Extracardiac minimal invasive implantation of cardioverter defibrillator in young children.

Implantable cardioverter defibrillator (ICD) placement in young children remains a challenge due to device-patient size mismatch and the important choice between an endovenous or an epicardial approach for lead implantation. We treated three children, with respectively Long QT-syndrome, Brugada syndrome and Brugada syndrome with sick sinus syndrome, ranging from 9 months to 7 years with a subxyphoidal ICD and extracardiac lead implantation by minimally invasive techniques. In all cases the thresholds were excellent. The devices could be properly placed in the preperitoneal space without discomfort to the patients. The clinical course was uneventful and results were excellent.

Brugada syndrome: the prognostic dilemma and value of sincope.

Since its first description in 1992 as a new clinical entity, the Brugada syndrome has stimulated great interest among physicians and basic scientists. In 2002 and 2005, two consensus conferences have respectively defined the diagnostic criteria for the syndrome. Currently the diagnosis of Brugada syndrome is based on a combination of clinical events (syncope and/or sudden cardiac death due to malignant ventricular arrhythmias) and electrocardiographic features (pathognomonic ST-segment elevation morphology). In the last years, many advances have been done in the knowledge about the genetic basis, the cellular mechanisms responsible for the typical electrocardiography features, susceptibility to ventricular arrhythmias and risk stratification. The implantable cardioverter defibrillator remains the only therapeutic option of proven efficacy for primary and secondary prophylaxis of sudden cardiac death. Identification of high risk subjects is one of the major goals in clinical decision-making. Syncope is ubiquitously recognized as a bad prognostic marker in Brugada syndrome. However, young individuals with this disease may suffer from vaso-vagal instead of arrhythmic syncope. The prognostic significance of syncope in patients with Brugada syndrome is discussed in this review.

The Brugada syndrome: update 2006.

Over the last 14 years - since the Brugada syndrome was first recognized as a distinct clinical/electrocardiographical entity - a considerable number of papers have been published on its various aspects. It has been defined as the combination of a typical ST-segment elevation in the right precordial leads and a predisposition for malignant ventricular arrhythmias occurring in the absence of structural heart disease. From the outset, controversy arose about the diagnostic criteria to be applied. This issue has been clarified since the announcement of a first (2002) and second (2005) consensus report. Our review will discuss the clinical characteristics and different possible pathophysiological mechanisms underlying the specific ECG-abnormalities and susceptibility for malignant arrhythmias. Nowadays, the main issue of discussion revolves essentially around its prognostic features, especially in asymptomatic patients. This review will compare the results of different follow-up studies and yields a possible explanation for the differences in event rates and in the identification of useful sudden-death predictors. Finally, the most recent data concerning new diagnostic techniques, gene identification and future therapeutic options will also be discussed.

[ST segment elevation, right bundle branch block and sudden death: Brugada's syndrome]. / Elevación del ST, bloqueo de rama derecha y muerte súbita: síndrome de Brugada.

Brugada's syndrome is one of the main causes of sudden death in young adults without a structural heart disease. This is an electrical cardiac illness secondary to a mutation of SCN5A gene of chromosome 3 that has a dominant autosomic transmission pattern. This mutation implies the dysfunction of the sodium channel that increases the Ito, loosing the dome of the epicardiac action potential phase two. An "all or none" repolarization pattern ensues and gives rise to a phase two reentry. This kind of reentry is responsible for the initiation and perpetuation of malignant ventricular arrhythmias among these patients. The clinical characteristics of the syndrome are the right bundle branch block, ST segment elevation from V1 to V3 leads and sudden death or syncope. In some patients, a pharmacological test must be done with ajmaline or procainamide to unmask the electrocardiographic changes. At present, the only effective treatment is the implantable cardioverter defibrillator (ICD). This device has the capability to reduce mortality from 40% annually to 0% at ten years. Pharmacological treatment is not useful.

[Sudden death (VI). The Brugada syndrome and right myocardiopathies as a cause of sudden death. The differences and similarities]. / Muerte súbita (VI). El síndrome de Brugada y las miocardiopatías derechas como causa de muerte súbita. Diferencias y similitudes.

