Coartación de aorta en pediatría: características clínicas de niños y adolescentes asistidos en el Centro Hospitalario Pereira Rossell / Coarctation of the aorta in pediatrics: clinical characteristics of children and adolescents treated at the Pereira Rossell Hospital Center / Coarctação da aorta em pediatria: características clínicas de crianças e adolescentes atendidos no Centro Hospitalar Pereira Rossell

La coartación de aorta (CoAo) es una cardiopatía congénita caracterizada por un estrechamiento de la aorta descendente distal al origen de la arteria subclavia izquierda, lo que determina una obstrucción al flujo sanguíneo. Es imperativo realizar un diagnóstico precoz y tratamiento oportuno para evitar complicaciones y la muerte en algunos casos. Se describen las características clínicas y anatómicas, así como el tratamiento y la evolución en niños menores de 15 años asistidos en un hospital pediátrico de referencia de Uruguay.

Coarctation of the aorta (CoAo) is a congenital heart disease characterized by a narrowing of the descending aorta distal to the origin of the left subclavian artery, which determines an obstruction to blood flow. It is imperative to make an early diagnosis and timely treatment to avoid complications and death in some cases. The clinical and anatomical characteristics are described, as well as the treatment and evolution in children under 15 years of age attended in a reference pediatric hospital in Uruguay.

A coarctação da aorta (CoAo) é uma doença cardíaca congênita caracterizada por estreitamento da aorta descendente distal à origem da artéria subclávia esquerda, o que determina uma obstrução ao fluxo sanguíneo. É imperativo fazer um diagnóstico precoce e tratamento oportuno para evitar complicações e morte em alguns casos. São descritas as características clínicas e anatômicas, bem como o tratamento e a evolução em crianças menores de 15 anos atendidas em um hospital pediátrico de referência no Uruguai.

Retos en el diagnóstico prenatal de coartación de aorta: actualización y perspectiva actual / Challenges associated with the prenatal diagnosis of aortic coarctation: update and current perspectives

Resumen La coartación de aorta es una cardiopatía congénita frecuente y potencialmente mortal. Su diagnóstico prenatal es un reto, ya que está dificultado por características inherentes a la circulación fetal, siendo relativamente bajas sus tasas de detección (30-50%). A diferencia de lo que sucede con otras cardiopatías congénitas, el diagnóstico prenatal de la coartación de aorta es en la mayoría de los casos solo de sospecha y únicamente podrá confirmarse de forma posnatal. Su identificación es de gran importancia, ya que mejora el pronóstico neonatal, y se basa sobre todo en la visualización de signos indirectos, como asimetría de cavidades o grandes vasos, con dominancia derecha. La principal limitación de estos es su bajo valor predictivo positivo, en especial en edades gestacionales tardías. Existen otros signos directos con mayor especificidad, como la hipoplasia de arco, el cociente istmo/ductus o el shelf contraductal, que en ocasiones solo son evidentes en el tercer trimestre dado el carácter evolutivo de la enfermedad. No obstante, ningún parámetro aislado presenta un rendimiento diagnóstico adecuado, siendo la combinación de algunos en distintos modelos multiparamétricos la que ha presentado mejores valores predictivos. Estos permiten al clínico un mejor asesoramiento a los padres, así como una planificación de la asistencia perinatal.

Abstract Coarctation of the aorta is a relatively common and potentially fatal congenital cardiac defect. Prenatal diagnosis remains a challenge, as it is limited by the inherent characteristics of the fetal circulation, with overall low detection rates (30-50%). Opposite to other congenital cardiac defects, prenatal diagnosis of coarctation of the aorta will be a suspicion one and can only be confirmed postnatally. Its identification is of paramount importance as it improves neonatal prognosis and is mainly based on the identification of indirect signs such as cardiac or great vessels asymmetry with right dominance. The main limitation of these signs is their low positive predictive value, especially in later gestational ages. There are direct signs with higher specificity such as arch hypoplasia, the isthmus/ductus ratio or the contraductal shelf, which may only be apparent in the third trimester given the progressive nature of the disease. However, no isolated parameter has an adequate diagnostic performance and it is their combination in multiparametric models that has shown the best predictive values. These models allow clinicians to give parents better counselling as well as tailor perinatal management.

