Reversal of systolic dysfunction in noncompaction and Wolff-Parkinson-White syndrome after accessory pathway ablation.

In a previously healthy asymptomatic 18-year old male, Wolff-Parkinson-White syndrome (WPW) with left ventricular hypertrabeculation/noncompaction (LVHT) and systolic dysfunction was detected. Holter monitoring disclosed multiple long episodes of supraventricular tachycardia with a heart rate of about 110/min. After radiofrequency ablation of an epicardial posteroseptal accessory pathway with ante- and retrograde conduction, systolic function gradually normalized without any pharmacotherapy.

Subcutaneous implanted cardioverter-defibrillator in ventricular noncompaction, coronary artery disease and stroke.

Left ventricular hypertrabeculation/noncompaction (LVHT) is associated with arrhythmias. Guidelines for prevention of sudden cardiac death by implanted cardioverter-defibrillator (ICD) also apply to LVHT-patients. Right ventricular perforation by the ICD-lead is a matter of concern.

Takotsubo as Initial Manifestation of Non-Myopathic Cardiomyopathy Due to the Titin Variant c.1489G > T.

Unlabelled: : Whether patients with subclinical cardiomyopathy (CMP) are more prone to experience Takotsubo syndrome (TTS) than patients without CMP, is unknown. We present a patient with TTS as the initial manifestation of a hitherto unrecognized genetic CMP. : case report.

Wolff-Parkinson-White syndrome and noncompaction in Leber's hereditary optic neuropathy due to the variant m.3460G>A.

This report describes a 66-year-old Caucasian male who acutely developed severe, bilateral impairment of visual acuity at 24 years of age. Leber's hereditary optic neuropathy (LHON) was suspected but the diagnosis was not genetically confirmed until the age of 49 years when the primary LHON mutation m.3460G>A was detected.

Coronary ectasia in amyloid cardiomyopathy and neuropathy due to the transthyretin mutation c.323A>G.

Background: atrial fibrillation(AF) is a frequent manifestation of cardiac involvement in genetic and wild-type transthyretin-related familial amyloidosis(TTR-FA). However, ectasia of coronary arteries and ablation for AF have not been reported in TTR-FA.

Methods And Results: A 65yo male developed progressive sensori-motor polyneuropathy since age 59y.

Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy.

Pre-excitation-syndrome has not been reported as a phenotypic feature of facio-scapulo-humeral muscular dystrophy (FSH-MD). In a 39-year-old male with FSH-MD due to a reduced tandem repeat size in the D4Z4-locus on chromosome 4q35, cardiac involvement, manifesting as an incomplete right bundle-branch-block, tall T-waves in V 3-5, ST-elevation in V 2-4, and mild thickening of the left ventricular myocardium, was first recognised 10 years earlier. Follow-up at age 39 years revealed mild myocardial thickening, two intra-ventricular aberrant bands, and, surprisingly, intermittent pre-excitation on a routine electrocardiography.

Repeated radiofrequency ablation of atrial tachycardia in restrictive cardiomyopathy secondary to myofibrillar myopathy.

Myofibrillar myopathy is characterized by nonhyaline and hyaline lesions due to mutations in nuclear genes encoding for extra-myofibrillar or myofibrillar proteins. Cardiac involvement in myofibrillar myopathy may be phenotypically expressed as dilated, hypertrophic, or restrictive cardiomyopathy. Radiofrequency ablation of atrial fibrillation and flutter has so far not been reported in myofibrillar myopathy.

Catheter ablation of multiple accessory pathways in duchenne muscular dystrophy.

A 23-year-old male with Duchenne muscular dystrophy (DMD) experienced self-limiting palpitations at age 19 years for the first time. Palpitations recurred not earlier than at age 23 years, and were attributed to narrow complex tachycardia, which could be terminated with adenosine. Since electrocardiography showed a delta-wave, Wolff-Parkinson-White (WPW) syndrome was diagnosed, ajmaline prescribed and radio-frequency catheter ablation of three accessory pathways carried out one week later.

Novel c.367_369del LMNA mutation manifesting as severe arrhythmias, dilated cardiomyopathy, and myopathy.

Objective: The 3-bp deletion in exon 2 of the Lamin A/C (LMNA) gene has not been described in association with dilated cardiomyopathy, which is characterized by progressive heart failure, atrioventricular (AV) block, tachyarrhythmias, and variable skeletal muscle involvement.

Case Report: In a 43-year-old woman with a long-term history of palpitations and newly diagnosed AV blocks I and II, ventricular ectopic beats, inducible nonsustained ventricular tachycardias (VTs), cardiac arrest, and successful resuscitation, an implantable cardioverter defibrillator was successfully implanted. Her family history was positive for sudden cardiac death (her father and sister), dyspnea and heart failure (her grandmother and sister), palpitations (her brother), and elevated levels of creatine-kinase (CK) (her sister).

Flecainide versus ibutilide for immediate cardioversion of atrial fibrillation of recent onset.

Aims: This study compared the efficacy and safety of intravenous flecainide and ibutilide for immediate cardioversion of atrial fibrillation (AF).

Methods And Results: We conducted a prospective, randomised trial, including 207 patients with AF of recent onset (< or = 48 h). Flecainide was given over 20 min at a dose of 2 mg/kg body weight (maximum 200 mg), ibutilide was infused at a dose of 1 mg (or 0.

Reflux esophagitis in the pathogenesis of paroxysmal atrial fibrillation: results of a pilot study.

Background: We sought to assess whether proton pump inhibitor (PPI) therapy of gastroesophageal reflux disease (GERD) in patients with lone paroxysmal atrial fibrillation (PAF) leads to a reduction of PAF-related symptoms.

Methods: The records of patients with reflux esophagitis were screened for the diagnosis of lone PAF. All patients with reflux esophagitis and lone PAF were invited for a follow-up visit, at which PAF- and GERD-related symptoms, medication, and electrocardiogram were recorded.

Oral anticoagulation for ECG tremor artefact simulating atrial fibrillation.

That tremor simulates atrial fibrillation and causes oral anticoagulation has not been reported. In a 69-year-old patient with diabetes, arterial hypertension and recurrent strokes, hand tremor developed since 1998. In September 2000 atrial fibrillation was diagnosed upon a routine and 24-hour ambulatory ECG.