Comparative analysis of the effectiveness of coarctation surgery between neonates and infants

SUMMARY OBJECTIVE: This study aimed to compare the effectiveness of resection and extended end-to-end anastomosis between neonate and infant patients with coarctation. METHODS: This study was designed retrospectively and included 41 neonate (<30 days) and infant (30 days to 1 year) patients who were operated on using the resection and extended end-to-end anastomosis technique for aortic coarctation. Preoperative aortic annulus diameters and Z scores, all aortic arch diameters and Z scores, the presence of hypoplastic aortic segment, and the presence of prematurity were reviewed in both groups. Subsequently, we investigated whether these parameters were statistically related to the residual gradient in the operation area, whether there was a need for early re-intervention, and what was the incidence of mortality in the early postoperative period. In addition, the aortic arch Z scores of the patients at 6 months postoperatively were examined. RESULTS: While the mean age (p<0.001), body weight (p<0.001), and proximal arch Z score (p=0.029) were found to be significantly lower in the neonate group than in the infant group, the total length of the intensive care unit stay (p=0.013) and the total length of hospital stay (p=0.017) were found to be significantly higher. In addition, significant enlargement was detected in the proximal arch, distal arch, and isthmus segments in both patient groups. CONCLUSION: The resection and extended end-to-end anastomosis is an equally effective technique that can provide a marked decrease in gradient in the coarctation area and a significant enlargement of the aortic arch segments in the early period after coarctation repair in both neonate and infant patients.

Application of Computed Tomography Angiography in Preoperative Diagnosis of Coarctation of Aorta and Evaluation of Aortic Dilatation in Infants

ABSTRACT Objective: To evaluate the occurrence of aortic dilatation and its associated predictors with coarctation of the aorta (CoA) in infants using multi-slice computed tomography (MSCT). Methods: The clinical data of 47 infantile patients with CoA diagnosed by MSCT and 28 infantile patients with simple ventricular septal defect were analyzed retrospectively. Aortic diameters were measured at six different levels, and aortic sizes were compared by z score. The coarctation site-diaphragm ratio was used to describe the degree of narrowing. Relevant clinical data were collated and analyzed. Results: The dilation rate and z score of the ascending aorta in the severe CoA group were significantly higher than those in the mild CoA group (11 [52.38%] vs. 21 [80.77%], P=0.038 and 2.00 ± 0.48 vs. 2.36 ± 0.43, P=0.010). Pearson's correlation analysis found that the z score of the ascending aorta was negatively correlated with the coarctation site-diaphragm ratio value (r=-0.410, P=0.004). A logistic retrospective analysis found that an increased degree of coarctation was an independent predictor of aortic dilatation (adjusted odds ratio 0.002; 95% confidence interval 0.00-0.819; P=0.043). The z score of the ascending aorta in the severe CoA group was significantly higher than that in the ventricular septal defect group (P<0.05). Conclusion: Most infants with CoA can also have significant dilatation of the ascending aorta, and the degree of this dilatation is related to the degree of coarctation. Assessment of aortic diameter and related malformations by MSCT can predict the risk of aortic dilatation in infants with CoA.

Single-Lung Ventilation in Infants for Surgical Repair of Coarctation of The Aorta Without Cardiopulmonary Bypass

ABSTRACT Objective: To investigate the effect of improving the operative field and postoperative atelectasis of single-lung ventilation (SLV) in the surgical repair of coarctation of the aorta (CoA) in infants without the use of cardiopulmonary bypass (CPB). Methods: This was a retrospective cohort study. The clinical data of 28 infants (aged 1 to 4 months, weighing between 4.2 and 6 kg) who underwent surgical repair of CoA without CPB from January 2019 to May 2022 were analyzed. Fourteen infants received SLV with a bronchial blocker (Group S), and the other 14 infants received routine endotracheal intubation and bilateral lung ventilation (Group R). Results: In comparison to Group R, Group S exhibited improved exposure of the operative field, a lower postoperative atelectasis score (P<0.001), reduced prevalence of hypoxemia (P=0.01), and shorter durations of operation, mechanical ventilation, and ICU stay (P=0.01, P<0.001, P=0.03). There was no difference in preoperative information or perioperative respiratory and circulatory indicators before SLV, 10 minutes after SLV, and 10 minutes after the end of SLV between the two groups (P>0.05). Intraoperative bleeding, intraoperative positive end-expiratory pressure (PEEP), and systolic pressure gradient across the coarctation after operation were also not different between the two groups (P>0.05). Conclusion: This study demonstrates that employing SLV with a bronchial blocker is consistent with enhanced operative field, reduced operation duration, lower prevalence of intraoperative hypoxemia, and fewer postoperative complications during the surgical repair of CoA in infants without the use of CPB.