Coexistencia infrecuente de la enfermedad de Tay-Sachs, coartación aórtica y reflujo vesicoureteral de grado V / Rare coexistence of Tay-Sachs disease, coarctation of the aorta and grade V vesicoureteral reflux

La enfermedad de Tay-Sachs es una enfermedad metabólica hereditaria neurodegenerativa. Existen cuatro tipos según el inicio de los síntomas clínicos: infantil, infantil de inicio tardío, juvenil y adulto. El tipo infantil tiene el peor pronóstico. Recientemente, se describieron diferentes anomalías que acompañan a los trastornos metabólicos e influyen en el pronóstico. Presentamos el caso de un lactante con enfermedad de Tay-Sachs junto con coartación aórtica y reflujo vesicoureteral bilateral (RVU) de grado V. Se realizó el seguimiento del paciente en el consultorio externo de Cardiología Pediátrica. En la ecografía abdominal, se observó ectasia pielocalicial, y se detectó reflujo vesicoureteral bilateral de grado V en la cistouretrografía miccional. No se ha informado previamente la coexistencia de estas anomalías. Este caso pone de manifiesto que no se deben subestimar las anomalías del examen neurológico en los pacientes con una cirugía cardíaca reciente, porque podría perderse la oportunidad de diagnosticar enzimopatías congénitas.

Tay-Sachs disease is a neurodegenerative inherited metabolic disease. There are four forms classified by the time of first clinical symptoms: infantile, late infantile, juvenile and adult. Infantile form has the poorest prognosis. Lately, different abnormalities which accompany metabolic disorders and affect the prognosis have been described. We present an infant with Tay-Sachs disease accompanied by coarctation of the aorta and bilateral grade V vesicoureteral reflux (VUR). The patient was followed up in the outpatient clinic of Pediatric Cardiology. The abdominal ultrasonography showed pelvicalyceal ectasia; bilateral grade V VUR in voiding cystourethrography was found. This coexistence has not been previously reported. This case emphasizes that abnormalities in the neurological examination of cardiac postsurgical patients should not be underestimated because the opportunity to diagnose inborn errors of metabolism could be missed.

Coarctação de aorta abdominal justarrenal associada à hipertensão renovascular e claudicação incapacitante: tratamento utilizando ponte ilíaco-birrenal e técnica "paving and cracking" / Coarctation of the abdominal aorta associated with renovascular hypertension and incapacitating claudication: repair with iliac-birenal bypass and paving and cracking technique

Resumo Coarctação da aorta abdominal é uma causa rara de claudicação de membros inferiores e hipertensão refratária. O tratamento é complexo e exige conhecimento de diversas técnicas de reconstrução vascular. Apresentamos um caso de coarctação ao nível das artérias renais, seu tratamento e revisão da literatura. Paciente feminina, 65 anos, com hipertensão refratária desde os 35 anos, utilizando cinco medicações anti-hipertensivas em dose máxima. Pressão arterial média de 260/180mmHg e claudicação incapacitante (menos de 20 metros) bilateral. Angiotomografia computadorizada demonstrou coarctação de aorta justarrenal de 4 mm de maior diâmetro, calcificação circunferencial no local da estenose e tortuosidade da aorta infrarrenal. Foi submetida a tratamento híbrido, com ponte ilíaco-birrenal e implante de stent Advanta V12 no local da estenose. A paciente evoluiu satisfatoriamente e, 60 dias depois da cirurgia, apresentava-se com uma pressão arterial de 140/80mmHg, em uso de apenas duas medicações anti-hipertensivas e sem claudicação.

Abstract Coarctation of the abdominal aorta is a rare etiology of intermittent claudication and refractory hypertension. Treatment is complex and requires knowledge of several vascular reconstruction techniques. We report a case of aortic coarctation at the level of the renal arteries, describing its treatment and presenting a literature review. Female patient, 65 years old, with refractory hypertension since the age of 35, using five antihypertensive medications at maximum doses. Blood pressure was 260/180mmHg and she had disabling claudication (less than 20 meters). Computed tomography angiography showed a 4mm coarctation in the juxtarenal aorta, with circumferential calcification at the stenosis site, and tortuous infrarenal aorta. Hybrid repair was performed with an iliac-birenal bypass and implantation of an Advanta V12 stent at the stenosis site. The patient's postoperative course was satisfactory, she was free from claudication, and her blood pressure 60 days after surgery was 140/80mmHg, taking two antihypertensive medications.

