PREVENTION-ACHD: PRospEctiVE study on implaNTable cardioverter-defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease; Rationale and Design.

BACKGROUND: Many adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) may prevent SCD, but the evidence for primary prevention indications is still unsatisfactory. STUDY DESIGN: PREVENTION-ACHD is a prospective study with which we aim to prospectively validate a new risk score model for primary prevention of SCD in ACHD patients, as well as the currently existing guideline recommendations. Patients are screened using a novel risk score to predict SCD as well as current ICD indications according to an international Consensus Statement. Patients are followed up for two years. The primary endpoint is the occurrence of SCD and sustained ventricular arrhythmias. The Study was registered at ClinicalTrials.gov (NCT03957824). CONCLUSION: PREVENTION-ACHD is the first prospective study on SCD in ACHD patients. In the light of a growing and aging population of patients with more severe congenital heart defects, more robust clinical evidence on primary prevention of SCD is urgently needed.

Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease. / Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert.

BACKGROUND AND OBJECTIVES: Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM 160900), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1. MATERIAL AND METHODS: Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide. RECOMMENDATIONS: The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives. CONCLUSION: MD1 is a multisystemic disease that requires specialised multidisciplinary follow-up.

Relationship between ethanol tolerance, H+ -ATPase activity and the lipid composition of the plasma membrane in different wine yeast strains.

Ethanol tolerance, ATPase activity and the lipid composition of the plasma membrane to study potential relationship among them were examined in five different wine yeast strains. Yeast cells were subjected to ethanol stress (4% v/v). Principal component analysis of the results revealed that the wine yeasts studied can be distinguished in terms of ATPase activity and oleic acid (C18:1), and palmitoleic acid (C16:1), in plasma membrane. Multiple regression analysis was used to identify a potential influence of some components of the plasma membrane on ethanol tolerance and ATPase activity. Based on the results, the ergosterol, oleic acid and palmitoleic acid are highly correlated with ATPase activity and ethanol tolerance. Ethanol tolerance and the ATPase activity of the plasma membrane were correlated at the 96.64% level with the oleic acid and ergosterol in plasma membrane. The Saccharomyces cerevisiae var. capensis flor yeast strain, which exhibited the highest ergosterol concentration in plasma membrane when grown in the presence of 4% v/v ethanol, was found to be the most ethanol-tolerant.

Transient entrainment of bundle-branch reentry by atrial and ventricular stimulation: elucidation of the tachycardia mechanism through analysis of the surface ECG.

BACKGROUND: Different responses to entrainment have been reported in relation to the pacing site of a variety of tachycardias. However, transient entrainment of bundle-branch reentrant tachycardia (BBRT) has not been investigated systematically. METHODS AND RESULTS: We attempted entrainment of 13 BBRTs in 9 patients by pacing first the right ventricle and then the right atrium. The initial pacing cycle length (CL) was 10 ms faster than the tachycardia CL. Subsequent pacing sequences were performed with 5- to 10-ms CL decrements until tachycardia termination or loss of postatropine 1:1 AV conduction. Both full ventricular-paced and AV-conducted QRS complex references were obtained during sinus rhythm pacing from the same sites and with similar CL as during entrainment. Transient entrainment was achieved by ventricular and atrial stimulation in 11 and 8 tachycardias, respectively. Constant fusion was always present during entrainment by ventricular stimulation. There was no change in the QRS complex (orthodromically concealed fusion) during entrainment by atrial stimulation in 6 of 6 tachycardias with left bundle-branch block morphology and in 1 of 2 tachycardias with right bundle-branch block morphology. CONCLUSIONS: BBRT, especially if it has a left bundle-branch block morphology, can be differentiated from other wide-QRS-complex tachycardia mechanisms through analysis of the ECGs recorded during tachycardia entrainment by atrial and ventricular stimulation. This diagnostic approach may be especially useful when it is difficult to record a stable or sufficiently sized His bundle electrogram or when spontaneous changes in the ventricular CL precede similar changes in the His bundle CL.

Bundle-branch reentry and the postpacing interval after entrainment by right ventricular apex stimulation: a new approach to elucidate the mechanism of wide-QRS-complex tachycardia with atrioventricular dissociation.

