Fístula arteriovenosa para hemodiálisis en receptores de trasplante renal e Implicaciones en el síndrome cardiorenal tipo 4 / Arteriovenous fistula for hemodialysis in renal transplant recipients and implications in cardiorenal syndrome type 4

Introducción: La fístula arteriovenosa por vía quirúrgica para hemodiálisis constituye el acceso vascular de elección, para ese proceder depurador, no obstante, repercute negativamente sobre el aparato cardiovascular. Objetivo: Actualizar aspectos conceptuales y conductuales relativos a la permanencia de la fístula arteriovenosa para hemodiálisis posterior al trasplante renal. Métodos: Se revisó la literatura publicada en los últimos cinco años en las bases de datos; PubMed/Medline y Scopus y las fuentes de información; Cochrane Library y Ebsco. Resultados: La repercusión de la fístula arteriovenosa sobre el sistema cardiovascular tiene una marcada incidencia en diferentes alteraciones estructurales y funcionales del corazón que requieren de la adopción de una conducta destinada a evitar el riesgo. Se examinan puntos de vista convergentes y divergentes relacionados con la pertinencia de la fístula arteriovenosa como acceso vascular creado para hemodiálisis y la posibilidad de mantenerla luego del trasplante renal. Conclusiones: La fístula arteriovenosa supone el acceso vascular de apropiado para la supervivencia en hemodiálisis, sin embargo, las consecuencias de su permanencia sobre el aparato cardiovascular implican riesgo añadido de morbilidad y mortalidad(AU)

Introduction: Surgical arteriovenous fistula for hemodialysis is the vascular access of choice, this purifying procedure, however, has negative impact on the cardiovascular system. Objective: To bring up-to-date conceptual and behavioral aspects related to the permanence of the arteriovenous fistula for hemodialysis after renal transplantation. Methods: The literature published in the last five years was reviewed in PubMed/Medline and Scopus and information sources; Cochrane Library and Ebsco databases. Results: The repercussion of the arteriovenous fistula on the cardiovascular system has a marked incidence in different structural and functional alterations of the heart that require the adoption of a behavior to avoid risks. Convergent and divergent points of view related to the relevance of the arteriovenous fistula as a vascular access created for hemodialysis and the possibility of maintaining it after renal transplantation are examined. Conclusions: The arteriovenous fistula is the appropriate vascular access for survival in hemodialysis, however, the consequences of its permanence on the cardiovascular system imply an added risk of morbidity and mortality(AU)

Síndrome de Turner: nuestra experiencia en la creación del Registro Institucional y de la Unidad Interdisciplinaria en el Hospital Italiano de Buenos Aires: un primer paso para el mejor seguimiento / Turner syndrome: our experience in the creation of the Registry Institutional and Interdisciplinary Unit at the Italian Hospital of Buenos Aires: a first step to the best follow-up

El síndrome de Turner (ST) resulta de la ausencia completa o parcial del segundo cromosoma sexual en fenotipos femeninos. Tiene una incidencia de 1:2000- 2500 nacidas vivas. Recién en la última década se ha puesto atención a la salud de las adultas con ST. La mortalidad es 3 veces superior respecto de la población general debido al riesgo de disección aórtica por anomalías cardiovasculares estructurales y aterosclerosis vinculada a hipertensión arterial, diabetes, dislipidemia y obesidad. También presentan elevada prevalencia de enfermedades autoinmunitarias. Objetivo: evaluar la calidad del seguimiento clínico de pacientes adultas con ST, comparando los controles de salud preconformación y posconformación del Registro y de la Unidad Interdisciplinaria. En el año 2017 fuimos convocados para integrar el Programa de Enfermedades Raras del Hospital Italiano de Buenos Aires. A partir de la creación del Registro Institucional y del equipo multidisciplinario obtuvimos mejoría significativa en los controles por las especialidades de cardiología, endocrinología y otorrinolaringología, en los controles bioquímicos del metabolismo lipídico, hidrocarbonado, hepatograma, TSH y anticuerpos para celiaquía e imágenes cardiovasculares y densitometría ósea. En conclusión, el seguimiento sistematizado e institucional, mediante el Registro y la creación de la Unidad Interdisciplinaria de Síndrome de Turner, permitió encontrar las falencias del sistema de atención y optimizar el seguimiento de esta población. (AU)

