Impact of resident training on cardiac electrophysiological procedures.

Background: Modern management of cardiac arrhythmias often requires interventions in which young physicians must acquire a high level of expertise. However, concerns have been raised about the increase in side effects during procedures performed with resident involvement.

Aim: This study aims to identify the effects of resident training on cardiac electrophysiological procedures within a university centre.

Cause of death analysis and temporal trends in survival after liver transplantation for transthyretin familial amyloid polyneuropathy.

Background: Hereditary transthyretin amyloidosis (ATTR) is a multisystemic disease involving mainly the peripheral nervous system and the heart. Liver transplantation (LT) is the reference treatment for ATTR neuropathy and preoperative detection of high risk patients is crucial. We aimed to document the causes of death of ATTR patients after LT, their temporal trends, and to evaluate whether the available preoperative tools that predict the risk of death after LT for hereditary ATTR amyloidosis matched with these trends.

Cardiac Dysautonomia Predicts Long-Term Survival in Hereditary Transthyretin Amyloidosis After Liver Transplantation.

Objectives: This study sought to compare techniques evaluating cardiac dysautonomia and predicting the risk of death of patients with hereditary transthyretin amyloidosis (mATTR) after liver transplantation (LT).

Background: mATTR is a multisystemic disease involving mainly the heart and the peripheral nervous system. LT is the reference treatment, and pre-operative detection of high-risk patients is critical.

Right ventricular failure secondary to chronic overload in congenital heart diseases: benefits of cell therapy using human embryonic stem cell-derived cardiac progenitors.

Objective: Despite the increasing incidence of right ventricular (RV) failure in adult patients with congenital heart disease, current therapeutic options are still limited. By contrast to left-heart diseases, cell-based myocardial regeneration applied to the right ventricle is poorly studied, even though it may be a therapeutic solution. As human embryonic stem cell-derived cardiac progenitors seem to be good candidates owing to their proliferation capacity, our aim was to assess, in a large animal model of overloaded RV dysfunction, the feasibility and effects of such a cell therapy.

Circadian rhythm of blood pressure reflects the severity of cardiac impairment in familial amyloid polyneuropathy.

Background: Cardiac amyloidosis due to familial amyloid polyneuropathy (FAP) includes restrictive cardiomyopathy, thickened cardiac walls, conduction disorders and cardiac denervation. Impaired blood pressure variability has been documented in FAP related to the Val30Met mutation.

Aims: To document blood pressure variability in FAP patients with various mutation types and its relationship to the severity of cardiac involvement.

Prophylactic pacemaker implantation in familial amyloid polyneuropathy.

Background: Familial amyloid polyneuropathy (FAP) is an autosomic dominant disease with a high rate of conduction disorders and increased risk of sudden death. Prophylactic cardiac pacing may be considered in asymptomatic patients with FAP. However, the potential benefits are unknown.

Early detection of right ventricular functional abnormalities in patients with complex right premature ventricular contractions.

Background: Right ventricular (RV) premature contractions, although generally benign, may represent the first manifestation of arrhythmogenic RV cardiomyopathy. The diagnostic and prognostic value of RV functional abnormalities evidenced by equilibrium radionuclide angiocardiography (ERNA) with multiharmonic Fourier analysis has been validated in patients with severe RV arrhythmias suspected of being affected by arrhythmogenic RV cardiomyopathy. The aim of this study was to assess the prevalence of the same RV functional abnormalities in patients with frequent left bundle branch block pattern premature ventricular contractions (PVCs), without known heart disease, using ERNA as a screening tool.

Downregulation of the calcium current in human right atrial myocytes from patients in sinus rhythm but with a high risk of atrial fibrillation.

Aims: A decrease in L-type calcium current (ICaL) is an important mechanism favouring atrial fibrillation (AF). Here, we aimed to identify pathogenic factors associated with ICaL downregulation.

Methods And Results: Atrial myocytes were isolated from right atrial appendages obtained from 86 adult patients in sinus rhythm with coronary artery disease, aortic valve disease, or mitral valve disease (MVD).

Impact of liver transplantation on cardiac autonomic denervation in familial amyloid polyneuropathy.

Familial amyloid polyneuropathy (FAP) is a rare and severe hereditary form of amyloidosis, due to the deposition of a genetic variant transthyretin essentially produced by the liver, and characterized by both sensorimotor and autonomic neuropathy. Liver transplantation (LT) is the most effective treatment to stop the progression of the disease. Cardiac amyloid infiltration is usually associated with cardiac denervation, restrictive cardiomyopathy, conduction disturbances, and sometimes sudden death.

Moderate and chronic hemodynamic overload of sheep atria induces reversible cellular electrophysiologic abnormalities and atrial vulnerability.

Objectives: The aim of this study was to evaluate the myocardial consequences of a chronic volume overload of the left atrium (LA).

Background: Atrial dilation is a major risk factor for atrial fibrillation (AF), but the underlying mechanisms are poorly understood.

Methods: A left-right aorto-pulmonary artery shunt (APS) was created in sheep.