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Resumen El prolapso de la válvula mitral es una enfermedad ampliamente conocida, la cual es benigna en la mayoría de casos; sin embargo, puede estar en asociación con alteraciones del ritmo cardiaco, específicamente arritmias ventriculares malignas y muerte cardiaca súbita. Pese a que esta asociación y su desenlace están descritos en la literatura médica, su baja incidencia conduce a la falta de evidencia en cuanto a su manejo y estratificación. Se presenta el caso de una mujer joven, cuya manifestación inicial fue síncope, con posterior desarrollo de insuficiencia mitral grave, con hallazgo de extrasístoles ventriculares frecuentes, en quien se realizó tratamiento quirúrgico con plastia mitral y crioablación del músculo papilar anterolateral. La evolución clínica fue satisfactoria, ya que se evidenció mejoría de la sintomatología y control de la carga arrítmica.

Abstract Mitral valve prolapse is a well-known condition that is generally benign, but it can be associated with cardiac arrhythmias, particularly malignant ventricular arrhythmias and sudden cardiac death. This association and its outcome have been described in medical literature, but the low incidence leads to a lack of evidence regarding its management and stratification.The case of a young woman is presented, whose initial manifestation was syncope, with subsequent development of severe mitral insufficiency, followed by severe mitral regurgitation and the discovery of frequent ventricular extrasystoles. She underwent surgical repair with mitral valve plasty and cryoablation of the anterior papillary muscle. The clinical outcome was favorable, as evidenced by an improvement in symptoms and control of arrhythmia burden.

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BACKGROUND: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. METHODS: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. RESULTS: Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030). CONCLUSION: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.

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Resumen Introducción: La sobrevida de pacientes con síndrome de Marfan y compromiso de la raíz aórtica ha aumentado debido a la introducción de técnicas de reemplazo de raíz aórtica con preservación valvular. Objetivo: Identificar y caracterizar desenlaces a corto y largo plazo de pacientes con síndrome de Marfan intervenidos con la técnica de reemplazo de la raíz aórtica con reimplante de la válvula aórtica (procedimiento de Tirone David). Materiales y método: Serie de casos de pacientes con síndrome de Marfan sometidos al procedimiento quirúrgico de Tirone David entre 2002 y 2020. Se generaron curvas de Kaplan-Meier para evaluar el tiempo libre de reintervención aórtica y la sobrevida. Resultados: Se incluyeron 18 pacientes, con edad promedio de 29 años. Se identificó progresión de la enfermedad en el 35.3%, determinada por reintervención en 5 pacientes, disección aórtica tipo B en 3 pacientes, insuficiencia aórtica grave en 2 pacientes y aneurismas crónicos torácicos o abdominales en 5 pacientes. Se identificaron 3 muertes no relacionadas directamente con la progresión de la enfermedad. Conclusiones: En pacientes con síndrome de Marfan y patología de raíz aórtica, el procedimiento de reemplazo de la raíz aórtica con reimplante de la válvula aórtica es la técnica ideal por sus resultados en libertad de reintervención, sobrevida y calidad de vida. Sin embargo, la cirugía no resuelve el compromiso aórtico distal, el cual es el principal factor en la progresión de la enfermedad.

Abstract Introduction: The survival of patients with Marfan syndrome and aortic root involvement has increased over the past decades due to the introduction of valve-sparing aortic root replacement techniques. Objective: To identify and characterize the short- and long-term outcomes of patients with Marfan syndrome managed with aortic root replacement with aortic valve reimplantation (Tirone David procedure). Materials and method: A case series of patients diagnosed with Marfan syndrome who underwent the surgical procedure between 2002 and 2020. Kaplan-Meier curves were created to evaluate aortic reintervention-free time and survival. Results: 18 patients were included, the average age was 29 years; disease progression was identified in 35.3%, determined by reintervention in five patients, three patients had type B aortic dissection, two had severe aortic insufficiency and five had chronic thoracic or abdominal aneurysms. There were three deaths, not directly related to disease progression. Conclusions: In patients with Marfan syndrome and aortic root disease, aortic root replacement with aortic valve reimplantation is the ideal technique due to its results in results in avoiding the need for reintervention and improving survival and quality of life. However, surgery does not resolve distal aortic involvement, which is the main factor in disease progression.