Turner syndrome (TS) results from the complete or partial absence of the second sex chromosome in female phenotypes. It has an incidence of 1: 2000-2500 girls born alive. Only in the last decade has been paid attention to the health of adults women with TS. Mortality is 3 times higher than in the general population due to the risk of aortic dissection cause to structural cardiovascular anomalies and atherosclerosis related to hypertension, diabetes, dyslipidemia and obesity. They also have a high prevalence of autoimmune diseases. Until nowadays in Argentina do not exist a national registry of this disease that complies with the international follow-up recommendations for these patients. We proposed to develop the institutional register at 2014 and a multidisciplinary team was created to care and follow up girls and women with TS during 2015. It was indexed to Italian Hospital of Buenos Aires' Rare Diseases Program since 2017. After the creation of the institutional registry and the multidisciplinary team we obtained a significant improvement in cardiology, endocrinology and otorhinolaryngology schedule visits, in lipids and hydrocarbon metabolism, liver, thyroid and celiac diseases biochemical controls and in the performance of cardiovascular MNR and bone densitometry. In conclusion, the systematized and institutional follow-up, through the registry and the creation of the Interdisciplinary Unit of Turner Syndrome, allowed us to find the flaws of the care system and to optimize the follow up of this population. (AU)

Cardiovascular abnormalities in patients with oral cleft: a clinical-electrocardiographic-echocardiographic study

OBJECTIVES: The present study aims to describe the clinical, electrocardiographic, and echocardiographic cardiological findings in a group of patients with oral clefts. METHODS: This is a prospective cross-sectional study on 70 children (age range from 13 days to 19 years) with oral clefts who attended the multidisciplinary program of a university hospital from March 2013 to September 2014. The patients were evaluated by a pediatric cardiologist and underwent detailed anamnesis, physical examination, electrocardiogram, and echocardiogram. RESULTS: Sixty percent of the patients were male; 55.7% presented with cleft lip and palate, and 40.0% presented with health complaints. Comorbidities were found in 44.3%. Relevant pregnancy, neonatal, family and personal antecedents were present in 55.7%, 27.1%, 67.2%, and 24.3% of the patients, respectively. Regarding the antecedents, 15.2% of the patients presented with a cardiac murmur, 49.0% with a familial risk of developing plurimetabolic syndrome, and 6% with family antecedents of rheumatic fever. Electrocardiographic evaluation showed one case of atrioventricular block. Echocardiograms were abnormal in 35.7% of the exams, including 5 cases of mitral valve prolapse — one of which was diagnosed with rheumatic heart disease. CONCLUSION: The finding of a family risk of developing plurimetabolic syndrome and a diagnosis of rheumatic heart disease indicates that patients with oral clefts may be more prone to developing acquired heart disease. Thus, our findings highlight the importance of anamnesis and methodological triangulation (clinical-electrocardiographic-echocardiographic) in the investigation of patients with oral clefts and emphasize that cardiological follow-up to evaluate acquired and/or rhythm heart diseases is necessary. This strategy permits comorbidity prevention and individualized planned treatment.

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Ecocardiograma: da Solicitação do Exame pelo Pediatra à Realização pelo Cardiologista Pediátrico / Echocardiography: The Examination Request by the Pediatriciant Achieving the Pediatric Cardiologist

Fundamento: O ecocardiograma é uma ferramenta diagnóstica utilizada para avaliar anomalias cardíacas e esclarecer dúvidas quanto ao caráter benigno de certos achados do exame clínico cardiovascular. Objetivos: Identificar os principais motivos da solicitação do ecocardiograma pelo pediatra e avaliar o grau de ansiedade dos familiares gerado por essa conduta. Métodos: Foram incluídos pacientes com idade inferior a 18 anos, sem diagnóstico prévio de cardiopatia e encaminhados para o primeiro exame de ecocardiograma. Os familiares responsáveis pelos pacientes responderam a um questionário para avaliar o grau de ansiedade deles desde o pedido até a realização do ecocardiograma, com pontuação entre 0 e 19. Os dados foram analisados através de porcentagens, desvio padrão e teste T de Student. Resultados: Estudados 30 pacientes com idade média de 4,45 anos. O motivo de encaminhamento mais prevalente foi sopro cardíaco (23 casos), dos quais 70% não tiveram a hipótese de cardiopatia congênita confirmada após o ecocardiograma. A pontuação média no questionário de ansiedade foi 11 ± 6, sendo relacionado significativamente à presença de cardiopatia, com média de 13,0 ± 5,3 vs. 9,3 ± 5,2 no grupo sem a hipótese da mesma (p = 0,007). Conclusão: O achado de sopro foi o principal motivo para solicitação de ecocardiograma pelo pediatra. A confirmação de cardiopatia foi maior nos menores de 1 ano e com achado de sopro. Apesar do grau de ansiedade ter sido maior no grupo dos pacientes que tiveram o diagnóstico de cardiopatia congênita, este não foi desprezível nos familiares no grupo dos pacientes sem cardiopatia

