RESUMEN
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.
Asunto(s)
Síndrome de Marfan , Humanos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Masculino , Femenino , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Disección Aórtica/cirugía , Adulto Joven , Aneurisma de la Aorta/cirugíaRESUMEN
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.
Asunto(s)
Aneurisma Roto , Implantación de Prótesis Vascular , Anomalías Cardiovasculares , Divertículo , Procedimientos Endovasculares , Humanos , Masculino , Persona de Mediana Edad , Anciano , Femenino , Aorta Torácica/cirugía , Implantación de Prótesis Vascular/métodos , Anomalías Cardiovasculares/cirugía , Aneurisma Roto/diagnóstico por imagen , Aneurisma Roto/cirugía , Aneurisma Roto/complicaciones , Procedimientos Endovasculares/métodos , Arteria Subclavia/cirugía , Divertículo/cirugíaRESUMEN
Popliteal arteriovenous fistulae (PAF) are anomalous communications between the arterial and venous systems of the lower extremity. They are usually secondary to trauma and are rarely associated with additional vascular defects. The coexistence of a PAF and a venous aneurysm is rare and usually occurs in patients with connective tissue disorders. Evidence regarding the management of this type of anomaly is scarce. However, both open and endovascular approaches seem feasible alternatives for treating this condition. Here, we describe a spontaneous popliteal arteriovenous fistula associated with a venous aneurysm in a 42-year-old male patient who presented with a popliteal mass. Satisfactory endovascular closure of the fistula and exclusion of the venous aneurysm were achieved using an Amplatzer™ Vascular Plug II.
Asunto(s)
Aneurisma , Fístula Arteriovenosa , Procedimientos Endovasculares , Adulto , Aneurisma/diagnóstico por imagen , Aneurisma/etiología , Aneurisma/cirugía , Fístula Arteriovenosa/diagnóstico por imagen , Fístula Arteriovenosa/etiología , Fístula Arteriovenosa/cirugía , Humanos , Masculino , Resultado del TratamientoRESUMEN
Three-dimensional printing (3DP) has recently gained importance in the medical industry, especially in surgical specialties. It uses different techniques and materials based on patients' needs, which allows bioprofessionals to design and develop unique pieces using medical imaging provided by computed tomography (CT) and magnetic resonance imaging (MRI). Therefore, the Department of Biology and Medicine and the Department of Physics and Engineering, at the Bioastronautics and Space Mechatronics Research Group, have managed and supervised an international cooperation study, in order to present a general review of the innovative surgical applications, focused on anatomical systems, such as the nervous and craniofacial system, cardiovascular system, digestive system, genitourinary system, and musculoskeletal system. Finally, the integration with augmented, mixed, virtual reality is analyzed to show the advantages of personalized treatments, taking into account the improvements for preoperative, intraoperative planning, and medical training. Also, this article explores the creation of devices and tools for space surgery to get better outcomes under changing gravity conditions.
Asunto(s)
Impresión Tridimensional , Realidad Virtual , Humanos , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos X , Sistema UrogenitalRESUMEN
Chronic manifestations of Chagas disease present as disabling and life-threatening conditions affecting mainly the cardiovascular and gastrointestinal systems. Although meaningful research has outlined the different molecular mechanisms underlying Trypanosoma cruzi's infection and the host-parasite interactions that follow, prompt diagnosis and treatment remain a challenge, particularly in developing countries and also in those where the disease is considered non-endemic. This review intends to present an up-to-date review of the parasite's life cycle, genetic diversity, virulence factors, and infective mechanisms, as well as the epidemiology, clinical presentation, diagnosis, and treatment options of the main chronic complications of Chagas disease.
RESUMEN
Coronary artery fistulas are rare coronary abnormalities. Most of these fistulas have a congenital origin, and only a few are acquired. We report the case of a patient with late-acquired multiple coronary fistulas secondary to a stab wound, diagnosed in the setting of ischemic heart failure secondary to coronary steal syndrome. (Level of Difficulty: Intermediate.).
