Riccardo Riccardi

Short QT Syndrome: a familial cause of sudden death.

Background—A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse—a short QT interval. Several members of 2 different families were referred for syncope, palpitations, and resuscitated cardiac arrest in the presence of a positive family history for sudden cardiac death. Autopsy did not reveal any structural heart disease. All patients had a constantly and uniformly short QT interval at ECG. Methods and Results—Six patients from both families were submitted to extensive noninvasive and invasive work-up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 patients underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural heart disease was excluded. At baseline ECG, all patients exhibited a QT interval ≤280 ms (QTc ≤300 ms). During electrophysiological study, short atrial and ventricular refractory periods were documented in all and increased ventricular vulnerability to fibrillation in 3 of 4 patients. Conclusions—The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation. It is important to recognize this ECG pattern because it is related to a high risk of sudden death in young, otherwise healthy subjects.

Linear Cryoablation of the Left Atrium Versus Pulmonary Vein Cryoisolation in Patients With Permanent Atrial Fibrillation and Valvular Heart Disease. Correlation of Electroanatomic Mapping and Long-Term Clinical Results

Background—The aim of this study was to clarify the role of pulmonary vein isolation (PVI) alone versus left atrial linear lesions in the treatment of permanent atrial fibrillation (AF) in patients with left atrial dilatation and valvular disease. The primary end point was to assess the persistence of sinus rhythm (SR) off antiarrhythmic drugs (AADs) at 2-year follow-up and to correlate clinical outcome with surgical results validated with electroanatomic mapping (EAM). Methods and Results—A total of 105 patients with permanent AF undergoing valve surgery were assigned to 3 different groups: in groups “U” and “7,” left atrial linear cryoablation was performed, whereas in group “PV” patients, anatomic cryoisolation of pulmonary veins only was performed. In groups U and 7, SR was achieved in 57% of patients, whereas it was achieved in 20% of PV patients during 2-year follow-up. In the first 51 patients, the ablation schemes were validated with EAM. The EAM showed that the U lesion was never obtained: in 59% of these patients, a complete 7 lesion was achieved instead; in the 7 group, a complete 7 lesion was present in 65% of patients, whereas a complete PVI was obtained in 71% of patients. Considering patients in whom a complete 7 lesion was demonstrated with the EAM, SR without AADs was achieved in 86% of patients, whereas only 25% of patients with complete PVI were in SR without AADs. Conclusions—In patients with permanent AF, left atrial dilatation and valvular heart disease linear lesions in the posterior region of the left atrium are more effective than PVI alone. With cryoablation, the surgical intent is fulfilled in only approximately 65% of the cases. Knowing the real anatomic and electrophysiological effects of surgical ablation is necessary to correctly interpret the clinical outcome.

Limited posterior left atrial cryoablation in patients with chronic atrial fibrillation undergoing valvular heart surgery

OBJECTIVES We sought to evaluate whether a limited surgical cryoablation of the posterior region of the left atrium was safe and effective in the cure of atrial fibrillation (AF) in patients with associated valvular heart disease. BACKGROUND Extensive surgical ablation of AF is a complex and risky procedure. The posterior region of the left atrium seems to be important in the initiation and maintenance of AF. METHODS In 32 patients with chronic AF who underwent heart valve surgery, linear cryolesions connecting the four pulmonary veins and the posterior mitral annulus were performed. Eighteen patients with AF who underwent valvular surgery but refused cryoablation were considered as the control group. RESULTS Sinus rhythm (SR) was restored in 25 (78%) of 32 patients immediately after the operation. The cryoablation procedure required 20 +/- 4 min. There were no intraoperative and perioperative complications. During the hospital period, one patient died of septicemia. Thirty-one patients reached a minimum of nine months of follow-up. Two deaths occurred but were unrelated to the procedure. Twenty (69%) of 29 patients remained in SR with cryoablation alone, and 26 (90%) of 29 patients with cryoablation, drugs and radiofrequency ablation. Three (10%) of 29 patients remained in chronic AF. Right and left atrial contractility was evident in 24 (92%) of 26 patients in SR. In control group, two deaths occurred, and SR was present in only four (25%) of 16 patients. CONCLUSIONS Linear cryoablation with lesions connecting the four pulmonary veins and the mitral annulus is effective in restoration and maintenance of SR in patients with heart valve disease and chronic AF. Limited left atrial cryoablation may represent a valid alternative to the maze procedure, reducing myocardial ischemic time and risk of bleeding.

