Adult congenital heart disease training in Europe: current status, disparities and potential solutions.

OBJECTIVES: This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe. METHODS: A questionnaire was sent to ACHD cardiologists from 34 European countries. RESULTS: Representatives from 31 of 34 countries (91%) responded. ACHD cardiology was recognised by the respective ministry of Health in two countries (7%) as a subspecialty. Two countries (7%) have formally recognised ACHD training programmes, 15 (48%) have informal (neither accredited nor certified) training and 14 (45%) have very limited or no programme. Twenty-five countries (81%) described training ACHD doctors 'on the job'. The median number of ACHD centres per country was 4 (range 0-28), median number of ACHD surgical centres was 3 (0-26) and the median number of ACHD training centres was 2 (range 0-28). An established exit examination in ACHD was conducted in only one country (3%) and formal certification provided by two countries (7%). ACHD cardiologist number versus gross domestic product Pearson correlation coefficient=0.789 (p<0.001). CONCLUSION: Formal or accredited training in ACHD is rare among European countries. Many countries have very limited or no training and resort to 'train people on the job'. Few countries provide either an exit examination or certification. Efforts to harmonise training and establish standards in exit examination and certification may improve training and consequently promote the alignment of high-quality patient care.

Stabilization of unstable reentrant atrial tachycardias via fractionated continuous electrical activity ablation (CHAOS study).

BACKGROUND: Unstable reentrant atrial tachycardias (ATs) (i.e., those with frequent circuit modification or conversion to atrial fibrillation) are challenging to ablate. We tested a strategy to achieve arrhythmia stabilization into mappable stable ATs based on the detection and ablation of rotors. METHODS: All consecutive patients from May 2017 to December 2019 were included. Mapping was performed using conventional high-density mapping catheters (IntellaMap ORION, PentaRay NAV, or Advisor HD Grid). Rotors were subjectively identified as fractionated continuous (or quasi-continuous) electrograms on 1-2 adjacent bipoles, without dedicated software. In patients without detectable rotors, sites with spatiotemporal dispersion (i.e., all the cycle length comprised within the mapping catheter) plus non-continuous fractionation on single bipoles were targeted. Ablation success was defined as conversion to a stable AT or sinus rhythm. RESULTS: Ninety-seven patients with reentrant ATs were ablated. Of these, 18 (18.6%) presented unstable circuits. Thirteen (72%) patients had detectable rotors (median 2 [1-3] rotors per patient); focal ablation was successful in 12 (92%). In the other 5 patients, 17 sites with spatiotemporal dispersion were identified and targeted. Globally, and excluding 1 patient with spontaneous AT stabilization, ablation success was achieved in 16/17 patients (94.1%). One-year freedom from atrial arrhythmias was similar between patients with unstable and stable ATs (66.7% vs. 65.8%, p = 0.946). CONCLUSIONS: Most unstable reentrant ATs show detectable rotors, identified as sites with single-bipole fractionated quasi-continuous signals, or spatiotemporal dispersion plus non-continuous fractionation. Ablation of these sites is highly effective to stabilize the AT or convert it into sinus rhythm.

Characterization of high-power and very-high-power short-duration radiofrequency lesions performed with a new-generation catheter and a temperature-control ablation mode.

INTRODUCTION: High-power short-duration (HPSD) has been proposed to shorten procedure times while maintaining efficacy and safety. We evaluated the differences in size and geometry between radiofrequency lesions obtained with this method and conventional ones. METHODS AND RESULTS: Twenty-eight sets of 10 perpendicular radiofrequency applications were performed with two commercially available catheters: a temperature-controlled HPSD catheter (QDot-Micro) and a conventional power-controlled catheter (Thermocool SmartTouch) on porcine left ventricle. Different power settings (35, 40, 50, and 90 W), contact force (CF; 10 and 20 g), ablation index (AI; 400 and 550), and application times were combined to create conventional (35-40 W), HPSD (50 W) and very-high-power short-duration (VHPSD; 90 W) lesions, that were cross-sectioned and measured. About 4-s VHPSD lesions were smaller, shallower, and thinner than HPSD performed with the QDot-Micro catheter in any scenario of CF or AI (61 ± 7.8 mm3 , 6.1 ± 0.3 mm wide, and 2.9 ± 0.1 mm deep with 10 g; 72.2 ± 0.5 mm3 , 6.8 ± 0.3 mm wide, and 2.9 ± 0.2 mm deep with 20 g). Conventional and HPSD lesions performed with the temperature-controlled catheter were generally bigger, deeper, and wider than the ones obtained with the power-controlled catheter, as well as more consistent in size. This was especially true with the lower CF and AI scenario, while differences were less notable with other setting combinations. CONCLUSION: VHPSD lesions performed with QDot-Micro catheter were smaller than any other lesions, which is especially attractive for posterior left atrial wall ablation. On the contrary, conventional-powered and HPSD lesions performed with this catheter were equally sized (or even bigger with lower CF and AI objectives), as well as more consistent in size, which would guarantee transmurality in other locations.