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BACKGROUND: Kommerell's aneurysm is a saccular or fusiform dilatation found in 3-8% of Kommerell's diverticulum cases. A non-dissecting rupture rate of 6% has been reported. If ruptured, emergent surgical correction is usually granted. However, evidence regarding the optimal surgical approach in this acute setting is scarce. In this case report series, we aim to describe our experience managing type-1 non-dissecting ruptured Kommerell's aneurysm with hybrid emergent surgical approaches. CASES PRESENTATION: From January 2005 to December 2020, three cases of type-1 non-dissecting ruptured Kommerell's aneurysm requiring emergent surgical repair were identified. The mean age was 66.67 ± 7.76 years, and 3/3 were male. The most common symptoms were atypical chest pain, dyspnoea, and headache (2/3). The mean aneurysm's diameter was 63.67 ± 5.69 mm. Frozen Elephant Trunk was the preferred surgical approach (2/3). The Non-Frozen Elephant Trunk patient underwent a hybrid procedure consisting of a supra-aortic debranching and a zone-2 stent-graft deployment. We found a mean clamp time of 140 ± 60.75 min, cardiac arrest time of 51.33 ± 3.06 min, and a hospital stay of 13.67 ± 5.51 days. The most common complications were surgical-site infection and shock (2/3). Only one patient died (1/3). CONCLUSION: Evidence of management for non-dissecting ruptured Kommerell's aneurysms is scarce. Additional, robust, and more extensive studies are required. The selection of the appropriate surgical approach is challenging, and each patient should be individualized. Frozen Elephant Trunk was feasible for patients requiring emergent surgical repair in our centre. However, other hybrid or open procedures can be performed.

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BACKGROUND: The lack of evidence on complications using mitral valve approaches leaves the choice of risk exposure to the surgeon's preference, based on individual experience, speed, ease, and quality of exposure. METHODS: The present study analysed patients undergoing mitral valve surgery using a superior transseptal approach or a left-atrial approach between 2006 and 2018. We included first-time elective mitral valve procedures, isolated, or combined, without a history of rhythm disturbances. We used propensity score matching based on 26 perioperative variables. The primary endpoint was the association between the superior transeptal approach and clinically significant adverse outcomes, including arrhythmias, need for a permanent pacemaker, cerebrovascular events, and mortality. RESULTS: A total of 652 patients met the inclusion criteria; 391 received the left atrial approach, and 261 received the superior transseptal approach. After matching, 96 patients were compared with 69 patients, respectively. The distribution of the preoperative and perioperative variables was similar. There was no difference in the incidence of supraventricular tachyarrhythmias or the need for treatment. The incidence of nodal rhythm (p = 0.008) and length of stay in intensive care (p = 0.04) were higher in the superior transseptal group, but the need for permanent pacemaker implantation was the same. Likewise, there was no difference in the need for anticoagulation due to arrhythmia, the incidence of cerebrovascular events or mortality in the postoperative period or in the long-term follow-up. CONCLUSION: We did not find an association with permanent heart rhythm disorders or any other significant adverse clinical outcome. Therefore, the superior transeptal approach is useful and safe for mitral valve exposure.

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Las enfermedades cardiovasculares incluyen cardiopatía isquémica, enfermedad cardiaca estructural, accidente cerebrovascular, insuficiencia cardíaca, enfermedad arterial periférica y otras afecciones cardíacas y vasculares, que constituyen la principal causa de morbimortalidad global. De igual forma, contribuyen de manera importante a la reducción de la calidad de vida y son la principal causa de costos a los sistemas de salud en el mundo. En el 2017, las enfermedades cardiovasculares ocasionaron aproximadamente 17,8 millones de muertes en todo el mundo, lo que corresponde a 330 millones de años de vida perdidos y otros 35,6 millones de años vividos con discapacidad.