In 1992 we described a new syndrome characterized by syncopal or sudden death episodes in patients with a structurally normal heart and a characteristic electrocardiogram 9 showing a pattern of right bundle branch block and ST segment elevation in right precordial leads V1 to V3. The disease is genetically determined with and autosomic dominant pattern of transmission. Until now three mutations and one polymorphism in the sodium cardiac channel gene have been identified in two families and one sporadic patient. As in many other genetically determined diseases, the disease is heterogeneous, caused by more than one gene. The syndrome has been identified in almost all countries in the world. Its incidence is difficult to evaluate, but it seems to be responsible for 4 to 10 sudden deaths per year per 10,000 inhabitants in areas like Laos or Thailand, and it represents the most frequent cause of death in young male adults in these countries. Up to 50% of all sudden deaths in patients with structurally normal heart are caused by the disease. The diagnosis can be easily made thanks to the characteristic electrocardiographic pattern. In some patients, the presence of concealed and intermittent forms might make the diagnosis more difficult. The electrocardiogram can be modulated by autonomic changes and administration of antiarrhythmic drugs. Beta-adrenergic stimulation normalizes the electrocardiogram, whereas ajmaline, flecainide or procainamide administration increase ST segment elevation. These drugs allow the unmasking of concealed or intermittent forms of the disease. Prognosis of patients with the syndrome is poor without an implantable defibrillator and antiarrhythmic drugs like amiodarone or betablockers do not protect against sudden death. The poor prognosis is similar in patients with a history of aborted sudden death or syncope and in asymptomatic patients in whom the abnormal electrocardiogram characteristic of the syndrome, was identified during a routine examination.

Pharmacological and device approach to therapy of inherited cardiac diseases associated with cardiac arrhythmias and sudden death.

A genetic origin in diseases like the long QT syndrome, the Brugada syndrome, or hypertrophic cardiomyopathy have been identified over the past years. These diseases have in common that they may result in sudden cardiac death of the patient. Recognition of patients based on their phenotype and application in clinical practice of the knowledge acquired on the genetic basis may have a major impact on how we approach them. In the long QT syndrome several mutations have been identified both in the sodium and in the potassium channels. The different electrophysiological effects of the mutations lead to a common phenotype: prolongation of the QT interval; but also to a common clinical impact: occurrence of malignant ventricular arrhythmias. Genetics should help us in treating in a more rational way our patients depending on the type of mutation. In the Brugada syndrome, mutations affecting the sodium channel have been so far identified. The results are electrophysiologically opposite to the ones observed in the long QT syndrome. Thus different mutations in the same gene lead to different functional consequences. Again, identification and study of the right mutation may lead to a more rational treatment directed to correct the malfunction of the channel.

Coronary artery bypass grafting and defibrillator implantation in patients with ventricular tachyarrhythmias and ischemic heart disease.

In patients with sustained ventricular tachyarrhythmias and myocardial ischemia due to multivessel coronary artery disease, it remains unclear whether revascularization is enough to control the arrhythmias or whether additional implantation of a defibrillator is indicated. We therefore reviewed our clinical strategy of performing both bypass surgery and implantation of a defibrillator in patients with syncopal ventricular tachycardia or fibrillation and significant multivessel coronary artery disease. We retrospectively reviewed the outcome of 18 patients with malignant ventricular tachyarrhythmias, significant multivessel coronary artery disease, and signs of myocardial ischemia who underwent both bypass surgery and defibrillator implantation. Data on these patients were compared to data from 232 other defibrillator patients with respect to baseline clinical variables, cardiac events, and mortality during follow-up. Except for underlying pathology, no other important differences in baseline characteristics were noted between the study patients and the other defibrillator patients. The cumulative occurrence of shocks during follow-up was comparable in both groups (66% vs 67%). The cumulative survival from all-cause mortality was 94% in the study patients and 78% in the others (P = NS). Pre- and postoperative electrophysiological testing was not useful to predict arrhythmia recurrences. In this population of patients with ventricular tachyarrhythmias and ischemia due to multivessel coronary artery disease, bypass surgery alone would not have prevented recurrences of arrhythmias. An excellent survival and a high incidence of shocks after both bypass surgery and defibrillator implantation were observed.

Long-term follow-up in patients with the permanent form of junctional reciprocating tachycardia treated with radiofrequency ablation.

