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Since the first robotic-assisted percutaneous coronary intervention procedure (R-PCI) was performed in 2004, there has been a steady evolution in robotic technology, combined with a growth in the number of robotic installations worldwide and operator experience. This review summarises the latest developments in R-PCI with a focus on developments in robotic technology, procedural complexity, tele-stenting and training methods, which have all contributed to the global expansion in R-PCI.

Objective One of the challenges in hypertrophic cardiomyopathy (HCM) is to determine the pathogenicity of genetic variants and to establish genotype/phenotype correlations. This study aimed to: (1) demonstrate that MYBPC3 c.2149–1G>A is a founder pathogenic variant, (2) describe the phenotype and clinical characteristics of mutation carriers and (3) compare these patients with those with the most frequent pathogenic HCM variants: MYBPC3 p.Arg502Trp/Gln.Methods We reviewed genetic tests performed in HCM probands at our institution. We carried out transcript analyses to demonstrate the splicing effect, and haplotype analyses to support the founder effect of MYBPC3 c.2149–1G>A. Carriers with this mutation were compared with those from MYBPC3 p.Arg502Trp/Gln in terms of presentation features, imaging and outcomes.Results MYBPC3 c.2149–1G>A was identified in 8 of 570 probands and 25 relatives. Penetrance was age and sex dependent, 50.0% of the carriers over age 36 years and 75.0% of the carriers over 40 years showing HCM. Penetrance was significantly higher in males: in carriers older than 30 years old, 100.0% of males vs 50.0% of females had a HCM phenotype (p=0.01). Males were also younger at diagnosis (32±13 vs 53±10 years old, pA resulted in an abnormal transcript that led to haploinsufficiency and was segregated in two haplotypes. However, both came from one founder haplotype. Affected carriers showed a better functional class and higher left ventricular ejection fraction (LVEF) than patients with MYBPC3 p.Arg502Trp/Gln (pA splicing variant is a founder mutation. Affected males show an early onset of HCM and with higher penetrance than women. Carriers show better functional class and higher LVEF than MYBPC3 p.Arg502Trp/Gln carriers, but a similar rate of major adverse outcomes.

To the Editor, Aorto-atrial fistulas are an extremely rare condition where an abnormal connection forms between the aortic structures and the cardiac atria. Among them, fistulas between the aorta and the left atrium (Ao-LA) are particularly uncommon, and mainly associated with complications like infectious endocarditis, paravalvular abscesses, aortic dissections, and cardiac surgery often damaging the aortic valve or root.1 This is the case of a patient with a iatrogenic Ao-LA fistula successfully treated with a percutaneous Amplatzer device. This is the case of an 82-year-old man admitted to the hospital due to acute heart failure. Three weeks earlier, he had undergone cardiac surgery with mitral and tricuspid valve repair through double annuloplasty plus left anterior descending coronary artery bypass with internal mammary artery graft. The transthoracic echocardiography revealed the presence of a preserved biventricular and valvular function, but also an abnormal systolic and diastolic jet in the left atrium (figure 1A and video 1 of the supplementary data). On the transoesophageal echocardiography this jet corresponded to an Ao-LA fistula through the aortic non-coronary sinus with a significant shunt across it (figure 1B,C and video 2 of the supplementary data). There were no images suggestive of infective endocarditis and repeated blood cultures remained negative...

Sr. Editor: Las fístulas entre la aorta y las aurículas son una patología extremadamente rara en la que se establece una conexión anómala entre las estructuras aórticas y las aurículas cardiacas. En concreto, las fístulas entre la aorta y la aurícula izquierda (Ao-AI) son particularmente poco comunes y están asociadas, principalmente, a complicaciones tales como endocarditis infecciosas, abscesos periprotésicos, disecciones aórticas y cirugías cardiacas con, a menudo, afectación de la válvula o la raíz aórtica1. Presentamos el caso de un paciente con una fístula Ao-AI iatrogénica tratada con éxito con un dispositivo percutáneo Amplatzer. Se trata de un hombre de 82 años hospitalizado con un cuadro de insuficiencia cardiaca aguda. Tres semanas antes, había sido intervenido por cirugía cardiaca con reparación de las válvulas mitral y tricúspide mediante doble anuloplastia y revascularización mediante un puente de arteria mamaria interna a la descendente anterior. El ecocardiograma transtorácico reveló la presencia de una función biventricular y valvular conservada, pero también un flujo anomalo sistólico y diastólico en la aurícula izquierda (figura 1A y vídeo 1 del material adicional). En el ecocardiograma transesofágico, este flujo se debía a una fístula Ao-AI a través del seno aórtico no coronario con un shunt significativo a su través (figura...

