Descending aortic translocation for left aortic arch with right descending aorta and coarctation of the aorta.

Left aortic arch with right descending aorta associated with coarctation of the aorta is a rare congenital cardiac anomaly. Conventional aortic arch repair in this condition may cause airway compression by the abnormally coursing descending aorta. We present the case of a neonate with this anomaly who underwent successful descending aortic translocation to prevent postoperative left main bronchial stenosis.

Treatment-refractory heart failure as a manifestation of aortic arch atresia.

BACKGROUND: Distal segment atresia (isthmus) is an extremely rare anatomical variant of obstructive aortic arch anomalies. CASE REPORT: We present the case of a newborn who, at 48 hours of life, presented a clinical picture of heart failure. The initial echocardiogram showed a congenital interrupted aortic arch type A, patent ductus arteriosus, and ventricular septal defect. Prostaglandins were initially indicated. Subsequently, a second echocardiogram showed the absence of ductus arteriosus; the CT angiography study confirmed this finding and revealed blood flow to the descending aorta through small intercostal blood vessels. The possibility of atresia of the distal segment (isthmus) of the aortic arch was considered and confirmed at the time of surgery. CONCLUSIONS: Aortic atresia should be considered a diagnostic possibility in the presence of type A interrupted aortic arch since the hemodynamic behavior between them is similar. Surgical medical treatment should be individualized since this condition is frequently an emergency in the neonatal period. However, this is not always the case, as other cases have been reported in schoolchildren and adults.

INTRODUCCIÓN: La atresia de segmento distal (istmo) de arco aórtico es una variante anatómica extremadamente rara de las anomalías obstructivas del arco aórtico. CASO CLÍNICO: Se presenta el caso de un recién nacido que a las 48 horas de vida presentó un cuadro clínico de insuficiencia cardiaca. El estudio de ecocardiograma inicial mostró una anomalía congénita de interrupción de arco aórtico tipo A, conducto arterioso y comunicación interventricular. De inicio se indicaron prostaglandinas. Posteriormente, el segundo ecocardiograma mostró la ausencia del conducto arterioso; el estudio de angiotomografía confirmó este hallazgo y también reveló flujo sanguíneo hacia aorta descendente a través de pequeños vasos sanguíneos intercostales. Se consideró la posibilidad de atresia del segmento distal (istmo) de arco aórtico y se confirmó al momento del acto quirúrgico. CONCLUSIONES: La atresia aórtica debe ser considerada como posibilidad diagnóstica en presencia de interrupción de arco aórtico tipo A, ya que el comportamiento hemodinámico entre ellos es similar. El tratamiento médico quirúrgico debe individualizarse, ya que es frecuente que sea una urgencia en el periodo neonatal. Sin embargo, no sucede así siempre, ya que se han reportado casos en escolares y adultos.

Cases of Ultrasound-Diagnosed Right Aortic Arch with Right Arterial Duct and the Treatment.

This paper aims to discuss the value of ultrasound to diagnose right aortic arch with right arterial duct. A retrospective analysis of fetal echocardiography characteristics of 10 fetuses who were diagnosed as right aortic arch with right arterial duct from December 2016 to March 2021 is made, and focus is put on the relationship between the aortic arch and arterial duct, and the position of aortic arch, arterial duct arch and trachea on the three vessels and trachea view (3VT). As a result, all 10 cases with right aortic arch and right arterial duct do not show aberrant left subclavian artery, and aortic arch with arterial duct are still connected as "V-shaped", and do not get vascular rings. In conclusion, 3VT can simply and clearly detect the right aortic arch, and the key to diagnosing the right aortic arch and right arterial duct is thorough inspection of the aortic arch, arterial duct, and trachea in their respective positions.

Merged bilateral arterial duct and circumflex retroesophageal right aortic arch in a fetus with normal intracardiac anatomy.

We report the case of a fetus with anamnios sequence and VACTERL syndrome, having a circumflex right aortic arch. Two arterial ducts join anteriorly to form a common vessel that connects to the pulmonary trunk with confluent pulmonary branches. Embryologically, the dorsal right 6th aortic arch did not disappear and the aortic arch development stopped in a symmetrical state with an exceptional "Y-shaped" merged bilateral arterial duct.

