Abstract: We describe a 65-year-old man with unexplained syncope, Brugada ECG pattern and right ventricular abnormalities. To reach a diagnosis of Brugada syndrome, a variety of diseases have to be excluded. This case report shows how difficult the differential diagnosis is, particularly with arrhythmogenic right ventricular cardiomyopathy, after documentation of minimal structural RV abnormalities; invasive examination may be required. In this case, three-dimensional electroanatomical bipolar voltage mapping revealed a scar area in the right ventricle in the absence of clear-cut kinetic abnormalities, but the sensitivity of this method and specificity in patients without confirmed arrhythmogenic right ventricular cardiomyopathy have not been defined.
Abstract: We describe a case of a 30-year-old man with Danon's disease, an X-linked genetic disorder due to deficiency of lysosomal-associated membrane protein 2 with secondary intracytoplasmatic glycogen and autophagic material storage. This disease is characterized by skeletal muscle involvement, mental retardation, ophthalmic abnormalities, and cardiac disease. In this patient, cardiac involvement was characterized by hypertrophic cardiomyopathy in young age, preexcitation, and parossistic atrioventricular block. The patient underwent to an implantable cardioverter defibrillator implantation for conduction disorders and for primary prevention of sudden death, a frequent event in Danon's disease. This case report describes cardiac involvement with conduction disorders and multiple pacemaker malfunctions in Danon's disease.
Abstract: Since 1971, in Italy every citizen engaged in competitive sports activity must periodically undergo preventive medical examinations to ascertain eligibility for sports participation. The medical examination includes rest and effort ECG. The Italian model has a very good sensitivity in identifying most pathologies at risk of sudden death particularly in the young (hypertrophic cadiomyopathy, arrhythmogenic right ventricular disease, chanelopathies). In this model, the ECG is very important, being able to disclose cardiac anomalies in most cases. The application of the Italian screening model has progressively reduced the incidence of sudden death in athletes.
Abstract: Brugada syndrome is associated with a considerable risk of sudden death in young and otherwise healthy adults. The syndrome is estimated to be responsible for at least 4% of all sudden deaths and at least 20% of sudden deaths in patients with structurally normal hearts. The diagnosis of Brugada syndrome is based on peculiar electrocardiogram (ECG) abnormalities classified by the European Society of Cardiology in three types: type 1 (coved-type) is the diagnostic pattern; type 2 (saddle-back type); and type 3 are considered significant if there is a conversion to a type 1, spontaneously or during administration of class I A/C anti-arrhythmic drugs (flecainide, etc.). There is a general agreement about the high risk of sudden death in patients with previous cardiac arrest, for whom an implantable defibrillator (ICD) is recommended. In contrast, controversy exists on the correct clinical behaviour in individuals without a history of previous cardiac arrest. To stratify the risk in patients with type 1 pattern, three major factors have been suggested: typical ECG pattern in the basal state; a history of syncope; and inducible ventricular tachycardia/ventricular fibrillation during electrophysiological study (EPS). However, the indication and usefulness of an EPS is debatable. In patients with a type 2 or 3 pattern a pharmacological test is indicated in the presence of symptoms or of a familial history. With regard to sports eligibility, patients with a history of cardiac arrest should have an ICD and they can practise (low intensity) sport only after the implant of the device. Patients without documented cardiac arrest but at high risk (basal type 1 ECG pattern, syncope and/or positive EPS) should also have an ICD and they can practise (low intensity) sport only after the implant of the device. In patients at low risk (type 1 ECG pattern in the absence of symptoms, without family history and negative EPS) the behaviour regarding sport eligibility is not a matter of debate. In cases with type 2 or 3 pattern, in the absence of familial history and symptoms, a permissive behaviour should be assumed.
Abstract: OBJECTIVE: Both atrial flutter and atrial fibrillation (AF) frequently develop in the same patient. There is therefore reasonable doubt that flutter ablation may not solve the clinical problem, owing to the occurrence/recurrence of AF. The aim of this study was to establish whether cavo-tricuspid isthmus ablation is curative in patients with common atrial flutter alone or combined with AF. METHODS: One hundred and forty-one patients (114 male, 27 female, mean age 63 +/- 10 years) who had cavo-tricuspid isthmus ablation were followed up for 44 +/- 20 months. Before ablation, 48 patients had only atrial flutter (group A), whereas 93 patients had both atrial flutter and AF. Among the latter, during antiarrhythmic therapy, 31 patients had only atrial flutter (group B1), whereas 62 patients (group B2) continued to experience both arrhythmias. RESULTS: During follow-up, 27% of group A and 61% of group B patients had documented recurrent AF (P < 0.001). AF recurred in 51% of group B1 and in 66% of group B2 patients (P = NS). Permanent AF occurred in 6% of group A, 3% of group B1 and 21% of group B2 (P < 0.01). Specific symptom scale scores significantly decreased in all groups, particularly in group A. Two patients of group B had cerebral ischaemic attacks. CONCLUSIONS: Over a long-term follow-up, cavo-tricuspid isthmus ablation is curative in >70% of patients with atrial flutter alone. Therefore, if no AF is documented, more extensive ablation is not needed. By contrast, cavo-tricuspid isthmus ablation is frequently unable to prevent AF in patients with both atrial flutter and AF, although in some cases a significant clinical benefit may be obtained.
Abstract: INTRODUCTION: Slow pathway (SP) ablation of AV nodal reentrant tachycardia (AVNRT) can be complicated by second- to third-degree AV block. We assessed the usefulness of pace mapping of Koch's triangle in preventing this complication. METHODS AND RESULTS: Nine hundred nine consecutive patients undergoing radiofrequency ablation of AVNRT were analyzed. Group 1 (n=487) underwent conventional slow pathway ablation. Group 2 (n=422) underwent ablation guided by pace mapping of Koch's triangle, which located the anterogradely conducting fast pathway (AFP) based on the shortest St-H interval obtained by stimulating the anteroseptal, midseptal, and posteroseptal aspects of Koch's triangle. In group 2, AFP was anteroseptal in 384 (91%), midseptal in 33 (7.8%), and posteroseptal or absent in 5 (1.2%). In 32 of 33 patients with midseptal AFP, slow pathway ablation was performed strictly in the posteroseptal area. In 4 of 5 patients with posteroseptal or no AFP, retrograde fast pathway was ablated. Two patients refused ablation. Persistent second- to third-degree AV block was induced in 7 (1.4%) of 487 group 1 patients versus 0 (0%) of 422 group 2 patients (P=0.038). Ablation was successful in all patients in whom ablation was performed. CONCLUSION: Pace mapping of Koch's triangle identifies patients in whom the AFP is absent or is abnormally close to the slow pathway. In these cases, guiding ablation helps to avoid AV block.
Abstract: Pulmonary veins are a well-recognized source of focal ectopies that may trigger atrial fibrillation. Many ablative strategies, in particular ablation of the four pulmonary vein ostia, have been developed in order to cure atrial fibrillation. In some patients, the predominant arrhythmia may be an ectopic atrial tachycardia arising from a pulmonary vein and atrial fibrillation may be only a consequence of rapid atrial activation. There is a paucity of data regarding the electrocardiographic and electrophysiological characteristics of pulmonary vein tachycardia and the ablation strategy of this arrhythmia. In the present paper, we describe a case of a young woman with an arrhythmic focus localized in the right superior pulmonary vein with episodes of atrial tachycardia, paroxysmal atrial fibrillation and atrial flutter, who was successfully treated with transcatheter ablation.