Impact of power and contact force on index-guided radiofrequency lesions in an ex vivo porcine heart model.

PURPOSE: Lesion size index (LSI) and ablation index (AI) are markers of lesion quality that incorporate power, contact force (CF) and time in a weighted formula to estimate lesion size. Although accurate predicting lesion depth in vitro, their precision in lesion size estimation has not been well established for certain power and CF settings. We conducted an experimental ex vivo study to analyse the effect of power and CF in size and morphology of ablation lesions in a porcine heart model. METHODS: Twenty-four sets of 10 perpendicular epicardial radiofrequency applications were performed with two commercially available catheters (TactiCath, Sensor Enabled; and SmartTouch) on porcine left ventricle submerged in 37 °C saline, combining different power (25, 30, 35, 40, 50 and 60 W) and CF (10 and 20 g) settings, and aiming at a lower (LSI/AI of 5/400) or higher (LSI/AI of 6/550) index. After each application, lesions were cross-sectioned and measured. RESULTS: Four hundred eighty lesions were performed. For a given target index and CF, significant differences in lesion volume and depth with different power were observed with both catheters, generally with smaller lesions using higher power. Lesions performed with CF of 10 g were particularly smaller with TactiCath compared to SmartTouch; lesions with CF of 20 g aiming a low LSI/AI were, however, bigger; lesions with CF of 20 g aiming a high LSI/AI were similar. In general, high-power lesions were wider and shallower than low-power lesions, especially with SmartTouch. CONCLUSION: Size and morphology of index-guided radiofrequency lesions varied significantly with different power and CF settings.

Propranolol syrup to tablets change triggers electrical storm in Jervell-Lange-Nielsen syndrome.

A 10-year-old girl with genetically confirmed Jervell-Lange-Nielsen syndrome treated with beta-blocker and developed electrical storm after changing propranolol syrup to tablets. Jervell-Lange-Nielsen is characterised by long QT and congenital sensorineural deafness, with high risk of malignant arrhythmias at early ages. Gastric involvement and achlorhydria may be present, with subsequent alteration of medication bioavailability which can trigger severe arrhythmic complications.

Morbilidad y estado de salud autopercibido, dos aproximaciones diferentes al estado de salud / Morbidity and self-perception of health, two different approaches to health status

OBJETIVO: Determinar el grado de asociación entre el estado de salud percibido y el obtenido a partir de información sobre morbilidad atendida. MÉTODO: Se estudió la población de cuatro áreas básicas de salud de la comarca del Baix Empordà (N=91.067), en el año 2016, con una muestra de 1202 personas a las que se realizó una encuesta de salud. Paralelamente, las personas encuestadas se clasificaron en estados de salud mediante el agrupador Clinical Risk Groups. El grado de asociación se analizó mediante regresión logística. RESULTADOS: Un 27% de las personas con más de dos enfermedades crónicas mayores o con condiciones de salud complejas afirmaron tener buena salud. Se detectó asociación entre percepción de salud y carga de enfermedad. El sexo, la situación laboral, la clase social y especialmente la edad también mostraron asociación con la percepción de salud. CONCLUSIONES: La inferencia del estado de salud a partir de registros clínicos sobre morbilidad atendida aproxima la percepción subjetiva de la salud, y viceversa. No obstante, determinados factores sociodemográficos modulan la percepción individual. Los resultados obtenidos son esperanzadores en relación con el desarrollo de nuevas metodologías de cálculo de indicadores de calidad de vida

OBJECTIVE: To determine the degree of association between perceived health status and that obtained from information on attended morbidity. METHOD: The population of four health areas of the Baix Empordà region (Catalonia, Spain) (N=91,067) was studied in 2016, by means of a sample of 1202 individuals. A health survey was conducted on sample individuals. At the same time, the respondents were classified by health status through the Clinical Risk Groups system. The degree of association was analysed by logistic regression. RESULTS: 27% of patients with more than two major chronic diseases or with complex health conditions stated they were in good health. An association was detected between health perception and disease burden. Sex, work situation, social class and especially age also showed association with the perception of health. CONCLUSIONS: The inference of health status from clinical records on attended morbidity approximates to the subjective perception of health and vice versa. However, certain sociodemographic factors modulate individual perception. The results obtained are encouraging in relation to the development of new methodologies for calculating quality of life indicators

[Morbidity and self-perception of health, two different approaches to health status]. / Morbilidad y estado de salud autopercibido, dos aproximaciones diferentes al estado de salud.