Swing-back and trap-door technique repair for interrupted aortic arch with right-sided descending aorta.

A right-sided descending aorta with a left aortic arch is a rare congenital anomaly in which the aortic arch crosses the midline from the left side of the trachea coursing to the descending aorta in the right thoracic cavity. The surgical repair of an interrupted aortic arch with a right-sided descending aorta carries great risks of bronchial and oesophageal obstruction. Herein, we describe a case of successful surgical repair of an interrupted aortic arch with a right-sided descending aorta using the swing-back and trap-door techniques.

The python aortic arch.

Aortic arch anomalies are rare congenital cardiovascular malformations. We present a case of a 9-year-old asymptomatic boy with an unusual, unknown arch anomaly.

An unusual case of interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta.

Interrupted aortic arch is a rare congenital anomaly in newborns and infants and is commonly associated with other cardiovascular anomalies. Here, we report an unusual case of type A interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta. Patent ductus arteriosus reconstituted the descending thoracic aorta. Proximal segments of the left common carotid and left subclavian arteries were atretic. Echocardiography-gated multidetector CT angiography not only identified the type of aortic arch interruption in the neonate but also delineated the exact anatomical details.

Síndrome de Turner en una adolescente / Turner syndrome in an adolescent

Se presenta el caso clínico de una adolescente ecuatoriana de 17 años de edad, blanca, quien nació producto de un embarazo normal y parto eutócico. Posterior al nacimiento se le realizó estudio genético que mostró la presencia de un cariotipo X0, por lo cual se le diagnosticó síndrome de Turner. A los 3 años fue operada de hipoplasia del arco aórtico con una evolución favorable. En la actualidad la paciente tiene baja talla con orejas de baja implantación, así como implantación alta del cabello en la nuca, tórax plano en forma de escudo, escaso bello pubiano y ausencia de menstruación

The case report of a 17 years Ecuadorian white adolescent is presented who was born from a normal pregnancy and eutocic delivery. After birth, a genetic study that showed the presence of a X0 Karyotype was carried out, reason why she was diagnosed Turner syndrome. She was operated of hypoplastic aortic arch when she was 3 years with a favorable clinical course. At the present time the patient has short height with ears of low implantation, as well as high implantation of the hair in the back of the neck, flat thorax in shield form, scarce pubic hair and amenorrhea

How frequent is the fifth arch artery?

Although usually shown in embryology textbooks, the presence of the fifth pair of pharyngeal arch arteries has long been controversial. To the best of our knowledge, six pairs of bilaterally symmetrical arteries developing within the pharyngeal arches are yet to be found in any mammalian or avian species. Collateral channels between the distal ends of the fourth and sixth arch arteries, in contrast, have been found in up to half of all developing mouse embryos. In only one human embryo, again to the best of our knowledge, has a channel been found that extends from the aortic sac to the dorsal aorta, and hence qualifies as an arch artery. Despite these confounding factors in terms of the developmental heritage of the fifth arch arteries, the purported channels are invoked with increasing frequency to describe various lesions discovered in the setting of the congenitally malformed heart. Persistence of the artery of the fifth arch was initially proposed to explain double-barrelled aorta. It was subsequently proposed to account for various systemic-to-pulmonary channels feeding the pulmonary circulation in the setting of pulmonary atresia. It has also been claimed to persist so as to explain abnormal branching of the brachiocephalic arteries from the aortic arch. In the light of the ongoing doubts concerning the existence of the arteries of the fifth arch themselves, we have reviewed the various descriptions of purported fifth arch arteries within the world literature. We have then sought to validate the descriptions on the basis of our own understanding of development, for this purpose providing images of the remoulding arch arteries in the mouse so as to substantiate our conclusions. While accepting that our own interpretations are speculative, we suggest that more convincing alternative explanations can be advanced to account for the majority of lesions currently interpreted on the basis of persistence of the arteries of the fifth arches. Although the interpretations do not necessarily change the therapeutic approaches to the channels, appropriate description is important in terms of their classification.

Crossed pulmonary arteries with hypoplasia of the transverse aortic arch.