Background: Echocardiography is a diagnostic tool used to evaluate cardiac anomalies and clarify doubts about the benign nature of certain findings of cardiovascular clinical examination. Objectives: To identify the main reasons for the request of echocardiography by pediatrician and evaluate the degree of anxiety of the family generated by this conduct. Methods: We included patients younger than 18 years without previous diagnosis of heart disease and referred for the first echocardiogram. Family members responsible for the patients answered a questionnaire to assess the degree of their anxiety from order to the performance of the echocardiogram, with scores between 0 and 19. Data were analyzed using percentages, standard deviation and Student's t test. Results: Studied 30 patients with a mean age of 4.45 years. The most prevalent reason for referral was heart murmur (23 cases), of which 70% patients had no congenital heart disease hypothesis confirmed, after echocardiography. The average score on the anxiety questionnaire was 11 ± 6 significantly associated with the presence of heart disease, with a mean of 13.0 ± 5.3 vs. 9.3 ± 5.2 in the group without the possibility of it (p = 0.007). Conclusion: The murmurs findings was the main reason for echocardiography request by the pediatrician. Confirmation of heart disease was higher in children under 1 year and with murmurs findings. Despite the anxiety level was higher in the group of patients who were diagnosed with congenital heart disease, this was not negligible in the family in the group of patients without heart disease

Hallazgo de arteria lusoria en el estudio de otitis media a repetición: a propósito de una observación pediátrica y revista breve de la literatura / Arteria Lusoria revealed by a accute media otitis: about a pediatric observation and brieve review of literatura

Arteria Lusoria (AL) o arteria subclavia aberrante derecha es una malformación vascular rara. Es la más común de las malformaciones del arco aórtico. Fue descrita por primera vez por Hunalud en 1735. La etiología no está clarificada hasta hoy en día. AL puede formar parte de un cuadro sindrómico (síndrome de Down, síndrome de Edwards) o de una malformación cardiaca compleja. Es casi siempre asintomática, pero algunas veces puede revelarse por signos respiratorios o disfagia. Se elige habitualmente el tratamiento conservador. Les presentamos el caso de un niño de 2 años que presentó una AL revelada atipicamente por otitis media a repetición.

Arteria Lusoria (AL) or aberrant right subclavian artery is a rare congenital aortic arch malformation. Hunauld described it for the first time in 1735.The cause is until now no totally clarified. Usually it is associated with chromosomal disorders such as Down's syndrome, Edwards's syndrome or appears in childhood with complexes cardiovascular abnormalities. Patients are frequently asymptomatic, but it may be revealed by respiratories symptoms or by a dysphagia. The treatment is generally conservative. We expose a case report about a child of 2 years old with AL diagnosed after several episodes of purulent otitis.

Aberrant right subclavian artery-esophageal fistula: massive upper gastrointestinal hemorrhage secondary to prolonged intubation / Fístula de artéria subclávia direita anômala com esôfago - hemorragia digestiva alta maciça secundária a intubação gástrica prolongada

ABSTRACT Aberrant right subclavian artery-esophageal fistula is a rare but potentially fatal complication. It may be associated with procedures, such as tracheostomy and tracheal or esophageal intubation, and yields massive upper gastrointestinal bleeding difficult to identify and to control. A high index of suspicion is essential for early diagnosis and better prognosis. We report a rare case of a patient who survived after emergent surgical procedure for massive upper gastrointestinal bleeding secondary to aberrant right subclavian artery-esophageal fistula after prolonged intubation.