RESUMEN
Despite nearly a century of research and accounting for the highest disease burden of any parasitic disease in the Western Hemisphere, Chagas disease (CD) is still a challenging diagnosis, primarily due to its poor recognition outside of Latin America. Although initially considered endemic to Central and South America, globalization, urbanization, and increased migration have spread the disease worldwide in the last few years, making it a significant public health threat. The international medical community's apparent lack of interest in this disease that was previously thought to be geographically restricted has delayed research on the complex host-parasite relationship that determines myocardial involvement and its differential behavior from other forms of cardiomyopathy, particularly regarding treatment strategies. Multiple cellular and molecular mechanisms that contribute to degenerative, inflammatory, and fibrotic myocardial responses have been identified and warrant further research to expand the therapeutic arsenal and impact the high burden attributed to CD. Altogether, cardiac dysautonomia, microvascular disturbances, parasite-mediated myocardial damage, and chronic immune-mediated injury are responsible for the disease's clinical manifestations, ranging from asymptomatic disease to severe cardiac and gastrointestinal involvement. It is crucial for healthcare workers to better understand CD transmission and disease dynamics, including its behavior on both its acute and chronic phases, to make adequate and evidence-based decisions regarding the disease. This review aims to summarize the most recent information on the epidemiology, pathogenesis, clinical presentation, diagnosis, screening, and treatment of CD, emphasizing on Chagasic cardiomyopathy's (Ch-CMP) clinical presentation and pathobiological mechanisms leading to sudden cardiac death.
Asunto(s)
Humanos , Femenino , Adulto , Síndrome Antifosfolípido/diagnóstico por imagen , Hipocinesia/diagnóstico por imagen , Síndrome Coronario Agudo/terapia , Síndrome Coronario Agudo/diagnóstico por imagen , Arteria Coronaria Izquierda Anómala/patología , Dolor en el Pecho , Ecocardiografía/métodos , Cateterismo Cardíaco/métodos , Heparina/química , Enoxaparina/química , Electrocardiografía/métodos , Angiografía por Tomografía Computarizada/métodos , Anticoagulantes/químicaRESUMEN
Pseudoaneurysms are rare complications of Blalock-Taussig fistulas. We present the case of an abscessed right pulmonary aneurysm after a Blalock-Taussig fistula in the context of a Salmonella bacteremia.
Asunto(s)
Aneurisma Falso/diagnóstico por imagen , Procedimiento de Blalock-Taussing/efectos adversos , Angiografía por Tomografía Computarizada , Arteria Pulmonar/diagnóstico por imagen , Aneurisma Falso/etiología , Aneurisma Falso/cirugía , Femenino , Humanos , Lactante , Arteria Pulmonar/cirugía , Tetralogía de Fallot/cirugíaRESUMEN
BACKGROUND: Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies. METHODS: An observational retrospective study was conducted at a tertiary referral centre for Colombian and Latin American paediatric cardiology. We analysed all cases of primary cardiomyopathies in children younger than 18 years of age who presented at our institution between 2010 and 2016. Cases of cardiomyopathies were classified according to World Health Organization guidelines. RESULTS: From a total of 29,533 children who attended our institution during the study period, 89 new cases of primary cardiomyopathies were identified. The median age at diagnosis was 11 years (interquartile range 4-9). Dilated cardiomyopathy accounted for 57.3% (n = 51) of cases; hypertrophic cardiomyopathy, 12.3% (n = 11); restrictive cardiomyopathy, 8.9% (n = 8); non-compacted cardiomyopathy, 7.8% (n = 7); arrhythmogenic ventricular cardiomyopathy, 6.7% (n = 6); and unspecified cardiomyopathy, 6.7% (n = 6). Heart failure was observed in 53.93% of the patients. The overall mortality was 12.36% (n = 11), which included two of eight patients who underwent cardiac transplantation.