Endovascular treatment of coarctation of the aorta with a self-expanding endoprosthesis: how i do it using the braile dominus ® coarctation aorta device

Abstract The coarctation of the aorta is a relatively highly prevalent congenital heart disease and may be diagnosed as an underline cause of hypertension in adolescents and adults. The gold standard treatment for coarctation of the aorta in these patients is being replaced - from open surgery to endovascular therapy. Some prostheses have been developed to treat the coarctation with less acute and chronic complications. The Dominus® Coarctation Aorta (Braile Biomédica) is the first self-expandable prosthesis created specifically to treat coarctation of the aorta, reducing possible acute complications, like aortic rupture or aortic dissection. Here, we discuss the step-by-step method for using this prosthesis.

Isolated Coarctation Repair in Neonates and Infants Through Left Thoracotomy: Short-Term Outcomes

Abstract Introduction: End-to-end anastomosis and extended end-to-end anastomosis are typically used as surgical approaches to coarctation of the aorta (CoAo) with access at the subclavian artery or an interposition graft. The objective of this study is to analyze the impact of surgical and anatomical characteristics and techniques on early outcomes after surgical treatment of CoAo without cardiopulmonary bypass through left thoracotomy. Methods: This is a quantitative, observational, and cross-sectional analysis of patients who underwent repair of CoAo between July 1, 2010 and December 31, 2017. Seventy-two patients were divided into three groups according to age: 34 in group A (≤ 30 days), 24 in group B (31 days to one year), and 14 in group C (≥ 1 year to 18 years). Results: Aortic arch hypoplasia was associated in 30.8% of the cases, followed by ventricular septal defect (13.2%). The preductal location was more frequent in group A (73.5%), ductal in group B (41.7%), and postductal in group C (71.4%). Long coarcted segment was predominant in groups A and C (61.8% and 71.4%, respectively) and localized in group B (58.3%). Extended end-to-end anastomosis technique was prevalent (68%), mainly in group A (91.2%). Mortality in 30 days was 1.4%. Conclusion: Most of the patients were children under one year of age, and extended end-to-end anastomosis was the most used technique, secondary to arch hypoplasia. Further, overall mortality was low in spite of moderate morbidity in the first 30 postoperative days.

Double-lumen Aortic Arch: Persistence of the Fifth Aortic Arch?

Abstract Double-lumen aortic arch is a rare congenital anomaly related to persistence of the fifth aortic arch. It may be found alone or in association with other anatomical changes of the heart. We report a case of double-lumen aortic arch associated with coarctation of the aorta and patent ductus arteriosus in a child with a congenital malformation known as the VACTERL association (vertebral defects, imperforate anus, cardiopathy, tracheoesophageal fistula, renal abnormalities and limb anomalies).

Caso Clínico: Resolución quirúrgica de coartación de aorta en un paciente pediátrico / Case Report: Surgical resolution of aortic coarctation in pediatric patient

INTRODUCCIÓN: La coartación de aorta es una cardiopatía congénita con una incidencia de 4 por cada 10 000 nacidos vivos y puede asociarse o no a ductus arterioso persistente u otras malformaciones. Suele ser asintomático, normalmente se diagnostica mediante los signos clásicos; con gradiente de presión arterial entre extremidades, pulsos reducidos en extremidades inferiores, hipertensión arterial en miembros superiores o, en casos graves, fallo cardiaco izquierdo. Su resolución puede ser percutánea o quirúrgica, dependiendo de la edad y sus características. CASO CLÍNICO: Paciente masculino de 6 años, asintomático, con sospecha de coartación aórtica, por presentar una diferencia entre presiones arteriales de miembros superiores e inferiores, extremidades inferiores con pulsos reducidos y soplo sistólico en foco aórtico. Se solicitó ecocardiograma que informó válvula aórtica bicúspide con rafe, insuficiencia ligera y coartación de aorta; angiotomografía evidenció coartación de aorta yuxtaductal; cateterismo cardiaco objetivó casi nulo paso de contraste a través del defecto, por lo que se programó tratamiento quirúrgico. EVOLUCIÓN: La corrección quirúrgica se realizó mediante coartectomía con anastomosis termino-terminal más cierre del conducto arterioso. Posterior a la intervención se logró objetivar una mejora relevante en el gradiente de presión entre las extremidades. En el postquirúrgico el paciente presentó hipertensión que logró ser controlada, evolucionó favorablemente y recibió el alta a los 4 días sin tratamiento antihipertensivo ambulatorio. CONCLUSIÓN: La expectativa de vida en pacientes intervenidos para corregir su cardiopatía congénita es superior en relación a aquellos que no son intervenidos, por lo que el diagnóstico oportuno supone una herramienta importante para mejorar la calidad y esperanza de vida.