Infectious endarteritis in aortic coarctation: two spectra of an infrequent disease / Endarterite infecciosa na coarctação da aorta: dois espectros de uma doença pouco frequente

ABSTRACT Objective: To describe two different degrees of clinical commitment and results in the evolution of infectious endarteritis in patients without a previous diagnosis of aortic coarctation. Case description: Two male patients aged 13 and 9 years old were admitted. The first due to a fever for 2 months, which started after dental cleaning, and the second due to high blood pressure, both patients with asthenia and weight loss. In the first case, the transthoracic echocardiogram showed aortic coarctation, and the transesophageal echocardiogram showed the presence of vegetations in the post-coarctation area, without pseudoaneurysms, with blood culture positive for Streptococcus mitis. This patient was treated for six weeks with crystalline penicillin, resolving the infection without complications. The second case was assessed for high blood pressure with a history of fever, and was treated with antibiotics. When performing a transthoracic echocardiogram, aortic coarctation was observed with a saccular image classified as a pseudoaneurysm by angiography and tomography. Blood culture was negative, and the patient developed an episode of hematemesis whose initial etiology could not be determined. Before surgical repair, he had a second episode of copious hematemesis with hypovolemic shock and death. Comments: We need to have a high index of clinical suspicion to establish the diagnosis of aortic coarctation complicated by endarteritis and start the appropriate antibiotic treatment, always maintaining surveillance for the early detection of pseudoaneurysms.

RESUMO Objetivo: Descrever dois diferentes graus de comprometimento clínico e resultados na evolução de endarterite infecciosa em pacientes sem diagnóstico prévio de coarctação da aorta. Descrição do caso: Dois pacientes do sexo masculino com idades entre 13 e nove anos foram internados. O primeiro por febre durante dois meses, iniciada após limpeza dentária. O segundo por hipertensão arterial. Ambos com astenia e perda de peso. No primeiro caso, o ecocardiograma transtorácico mostrou coarctação da aorta e o ecocardiograma transesofágico revelou vegetações na área pós-coarctação, sem pseudoaneurismas. A hemocultura foi positiva para de Streptococcus mitis. Este paciente foi tratado por seis semanas com penicilina cristalina, resolvendo a infecção sem complicações. O segundo caso foi avaliado pela presença de hipertensão arterial, com história de febre tratada com antibióticos. Ao realizar o ecocardiograma transtorácico, observou-se coarctação da aorta com imagem sacular classificada como pseudoaneurisma pela angiografia e tomografia. A hemocultura foi negativa. O paciente desenvolveu um episódio de hematêmese, cuja etiologia inicial não pôde ser determinada. Antes da correção cirúrgica, apresentou um segundo episódio de hematêmese profusa, com choque hipovolêmico e óbito. Comentários: Devemos ter um alto índice de suspeição clínica para poder estabelecer o diagnóstico de coarctação da aorta complicada com endarterite e iniciar o tratamento antibiótico adequado. É preciso manter a vigilância para a detecção precoce de pseudoaneurismas.

Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair

ABSTRACT Introduction: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. Methods: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. Results: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. Conclusion: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

Echo-guiding percutaneous aortic stent implantation for coarctation of the aortic: A case report / 中国胸心血管外科临床杂志

@#Currently, transcatheter intervention has emerged as a first-line treatment for coarctation of the aortic. Due to the radiation exposure associated with catheter interventional therapy, there are numerous restrictions, which harms both patients and medical personnel and is dependent on sizable radiation apparatus. Here, we report for the first time a case of echo-guiding percutaneous aortic stent implantation for a 27 years female patient of reproductive age. After discharge, the patient's aortic coarctation pressure decreased to 18 mm Hg, and the surgical results were satisfactory.