ABSTRACT Introduction and objectives: Recipients of a heart transplant need to receive serial endomyocardial biopsies (EMB) to discard rejection, a procedure that is usually performed through the femoral or jugular vein. Over the last few years, we have developed a technique to perform EMBs using the brachial venous access that we have implemented as the preferential access route. In this article, we describe the technique and the initial clinical experience of 2 different centers. Methods: Between 2004 and 2016, we developed and implemented a brachial venous access technique. We registered the main clinical and procedural variables of all the brachial biopsies performed in both centers and compared them with a retrospective series of femoral and jugular procedures. Results: Brachial EMBs were successfully performed 544 of the time with no major complications. The number of brachial procedures per patient rose from 1 to 14. Over the same period of time 1054 femoral and 686 jugular procedures were performed. The total procedural time was similar with different access routes (mean for brachial/femoral/jugular access: 28/26/29 min., P = .31) while fluoroscopy time was shorter in jugular procedures (mean 5/5/3 min. respectively; P < .001). The brachial procedure was recalled as the least painful procedure of all compared to the jugular or femoral ones (2/8/9 score on a scale from 1 to 10; P = .001) with an overall patient preference towards the brachial access. Conclusions: The venous brachial access route is a good alternative to the central venous one to perform EMBs and is the route of choice in our centers. Also, it has high feasibility and safety and brings additional comfort to patients.

RESUMEN Introducción y objetivos: Los pacientes receptores de un trasplante cardiaco necesitan someterse a biopsias endomiocárdicas (BEM) para descartar el rechazo, procedimiento que habitualmente se realiza por acceso venoso yugular o femoral. En los últimos años hemos desarrollado una técnica de biopsia por vía braquial, que hemos implementado como acceso preferente. En este artículo describimos la técnica y la experiencia clínica inicial de 2 centros empleando el acceso braquial. Métodos: Entre 2004 y 2016 desarrollamos e implementamos la técnica de biopsia por vía venosa braquial. Se registraron las principales variables clínicas y del procedimiento de todas las BEM realizadas por vía braquial en ambos centros, y se compararon las características con los procedimientos realizados por vía femoral y yugular. Resultados: Se realizó la BEM por vía braquial en 544 casos, sin complicaciones mayores. El número de procedimientos braquiales por paciente varió entre 1 y 14. En el mismo periodo se realizaron 1.054 BEM femorales y 686 yugulares. La duración total del procedimiento fue similar por los distintos accesos (mediana braquial/femoral/yugular: 28/26/29 min; p = 0,31), con un menor tiempo de escopia por vía yugular (mediana 5/5/3 min, respectivamente; p < 0,001). Los procedimientos realizados por vía braquial se valoraron como menos dolorosos que los realizados por vía yugular o femoral (2/8/9 en la escala de dolor EVA de 1-10, respectivamente; p = 0,001), y fue la vía de elección por parte de los pacientes. Conclusiones: La BEM por vía venosa braquial es una buena alternativa a la punción venosa central y la vía de elección en nuestros centros, con altas factibilidad y seguridad, y mayor comodidad para el paciente.

Background: Endothelial and microvascular dysfunction are frequently found in the non-culprit territory in patients with acute myocardial infarction (AMI). We aimed to determine whether an impaired coronary physiology of the non-culprit territory impacts long-term prognosis. Methods: FISIOIAM was an observational single-center study which included patients with AMI and another coronary artery lesion in a different territory. Intracoronary physiology of the non-culprit artery was analyzed early after primary percutaneous coronary intervention of the culprit artery, using fractional flow reserve (FFR), index of microcirculatory resistance (IMR), coronary flow reserve (CFR), endothelium-dependent CFR (eCFR) and macrovascular endothelial function . Patients were followed for a composite outcome of cardiovascular death, non-fatal myocardial infarction, coronary revascularization, and hospitalization due to heart failure or unstable angina. Results: A total of 84 patients (mean age: 62 ± 10 years) were included and functional abnormalities were detected in 93% of them. During follow-up (median of 1422 days; interquartile range, 1287–1634), 13.1% of the patients experienced at least one adverse cardiovascular event. Kaplan-Meier analysis revealed that patients with a CFR

Sudden cardiac death (SCD) in young patients without structural heart disease is frequently due to inherited channelopathies such as long QT syndrome (LQTS), Brugada syndrome or Catecholaminergic polymorphic ventricular tachycardia. Accordingly, the addition of genetic testing to clinical data may be useful to identify the cause of the sudden death in this population. Mutations in the KCNQ1 encoded Kv7.1 channel are related to type 1 LQTS, familial atrial fibrillation (AF), short QT syndrome, and SCD. We present a clinical case where the presence of AF after resuscitation in a young man with cardiac arrest was the key clinical data to suspect an inherited disorder and genetic testing was the main determinant for identifying the cause of the cardiac arrest. The KCNQ1 p.Arg231His mutation explained the combined phenotype of AF and susceptibility to ventricular arrhythmias. The case highlights the importance of continued research in genetics and molecular mechanisms of channelopathies.