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

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.

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

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.

Infectious endarteritis in aortic coarctation: two spectra of an infrequent disease.

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.

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

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.

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

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.

Young Man With Coarctation of the Aorta.

This case report discusses a diagnosis of double-orifice mitral valve in a young man with coarctation of the aorta.

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

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.

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.

Surgical Management for Late Presentation of Interrupted Aortic Arch with Aortopulmonary Window.

We report a case of advanced age at presentation of interrupted aortic arch with aortopulmonary window. Although the association between these two defects is well established, their overall prevalence is extremely low. This case presents us with an excellent opportunity to discuss decision making regarding aortic arch repair techniques according to age at which primary surgical correction is intended.

Hybrid Procedure for Coexistence of Coarctation of the Aorta and Aberrant Right Subclavian Artery: A Case Report and Review of the Literature.

Background: Coarctation of the aorta (CoA) can either present alone as an isolated condition or in association with other aortic arch or cardiac anomalies. One percent of patients with CoA have concomitant an aberrant right subclavian artery (ARSA). Purpose: We report the case of a 35-year-old woman with uncontrolled hypertension who was found to have CoA and ARSA. Results: The patient was treated successfully using a hybrid procedure comprising ARSA ligation and subclavian to carotid transposition, followed by thoracic endovascular aortic repair. Conclusions: Patients with CoA should be carefully studied, considering the possible coexistence of other congenital aortic arch defects, such as ARSA. Hybrid repair is a safe and effective approach for this condition.

Fetal neuroimaging findings in PHACE syndrome: case report and review of the literature.

OBJECTIVE: To describe the main prenatal sonographic and magnetic resonance imaging (MRI) features leading to the diagnosis of Posterior fossa malformations, Hemangiomas of the face, Arterial anomalies, Coarctation of the aorta and cardiac defects, and Eye abnormalities (PHACE) syndrome. The literature was also reviewed in order to determine the main neuroimaging features of fetuses with this condition. RESULTS: The index case was referred at 24 weeks' gestation with the probable diagnosis of Dandy-Walker malformation. Prenatal sonographic examination revealed hypoplasia of the left cerebellar hemisphere, hypoplasia of the cerebellar vermis, and enlarged cisterna magna (the "tilted telephone receiver sign"). Fetal MRI at 30 weeks confirmed the findings and also revealed an ipsilateral retrocerebellar cyst communicating with the asymmetrical dilated fourth ventricle, upward displacement of the left cerebellar hemisphere, and elevation of the ipsilateral tentorium. Postnatally, a large left facial segmental hemangioma as well as ipsilateral vascular intracranial malformations were identified, confirming the diagnosis of PHACE syndrome. A review of the literature revealed 11 reports describing 22 fetuses with prenatal imaging studies, including ours, confirming the high prevalence of specific posterior fossa abnormalities associated with PHACE syndrome. CONCLUSION: Our case and those reported in the literature support the observation that PHACE syndrome presents with characteristic features affecting the posterior fossa, which can be identified through prenatal sonography and fetal MRI.

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.

Endovascular Treatment of Coarctation of the Aorta with a Self-Expanding Endoprosthesis: How I Do It Using the Braile Dominus® Coarctation Aorta Device.

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.

Prenatal diagnosis of aortic arch anomalies: Echocardiography, 3D-ultrasonography, and computed tomography angiogram findings. A case-report.

We report the case of a fetus presenting aortic arch anomalies associated with a ventricular septal defect (VSD). This fetus, which was referred at 25 weeks of gestation, was suspected to have coarctation of aorta (CoA) evidenced by enlarged right chambers at the four-chamber view during a routine obstetric ultrasonographic scan. The prenatal diagnosis of CoA remains a challenge. Here, we review the ultrasonographic findings that could contribute to this diagnosis.

Myocardial strain pattern progress in patients with Coarctation of the Aorta undergoing aortic stenting.

BACKGROUND AND AIM: Ventricular function evaluation in coarctation of the aorta (CoA) has become more sophisticated and precise with speckle tracking, revealing subclinical changes. However, CoA stenting treatment effects in on myocardial strain are still controversial. This study aimed to estimate the extent to which changes in left ventricular global longitudinal strain (LV GLS) occur in patients with CoA who undergo stenting. METHODS: The study included 21 patients with CoA (median age: 15 years [8-39]) and 21 healthy individuals matched by age and gender. Clinical and echocardiographic evaluations were performed 1 day before, 6 months, and 1 year after stenting. Correlations between LV GLS and arm-leg gradient, isthmus gradient on echocardiogram, age at intervention, left ventricular mass, and ejection fraction were tested. RESULTS: Before treatment, patients with CoA had lower LV GLS than the control group (-18.4% ± 1.96 vs -21.5% ± 1.37; P < .01), showing significant increase to -19.4% ± 2.1 at 6 months and -20.7% ± 2.19 at 1 year, P < .001. Only 28.5% (6 patients) had preserved GLS before treatment, improving to 80.9% (17 patients) in 1 year. The only variable correlated with low LV GLS values before treatment was age at intervention (Spearman's index = -0.571; P = .007). CONCLUSION: Percutaneous therapy showed significant LV GLS improvement 12 months after aortic stenting. Older patients have lower GLS, suggesting that early intervention may have positive effects on preservation of LV systolic function.

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)

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

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. 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.

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.