RESUMO A fístula de artéria subclávia direita anômala com o esôfago é uma complicação rara, mas potencialmente fatal. Pode estar associada a procedimentos como traqueostomia e intubação traqueal ou esofágica e originar hemorragia digestiva alta maciça, de difícil identificação e controle. Um elevado índice de suspeição é essencial para o diagnóstico precoce e a melhoria do prognóstico. Relatamos caso raro de doente que sobreviveu após intervenção cirúrgica emergente por hemorragia digestiva alta maciça secundária a fístula de artéria subclávia direita anômala com esôfago, após intubação gástrica prolongada.

Foramen oval permeable / Patent foramen ovale

La frecuencia del foramen oval permeable es del 25% en la población general. En 40% de los eventos cerebrales isquémicos no hay una causa demostrable, pero en este grupo de pacientes el 46% tienen foramen oval permeable demostrable; una vez que este es capaz de permitir un cortocircuito de derecha a izquierda, existe la posibilidad del paso de un trombo que cause un evento isquémico vascular cerebral. Caso clínico: Paciente de 24 años que sufrió dos episodios de ataque isquémico transitorio sin causa determinada, dentro de los estudios de imágenes se le realizó eco-cardiograma y se encontró presencia de foramen oval permeable. Una vez que se diagnosticó la presencia del foramen oval sin otra causa que explicara el evento isquémico transitorio y además por tener cortocircuito espontáneo, se decidió el cierre percutáneo. Conclusión: en este caso se decidió por el procedimiento quirúrgico y se tomó el tamaño del foramen, los ataques isquémicos reincidentes y la edad de la paciente. Los resultados en un periodo de 2 años han sido totalmente satisfactorios...(AU)

"Aunque usted no lo crea": doble arco aórtico descubierto en búsqueda de un cuerpo extraño / "Believe it or not": Double aortic arch discovered in search of a foreign body

Se presenta caso de paciente de dos años de edad, quien ingresó al hospital con sospecha fundada de cuerpo extraño en vía aérea, luego de haber presentado un evento asfíctico. Sin embargo, al realizar la broncoscopía, se encontró una compresión extrínseca pulsátil diagnóstica de anillo vascular. Se confirmó el diagnóstico con la angiotomografía que reveló doble arco aórtico completo, además de mos- trar el cuerpo extraño vegetal (semilla de licha o rambután, Naphelium lappaceum ) ubicado en el tercio distal del esófago. A pesar de las limitantes propias de la institución logramos realizar el diagnóstico clínico, e imagenológico de la paciente además de resolver la patología de urgencia extrayendo el cuerpo extraño en vía digestiva en 24 horas tras su ingreso...(AU)

Omega-3 intake in people with epilepsy under regular hemodialysis program: here to stay / Suplementacao de omega 3 em pessoas com epilepsia submetidas a um programa regular de hemidialise: veio para ficar

Among the many risk factors suggested for sudden unexpected death in epilepsy (SUDEP), higher frequency of seizures is a very consistent issue. Furthermore, it has been established that hemodialysis-associated seizure is a complication of the dialysis procedure. Thus, since a possible relation between cardiovascular abnormalities and SUDEP among patients with chronic renal insufficiency in regular hemodialysis program should not be neglected, we propose in this paper that omega-3 fatty acids offer opportunities for prevention of sudden cardiac death or improved treatment in people with epilepsy under the regular hemodialysis program.

Dentre os fatores de risco sugeridos para a morte súbita inesperada em epilepsia (SUDEP), maior frequência de crises epilépticas é uma questão muito consistente. Além disso, tem sido estabelecido que as crises epilépticas associadas à hemodiálise são uma complicação do procedimento dialítico. Desse modo, como a existência de uma possível associação entre as alterações cardiovasculares e a SUDEP entre indivíduos com insuficiência renal crônica em tratamento dialítico não deve ser negligenciada, foi proposto neste artigo que o ômega 3 pode atuar na prevenção da morte súbita cardíaca ou melhorar o tratamento de pessoas com epilepsia inseridas em um programa regular de hemodiálise.

Evaluation of Cardiovascular Anomalies in Patients with Asymptomatic Turner Syndrome Using Multidetector Computed Tomography

Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.