Catheter ablation of permanent junctional reciprocating tachycardia with radiofrequency current

OBJECTIVES This study evaluated accessory pathway location, its relation to retrograde P wave polarity on the surface electrocardiogram and radiofrequency ablation efficacy and safety in a large group of patients with permanent junctional reciprocating tachycardia. BACKGROUND Permanent junctional reciprocating tachycardia is an uncommon form of reciprocating tachycardia, almost incessant from infancy and usually refractory to drug therapy. It is characterized by RP > PR interval and usually by negative P waves in leads II, III, aVF and V4 to V6. Retrograde conduction occurs through an accessory pathway with slow and decremental properties. Although this accessory pathway has been classically located in the posteroseptal zone, other locations have been recently reported. METHODS The study included 32 patients (20 men, 12 women, mean [+/- SD] age 29 +/- 15 years) with a diagnosis of permanent junctional reciprocating tachycardia confirmed at electrophysiologic study. Seven patients had depressed left ventricular function. Radiofrequency energy was applied at the site of the earliest retrograde atrial activation during tachycardia. RESULTS There were 33 accessory pathways. The site of the earliest retrograde atrial activation was posteroseptal in 25 patients (76%), midseptal in 4 (12%), right posterior in 1 (3%), right lateral in 1 (3%), left posterior in 1 (3%) and left lateral in 1 (3%). Thirty pathways were ablated with a right approach; in 11 patients with posteroseptal pathway the ablation was performed through the coronary sinus. Three pathways were ablated with a left approach. Positive retrograde P wave in lead I suggested that ablation could be performed from the right side; if negative, it did not exclude ablation from this approach. All the accessory pathways were successfully ablated, with a median of 3 and a mean of 5.6 +/- 5 radiofrequency applications of 70 +/- 26 s in duration. In two patients with the accessory pathway in the midseptal zone, a transient second- and third-degree atrioventricular block, respectively, was observed after ablation. At a mean follow-up of 18 +/- 12 months, 31 patients (97%) are asymptomatic without antiarrhythmic therapy (95% confidence interval [CI] 84% to 99%). Recurrences were observed in four patients (13%) (95% CI 4% to 29%), three of whom had the accessory pathway ablated successfully at a second session. All patients with depressed left ventricular function showed a marked improvement after successful ablation. CONCLUSIONS In our experience, most of the patients with permanent junctional reciprocating tachycardia had posteroseptal pathways; all these pathways were ablated from the right side. P wave configuration may be helpful in suggesting the approach to the site of ablation. Catheter ablation using radiofrequency energy is an effective therapy for permanent junctional reciprocating tachycardia.

Risk stratification of the patients with Brugada type electrocardiogram: a community-based prospective study

AIMS Risk stratification of patients with Brugada electrocardiogram (ECG) is being strongly debated. Conflicting results have been suggested from international registries, which enrolled non-consecutive cases, studied with different programmed electrical stimulation (PES) protocols. The aim of this study was to prospectively evaluate the incidence of arrhythmic events and the prognostic role of clinical presentation, ECG, and of a standardized PES protocol in consecutive cases from a community-based population. METHODS AND RESULTS A total of 166 consecutive patients (45 +/- 14 years) with Brugada ECG were enrolled. Type 1 ECG was observed spontaneously in 72 (43%) and after pharmacological testing in 94 (57%). One hundred and three (62%) were asymptomatic, 58 (35%) had syncope, and five (3%) had a prior cardiac arrest. One hundred and thirty-five (81%) underwent PES with two extra stimuli up to ventricular refractoriness and 34% had ventricular fibrillation (VF) induced. Arrhythmic events occurred in nine patients at a mean follow-up of 30 +/- 21 months (2.2 events per 100 person-year): in three (60%) patients with aborted sudden death (aSD), five (8.6%) of those with syncope, and one (1%) of the asymptomatic. The only predictors of events were a history of syncope or aSD (P = 0.02) and induction at PES (P = 0.004). CONCLUSION Clinical presentation is the most important parameter in the risk stratification of patients with Brugada ECG. Programmed electrical stimulation seems valuable, particularly in patients with previous syncope.

Different patterns of atrial activation in idiopathic atrial fibrillation: simultaneous multisite atrial mapping in patients with paroxysmal and chronic atrial fibrillation