BACKGROUND: Coarctation of the aorta is a congenital heart disease with an incidence of 4 per 10 000 live births, it may or may not be associated with patent ductus arteriosus as well as other malformations. It is usually asymptomatic and diagnosed by its classic signs such as; arterial pressure gradient between extremities, reduced pulses in the lower extremities, arterial hypertension in the upper extremities or, in severe cases, left heart failure. Its resolution can be percutaneous or surgical, depending on the patient's age and the characteristics of the defect. CASE REPORTS: A 6-year-old male patient, asymptomatic, with suspected aortic coarctation, due to a difference between arterial pressures in the upper and lower limbs, lower limbs with reduced pulses, and a systolic murmur in the aortic focus. An echocardiogram was requested, which reported a bicuspid aortic valve with raphe, mild regurgitation, and coarctation of the aorta; CT angiography showed coarctation of the juxtaductal aorta; cardiac catheterization showed almost no passage of contrast through the defect, so surgical treatment was scheduled. EVOLUTION: Surgical correction was performed by coartectomy with end-to-end anastomosis and closure of the ductus arteriosus. After the intervention, a relevant improvement in the pressure gradient between the extremities was observed. In the postoperative period the patient presented hypertension, that we managed to control, the patient progressed favorably and was discharged after 4 days without antihypertensive treatment. CONCLUSION: Life expectancy in patients who underwent surgery to correct a congenital heart disease is higher than in those who don't, so timely diagnosis is an important tool to improve life quality and life expectancy.

Caracterização clínico-epidemiológica de crianças e adolescentes portadores de cardiopatia congênita / Clinical-epidemiological characterization of children and adolescents with congenital heart disease / Caracterización clínico-epidemiológica de niños y adolescentes con cardiopatía congénita

Objetivo: Analisar características clínico-epidemiológicas de crianças portadoras de cardiopatia congênita residentes do município de Rio das Ostras referenciadas para unidades especializadas no estado do Rio de Janeiro. Método: Pesquisa descritiva, transversal realizada em duas unidades hospitalares no município do Rio de Janeiro, através da técnica documental retrospectiva por meio de dados nos prontuários. A coleta ocorreu entre setembro de 2018 a fevereiro de 2019. Resultados: Foram analisados 48 prontuários, com maior proporção entre os escolares (33,3%); do sexo masculino (58,3%); Em relação as cardiopatias, verificou-se com maior proporção as acianóticas (62,5%); destacando a comunicação interatrial com (14,6%); seguida da comunicação interventricular (12,5%) e coarctação da aorta (12,5%). Conclusão: A identificação dessas crianças no município de Rio das Ostras e a distribuição destas pelo território nacional são informações imprescindíveis para o planejamento e implementação de programas e políticas públicas que atendam as reais demandas deste segmento populacional

Objetivo: Analizar las características clínicas y epidemiológicas de los niños con cardiopatía congénita que viven en la ciudad de Rio das Ostras remitidos a unidades especializadas en el estado de Río de Janeiro. Método: Investigación descriptiva, transversal realizada en dos hospitales de la ciudad de Río de Janeiro, a través de una técnica documental retrospectiva a través de datos en registros médicos. La recolección se realizó entre septiembre de 2018 y febrero de 2019. Resultados: Se analizaron 48 registros médicos, con una mayor proporción entre los estudiantes (33.3%); hombre (58,3%); Con respecto a la enfermedad cardíaca, hubo una mayor proporción de acianóticos (62.5%); destacando la comunicación interauricular con (14,6%); seguido de comunicación interventricular (12.5%) y coartación aórtica (12.5%). Conclusión: La identificación de estos niños en la ciudad de Rio das Ostras y su distribución en todo el territorio nacional son información esencial para la planificación e implementación de programas y políticas públicas que satisfagan las demandas reales de este segmento de la población