OBJECTIVE: To determine the degree of association between perceived health status and that obtained from information on attended morbidity. METHOD: The population of four health areas of the Baix Empordà region (Catalonia, Spain) (N=91,067) was studied in 2016, by means of a sample of 1202 individuals. A health survey was conducted on sample individuals. At the same time, the respondents were classified by health status through the Clinical Risk Groups system. The degree of association was analysed by logistic regression. RESULTS: 27% of patients with more than two major chronic diseases or with complex health conditions stated they were in good health. An association was detected between health perception and disease burden. Sex, work situation, social class and especially age also showed association with the perception of health. CONCLUSIONS: The inference of health status from clinical records on attended morbidity approximates to the subjective perception of health and vice versa. However, certain sociodemographic factors modulate individual perception. The results obtained are encouraging in relation to the development of new methodologies for calculating quality of life indicators.

Ablación con catéter de taquicardia auricular perihisiana desde el seno de Valsalva no coronario tras reemplazo de válvula aórtica / Catheter ablation of peri-hisian atrial tachycardia from the noncoronary sinus of Valsalva after aortic valve replacement

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Tratamiento percutáneo de las lesiones evolutivas del switch auricular en pacientes con transposición de grandes arterias / Percutaneous treatment of follow-up lesions after atrial switch in patients with transposition of the great arteries

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Contact force-sensing catheters: performance in an ex vivo porcine heart model.

PURPOSE: Contact force (CF) catheters are useful to address proper contact during ablation. However, interactions between the ablation process, or its associated irrigation flow changes, with the CF sensing may translate into unexpected CF value fluctuations. We aimed to test for unintentional CF value variations during radiofrequency applications at a fixed applied force, with two commercially available catheters (TactiCath™ and SmartTouch™), and to evaluate its theoretical clinical significance by correlating CF-derived automatic ablation algorithms (force-time integral and lesion index) and actual lesion size at two standard CF values. METHODS: Four series of 20 perpendicular epicardial ablations (20 W, 60 s, 17 ml/min) were performed on porcine left ventricle submerged in 37 °C saline. Catheters were mechanically fixed at a constant position and evaluated at 10 and 20 g. CF values were digitally analysed before each application changing irrigation rate (2-17-30 ml/min), and during ablation. Finally, lesions were quantified. RESULTS: Increasing irrigation before ablation led to a slight but significant CF decrease. During ablation, CF showed a reproducible pattern: fast initial decrease, subsequent increase until higher-than-initial values and final plateau phase (CF variation up to 69% at 10 g). CF variability was significantly higher at 10 g and using TactiCath™. There were no major differences in lesion size between catheters at the same initial CF. CF only correlated mildly to lesion measures, and automatic algorithms globally failed to predict lesion size. CONCLUSIONS: CF measured values spontaneously vary during ablation following a predictable pattern (initial decrease, subsequent increase and final plateau). This is especially remarkable applying lower CF.

Gestación tras cirugía de Fontan-Kreutzer / Pregnancy after Fontan-Kreutzer surgery

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Cambios electrocardiogáficos y arritmias en pacientes pediátricos con rabdomioma / Electrocardiographical changes and arrhythmias in pediatric patients with rabdomiome

La incidencia de tumores cardíacos en pediatría es menor de 0,1%, en su mayoría son de carácter benigno y másde la mitad rabdomiomas. La historia natural de estos tumores es de regresión y la mayoría no produce síntomas. No es infrecuente la asociación con cambios electrocardiográficos, dependiendo de la localización tumoral, y ocasionalmente su presentación concomitante con taquicardias ventriculares, bloqueos aurículo-ventriculares y supraventriculares por vías anómalas.Revisamos nuestra experiencia en pacientes diagnosticados con rabdomioma asociados a arritmias y suevolución. 18 pacientes, nueve de ellos varones diagnosticados con tumoración cardíaca y etiquetados como rabdomioma por imagenología, a una edad media de 7,7 meses (uno prenatalmente). 22% (4) presentaban esclerosis tuberosa asociada. La media de seguimiento es de 17 años. De los 18 pacientes, siete presentaban bloqueo de rama derecha (dos aislado, tres con bloqueo aurículo-ventricular de primer grado, dos con bloqueo aurículo-ventricular de segundo grado y extrasístoles ventriculares), tres debutaron con taquicardia o fibrilación ventricular, uno con bradicardia neonatal y siete con preexcitación intermitente (cuatro con taquicardia supraventricular), cinco fallecieron durante meses posteriores al diagnóstico, dos de ellos tempranamente en relación con arritmias ventriculares incontrolables, uno por problemas hemodinámicos obstructivos previo a cirugía y dos en el postoperatorio inmediato (ninguno de ellos tenía el diagnóstico de esclerosis tuberosa). De los 13 pacientes restantes, seis requirieron ser intervenidos por obstrucción tumoral. En uno se implantó un marcapasos AAI por disfunción sinusal evolutiva postoperatoria y en un paciente persiste preexcitación electrocardiográfica, sin taquicardia y en tratamiento con betabloqueantes, y en dos bloqueo de rama derecha, uno de ellos asociado a bloqueo aurículo ventricular de primer grado...