Establishment of a rat model of myocardial hypertrophy by a modified abdominal aortic coarctation method / 解剖学报

Objective To compare effectiveness between the modified and traditional pressure-overload myocardial hypertrophy(POMH) model by abdominal aorta coarctation (AAC) method. Methods Totally 45 rats were divided into three groups(n = 15 per group), sham group, traditional group, and modified group. In the traditional group, the diameter ol the abdominal aorta was narrowed to 0. 70 mm through a midline incision for 4 weeks; in the modified group, the diameter of the abdominal aorta was narrowed above the left kidney to 0. 45 mm for 1 week, and then the narrowing was lifted postoperatively. The cardiac index, heart weight (HW) /body weight (BW) and left ventricular index, left ventricular weight (LVW)/BW were measured from the heart specimens, and the cross-sectional area of cardiac myocytes, myocardial collagen area, and myocardial collagen area Iraction were measured in the pathological sections by HE staining and Masson staining. Results Compared with the sham group, the differences in end-systolic interventricular septum thickness (IVSs), left ventricular end-systolic posterior wall thickness (LVPWs), HW/BW, LVW/BW, cardiomyocyte cross-sectional area, myocardial collagen area, myocardial collagen area fraction, and brain natriuretic peptide (BNP) expression levels were statistically significant (P0. 05). Conclusion The modified abdominal aortic constriction method used in this experiment is time-saving, stable, homogeneous and easy to replicate, and is a more ideal approach to establish a rat model of POMH.

Enzima convertidora de angiotensina homóloga (ECA2) y actividad de ROCK en la falla cardíaca experimental / ACE 2 and ROCK activity in experimental congestive heart failure

Antecedentes: La ECA2 ha mostrado ser un regulador esencial de la funcionalidad cardíaca. En un modelo experimental de insuficiencia cardíaca (IC) con Fier, modelo de coartación de aorta (COA), se encontró activación de la vía Rho-kinasa. La inhibición de esta vía con fasudil no mejoró el remodelado cardíaco ni la disfunción sistólica. Se desconoce en este modelo, si el deterioro de la función cardíaca y activación de la vía rho-kinasa se asocia con una disminución de la ECA2 cardíaca y si la inhibición de Rho-kinasa tiene un efecto sobre la expresión de ECA2. Objetivo: Nuestro objetivo es determinar si en la falla cardaca experimental por coartación aórtica, los niveles proteicos de ECA2 en el miocardio se asocian a disfunción sistólica y cual es su interacción con la actividad de ROCK en el miocardio. Métodos: Ratones C57BL6J machos de 7-8 semanas se randomizaron en 3 grupos experimentales. Grupo COA por anudación de la aorta + vehículo; Grupo COA + Fasudil (100 mg/Kg día) por bomba osmótica desde la semana 5 post-cirugía; y grupo control o Sham. Se determinaron las dimensiones y función cardíaca por ecocardiografía. Posterior a la eutanasia, se determinaron los niveles de ECA2 del VI por Western-blot y actividad de la Rho-kinasa Resultados: En los grupos COA+vehículo y COA-FAS hubo deterioro de la función cardíaca, reflejada por la reducción de la FE (47,9 ± 1,53 y 45,5 ± 2,10, p < 0,05, respectivamente) versus SHAM (68,6 ± 1,19). Además, aumentaron las dimensiones cardíacas y hubo desarrollo de hipertrofia (0,53 ± 0,02 / 0,53 ± 0,01, p < 0,05) medida por aumento de la masa cardíaca relativa respecto del grupo SHAM (0,40 ± 0,01). En los grupos COA+vehículo y COA-FAS se encontró una disminución significativa del 35% en la expresión de ECA2 cardíaca respecto al grupo control. Conclusiones: La disfunción sistólica por coartación aórtica se asocia con aumento de la actividad de Rho-kinasa y significativa disminución de la expresión de ECA2. La inhibición de Rho-kinasa no mejoró el remodelado cardíaco, la disfunción sistólica y tampoco modificó los niveles de ECA2 cardíaca.

