: To evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).

Methods: In this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.

Transcatheter Mitral Valve Repair in Cardiogenic Shock and Mitral Regurgitation: A Patient-Level, Multicenter Analysis.

Objectives: The aim of this study was to evaluate the outcome of transcatheter mitral valve repair (TMVr) in patients with cardiogenic shock and significant mitral regurgitation (MR).

Background: Patients in cardiogenic shock with severe MR have a poor prognosis in the setting of conventional medical therapy. Because of its favorable safety profile, TMVr is being increasingly used as an acute therapy in this population, though its efficacy remains unknown.

Modifications of medical treatment and outcome after percutaneous correction of secondary mitral regurgitation.

Aims: The optimization of guideline-directed medical therapy (GDMT) in reduced ejection fraction heart failure (HFrEF) is associated with improved survival and can reduce the severity of secondary mitral regurgitation (SMR). Highest tolerated doses should be achieved before percutaneous mitral valve repair (pMVR) and drugs titration further pursued after procedure. The degree of GDMT titration in patients with HFrEF and SMR treated with pMVR remains unexplored.

Differences in cardiac phenotype and natural history of laminopathies with and without neuromuscular onset.

Objective: To investigate differences in cardiac manifestations of patients affected by laminopathy, according to the presence or absence of neuromuscular involvement at presentation.

Methods: We prospectively analyzed 40 consecutive patients with a diagnosis of laminopathy followed at a single centre between 1998 and 2017. Additionally, reports of clinical evaluations and tests prior to referral at our centre were retrospectively evaluated.

Antithrombotic Management during Percutaneous Mitral Valve Repair with the Mitraclip System in a Patient with Heparin-Induced Thrombocytopenia.

Interventional cardiology procedures require full anticoagulation to prevent thrombus formation on catheters and devices with potential development of embolic complications. Bivalirudin, a short half-life direct thrombin inhibitor, has been largely used during percutaneous coronary interventions and represents the preferred alternative to heparin in patients with heparin-induced thrombocytopenia (HIT). However, few data are available about intraprocedural use of bivalirudin during transcatheter structural heart disease interventions.

Predicting device failure after percutaneous repair of functional mitral regurgitation in advanced heart failure: Implications for patient selection.

Background: Patients with heart failure (HF) and severe symptomatic functional mitral regurgitation (FMR) may benefit from MitraClip implantation. With increasing numbers of patients being treated the success of procedure becomes a key issue. We sought to investigate the pre-procedural predictors of device failure in patients with advanced HF treated with MitraClip.

Percutaneous mitral valve repair: The last chance for symptoms improvement in advanced refractory chronic heart failure?

Background: The role of percutaneous mitral valve repair (PMVR) in patients with end-stage heart failure (HF) and functional mitral regurgitation (FMR) is unclear.

Methods: Seventy-five consecutive patients with FMR grade≥3+ and severe HF symptoms despite optimal medical therapy and resynchronization therapy underwent PMVR with the MitraClip system (Abbott, Abbott Park, IL, USA) at 3 centers. Clinical evaluation, echocardiography and pro-BNP measurement were performed at baseline and at 6-month.

Balloon aortic valvuloplasty as a bridge-to-decision in high risk patients with aortic stenosis: a new paradigm for the heart team decision making.

Background: Whilst the majority of the patients with severe aortic stenosis can be directly addressed to surgical aortic valve replacement (AVR) or transcatheter aortic valve implantation (TAVI), in some instances additional information may be needed to complete the diagnostic workout. We evaluated the role of balloon aortic valvuloplasty (BAV) as a bridge-to-decision (BTD) in selected high-risk patients.

Methods: Between 2007 and 2012, the heart team in our Institution required BTD BAV in 202 patients.

Usefulness of Electrocardiographic Patterns at Presentation to Predict Long-term Risk of Cardiac Death in Patients With Hypertrophic Cardiomyopathy.

The objective of this study was to investigate the prognostic significance of 12-lead electrocardiogram (ECG) patterns in a large multicenter cohort of patients with hypertrophic cardiomyopathy; 1,004 consecutive patients with hypertrophic cardiomyopathy and a recorded standard ECG (64% men, mean age 50 ± 16 years) were evaluated at 4 Italian centers. The study end points were sudden cardiac death (SCD) or surrogates, including appropriate implanted cardiac defibrillator discharge and resuscitated cardiac arrest and major cardiovascular events (including SCD or surrogates and death due to heart failure, cardioembolic stroke, or heart transplantation). Prevalence of baseline electrocardiographic characteristics was: normal ECG 4%, ST-segment depression 56%, pseudonecrosis waves 33%, "pseudo-ST-segment elevation myocardial infarction (STEMI)" pattern 17%, QRS duration ≥120 ms 17%, giant inverted T waves 6%, and low QRS voltages 3%.

Risk of Adverse Cardiac and Bleeding Events Following Cardiac and Noncardiac Surgery in Patients With Coronary Stent: How Important Is the Interplay Between Stent Type and Time From Stenting to Surgery?

Background: Epidemiology and consequences of surgery in patients with coronary stents are not clearly defined, as well as the impact of different stent types in relationship with timing of surgery.

Methods And Results: Among 39 362 patients with previous coronary stenting enrolled in a multicenter prospective registry and followed for 5 years, 13 128 patients underwent 17 226 surgical procedures. The cumulative incidence of surgery at 30 days, 6 months, 1 year, and 5 years was 3.

Repeated Aortic Balloon Valvuloplasty in Elderly Patients With Aortic Stenosis Who Are Not Candidates for Definitive Treatment.

Aims: A sizable group of patients with symptomatic aortic stenosis can undergo neither surgical aortic valve replacement nor transcatheter aortic valve implantation. The aim of this study was to assess the potential role of repeated balloon aortic valvuloplasty (BAV) in these patients.

Methods: Within our local prospective BAV registry, we retrospectively selected 105 patients who underwent ≥2 BAV procedures between 2005 and 2012 because of persisting contraindications to definitive treatment after first BAV.

[Gender effect on cardiomyopathy].

The role of a gender effect (that means differences in clinical manifestations, access to therapies and response to treatments according to gender) in cardiomyopathies remains a matter of debate. Although recent studies have evaluated the differences in the clinical features and prognosis between the two sexes, many issues remain to be elucidated. At present, the only sex-specific condition that affects females is peripartum cardiomyopathy.

Heart transplantation in infants with idiopathic hypertrophic cardiomyopathy.

Whereas it is well known that idiopathic HCM can present in newborns and infants, little information is available on HT in this very young age group. We report a series of 17 infants with idiopathic HCM, including two neonates with rapidly progressive severe HF for whom HT was necessary. When HF manifests in a newborn/infant with idiopathic HCM and extreme cavity size reduction, the possibility of a rapidly progressive clinical course should be anticipated and HT may become the only available therapeutic solution.