Objective: To analyze the clinical and epidemiological characteristics of children with congenital heart disease living in the city of Rio das Ostras referred to specialized units in the state of Rio de Janeiro. Method: Descriptive, cross-sectional research conducted in two hospitals in the city of Rio de Janeiro, through retrospective documentary technique through data in medical records. The collection took place between September 2018 and February 2019. Results: 48 medical records were analyzed, with a higher proportion among students (33.3%); male (58.3%); Regarding heart disease, there was a greater proportion of acyanotic (62.5%); highlighting interatrial communication with (14.6%); followed by interventricular communication (12.5%) and aortic coarctation (12.5%). Conclusion: The identification of these children in the city of Rio das Ostras and their distribution throughout the national territory are essential information for the planning and implementation of programs and public policies that meet the real demands of this population segment

Pseudocoartación aórtica en niños con ductus arterioso persistente: dos casos de una patología extremadamente infrecuente en menos de 48 horas / Aortic pseudocoarctation in children with persistent ductus arteriosus: two cases of an extremely uncommon pathology in less than 48 hours

Introducción: mientras la persistencia del ductus arterioso (PDA) es la cardiopatía congénita más frecuente, encontrar una pseudocoartación aórtica es muy poco frecuente y lo es más hallarlo en niños y solo hay unos cuantos casos reportados de este hallazgo en niños asociado a PDA. Material y Métodos: se describen los casos de 2 pacientes (1 con diagnóstico preoperatorio y otro sin él) con esta asociación: tanto los datos preoperatorios, los hallazgos transoperatorios, y su manejo trans y postoperatorio que operamos en menos de 48 horas. (AU)

Introduction: while the persistence of ductus arteriosus (PDA) is the most frequent congenital heart disease, finding an aortic pseudocoarctation is very rare and more find it in children and there are only a few reported cases of this finding in children associated with PDA. Material and Methods: we describe the cases of 2 patients (1 with preoperative diagnosis and another without it) with this association: the preoperative data, the transoperative findings, and their trans and postoperative management that we operated on in less than 48 hours. (AU)

Características de los pacientes pediátricos operados de coartación aórtica en los años 2009 a 2018 en el Instituto Nacional de Cardiología Ignacio Chávez / Characteristics of pediatric patients operated of aortic coartation in the years 2009 to 2018 at the National Institute of Cardiology Ignacio Chávez

Resumen Objetivo: Determinar las características radiográficas, electrocardiográficas, angiográficas y quirúrgicas de los pacientes operados con coartación aórtica en el Instituto Nacional de Cardiología Ignacio Chávez entre los años 2009 y 2018. Métodos: Se recabaron del expediente electrónico las variables consideradas relevantes para el estudio y se registraron en la hoja de captura Excel previamente diseñada. Se analizaron diversas variables radiográficas, electrocardiográficas, angiográficas y quirúrgicas. Se valoró la evolución clínica posquirúrgica, complicaciones, requerimiento de procedimientos intervencionistas o quirúrgicos, y se valoró la respuesta a estos y también los diferentes desenlaces. Resultados: Se reportan 187 cirugías de pacientes desde los 2 días de vida hasta los 12 años, en donde se mencionan las características radiológicas, electrocardiográficas y edad de presentación. El total de fallecimientos en los 9 años fue de 17 casos, los cuales en su gran mayoría (94%) fueron antes del primer año de vida. La técnica quirúrgica más utilizada (90%) fue la coartectomía extendida con anastomosis terminoterminal. Las complicaciones posquirúrgicas registradas fueron en orden descendente: insuficiencia ventricular izquierda, hipertensión arterial sistémica, insuficiencia renal que requirió diálisis peritoneal, etc. Conclusiones: La población registrada que requirió una intervención quirúrgica en el Instituto Nacional de Cardiología fue en su mayoría «lactante menor¼, con una mediana de 3 meses de edad en el momento de la cirugía. Respecto a la técnica quirúrgica empleada, la que tuvo mejores resultados y que no demostró tener una asociación significativa con el número de defunciones fue la coartectomía extendida con anastomosis terminoterminal.