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BACKGROUND: The approach to coarctation of the aorta with hypoplastic aortic arch is controversial. We evaluated the outcomes in patients with coarctation of the aorta with or without hypoplastic aortic arch operated through a posterior left lateral thoracotomy. METHODS: A retrospective cohort of patients with aortic coarctation, who underwent repair between January 2009 and October 2017, was analyzed. Preoperative, postoperative, and echocardiographic characteristics were reviewed. Statistical analysis examined survival, freedom from reintervention, and freedom from recoarctation. RESULTS: In nine years, 389 patients who underwent surgical treatment for coarctation of the aorta were identified; after exclusion criteria and complete echocardiographic reports, 143 patients were analyzed, of which 29 patients had hypoplastic aortic arch. The modification in the extended end-to-end anastomosis technique was a wide dissection and mobilization of the descending aorta that was achieved due to the ligation and division of 3 to 5 intercostal vessels. In both groups, patients were close to one month of age and had a median weight of 3.6 and 3.4 kg for hypoplastic and nonhypoplastic arch, respectively. In postoperative events, there was no statistically significant difference between the groups ( P = .57 for renal failure, P = .057 for transient, nonpermanent neurologic events, P = .496 for sepsis), as for intensive care unit ( P = .502) and total in-hospital stay ( P = .929). There was one case of postoperative mortality in each group and both were associated with noncardiac comorbidities. Regarding survival (log-rank = 0.060), freedom from reintervention (log-rank = 0.073), and freedom from recoarctation (log-rank = 0.568), there was no statistically significant difference between the groups. CONCLUSION: We believe that it is the modified technique that allowed greater mobilization of the aorta and successful repair of hypoplastic arch through thoracotomy, without an increase in paraplegia or other adverse outcomes.

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BACKGROUND: Recent evidence has showed us that quality of mitral valve repair is strongly related to volume. However, this study shows how low-volume centers can achieve results in mitral valve repair surgery comparable to those reported by referral centers. It compares outcomes of mitral valve repair using resection versus noresection techniques, tendencies, and rates of repair. METHODS: Between 2004 and 2017, 200 patients underwent mitral valve repair for degenerative mitral valve disease at Fundación Cardioinfantil-Institute of Cardiology. Fifty-eight (29%) patients underwent resection and 142 (71%) noresection. RESULTS: Follow-up was 94% complete, mean follow-up time was 2.3 years. There was no 30-day mortality. Five patients required mitral valve replacement after an average of 5.3 years (Resection = 2; Noresection = 3). Freedom from severe mitral regurgitation was 98% at 6.6 years of follow-up for the noresection group, and 92.5% at 7 years for the resection group (log rank: 0.888). At last follow-up, two patients died of cardiovascular disease related to mitral valve, 181 patients (86%) showed no or grade I mitral regurgitation. Patients with previous myocardial infarction had increased risk of recurrent mitral regurgitation (p = 0,030). Within four years, we inverted the proportion of mitral valve replacement and repair, and in 2016 we achieved a mitral valve repair rate of 96%. CONCLUSION: This study suggests that resection and noresection techniques are safe and effective. Recurrence of severe mitral regurgitation and need for mitral valve replacement are rare. We show that low-volume centers can achieve results comparable to those reported worldwide by establishing a mitral valve repair team. We encourage hospitals to follow this model of mitral valve repair program to decrease the proportion of mitral valve replacement, while increasing mitral valve repair.

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BACKGROUND: Mechanical circulatory support (MCS) represents an effective urgent therapy for patients with cardiac arrest or end-stage cardiac failure. However, its use in developing countries as a bridge therapy remains controversial due to costs and limited duration. This study presents five patients who underwent MSC as bridge therapy for heart transplantation in a developing country. CASE PRESENTATION: We present five patients who underwent MCS as bridge therapy for heart transplant between 2010 and 2015 at Fundación Cardioinfantil-Instituto de Cardiología. Four were male, median age was 36 (23-50) years. One patient had an ischemic cardiomyopathy, one a lymphocytic myocarditis, two had electrical storms (recurrent ventricular tachycardia) and one an ischemic cardiomyopathy with an electrical storm. Extracorporeal life support (ECLS) was used in three patients, left ventricular assistance in one, and double ventricular assistance in one (Levitronix® Centrimag®). Median assistance time was 8 (2.5-13) days. Due to the inability of cardiopulmonary bypass weaning, two patients required ECLS after transplant. One patient died in the intensive care unit due to type I graft rejection. Endpoints assessed were 30-day mortality, duration of bridge therapy and complications related to MCS. Patients that died on ECLS, or were successfully weaned off ECLS were not included in this study. CONCLUSIONS: MCS is often the only option of support for critically ill patients waiting for a heart transplant and could be considered as a short-term bridge therapy.