Background: ACE2 has been described as an essential regulator of cardiac function. In an experimental model of heart failure (HF) and heart failure reduced ejection fraction (HFrEF), the aortic coarctation (COA) model, activation of the Rho-kinase pathway of cardiac remodeling was found. Inhibition of this pathway did not improve cardiac remodeling or systolic ventricular dysfunction. It is unknown in this model whether the impairment of cardiac function and activation of the rho-kinase pathway is associated with a decrease in ACE2 and whether rho-kinase inhibition has an effect on ACE2 expression. Objective: To determine if in experimental heart failure due to aortic coarctation, ACE2 protein levels in the myocardium are associated with systolic dysfunction and what is its interaction with ROCK activity in the myocardium. Methods: Male C57BL6J mice aged 7-8 weeks were divided into 3 groups and anesthetized: One group underwent COA+ vehicle; A second group COA + Fasudil (100 mg/Kg/d) by osmotic pump from week 5 post-surgery and; the third group, control(SHAM). Echocardiograms were performed to determine cardiac dimensions and systolic function. Rats were then euthanized. Ventricular expression of ACE2, activity of the Rho-kinase pathway by MYPT-1 phosphorylation, relative cardiac mass, area and perimeter of cardiomyocytes were determined by Western blot. Results: In both COA+vehicle and COA+FAS groups there was deterioration of cardiac function, reflected in the reduction of EF (47.9 ± 1.53 and 45.5 ± 2.10, p < 0.05, respectively) versus the SHAM group (68.6 ± 1.19). In addition, cardiac dimensions and hypertrophy increased (0.53 ± 0.02 / 0.53 ± 0.01, p < 0.05) due to increased relative cardiac mass compared to the SHAM group (0.40 ± 0.01). In the COA+vehicle and COA+FAS groups a significant decrease of 35% in cardiac ACE2 expression was found compared to the control group. Conclusions: Systolic dysfunction due to aortic coarctation is associated with increased Rhokinase activity and a significant decrease in ACE2 expression. Rho-kinase inhibition did not improve cardiac remodeling, systolic dysfunction, nor did it change cardiac ACE2 levels.

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.

Ascending Aortic Coarctation - an Atypical Location in a Non-Takayasu Arteritis Female Patient

ABSTRACT Coarctation of the aorta is a well-known congenital cardiovascular disorder that typically occurs within proximity to the ductus arteriosus. The ascending aorta, distal descending aorta, and abdominal aorta are segments which are prone to development of an atypical coarctation. The etiologies of atypical cases are usually associated with various types of vasculitis syndromes or underlying genetic disorders. In this report, we present a 24-year-old female patient with an ascending aortic coarctation which developed secondary to an atherosclerotic process.

Using Revolution™ CT Angiography to Assess Complex Coarctation of the Aorta in Infants and Its Association with a Prolonged Postoperative Cardiac ICU Stay

ABSTRACT Objective: To investigate the accuracy of aortic dimensions measured by Revolution™ computed tomography (CT) in infants with complex coarctation of the aorta (CoA) and to further analyze the utility of the degree of CoA in predicting the risk of prolonged postoperative cardiac intensive care unit stay. Methods: A total of 30 infants with complex CoA who underwent surgical correction from January 2020 to July 2022 were retrospectively enrolled. General demographic data, preoperative imaging, and perioperative outcomes were collected. Univariate and multivariate analyses were performed to investigate predictors of prolonged postoperative cardiac intensive care unit stay, and the reliability of the CT measurements was assessed by the intraclass correlation coefficient. Results: All infants were divided into a mild or severe CoA group. The duration of mechanical ventilation and cardiac intensive care unit stay in the mild CoA group were significantly lower than those in the severe CoA group. After multivariate analysis, we found that the degree of CoA and age at surgery were significant predictors of prolonged postoperative cardiac intensive care unit stay. The intraclass correlation coefficient between CT measurements and intraoperative measurements was between 0.937 and 0.975, and the measurement results had good reliability. Conclusion: CT angiography can provide a comprehensive and accurate preoperative evaluation of aortic dimensions measured in infants with complex CoA. The degree of CoA is an independent risk factor for prolonged postoperative cardiac intensive care unit stay in infants with complex CoA.