OBJECTIVES We aimed to evaluate: 1) the behavior of electrical activity simultaneously in different atrial regions during atrial fibrillation (AF); 2) the difference of atrial activation between paroxysmal and chronic AF; 3) the atrial refractoriness dispersion; and 4) the correlation between the effective refractory periods (ERPs) and the FF intervals. BACKGROUND Little data exist on the electrophysiologic characteristics of the different atrial regions in patients with AF. A more detailed knowledge of the electrical activity during AF may provide further insights to improve treatment of AF. METHODS Right and left atria were extensively mapped in 30 patients with idiopathic AF (18 paroxysmal and 12 chronic). In different atrial locations, we analyzed 1) the FF interval duration; and 2) the grade of organization and, in case of organized electrical activity, the direction of atrial activation. Furthermore, in patients with paroxysmal AF, we determined the atrial ERP, evaluated the ERP dispersion and assessed the presence of a correlation between the ERPs and the FF intervals. RESULTS In patients with chronic AF, we observed a shortening of the FF intervals and a greater prevalence of disorganized activity in all the atrial sites examined. In patients with paroxysmal AF, a significant dispersion of refractoriness was observed. The right lateral wall showed longer FF intervals and more organized atrial activity and, unexpectedly, the shortest mean ERPs. In contrast, the septal area showed shorter FF intervals, greater disorganization and the longest mean ERPs. CONCLUSIONS Electrical activity during AF showed a significant spatial inhomogeneity, which was more evident in patients with paroxysmal AF. The mean FF intervals did not correlate with the mean ERPs.

Stress and pharmacologic tests as methods to identify patients with Wolff-Parkinson-White syndrome at risk of sudden death

Noninvasive stress and pharmacologic tests with procainamide and propafenone were studied as methods to identify patients with Wolff-Parkinson-White syndrome (WPW) who would otherwise be judged at risk of sudden death on the basis of electrophysiologic criteria: the shortest RR interval during induced atrial fibrillation less than or equal to 250 ms or accessory pathway anterograde effective refractory period less than or equal to 250 ms. Sixty-five patients were studied. Twenty-four patients fulfilled the electrophysiologic risk criteria (group A) and 41 patients fulfilled none of these criteria (group B). Persistence of preexcitation during stress test showed a sensitivity of 96% and a specificity of 17% to identify group A patients; its positive predictive value was 40% and negative predictive value 88%. With both procainamide and propafenone tests persistence of preexcitation identified group A patients with a sensitivity of 96% and a specificity of 51%; their positive and negative predictive value were, respectively, 53 and 95%. Stress and pharmacologic tests have good sensitivity and negative predictive value, but low specificity and positive predictive value.

Cryoenergy Catheter Ablation: A New Technique for Treatment of Permanent Junctional Reciprocating Tachycardia in Children

Introduction: Permanent junctional reciprocating tachycardia (PJRT) is an infrequent form of reciprocating tachycardia, almost incessant from childhood and usually refractory to drug therapy. Radiofrequency catheter ablation currently is the first‐line therapy for PJRT, but its application in the septal region may be associated with complications. In contrast, cryoenergy has several advantages, such as the ability to test the effects of ablation while the lesion is still forming, thus reducing the number of ineffective, useless, and potentially harmful lesions. The aim of this study was to investigate the potential clinical utility of percutaneous cryoenergy catheter ablation for treatment of pediatric patients with PJRT.

Cryothermic Ablation Within the Coronary Sinus of an Epicardial Posterolateral Pathway

Cryoablation Within the Coronary Sinus. Radiofrequency ablation inside the coronary sinus (CS) is associated with the risk of vein stenosis, or perforation or damage to the circumflex artery. Cryothermic ablation has proved less harmful, but there are no data on its use within a venous structure. A patient with a posterolateral accessory pathway underwent several unsuccessful attempts at endocardial ablation. Ablation then was performed with cool energy within the CS, where the shortest VA interval and a possible Kent potential were recorded. Selective left coronary angiography showed a normal circumflex artery and the absence of lesions of the CS. Six months later, the patient was asymptomatic without taking antiarrhythmic drugs. We conclude that cryoablation within the CS may be effective and safe.

Interruption of the Inferior Extension of the Compact Atrioventricular Node Underlies Successful Radio Frequency Ablation of Atrioventricular Nodal Reentrant Tachycardia

A recent anatomic study has revived interest in the inferior extensions of the compact atrioventricular node in humans. The rightward extension is on the right atrial aspect, close to the septal attachment of the tricuspid valve leaflet and, hence, closely related to the anticipated slow pathway considered to play a role in atrioventricular nodal reentrant tachycardia (AVNRT). This report documents a patient, 65 years of age, with dilated cardiomyopathy and AVNRT. The tachycardia was successfully terminated using selective radiofrequency (RF) ablation, delivered at a site where a slow potential was recorded and validated by atrial pacing, located between the tricuspid valve and the os of the coronary sinus (CS), close to its superior rim. In subsequent years the patient developed progressive heart failure and eventually died. Histopathologic examination revealed extensive scar tissue at the site of the burn, extending onto the crest of the underlying ventricular septum. Serial sections revealed the compact AV node superiorly and an inferior extension surfacing from the scar which could be traced inferiorly beyond the os of the CS. This is the first documentation of RF ablation interrupting an inferior extension of the compact AV node in a patient successfully ablated for AVNRT. The observation suggests that the slow pathway in this patient found its anatomic substrate in the inferior extension of the compact AV node.