This study sought to determine the long-term follow-up, safety, and efficacy of radiofrequency catheter ablation of patients with the permanent form of junctional reciprocating tachycardia (PJRT). We assessed the reversibility of tachycardia induced LV dysfunction and we detailed the location and electrophysiological characteristics of these retrograde atrioventricular decremental pathways. PJRT is an infrequent form of reciprocating tachycardia, commonly incessant, and usually drug refractory. The ECG hallmarks include an RP interval > PR with inverted P waves in leads II, III, a VF, and V3-V6. During tachycardia, retrograde VA conduction occurs over an accessory pathway with slow and decremental conduction properties, located predominantly in the posteroseptal zone. It is known that long-lasting and incessant tachycardia may result in tachycardia induced severe ventricular dysfunction. We included 36 patients (13 men, 23 women, mean +/- SD, aged 44 +/- 22 years) with the diagnosis of PJRT. Seven patients had tachycardia induced left ventricular dysfunction. Radiofrequency energy was delivered at the site of earliest retrograde atrial activation during ventricular pacing or during reciprocating tachycardia. All patients were followed at the outpatient clinic and serial echocardiograms were performed in those who presented with depressed LV function. Radiofrequency ablation was performed in 36 decremental accessory pathways. Earliest retrograde atrial activation was right posteroseptal in 32 patients (88%), right mid-septal in 2 (6%), right posterolateral in 1 (3%), and left anterolateral in 1 (3%). Thirty-five accessory pathways were successfully ablated with a mean of 5 +/- 3 applications. A mid-septal accessory pathway could not be ablated. After a mean follow-up of 21 +/- 16 months (range 1-64) 34 patients are asymptomatic. There were recurrences in 8 patients after the initial successful ablation (mean of 1.2 months), 5 were ablated in a second ablation procedure, 2 patients required a third procedure, and 1 patient required four ablation sessions. All patients with LV dysfunction experienced a remarkable improvement after ablation. Mean preablation LV ejection fraction in patients with tachycardiomyopathy was 28% +/- 6% and rose to 51% +/- 16% after ablation (P < 0.02). Our study supports the concept that radiofrequency catheter ablation is a safe and effective treatment for patients with PJRT. Radiofrequency ablation should be the treatment of choice in these patients because this arrhythmia is usually drug refractory. The majority of accessory pathways are located in the posteroseptal zone. Cessation of the arrhythmia after successful ablation results in recovery of LV dysfunction.

Early benefit of implantable cardioverter defibrillator therapy in patients waiting for cardiac transplantation.

The ICD can effectively recognize and treat ventricular arrhythmias that can lead to sudden death. Sudden death is a major problem in patients awaiting heart transplantation. We reviewed our experience with the ICD in patients with malignant ventricular arrhythmias waiting for cardiac transplantation. Nineteen patients were included. Seventeen were men, mean age was 54 +/- 11 years (range 17-66) and the left ventricular ejection fraction was 22% +/- 10% (range 9%-46%). After a mean follow-up of 6 +/- 5 months (range 1-20 months), 17 patients reached heart transplantation. One patient died and the other is waiting for a transplant. Before transplantation 71% of patients received an appropriate discharge. The mean time to the first appropriate discharge was 2 +/- 2 months (range < 1-6 months), which was significantly shorter than the mean time to first discharge in the other patients (n = 182) receiving a defibrillator in our center (11 +/- 10 months; range 1-58 months) (P < 0.0004). In conclusion, cardiac transplantation candidates with life-threatening ventricular arrhythmias can effectively be protected against sudden arrhythmic death by ICD. These patients have a high incidence of appropriate shocks occurring very early after implantation.

[Hemodynamic deterioration in patients submitted to ablation of the atrioventricular node]. / Deterioro hemodinámico en pacientes sometidos a ablación del nodo auriculoventricular.

INTRODUCTION: Radiofrequency ablation of the atrioventricular conduction system has become an established therapy for patients with drug-refractory atrial fibrillation. We observed 14 patients with hemodynamic deterioration related to worsening of mitral regurgitation after the procedure. PATIENTS AND METHODS: We retrospectively evaluated 256 consecutive patients with drug-refractory atrial fibrillation referred for radiofrequency ablation of the AV node and implantation of a pacemaker. Because we found hemodynamic deterioration related to worsening mitral regurgitation, we compared the clinical history, electrophysiologic and echocardiographic data from the patients with hemodynamic deterioration and worsening mitral regurgitation (group A) with those without hemodynamic deterioration (group B). RESULTS: Fourteen out of 256 patients (group A) undergoing ablation of the atrioventricular conduction system deteriorated with acute pulmonary edema (3 patients) or congestive heart failure (11 patients) at a mean of 6 weeks after the ablation procedure. Four of these patients were referred for mitral valve surgery. The length of the procedure and the number of applications during ablation were similar in both groups. Compared with group B patients, group A patients had significantly higher left ventricular end-diastolic diameters (64 +/- 6 mm vs 56 +/- 9 mm; p < 0.05) at baseline despite similar left ventricular end-systolic diameters, fractional shortening and grade of mitral regurgitation (1.15 +/- 1.05 vs 1.11 +/- 0.97). Moreover, whereas no change was observed in left ventricular end-diastolic diameter, left ventricular end-systolic diameter, fractional shortening and grade of mitral regurgitation in group B patients after ablation, group A patients experienced a significant increase in left ventricular end-diastolic diameter (64 +/- 6 mm vs 72 +/- 9 mm; p < 0.01) and grade of mitral regurgitation (1.15 +/- 1.05 vs 2.90 +/- 1.15; p < 0.01). In patients operated on no ablation related structural damage to the mitral valve apparatus could be detected. The worsening of the mitral regurgitation was related to dilation of the mitral valve annulus. CONCLUSIONS: Hemodynamic deterioration together with progression of mitral regurgitation is a potential complication of ablation of the atrioventricular conduction system.