Agenesia da veia cava inferior / Agenesis of the inferior vena cava

Agenesia da veia cava inferior é uma malformação rara. Sua causa mais comum é a disgenesia durante a embriogênese, mas também pode estar relacionada a trombose intrauterina ou perinatal. Normalmente é assintomática, em associação, ou não, com outras malformações congênitas, e pode cursar com maior risco de insuficiência venosa crônica e trombose venosa profunda, especialmente em jovens. Seu diagnóstico frequentemente é acidental, durante cirurgias abdominais ou procedimentos radiológicos. Relatamos cinco casos de agenesia da veia cava inferior detectada durante procedimentos eletrofisiológicos.

Agenesis of the inferior vena cava is a rare malformation. Its most common cause is dysgenesis during embryogenesis, but it may also be related to intrauterine or perinatal thrombosis. It is usually asymptomatic, associated or not with other congenital malformations and may be related to increased risk of chronic venous insufficiency and deep vein thrombosis, especially in young individuals. Diagnosis is often incidental, during abdominal surgery or radiological procedures. We reported five cases of agenesis of the inferior vena cava detected during electrophysiological procedures.

Compression of undiagnosed aberrant right subclavian artery during transesophageal echocardiography probe insertion.

Transesophageal echocardiography (TEE) has become an important monitoring tool for the anesthesiologist during repair of intracardiac defects. Although the incidence of reported complications associated with its use is low, one should be careful during the insertion and use of TEE probe, as it may result in potential devastating problems. We present a case of undiagnosed aberrant right subclavian artery (ARSA) that got compressed by the TEE probe during its insertion. It was noticed because of the presence of the right radial artery catheter, else it would have passed unnoticed.

Congenital duodenal obstruction due to a preduodenal portal vein.

Only about 80 cases of pre-duodenal portal vein (PDPV), a rare congenital anomaly of portal vein development that was first described by Knight in 1921, have been reported till date.[1] We report a neonate with duodenal obstruction caused by PDPV and briefly review the available literature.

Moebius syndrome with total anomalous pulmonary venous connection.

Moebius syndrome is characterized by congenital complete or partial facial nerve palsy with or without paralysis of cranial nerves and often associated with other malformations. Cardiac anomalies though known are very rare and till date only 4 cases have been reported. We present a case of Moebius syndrome with supracardiac total anomalous pulmonary venous connection which has not yet been reported in literature.

Vascular rings: an important cause of persistent respiratory symptoms in infants and children.

OBJECTIVE: To study the pattern of symptomatic vascular rings in infants and children with particular reference to clinical features, investigations and management. SETTING: Tertiary Care Paediatric Cardiology Division at the Royal Hospital, Muscat, Sultanate of Oman. DESIGN: Retrospective study. METHODS: Data on all patients aged less than 13 years diagnosed to have symptomatic vascular rings at the Royal Hospital, Muscat, Oman from 1992 to 2001 were retrieved from hospital records. RESULTS: Sixteen patients, 8 males and 8 females, aged 15 days to 36 months were identified, and included 12 with double aortic arch and 4 with right aortic arch, aberrant left subclavian artery and ligamentum arteriosum. Associated intracardiac defects were not found in any. Noisy breathing (7/16), stridor (6/16), respiratory distress (5/16) and recurrent respiratory infections (5/16) were the common modes of presentation. The duration from onset of symptoms to diagnosis ranged from 3 weeks to 24 months. Besides suggestive clinical features, the diagnosis was suspected from the chest radiograph (presence of right aortic arch) in 7 patients and from 2-Dimensional echocardiography and Doppler studies in 5 patients. Barium swallow studies, however, documented the abnormal esophageal indentation in all the 16 patients. Confirmatory imaging techniques included digital subtraction angiography in 7/16, cardiac catheterization and angiography in 5/16, and CT and MRI of the chest in 2 each. All 16 patients underwent successful surgical repair, and 13/16 became asymptomatic. 3 patients continued to suffer from occasional respiratory infections. CONCLUSIONS: A high index of clinical suspicion coupled with the use of barium oesophagraphy enabled early diagnosis in patients with symptomatic vascular rings, and surgical repair could be performed successfully in all patients.