BACKGROUND: Diagnosis of bundle-branch reentry ventricular tachycardia (BBR-VT) by the standard approach is challenging, and this may lead to nonrecognition of this tachycardia mechanism. Because the postpacing interval (PPI) after entrainment has been correlated with the distance from the pacing site to the reentrant circuit, BBR-VT entrainment by pacing from the right ventricular apex (RVA) should result in a PPI similar to the tachycardia cycle length (TCL). This factor may differentiate BBR-VT from other mechanisms of wide-QRS-complex tachycardia with AV dissociation, such as myocardial reentrant VT (MR-VT) or AV nodal reentrant tachycardia (AVNRT), in which the circuit is usually located away from the RVA. METHODS AND RESULTS: Transient entrainment by RVA pacing was attempted in 18 consecutive BBR-VTs and finally achieved in 13. Results were compared with those found in 59 consecutive MR-VTs and 50 consecutive AVNRTs. The mean PPI-TCL difference was significantly (P:<0.0001) shorter in the BBR-VT group (9+/-11 ms) than in the MR-VT (109+/-48 ms) and the AVNRT (150+/-29 ms) groups. No BBR-VT showed a PPI-TCL >30 ms (range -12 to 24 ms). Except for 2 MR-VTs, no MR-VT (range 21 to 211 ms) or AVNRT (range 100 to 215 ms) showed a PPI-TCL <30 ms. CONCLUSIONS: A PPI-TCL >30 ms, after entrainment by RVA stimulation, makes BBR-VT unlikely. Conversely, a PPI-TCL <30 ms is suggestive of BBR-VT but should lead to further investigation by use of conventional criteria.

Mechanism and location of atrial flutter in transplanted hearts: observations during transient entrainment from distant sites.

OBJECTIVES: This study was designed to elucidate the location and mechanism of typical atrial flutter in the transplanted heart. BACKGROUND: Although the F wave morphology in atrial flutter is similar in nontransplanted and transplanted hearts, the surgical incision needed for the atrial anastomosis may create a distinct electrophysiologic substrate of atrial flutter. METHODS: Entrainment from the lateral wall of the right atrium and interatrial septum was used to determine the location of atrial flutter in five patients with a transplanted heart and six patients with a nontransplanted heart. The difference between the first postpacing interval (FPPI) and the flutter cycle length (FCL) was used as an index of proximity to the circuit. RESULTS: In the transplant group, the FPPI was equal to the FCL at sites located close to the tricuspid annulus (TA); the mean differences (+/-SD) were 1 +/- 5 and -1 +/- 2 ms at the lateral wall and interatrial septum, respectively. However, from sites close to the surgical incision at the lateral wall and at the interatrial septum, these differences were significantly longer (29 +/- 12 and 27 +/- 9 ms, respectively, p < 0.05). In the nontransplant group, the FPPI was similar to the FCL at points in the lateral wall and interatrial septum close to the TA (mean difference 7 +/- 6 and 6 +/- 11 ms, respectively) and at sites close to the crista terminalis (CT) in the lateral wall (mean difference 4 +/- 4 ms). However, in sites separated from the TA at the interatrial septum the difference was markedly longer (35 +/- 11 ms, p < 0.05). CONCLUSIONS: Atrial flutter in transplanted hearts may best be explained by macroreentry around the tricuspid ring. In non-transplanted hearts a different structure (perhaps the CT?) may be the basis for atrial flutter at the lateral wall.

"Pseudodisappearance" of atrial electrogram during orthodromic tachycardia: new criteria for successful ablation of concealed left-sided accessory pathways.

OBJECTIVES: This study sought to analyze two new criteria along with other known predictors of success of radiofrequency ablation. Background. Although the overall success rate of radiofrequency ablation of accessory pathways is high, the individual predictive value of each of the established criteria is low. METHODS: We prospectively studied the local electrograms obtained before the application of radiofrequency energy in 33 patients with a left-sided concealed accessory pathway successfully ablated. Two new criteria ("pseudodisappearance" during tachycardia of a bipolar atrial electrogram visible during sinus rhythm and the presence of an "atrial notch" in the ascending limb of the unipolar ventricular electrogram during tachycardia) were studied along with other known predictors. Electrograms recorded at a total of 157 sites were analyzed (33 successful applications, 124 failures). RESULTS: Electrogram characteristics that were predictive of success during ablation on the basis of univariate analyses were a pseudodisappearance criterion (p<0.001), the presence of a Kent potential (p<0.005) and the presence of an "atrial notch" (p<0.005). After adjustment for between-patient differences, logistic regression analysis showed that only the "pseudodisappearance" criterion (odds ratio [OR] 7.2, 95% confidence interval [CI] 1.2 to 42.5, p<0.03) and the presence of a Kent potential (OR 2.4, 95% CI 1.01 to 5.79, p<0.05) had independent predictive value. CONCLUSIONS: The pseudodisappearance during tachycardia or ventricular pacing of a bipolar atrial electrogram present during sinus rhythm is associated with a good outcome during radiofrequency ablation of concealed accessory pathways. These observations may help to ablate accessory pathways and to avoid missing appropriate sites for ablation when the atrial activation is not clearly visible at the local electrogram.

