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.

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.

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.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

Marcadores sonograficos de cardiopatías congénitas: desproporción de las cavidades cardiacas / Sonographic markers of congenital heart disease: disproportion of the cardiac cavities

La coartación aórtica es una cardiopatía congénita (Cc) de muy difícil diagnóstico antenatal. La forma neonatal es de presentación crítica con dependencia ductal, por lo que la sospecha antenatal permite programar un adecuado manejo neonatal inmediato. En esta tercera publicación, destacamos la importancia de la visualización de una desproporción de las 4 cámaras como marcador sonográfico de Cc. En nuestra serie de Cc, hemos detectado 8 fetos con desproporción de las 4 cámaras; 3 de ellos resultaron portadores de coartación aórtica y 2 portadores de aneuploidias. Reportamos los hallazgos in utero, el resultado postnatal y la conducta más apropiada para la inclusión de este signo como marcador de coartación aórtica.

Coarctation of the aorta remain difficult to identify during the antenatal period. Prenatal detection of the shelf of the coarctation may be difficult to image in the fetus, perhaps related to the normal patency of the ductus arteriosus. The neonatal form is critical and ductus dependent. Antenatal detection could be useful to programme in utero transport or a more opportune intervention in the neonatal period. One of the markers of coarctation of the aorta is ventricular discrepancy or disproportion of the four-chamber view. Among 8 fetuses with disproportion diagnosed in our series, there were 3 coarctation and 2 chromosomal abnormalities. We discuss the clues for in utero detection of disproportion. We considered that the diagnosis of discrepancy between the chambers at the level of the four-chamber view is a tool for the prenatal diagnosis of coarctation of the aorta.

Follow-up results of balloon angioplasty for native coarctation of aorta.

Balloon angioplasty of native discrete (< 1 cm) coarctation of thoracic aorta was performed in 29 patients [aged 13 to 31 years (mean 21.5 +/- 5.8 years), 22 males and 7 females], from March 1993 to December 1995. The patients were followed up for two years. After angioplasty, peak systolic arterial pressure (PSAP) decreased from 173.5 +/- 18.1 mm Hg to 122.5 +/- 9.9 mm Hg (p < 0.001), peak systolic pressure gradient (PSG) decreased by 90.9 percent from 86.2 +/- 15.77 to 7.77 +/- 3.8 mm Hg (p < 0.001) and the coarcted segment diameter increased about three times from 3.7 +/- 1.6 to 10.9 +/- 3.3 mm (p < 0.001). PSG decreased to < 10 mm Hg in 27 patients (93%) whereas in the other two patients PSG was 17 and 13 mm Hg. Restenosis appeared in 2 patients (6.8%); aneurysm in one patient (3.4%) which remained static even after 1.3 years of follow-up; mild dissection in 3 patients (10.3%); temporary femoral artery occlusion in 2 (6.8%) patients who recovered with heparin injection. Though hypertension disappeared after balloon dilatation in all patients, it reappeared in 3 patients (10.3%) within 7 days and in 10 patients (34.5%) within 6 months. In conclusion, balloon angioplasty of CoA is a good alternative to surgical correction.

Intravenous digital subtraction angiography in the pre-operative evaluation of coarctation of the thoracic aorta.

Many authors have previously advocated the use of intravenous digital subtraction angiography (IV-DSA) in the pre-operative evaluation of coarctation of aorta with excellent results and good surgical correlation. We have studied 20 patients with coarctation of aorta by IV-DSA and compared the angiographic findings with those seen at surgery in nine patients. The IV-DSA examinations were performed by utilizing small-volume (25 ml), bolus injections of an ionic, iodinated water soluble contrast medium delivered in the mid-right atrium. Good quality diagnostic arterial images were obtained in 14 patients (70 per cent). Associated cardiac decompensation and inability to profile the coarct segment due to overlap by surrounding dilated blood vessels were responsible for the unsuccessful examinations. Whereas most IV-DSA findings were confirmed at surgery, the calibre and length of the coarct segment at IV-DSA were found to be unreliable predictors of the actual severity of the coarctation. The IV-DSA images failed to detect a localized diaphragm at the coarct site in two patients. Even though IV-DSA is useful for the diagnosis of aortic coarctation, the surgeon must be aware of its pitfalls in assessing the calibre and length of the coarct segment.

Aortoarteritis presenting as coarctation of aorta.

An interesting case of Aortoarteritis with clinical features simulating congenital coarctation of aorta (COA) is presented. Clinical pointers of differentiation of these conditions are discussed. Relevant literature is reviewed.