Abstract Objective: To determine the radiographic, electrocardiographic, angiographic and surgical characteristics of the patients operated with aortic coarctation at the Ignacio Chavez National Institute of Cardiology between 2009 and 2018. Methods: The variables considered relevant for the study were collected from the electronic file, recorded in the previously designed Excel capture sheet. Various radiographic, electrocardiographic, angiographic and surgical variables were analyzed. Post-surgical clinical evolution, complications, requirements for interventional or surgical procedures were assessed, and the response to these as well as the different outcomes was assessed. Results: 187 patient surgeries are reported from 2 days of age to 12 years, where the radiological, electrocardiographic characteristics and presentation age are mentioned. The total number of deaths in the 9 years was 17 cases, which were mostly (94%) before the first year of life. The most commonly used surgical technique (90%) was extended coartectomy with term-terminal anastomosis. The recorded postoperative complications were in descending order: ventricular failure, systemic arterial hypertension, renal failure that required peritoneal dialysis, etc. Conclusions: The registered population that required surgical intervention at the National Institute of Cardiology was mostly a minor infant with a median of 3 months in their surgery age. In view of the surgical technique used, the one that had better results and that did not show a significant association with the number of deaths was the extended coartectomy with end-to-end anastomosis.

Morning glory disc anomaly associated with large facial infantile hemangioma as the presenting signs of PHACE syndrome / Anomalia de Morning Glory associada à extenso hemangioma infantil como apresentação da Síndrome PHACE

ABSTRACT Infantile hemangioma, the most common benign tumor in infancy, is usually an isolated condition occurring in many different locations in the body. However, large infantile hemangioma may be associated with other systemic malformations, including central nervous system, cerebrovascular, cardiac, and ophthalmology abnormalities, a condition termed PHACE syndrome. In this paper, we describe a case of PHACE syndrome that was presented with the unique association of a large facial infantile hemangioma and morning glory anomaly.

RESUMO O hemangioma infantil é a causa mais comum de tumor benigno na infância e usualmente é uma condição isolada podendo ocorrer em diferentes regiões do corpo. No entanto, hemangiomas infantil extensos podem ser associados com outras malformações sistêmicas incluindo anomalias no sistema nervoso central, cerebrovasculares, cardíacas e oftalmológicas, uma condição denominada síndrome PHACE. Neste trabalho, descrevemos o caso de um paciente com síndrome PHACE que se apresentou com um extenso hemangioma facial e anomalia de "morning glory".

Coartación de aorta / Coarctation of the Aorta

Resumen La coartación de aorta es una cardiopatía congénita con altas tasas de morbilidad y mortalidad, que usualmente es subdiagnosticada a pesar de la disponibilidad de herramientas diagnósticas. El grado de severidad de las manifestaciones clínicas de la coartación de aorta va a depender del grado de obstrucción, así como de la presencia de defectos cardiacos y lesiones extracardíacas asociados. En la población pediátrica la modalidad terapéutica mayormente utilizada es la reparación quirúrgica; mientras que la angioplastia con balón y la colocación de una endoprótesis son menos utilizadas en esta población, ya que asocian mayor riesgo de reestenosis con la consecuente reintervención, estas técnicas son principalmente utilizadas en pacientes mayores. A pesar del éxito en la reparación de la coartación de aorta, los pacientes deben continuar un seguimiento estrecho a largo plazo, que incluye mediciones de la presión arterial de manera periódica, así como estudios por imagen de la estructura cardíaca, debido a la aparición tardía de complicaciones cardiovasculares asociadas.

Abstract Coarctation of the aorta is a congenital heart disease with high rates of morbidity and mortality, which is usually underdiagnosed despite the availability of diagnostic tests. The degree of severity of the clinical manifestations of coarctation of the aorta will depend on the level of obstruction, as well as the presence of cardiac defects and associated extracardiac lesions. In the pediatric population the most used therapeutic modality is surgical correction; while balloon angioplasty and stent placement are less used in this population, since they are associated with a greater risk of restenosis with the subsequent reintervention; these techniques are mostly used in older patients. Despite the success in the repair of coarctation of the aorta, patients should continue a close long-term follow-up, which includes periodic blood pressure measurements, as well as imaging studies that assess the cardiac structure, due to the late onset of associated cardiovascular complications.