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La estenosis aórtica es la valvulopatía primaria más frecuente en el mundo; alcanza una prevalencia estimada en 7,6 millones de personas mayores de 75 años en Europa y Estados Unidos, aunado al aumento en la expectativa de vida de la población mundial que hará que esta tasa continúe en ascenso1. En el momento del diagnóstico de esta valvulopatía, aproximadamente el 50% de los pacientes son asintomáticos y varios pueden tener a fracción de eyección (FE) preservada; a este tipo de pacientes se les ha clasificado en un estadio C1 según la guía de la Asociación americana del corazón/Colegio americano de Cardiología (AHA/ACC, sus siglas en inglés) del año 2014. Su manejo consiste en un seguimiento clínico y ecocardiográfico cada 6 a 12 meses (recomendación dada por expertos y basada en estudios retrospectivos) (2. Sin embargo, se benefician de un reemplazo valvular temprano aquellos que tienen riesgo quirúrgico bajo (puntaje < 4 según la Sociedad de cirujanos del tórax -STS, su sigla en inglés-) y tienen características adicionales de mal pronóstico, tales como síntomas desencadenados por una prueba de esfuerzo, calcificación severa de la válvula con limitación para su apertura, velocidad de flujo transvalvular > 5 m/s, gradiente medio > 60 mm Hg y aumento de la velocidad de flujo valvular de más de 0,3 m/s/año3. Recientemente, la actualización de la guía AHA/ACC de 2017 recomienda el reemplazo valvular aórtico quirúrgico tanto para los pacientes con estenosis aórtica severa sintomáticos (estadio D), como para los asintomáticos (estadio C) que cumplan con alguna indicación quirúrgica, siempre y cuando el riesgo quirúrgico sea bajo o intermedio3. Dicha recomendación podría interpretarse de dos formas: todos los pacientes con estenosis aórtica severa independientemente de los síntomas deberán ser llevados a reemplazo valvular aórtico quirúrgico, o los pacientes asintomáticos deberán tener alguna característica de mal pronóstico para ser llevados a cirugía; esta última interpretación es la que concuerda con el texto de las guías del 2014. Por su parte, la guía de la Sociedad Europea de Cardiología de 20174 propone que la observación en pacientes sin factores de mal pronóstico parece ser segura, mientras que es poco probable que la cirugía temprana traiga beneficio.

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Introducción: Los resultados del tratamiento quirúrgico de los aneurismas de aorta abdominal dependen de múltiples factores asociados al paciente y a la experiencia del grupo tratante. Objetivos: Describir los resultados obtenidos en el tratamiento quirúrgico de aneurismas de aorta abdominal por un grupo multidisciplinario y evaluar el impacto del volumen de casos año y de la experiencia adquirida. Métodos: Cohorte histórica de pacientes con diagnóstico de aneurisma de aorta abdominal sometidos a tratamiento quirúrgico desde junio de 1994 a junio de 2013, en la que se establecieron tres grupos: Grupo 1, aneurisma roto reparo abierto; Grupo 2, pacientes electivos reparo abierto; Grupo 3, pacientes electivos reparo endovascular. El desenlace primario a evaluar fue mortalidad hospitalaria, y el secundario, morbilidad asociada con el procedimiento. Para el análisis, la cohorte se dividió en dos: casos intervenidos de 1994 a 2002 y casos de 2002 a 2013, fundamentado en la implementación del programa endovascular en la institución en 2002. Resultados: Se intervinieron 573 pacientes en total. En el Grupo 1, 65; en el 2, 433, y en el 3, 75. La mortalidad global fue de 28,6, 2,8 y 1,3% respectivamente. Se demostró un incremento significativo en el volumen de casos (233%) y una reducción importante en la mortalidad de los casos abiertos después de 2002 (4,8 vs. 2,0%, p = 0,19). Conclusiones: La implemetación de la terapia endovascular ha contribuido a aumentar el número de pacientes tratados con aneurisma de aorta abdominal en nuestra institución. El aumento del volumen se tradujo en disminución efectiva de la mortalidad.