Different humoral responses during head-up tilt testing among patients with neurocardiogenic syncope.

Neurocardiogenic dysfunction is believed to result from activation of ventricular mechanoreceptors. To asses other humoral and circulatory mechanisms activated during vasovagal syncope, epinephrine, norepinephrine, renin, and aldosterone levels were measured during head-up tilt testing. Twenty-three patients referred because of vasovagal syncope underwent passive head-up tilt testing (80 degrees). Blood samples were taken at baseline, after 30 minutes of supine rest and at syncope. Five patients (four men, one woman; mean age 46 +/- 27 years) had cardioinhibitory syncope. Seven patients (five men, two women; mean age 40 +/- 12 years) had vasodepressor syncope. Eleven patients (eight men, three women; mean age 55 +/- 21 years) had negative results of head-up tilt tests. Among patients with cardioinhibitory syncope, norepinephrine concentration rose significantly from baseline to syncope (0.44 +/- 0.12 ng/ml versus 1.14 +/- 0.72 ng/ml; p < 0.05), whereas no significant change was observed in epinephrine (0.08 +/- 0.03 ng/ml versus 2.74 +/- 2.85 ng/ml; p = not significant [NS]), renin (5.68 +/- 3.03 pg/ml versus 19.58 +/- 11.47 pg/ml; p = NS), or aldosterone concentration (66.60 +/- 16.10 ng/ml versus 109.00 +/- 44.70 ng/ml; p = NS). Patients with vasodepressor syncope had a significant rise in renin (9.03 +/- 4.56 pg/ml versus 52.53 +/- 41.63 pg/ml; p < 0.05) and aldosterone concentration (95.43 +/- 103.03 ng/ml versus 249.57 +/- 191.54 ng/ml; p < 0.05), whereas no change in level of epinephrine (0.12 +/- 0.12 ng/ml versus 0.28 +/- 0.33 ng/ml; p = NS) or norepinephrine (0.60 +/- 0.26 ng/ml versus 0.86 +/- 0.53 ng/ml; p = NS) was detected. Among patients with negative results of tilt tests, levels of renin (7.94 +/- 7.19 pg/ml versus 27.71 +/- 18.50 pg/ml; p < 0.01) and aldosterone (64.64 +/- 28.33 ng/ml versus 160.91 +/- 79.58 ng/ml; p < 0.01) rose significantly, whereas no change was seen in epinephrine (0.12 +/- 0.14 ng/ml versus 0.23 +/- 0.31; p = NS) or norepinephrine concentration (0.54 +/- 0.21 ng/ml versus 0.82 +/- 0.52; p = NS). Patients with cardioinhibitory syncope were characterized by a rise in norepinephrine level and blunted activation of the renin-angiotensin-aldosterone axis at syncope. Unlike patients with cardioinhibitory syncope, the renin-angiotensin-aldosterone axis is activated in patients with vasodepressor syncope and patients with a negative result of head-up tilt test without a statistically significant increase in catecholamine levels. Patients with cardioinhibitory syncope have higher epinephrine levels at syncope compared with patients with a negative result of head-up tilt test and patients with vasodepressor syncope.

Hemodynamic deterioration following radiofrequency ablation of the atrioventricular conduction system.