Coartación de aorta: causa de hipertensión arterial poco reconocida en el primer nivel de atención / Coarctation of the aorta: cause of low-recognized high blood pressure at the first level of care

La coartación de aorta es una causa poco frecuente de hipertensión arterial secundaria, asimismo representa aproximadamente el 5-10% de las cardiopatías congénitas. El presente reporte relata el caso de una mujer de 42 años, con hipertensión arterial refractaria, quien recibió un diagnóstico y manejo inadecuado en el primer nivel de atención. Finalmente, se le diagnosticó una coartación de aorta. Se resalta la importancia de una anamnesis detallada y el examen clínico en la práctica médica rutinaria y una buena anamnesis como herramienta principal de todo acto médico, así como la derivación oportuna a la especialidad correspondiente para su manejo. (AU)

Aorta coarctation is a rare cause of secondary high blood pressure, and also accounts for approximately 5-10% of congenital heart disease. This report relates the case of a 42-year-old woman, with a history of refractory high blood pressure, who received an inadequate diagnosis and management at the first level of care. Eventually, she was diagnosed with a coarctation of the aorta. It highlights the importance of detailed anamnesis and clinical examination in routine medical practice and good anamnesis as the main tool of any medical act, as well as the timely referral to the corresponding specialty for its management. (AU)

Coarctation of The Aorta: A Case-Series from a Tertiary Care Hospital

Abstract Background: Coarctation of the aorta is a congenital segmental narrowing of the aortic arch with severe hemodynamic repercussions and increased cardiovascular mortality. Early surgical correction and life-time echocardiographic follow-up must be performed to improve prognosis. However, this goal has been challenged by high rates of underdiagnosis, which delay surgical correction, and by recoarctation in up to one third of operated patients. Objectives: The objectives of this study were: (i) to register the frequency of common clinical signs at diagnosis of coarctation of the aorta; (ii) to describe the course of echocardiographic parameters before and during the follow-up of coartectomized subjects; (iii) to analyze the clinical prognosis of patients according to baseline characteristics, occurrence of recoarctation and associated malformations. Methods: Case-series of 72 patients coarctectomized between June 1996 and November 2016 in a tertiary care hospital. Clinical, echocardiographic and surgical variables were considered. All patients were submitted to coarctectomy by posterolateral thoracotomy and end-to-end anastomosis. Data were classified as parametric or non-parametric by Kolmogorov-Smirnov test. Parametric data were expressed as mean and standard deviation, and non-parametric data as median and interquartile range. Continuous variables were analyzed using paired t-tests, and categorical variables were compared by chi-square test. For all analysis, a p-value of less than 0.05 was considered statistically significant. Statistical analysis was performed using SPSS, version 20.0 (IBM, Chicago, IL, USA). Results: The mean follow-up time was 5.8 years (range: 0-20 years). At diagnosis, most patients had heart murmur (88%), non-palpable pulse in the lower limbs (50%), left ventricular hypertrophy (78%), and bicuspid aortic valve (33%), with a mean aortic peak gradient of 55 mmHg. After surgical correction, those without recoarctation were less symptomatic (60 vs 4.5%; p < 0.001), had lower aortic peak gradient (54 ± 3.8 vs 13 ± 0.8; p = 0.01) and left ventricle mass (95 ± 9.2 vs. 63 ± 11; p = 0.01), and the most common complications were late hypertension (39.2%), and recoarctation (27.6%). Recoarcted patients did not show improvement of neither clinical nor echocardiographic variables. Age at repair and bicuspid aortic valve groups had comparable results with controls. Surgical procedure was safe; mean time of hospitalization was 10 days and mean surgery time 2.3 hours. Conclusions: Coarctectomy improves cardiac symptoms and left ventricular hypertrophy, with a slight effect on the incidence of hypertension. Recoarctation occurs in one-third of patients and draws attention for the need of lifelong surveillance by echocardiography.