BACKGROUND: The entity of crossed pulmonary arteries was first described by Jue, Lockman, and Edwards in 1966, in a patient with trisomy 18. Since then, several series have been described, both in terms of the isolated anatomic variant, or its association with other intracardiac or extracardiac anomalies. We describe a rare association that has previously not been reported. Methods and results Institutional Review Board approval for a retrospective chart review was obtained. Over the period 2011 through 2013, we have encountered six patients in whom the crossed origins of the pulmonary arteries from the pulmonary trunk were associated with hypoplasia of the transverse aortic arch, an association that, to the best of our knowledge, has previously not been reported. In all of the patients, the isthmic component of the aortic arch was inserted in an end-to-side manner into the ductal arch, with additional discrete coarctation in half of the patients. CONCLUSION: To the best of our knowledge, no cases of crossed pulmonary arteries have been described in association with hypoplasia of the transverse aortic arch. We draw comparisons between the cases with exclusively tubular hypoplasia, and those with the added problem of the more typical isthmic variant of aortic coarctation. In all cases, the ability to reconstruct cross-sectional images added significantly to the diagnosis and understanding of these complex lesions. These findings have specific surgical implications, which are discussed.

Cervical aortic arch with multiple aortic aneurysms that required two aortic replacements with a 34-year interval.

A 57-year old female was referred to our hospital after symptoms of hoarseness and dysphagia for a few years. She was diagnosed with a cervical vessel anomaly during childhood and had undergone surgery for a right-sided thoracic descending aortic aneurysm through a right thoracotomy at the age of 23 years. Now, enhanced computed tomography revealed newly developed multiple aneurysms in the aorta between the left carotid artery and the proximal descending aorta in the left hemithorax. A successful aortic surgical repair was accomplished via a left posterolateral thoracotomy. To our knowledge, this is the first case of a cervical aortic arch with formation of multiple aneurysms that required multiple surgeries via bilateral thoracotomies. This case suggests the inherent fragility and possibility of later aneurysmal formation in malformed vessels, thereby emphasizing the importance of periodical imaging tests in this rare congenital anomaly.

Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses.

OBJECTIVE: To analyse the main characteristics, associated conditions and outcome of right aortic arch (RAA) detected in fetal life, and to assess if further investigation is required in cases of isolated RAA. METHODS: Retrospective observational study of all fetuses diagnosed with a RAA between 2004 and 2012 at a tertiary centre for fetal cardiology. RESULTS: A RAA was identified in 98 fetuses: 27 had normal intracardiac anatomy and 71 were associated with other congenital heart disease (CHD); conotruncal anomalies being the most common. An aberrant left subclavian artery was diagnosed in 18.4% of cases, a double aortic arch in 6.1%, and 12.2% had a vascular ring confirmed after birth. Overall, an extracardiac anomaly was present in 31.6% of the patients and a chromosomal anomaly in 15.3%, with half of the latter cases being 22q11.2 microdeletion. Extracardiac and chromosomal anomalies were more commonly associated with RAA with structural CHD (39.4% and 19.7%, respectively), compared to cases of RAA with normal intracardiac anatomy (11.1% and 3.7%, respectively) (p<0.05). First year mortality was 10.3%, with all deaths being in cases with associated structural CHD. CONCLUSIONS: Detailed fetal extracardiac examination should be undertaken in all cases of RAA. Isolated RAA has a good prognosis, and in the majority of the patients it is an asymptomatic vascular variant with a relatively low risk for chromosomal anomaly. The prognosis of RAA with CHD depends on the complexity of the CHD and/or the associated extracardiac anomalies. In these cases, there is a higher risk for chromosomal anomaly, particularly 22q11.2 microdeletion.

Multi-detector computed tomography demonstration of double-lumen aortic arch--persistent fifth arch--as an isolated anomaly in an adult.

Persistent fifth - double-lumen - aortic arch is a rarely seen entity, which is usually associated with other cardiac anomalies; it has been previously reported only in children. We report a new case of double-lumen aortic arch with a systemic-to-systemic connection diagnosed incidentally during cardiac multi-detector computer tomography evaluation of coronary artery bypass grafts in an adult.