The incidence of cardiac tumors in children is < 0,1%; most of them are benign and more than half of them arerhabdomyosarcoma. The majority regresses without symptoms. Frequently, depending on the tumors location, electrocardiographic changes can be seen, occasionally with ventricular tachyarrhythmia, auricular-ventricular blockage and supraventricular tachyarrhythmia due to accessory pathways. Our experience with patients who had rhabdomyosarcoma with arrhythmias and their evolution is described in thispaper. 18 patients were included, nine boys with the diagnosis of rhabdomyosarcoma through image with an age average of 7,7 months old (one was done prenatal). Four patients (22%) had tuberous sclerosis associated. Follow–up was done for an average of 17 years. Of the 18 patients, seven had sinoatrial block (two isolated, three with first degree AV block, two with second degree block and ventricular extrasystoles), three started with VT/VF, one had neonatal bradyarrhythmia and seven with intermitent preexcitement (four with SVT), five died months after the diagnosis, two died prematurelydue to uncontrollable ventricular arrhythmias, one had preoperative hemodynamic problems and two diedinmedialtely after surgery (none had tuberous sclerosis). Of the 13 patients left, six needed surgery due to tumoralobstruction. One patient needed a cardiac pacing and another patient persist with electrocardiographicpreexcitation, without tachyarrhythmia who is treated with â-blockers. Two have sinoatrial block, one of them firstdegree block. Of the rest three were operated and 16 had tumoral regression.Electrocardiographic alterations and arrythmias associated to tumors are frequent, malignant arrythmias can befatal but most of them dissapear when surgery of the tumor is practiced.

[Unusual cyanosing heart defect due to supravalvular tricuspid obstruction in an infant]. / Una forma rara de cardiopatía cianosante por obstrucción supravalvular tricuspídea en el lactante.

We present a case involving a rare form of cyanotic congenital heart disease in a 2-month-old neonate. The initial diagnosis was thought to be tricuspid dysplasia with right-to-left shunting through an interatrial communication. However, surgery showed the presence of a pedunculated mass that prolapsed into the tricuspid valve orifice, thereby causing severe obstruction to right ventricular filling. This mass was attached to the lower part of the atrial septum. Pathological examination showed fibrotic myxoid tissue. After the mass was resected surgically, the tricuspid valve appeared normal. We comment on the pathogenesis and the differential diagnosis of this rare entity.

Una forma rara de cardiopatía cianosante por obstrucción supravalvular tricuspídea en el lactante / Unusual cyanosing heart defect due to supravalvular tricuspid obstruction in an infant

Presentamos el caso de un lactante de 2 meses con cianosis desde el primer día de vida. El diagnóstico de sospecha inicial fue displasia tricuspídea, con shunt derecha-izquierda a través de una comunicación interauricular. Fue necesario llegar al acto quirúrgico para objetivar un «bolsón» de tejido supratricuspídeo adherido al septo interauricular bajo, que dificultaba el llenado del ventrículo derecho. Tras su resección se evidenció el aspecto macroscópico normal de la válvula tricúspide. La histología fue compatible con tejido fibromixoide. Se discuten la etiopatogenia y posibilidades diagnósticas de este hallazgo inhabitual

We present a case involving a rare form of cyanotic congenital heart disease in a 2-month-old neonate. The initial diagnosis was thought to be tricuspid dysplasia with right-to-left shunting through an interatrial communication. However, surgery showed the presence of a pedunculated mass that prolapsed into the tricuspid valve orifice, thereby causing severe obstruction to right ventricular filling. This mass was attached to the lower part of the atrial septum. Pathological examination showed fibrotic myxoid tissue. After the mass was resected surgically, the tricuspid valve appeared normal. We comment on the pathogenesis and the differential diagnosis of this rare entity