Surgical management of neonatal coarctation of the aorta with aortic arch hypoplasia: A retrospective study in a single center / 中国胸心血管外科临床杂志

@#Objective     To summarize the surgical treatment experience in neonates with coarctation of the aorta (CoA) and aortic arch hypoplasia (AAH). Methods     The neonates with CoA and AAH who underwent surgical treatment in the Department of Pediatric Cardiac Surgery of Guangdong Provincial People's Hospital from 2013 to 2020 were retrospectively enrolled. The postoperative complications, long-term survival rate, and freedom from aortic reobstruction were analyzed. Patients undergoing extended end-to-end anastomosis were allocated into an extended end-to-end group, those undergoing extended end-to-side anastomosis into an extended end-to-side group, and those undergoing pulmonary autograft patch aortoplasty into a patch aortoplasty group. Results     Finally 44 patients were  enrolled, including 37 males and 7 females, aged 5.00-30.00 (19.34±7.61) days and weighted 2.00-4.50 (3.30±0.60) kg. There were 19 patients of extended end-to-end anastomosis, 19 patients of extended end-to-side anastomosis, and 6 patients of pulmonary autograft patch aortoplasty. The mean values of the Z scores of the proximal, distal, and isthmus of the aortic arch were –2.91±1.52, –3.40±1.30, and –4.04±1.98, respectively. The mean follow-up time was 45.6±3.7 months. There were 2 early deaths and no late deaths. Aortic reobstruction occurred in 8 patients, and 3 patients underwent reoperation intervention. The 5-year rate of freedom from reobstruction was 78.8%. The Cox multivariable regression analysis showed that the related factors for postoperative reobstruction were the Z score of the preoperative proximal aortic arch (HR=0.152, 95%CI 0.038-0.601, P=0.007) and the postoperative left main bronchus compression (HR=15.261, 95%CI 1.104-210.978, P=0.042). Conclusion     Three surgical procedures for neonates with CoA and AAH are safe and effective, but the aortic reobstruction rate in long term is not low. The smaller Z score of the preoperative proximal aortic arch and the postoperative left main bronchus compression are risk factors for long-term aortic reobstruction.

Construction and validation of an early predictive model for intraoperative massive transfusion of red blood cells in patients with Stanford type A aortic coarctation / 中国输血杂志

【Objective】 To analyze the risk factors for intraoperative massive red blood cell (RBC) transfusion in patients with Stanford type A aortic dissection (TAAD), in order to develop a risk-prediction model and validate its predictive effect. 【Methods】 The clinical data of 233 patients with TAAD admitted to our hospital from July 2018 to June 2021 (modeling set) were retrospectively analyzed. They were divided into routine transfusion group (n=128, RBC≤8 U) and massive transfusion group (n=105, RBC>8 U). Risk factors for intraoperative massive RBC transfusion in TAAD patients were analyzed by multivariate logistic regression and a risk prediction model was developed. Calibration curve and receiver operating characteristic (ROC) curve were used to assess the accuracy and discrimination of the model. In addition, 61 TAAD patients admitted to our hospital from July 2021 to May 2022 (validation set) were used for external validation. 【Results】 The rate of intraoperative massive RBC transfusion in 233 TAAD patients was 45.06% (95% CI: 38.59%-51.69%). Logistic analysis showed that women, age >50 years, preoperative Hb≤131.50 g/L, intraoperative bleeding >720 mL, and CPB time >155 min were independent risk factors for massive intraoperative RBC transfusion (P<0.05). The intraoperative risk prediction model formula for massive RBC infusion was: -4.427+ 0.925×gender+ 1.461×age+ 2.081×preoperative Hb+ 1.573×bleeding volume+ 2.823×CPB time. The area under the ROC curve of the modeling set and validation set were 0.904 (95% CI: 0.865-0.943) vs 0.868 (95%CI: 0.779-0.958), and the slopes of the calibration curves all converged to 1, indicating that the model predicted the risk of intraoperative massive RBC infusion in TAAD patients in good consistency with the actual risk of massive infusion. The decision curve shows that the model exhibits a positive net benefit with a threshold probability of 0.15-0.67 and has a high clinical application value. 【Conclusion】 The prediction model constructed based on the risk factors of intraoperative massive RBC infusion in TAAD patients can effectively predict the risk of intraoperative massive RBC infusion with high clinical predictive efficacy.