Introduction: Surgical treatment outcomes for infrarenal abdominal aortic aneurysm repair depend on patient factors and attending vascular team expertise. Objetives: To describe the outcomes of surgical treatment of abdominal aortic aneurysm performed by an interdisciplinary team and to evaluate the effects of annual caseload and acquired expertise. Methods: Historic cohort of patients with abdominal aortic aneurysm diagnosis surgically treated from January 1994 to June 2013. Three groups were established: Group 1 was for open ruptured abdominal aortic aneurysm repair, Group 2 was for open elective repair and Group 3 was for elective endovascular aneurysm repairs. Primary outcome was in-hospital mortality, and secondary outcomes were peri-procedural morbidity. For analysis the cohort was divided in two: cases repaired from 1994 to 2002 and from 2002 to 2013. This was based in the implementation of endovascular therapy from 2002 at the institution. Results: 573 patients were treated; Group 1: 65 patients; Group 2: 433 patients; Group 3: 75 patients. Mortality was 28.6%, 2.8% and 1.3% respectively. A significant rise in annual caseload (233%) was demonstrated and a trend toward lowering in surgical mortality (4.8 vs. 2.0%, p = 0.19) after 2002. Conclusions: Implementation of endovascular repair has contributed to rising annual caseload of patients with abdominal aortic aneurysm at our institution. This rising in volume correlates with effective lowering in mortality.

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Introducción: La mediastinitis, entidad de etiología multifactorial, ocurre hasta en 4% de los pacientes sometidos a revascularización miocárdica, y se reporta una mortalidad hospitalaria de 14% a 47%, que genera aumento en los costos de atención y deterioro de la calidad de vida así como de la sobrevida del paciente a largo plazo. Objetivo: Determinar cuáles antecedentes clínicos del paciente y factores relacionados con el procedimiento quirúrgico se asocian con la aparición de mediastinitis. Materiales y métodos: Diseño de casos y controles anidado en una cohorte histórica de pacientes sometidos a revascularización miocárdica en el periodo de enero de 2005 a julio de 2011. Los pacientes con mediastinitis se compararon con un grupo control sin mediastinitis tomados del mismo grupo de riesgo en una relación 1:4, y pareados por fecha de cirugía. El diagnóstico de mediastinitis se hizo con criterios clínicos, de laboratorio y hallazgos quirúrgicos. Resultados: Se identificaron treinta casos en el periodo relacionado. Los factores asociados a la aparición del evento fueron: diabetes mellitus OR 2,3 (1,1-4,9), uso de circulación extracorpórea OR 2,4 (1,1-5,5), tiempo de perfusión OR 1,1 (1,1-1,3) y pacientes mayores de 70 años OR 1,1 (1,2-1,4). Conclusiones: La mediastinitis sigue siendo una complicación de baja prevalencia con consecuencias devastadoras. El impacto clínico y económico de esta complicación debe obligar a los grupos quirúrgicos a crear estrategias de prevención con base en el conocimiento de los factores de riesgo de su población.

Introduction: Mediastinitis is a multifactorial entity which occurs in up to 4% of patients undergoing coronary artery bypass graft (CABG). It has a hospital mortality of 14% to 47%, which generates increased costs of care and deterioration of the quality of life and patient survival in the long term. Objective: To determine which clinical antecedents and factors related to the surgical procedure are associated with the occurrence of mediastinitis. Materials and methods: A nested case control study in a historical cohort of patients undergoing CABG in the period January 2005 to July 2011. Patients with mediastinitis were compared with a control group without mediastinitis taken from the same risk group in a 1:4 ratio and matched for date of surgery. The diagnosis of mediastinitis was made on clinical and laboratory criteria and surgical findings. Results: Thirty cases were identified in the related period. Factors associated with the occurrence of the event were: diabetes mellitus OR 2.3 (1.1 to 4.9), use of extracorporeal circulation OR 2.4 (1.1-5.5), OR 1 perfusion time, 1 (1.1 to 1.3) and patients older than 70 years OR 1.1 (1.2-1.4). Conclusions: Mediastinitis remains complications of low prevalence with devastating consequences. The clinical and economic impact of this complication should compel surgical groups to create prevention strategies based on knowledge of the risk factors of the population.

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