[Characteristics of suicides in Navarra by gender (2010-2013)]. / Características de los suicidios consumados en Navarra en función del sexo (2010-2013).

OBJECTIVE: To determine the number of suicides and the main social and demographic characteristics, time frames and methods involved during the period 2010-2013 in Navarra by gender. METHODS: A study of the Electronic Clinical Records provided by Navarra's Healthcare Service in which suicides in Navarra are detailed. RESULTS: One hundred and eighty individuals committed suicide. The number of suicides remained stable: 41 in 2010, 51 in 2011 and 44 in 2012 and 2013. Seventy-five point four percent were males (n=136) and 24.6 % (n=44) were females. In the 13 to 26 age group, 12 (92.3%) out of 13 suicides were committed by males. In terms of employment status, 49.3% (n=70) were pensioners. The highest rate of suicides was reached in summer (n=71) and spring (n=39). Monday was the day with the highest rate of suicides (n=37) and the time period between 8:00 and 12:00 hours was when the highest number of suicides (n=80) took place. The most usual ways of committing suicide were hanging (n=80), falling from a height (n=41) and pharmacological overdose (n=23). Males used violent methods more frequently. It is necessary to highlight the fact that in some of the sociodemographic characteristics there was up to 60% of cases without enough information. CONCLUSION: The results obtained show some specific features of the phenomenon of suicide in Navarra that should be considered for its prevention. Furthermore, the implementation of effective protocols of data collection is recommended to develop prevention strategies.

Determination of trichloroanisole and trichlorophenol in wineries' ambient air by passive sampling and thermal desorption-gas chromatography coupled to tandem mass spectrometry.

The present paper describes the calibration of selected passive samplers used in the quantitation of trichlorophenol and trichloroanisole in wineries' ambient air, by calculating the corresponding sampling rates. The method is based on passive sampling with sorbent tubes and involves thermal desorption-gas chromatography-triple quadrupole mass spectrometry analysis. Three commercially available sorbents were tested using sampling cartridges with a radial design instead of axial ones. The best results were found for Tenax TA™. Sampling rates (R-values) for the selected sorbents were determined. Passive sampling was also used for accurately determining the amount of compounds present in the air. Adequate correlation coefficients between the mass of the target analytes and exposure time were obtained. The proposed validated method is a useful tool for the early detection of trichloroanisole and its precursor trichlorophenol in wineries' ambient air while avoiding contamination of wine or winery facilities.

Radiofrequency catheter ablation of ventricular tachycardia from right ventricle late after myocardial infarction.

In summary, this case illustrates how complex VT circuits may be. If the findings of this case are substantiated with additional cases, mapping and radiofrequency energy application from right ventricle would have to be considered in VT with left bundle branch blocks QRS morphology, whenever ablation from the left ventricule is ineffective.

Randomized, prospective comparison of four burst pacing algorithms for spontaneous ventricular tachycardia.

"Less aggressive" burst stimulation is more effective in terminating spontaneous monomorphic ventricular tachycardia with a lesser acceleration rate. Higher ventricular tachycardia cycle length and use of 91% coupling interval were independent predictors for pacing termination.

Implantable cardioverter defibrillator as therapeutic option for sudden cardiac death secondary to severe coronary vasospasm.

We report a case of a patient with angina at rest followed by sudden cardiac death secondary to ventricular fibrillation. Cardiac catheterization did not show significant coronary stenosis but after an acetylcholine test, a severe coronary spasm was induced. Despite intensive medical therapy, vasospasm was not prevented. Finally, the patient received an implantable cardioverter defibrillator (ICD) to avoid the risk of sudden cardiac death. In our opinion, ICD in addition to medical therapy may be useful in patients with sudden cardiac death secondary to coronary vasospasm.

