Acute Myocarditis-Like Episode in a Curly-Haired Young Boy-Red Flags for Familial Arrhythmogenic Cardiomyopathy.

The present case report describes a mother and son with arrhythmogenic cardiomyopathy (ACM) with early and greater left ventricle (LV) involvement. The presence of curly hair in both, together with the resuscitated sudden cardiac death of the mother, allowed timely genetic testing, which found a pathogenic nonsense mutation of the desmoplakin gene. While asymptomatic from an arrhythmic point of view, the son's evolution was characterized by a well-documented exercise-induced myocarditis-like stage.

Flecainide is a safe and effective treatment for pre-excited atrial fibrillation rapidly conducted to the ventricle in pregnant women: a case series.

Background: Pregnancy is associated with an increased incidence of cardiac arrhythmias likely due to hormonal, haemodynamic, and autonomic changes. Yet, there is little data available regarding the efficacy and safety of anti-arrhythmic agents to prevent pre-excited atrial fibrillation (AF) in pregnant women.

Case Summary: We report on three pregnant women who developed AF rapidly conducted to the ventricle through an overt accessory pathway as the first manifestation of Wolff-Parkinson-White syndrome.

Usefulness of the genetic risk score to identify phenocopies in families with familial hypercholesterolemia?

Familial hypercholesterolemia (FH) is caused by mutations in LDLR (low-density lipoprotein receptor), APOB (apolipoprotein B), PCSK9 (proprotein convertase subtilisin/kexin type 9), or APOE (apolipoprotein E) genes in approximately 80% of the cases. Polygenic forms of hypercholesterolemia may be present among patients clinically diagnosed with FH but with no identified mutation (FH mutation-negative (FH/M-)). To address whether polygenic forms may explain phenocopies in FH families, we calculated a 6-single-nucleotide polymorphism (SNP) genetic risk score (GRS) in all members from five French FH families where a mutation was identified (FH/M+) as well as some phenocopies (FH/M-).

Delayed diagnosis of Brugada syndrome in a patient with aborted sudden cardiac death and initial negative flecainide challenge.

A negative flecainide challenge does not rule out Brugada syndrome even in the presence of nonfatal cardiac arrest as the first manifestation of the disease. This should prompt clinicians to ensure long-term ECG follow-up and consider repeating a drug test with another sodium channel blocker.

Additional coronary sinus shocking lead as rescue therapy after multiple internal and external defibrillation failures.

High defibrillation threshold (DFT) and defibrillation failure can lead to intractable ventricular arrhythmias. Additional coronary sinus coil is an effective strategy to achieve marked reduction in DFT. However, physicians should retain this might prevent future coronary sinus lead placement in case the patient would develop complete left bundle branch block.

The very high cardiovascular risk in heterozygous familial hypercholesterolemia: Analysis of 734 French patients.

Background: Heterozygous familial hypercholesterolemia (heFH) is a genetic disease causing high levels of low-density lipoprotein cholesterol (LDL-C). Although this population is at high cardiovascular (CV) risk, the risk is variable within patients depending on additional risk factors. CV disease risk groups have been defined by the Nouvelle Société Francophone d'Athérosclérose (NSFA) and by the National Lipid Association recommendations.

Improvement in LDL-cholesterol levels of patients with familial hypercholesterolemia: can we do better? Analysis of results obtained during the past two decades in 1669 French subjects.

Background: Heterozygous Familial Hypercholesterolemia (heFH) is an autosomal disease that affects about 1/500 people. It is characterized by markedly elevated plasma LDL-cholesterol (C) levels and an increased risk of cardiovascular disease (CVD). The aim of this study was to measure changes in LDL-C levels in heFH patients over two decades, and to evaluate if patients achieved LDL-C targets.

Persistent abnormal intraventricular conduction after myocarditis revealed by recurrent syncopes.

A 24-year-old male was admitted to our hospital for recurrent syncopes. Several ECG showed paroxysmal second degree atrioventricular block with the QRS axis changing between conducted beats and escape beats. Transvenous cardiac pacing was performed until permanent recapture of atrioventricular conduction (day 4).

False positive result of positron emission tomography in a patient with suspected defibrillator system infection.

The infection of an implanted pacemaker or defibrillator is often difficult to diagnose. Positron emission tomography-computerized tomography (PET-CT) has recently been shown to be of great interest in this difficult clinical setting. We report the case of a patient with suspected implantable cardioverter-defibrillator (ICD) infection.

Accelerated coronary atherosclerosis revealed by arrhythmogenic coronary spasm.

Coronary artery spasm is sometimes associated with life-threatening ventricular arrhythmias. Based on intravascular ultrasound findings, it appears that coronary artery spasm promotes negative arterial remodeling, suggesting that patients with coronary artery spasm might be at higher risk of accelerated coronary atherosclerosis. We report the cases of 3 patients with ventricular arrhythmia secondary to coronary artery spasm complicated by accelerated coronary atherosclerosis.

Malignant neoplasms following cardiac transplantation.

Objective: Malignancies have long been recognized as a complication of long lasting immunosuppressive therapy. We reviewed our experience to investigate the incidence and the spectrum of non cutaneous de novo malignant neoplasms.

Methods: Between March 1987 and March 1996, 296 patients underwent 303 cardiac transplantation in our service.