Radiofrequency ablation of the atrioventricular conduction system (ACS) has become an established therapy for patients with drug refractory atrial fibrillation. We observed eight patients with hemodynamic deterioration after radiofrequency ablation of the atrioventricular conduction system. As we found hemodynamic deterioration related to worsening mitral regurgitation, we compared the clinical history, electrophysiological, and echocardiographic data from the patients with hemodynamic deterioration and worsening mitral regurgitation (group 1) to those without hemodynamic deterioration and stable mitral regurgitation after the procedure (group 2). Eight out of 108 patients (7.4%) undergoing ablation of the ACS deteriorated hemodynamically with acute pulmonary edema in three and congestive heart failure in five patients occurring at a mean of 3 and 8 weeks, respectively, after the procedure. Three of these patients were referred for mitral valve surgery. Two patients underwent ablation using a left-sided approach. A right-sided approach was used in five patients. In one patient, a left- and right-sided approach was used. Compared to group 2 patients, group 1 patients had significantly higher left ventricular end-diastolic diameters (64 +/- 6 mm vs 56 +/- 9 mm) at baseline despite similar fractional shortening (32% +/- 11% vs 34% +/- 13%), left ventricular end-systolic diameters (43 +/- 9 mm vs 36 +/- 7 mm) and degree of mitral regurgitation (1.4 +/- 1.1 vs 1.4 +/- 0.7) on echocardiographic analysis. Thus, hemodynamic deterioration together with progression of mitral regurgitation is a potential complication of ablation of the ACS (up to 7.4%). Patients with high left ventricular end-diastolic diameters and moderate mitral regurgitation at baseline seem prone to this complication.

Ventricular fibrillation and sudden death after radiofrequency catheter ablation of the atrioventricular junction.

Two hundred thirty-five patients underwent RF catheter ablation of AV conduction for symptomatic drug refractory AF (84%), atrial flutter (9%), and atrial tachycardia (7%). In the first 100 patients, postablation pacing was not prospectively set at any specific rate and was always < or = 70 beats/min. In the next 135 patients, postablation pacing was prospectively set at 90 beats/min for 1-3 months. Six of the first 100 patients (6%) had VF or sudden death after the RF procedure and none (0%) of the next 135 patients did (P < 0.05). One of the six patients had recurrent VF 4 days after the ablation. Five patients were successfully resuscitated and one patient died. There were no statistically significant differences between patients with and without (aborted) sudden death or between the first 100 and the next 135 patients with respect to age, sex, underlying heart disease, EF, number of RF applications, or left-or right-sided approach of the procedure. VF mostly occurred during episodes of slow ventricular escape rhythms or during slow ventricular pacing. We conclude that malignant ventricular arrhythmias and sudden death are possible complications of RF ablation of the AV function. The mechanism of these complications could have a bradycardia dependent nature and it seems that the occurrence of malignant arrhythmias can be prevented by temporarily pacing the heart at relatively fast rates immediately after ablation.

Some electrocardiographic patterns predicting sudden cardiac death that every doctor should recognize.

In recent years major advances have been made in the recognition and treatment of candidates to sudden cardiac death. These advances include very sophisticated diagnostic and therapeutic techniques, such as genetic testing and the implantable cardioverter-defibrillator, new knowledge coming from large multicenter trials, particularly about the poor efficacy of so-called "antiarrhythmic" drugs, but also very important advances have been made in improving the diagnostic value of simple techniques such as the twelve-lead electrocardiogram. In this article six different electrocardiographic patterns associated to sudden cardiac death are described. Some patterns, like left ventricular hypertrophy or low voltage in the limb electrocardiographic leads, are frequent and the incidence of sudden death in these patients is relatively low, although clearly higher as compared to individuals with a normal electrocardiogram. On the other hand, other patterns which are rarer (like the long QT syndrome or the syndrome of right bundle branch block and ST segment elevation in V1-V3) are associated to a very high rate of sudden death. Because sudden death can be prevented in many cases, every doctor should be able to recognize these types of electrocardiograms.

Electrocardiographic identification of mid-septal accessory pathways in close proximity to the atrioventricular conduction system.

In order to identify ECG characteristics of overt mid-septal accessory pathways (APs) predictive of close proximity to the AV conduction system we analyzed data from patients who underwent successful RF catheter ablation of a mid-septal AP. Mean patient age was 31 +/- 16 years, and 13 were male. The 40 degrees right anterior oblique view was used to divide the mid-septal area into 3 zones: 1 (anterior portion); 2 (intermediate); and 3 (posterior portion). The 12-lead ECG was analyzed with regard to delta wave polarity and R/S transition in the precordial leads. The findings from patients ablated at zone 3 were compared to those at zones 1 and 2. All patients had a positive delta wave in the leads I, II, aVL, and negative delta wave in the leads III and aVR. The R/S transition occurred in lead V2 in 80% of patients. The delta wave in lead aVF was the only ECG characteristic that correlated with the AP ablation zone. Six of 8 patients ablated at zone 3 had a negative delta wave in lead aVF while 6 out of 7 patients ablated at zone 1 or 2 had a positive or isoelectric delta wave in lead aVF (P = 0.03). A positive or isoelectric delta wave in lead aVF identifies mid-septal AP in close proximity to the AV conduction system.