[Current indications for implantable automatic defibrillators]. / Indicaciones actuales del desfibrilador automático implantable.

Since the first implantation in man in 1980 implantable cardioverter defibrillator technology has greatly improved and the number of devices implanted has increased considerably in recent years. Non-thoracotomy lead systems and biphasic shocks are now the approach of choice, offering nearly a 100% success rate. This paper version reviews the current indications for the implantation of implantable cardioverter defibrillator and is an upgraded of an article previously published by the Arrhythmia's Section of the Spanish Society of Cardiology. Recommendations for qualification of centres implanting defibrillators and follow up are also addressed.

[Sudden cardiac death out of the hospital and early defibrillation]. / Muerte súbita cardíaca extrahospitalaria y desfibrilación precoz.

Since most sudden cardiac death victims show neither symptoms before the event nor other signs or risk factors that would have identified them as a high risk population before their cardiac arrest, emergency out-of-hospital medical services must be improved in order to obtain a higher survival in these patients. Early defibrillation is an essential part of the chain of survival that also includes the early identification of the victim, activation of the emergency medical system, immediate arrival of trained personnel who can perform basic cardiopulmonary resuscitation and early initiation of advanced cardiac life support that would raise the survival rate for sudden cardiac arrest victims. Many studies have demonstrated the enormous importance of early defibrillation in patients with a cardiac arrest due to ventricular fibrillation. The most important predictor of survival in these individuals is the time that elapses until electric defibrillation, the longer the time to defbrillation the lower the number of patients who are eventually discharged. Multiple studies have demonstrated that automatic external defibrillation will reduce the time elapsed to defibrillation and thus improve survival. For these reason, public access defibrillation to allow the use of automatic external defibrillators by minimally trained members of the lay public, has received increasing interest on the part of a groving number of companies, cities or countries. The automatic external defibrillaton, as performed by a lay person is being investigated. The liberalization of its application, if is demonstrated to be effective, will need to be accompanied by legal measures to endorse it and appropriate health education, probably during secondary education.

[Cardioinhibitory vagal response not related to pain, and induced by radiofrequency application during ablation of right posteroseptal accessory pathway]. / Respuesta vagal cardioinhibidora, no mediada por dolor, secundaria a la aplicación de radiofrecuencia durante la ablación de una vía accesoria posteroseptal derecha.

Inappropriate sinus tachycardia is the most common arrhythmia induced by radiofrequency energy delivery in the posteroseptal area. It has been suggested that this could be secondary to parasymphathetic nerve injury. We report a patient with extreme sinus bradycardia and PR interval prolongation induced by radiofrequency energy delivered in the coronary sinus ostium area, but not related to any other stimulus. The most probable mechanism of the disorder was transient stimulation of the vagal afferent nerve fibers located in this anatomical area.

[Oblique orientation of the accessory pathway demonstrated by radiofrequency application]. / Demostración del trayecto cruzado de una vía accesoria mediante aplicación de radiofrecuencia.

Activation mapping of atrial and ventricular insertion has suggested an oblique orientation of some accessory pathways. However, this aspect has not been demonstrated by radiofrequency application. This report presents two patients with Wolff-Parkinson-White syndrome and an accessory pathway with bidirectional conduction and oblique orientation. The accessory pathway oblique orientation was demonstrated by transient and permanent conduction abolition following radiofrequency application in two separate ventricular and atrial sites. These findings may explain the failure to ablate accessory pathway by radiofrequency application in the ventricular side of the mitral annulus guided by retrograde atrial activation occasionally observed in patients with a concealed accessory pathway.

[Focal auricular fibrillation: catheter ablation with radiofrequency]. / Fibrilación auricular focal: ablación con catéter mediante radiofrecuencia.

Although atrial fibrillation is usually caused by multiple circulating reentrant wavelets, a focal origin has recently been reported. This focal source could be successfully treated by discrete radiofrequency catheter ablation. We report a patient without structural heart disease, but with multiple episodes of palpitations related to atrial premature beats, irregular atrial tachycardia and atrial fibrillation. The electrophysiological study demonstrated that all the atrial arrhythmias were due to the same focus firing irregularly and exhibiting a unique and centrifugal pattern of activation. Successful radiofrequency catheter ablation of the focus was performed, without complications. After three months follow-up the patient is asymptomatic and she has not presented arrhythmia recurrences.