COARCTATION OF AORTA IN A 33 YEARS-OLD UNCONTROLLED HYPERTENSIVE FEMALE PATIENT – A CASE REPORT

We report a 33-year-female patient of hypertension detected at the age of 19 year. Her blood pressure was not well controlled. The case was investigated for secondary hypertension. CT Thoracic and Abdominal aorta Angiogram of the present case showing the narrowing in the thoracic aorta extending at the level of T8 to T10 vertebral body level for the length of 7.5cm. Reconstructed CT of the case showed multiple tortuous collaterals between the branches of internal mammary artery and external iliac artery and between axillary and intercostal artery. Relevant history was reviewed and discussed.

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.

Tratamiento en un tiempo quirúrgico de una coartación aortica asociada a valvulopatía aórtica bicúspide severa. Reporte de un caso / One stage surgical treatment of aortic coarctation associated with bicuspid aortic valve. Report of one case

Percutaneous surgery is the treatment of choice of isolated aortic coarctation in adults However, when there are other heart problems related to aortic coarctation, its surgical management may vary. We report a 41-year-old male presenting with aortic coarctation associated with severe, symptomatic, bicuspid aortic valve lesions and significant left ventricular dysfunction. He underwent open heart surgery for the surgical resolution of these problems. One year after surgery the results are satisfactory with no evidence of postoperative complications and a significant improvement of patient symptoms and left ventricular function.

Common Arterial Trunk with Interrupted Aortic Arch

Abstract Clinical data: Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries. Diagnosis: Association of aortic arch interruption type A is uncommon and should be considered. Operation: Debanding of pulmonary arteries allowing for possible future complete repair.

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.

Coarctation of the aorta in a young female treated with left subclavian artery to descending thoracic aorta bypass: a case report / Coarctação de aorta em jovem tratada com artéria subclávia esquerda para bypass de aorta torácica descendente: relato de caso

Abstract Coarctation of the aorta is a rare congenital abnormality, with an incidence of 6-8% of all congenital heart problems. It is usually diagnosed in childhood during routine clinical examination and adults mostly present with hypertension. Various investigations like transthoracic echocardiography, computed tomography, and magnetic resonance angiography can help with diagnosis. Prognosis depends on age at presentation and the severity of coarctation. Treatment options available are open and endovascular repair. Extra-anatomical bypass is the preferred option in cases with unfavorable anatomy. Long term follow up is required post repair due to risk of restenosis and aneurysm formation. Here is a case in which a young female presented with hypertension, was diagnosed with coarctation of the aorta, and was treated a left subclavian artery to descending thoracic aorta bypass. Her postoperative course was uneventful and she had improvement in hypertension.

Resumo A coarctação da aorta é uma anomalia congênita rara, com incidência de 6-8% entre todos os problemas cardíacos congênitos. É geralmente diagnosticada na infância durante o exame clínico de rotina, e os adultos normalmente apresentam hipertensão. Diversas investigações, como ecocardiografia transtorácica, tomografia computadorizada e angiorressonância magnética, podem auxiliar no diagnóstico. O prognóstico depende da idade na apresentação e da gravidade da coarctação. As opções de tratamento disponíveis são o reparo aberto e o reparo endovascular. O bypass extra-anatômico é a opção preferida em caso de anatomia desfavorável. O acompanhamento de longo prazo é necessário após o reparo devido ao risco de reestenose e formação de aneurisma. Descrevemos o caso de uma jovem do sexo feminino que apresentou hipertensão, a qual foi diagnosticada como coarctação da aorta. Foi realizada derivação da artéria subclávia esquerda para a aorta torácica descendente. O pós-operatório transcorreu sem intercorrências, e a paciente apresentou melhora da hipertensão.