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

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

Anomalous origin of coronary arteries with an interarterial course: pictorial essay / Origem anômala de artérias coronarianas com trajeto interarterial: ensaio iconográfico

Abstract Coronary arteries originating from the contralateral (noncoronary) sinus and having an interarterial course, in which they run from the ascending aorta to the pulmonary trunk, is a potentially fatal anomaly. Computed tomography (CT) angiography facilitates the recognition and therapeutic planning of such anomalies because of its ability to acquire high-resolution images of the entire course of the coronary artery, as well as of the accompanying atherosclerotic involvement. The right coronary artery originating from the left coronary sinus is the most prevalent anomaly of this type and usually implies a better prognosis, the interarterial course being classified as "high" or "low", depending on whether it is above or below the level of the pulmonary valve, with consequent stratification of the risk and the treatment. However, it is known that there is a high risk of sudden death among patients with a left coronary artery of anomalous origin from the right sinus. In such cases, surgical treatment is recommended, regardless of whether there are symptoms or evidence of ischemia. Given the importance of those aspects, which can be identified on CT of the chest or CT angiography of the aorta, this pictorial essay aims to illustrate such anomalies to facilitate their recognition and description by radiologists who are not specialists in cardiac imaging.

Resumo O trajeto interarterial das artérias coronárias com origem em seio contralateral/não coronariano é uma anomalia potencialmente fatal caracterizada pelo trajeto das coronárias entre a aorta ascendente e o tronco da artéria pulmonar. A angiotomografia auxilia no reconhecimento e planejamento terapêutico dessas alterações, em virtude da sua capacidade em adquirir imagens de alta resolução de todo o trajeto coronariano, assim como do envolvimento aterosclerótico associado. A artéria coronária direita originada no seio coronariano esquerdo costuma ser mais prevalente e relacionada a um melhor prognóstico, sendo classificada em curso interarterial "alto" ou "baixo" de acordo com a altura do seu trajeto em relação à valva pulmonar, com consequente estratificação de risco e tratamento distintos. Sabe-se, entretanto, que há um elevado risco de morte súbita entre pacientes com artéria coronária esquerda de origem anômala a partir do seio direito, sendo recomendado tratamento cirúrgico, independentemente de sintomas ou evidência de isquemia. Em razão da importância desses achados que podem ser encontrados em exames de tomografia de tórax e angiotomografias de aorta, o presente ensaio tem por objetivo ilustrar as anomalias de trajeto das artérias coronárias, para facilitar seu reconhecimento e sua descrição por médicos radiologistas não especialistas em imagem cardíaca.

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Combination of ultrasound and MRI in the diagnosis of fetal thoracic abnormalities / 实用放射学杂志

Objective To compare and analyze the diagnostic value of prenatal ultrasound and MRI in fetal thoracic abnormalities, as well as the advantages and disadvantages, respectively, and to explore the clinical value of the combined use in diagnosing fetal thoracic abnormalities.Methods The prenatal ultrasound and MRI images of total 94 cases with thoracic abnormalities were analyzed retrospectively.All the patients received MRI exams within 2 days after the preliminary ultrasound diagnosis of abnormalities.All cases were confirmed by autopsy or postnatal follow-ups.Results 94 cases of thoracic abnormalities included 48 cases of cystic adenoma abnormalities, 33 cases of bronchopulmonary sequestration, 10 cases of diaphragmatic hernia and 3 cases of primary pulmonary hypoplasia.The diagnosis coincidence rate of ultrasound was 82.98% (78/94), while the combined use was 93.62% (88/94).There were statistical differences between the combined use and single ultrasound examination in detecting fetal thoracic abnormalities(P<0.05).Conclusion Both ultrasound and MRI could diagnose fetal thoracic abnormalities well and had its own advantages and disadvantages.The combined use of ultrasound and MRI could improve the sensitivity and specificity of prenatal diagnostic accuracy and have a better advantage in diagnosing fetal thoracic abnormalities.

Hernia diafragmática congénita de presentación tardía / Late-presenting congenital diaphragmatic hernia

La hernia diafragmática congénita de presentación tardía (HDCT) es una entidad infrecuente, representa del 5 a 20 % de todos los casos de hernia diafragmática congénita. La presentación clínica, diagnóstico y tratamiento difiere de la hernia de presentación neonatal, dada la ausencia de hipoplasia e hipertensión pulmonar. El amplio espectro clínico de la presentación tardía o su hallazgo radiológico incidental, pueden plantear un desafío diagnóstico; la demora en su detección y abordaje aumentan la morbimortalidad y ensombrecen el pronóstico. Presentamos el caso de una escolar de 8 años de edad a quien se le diagnostica una hernia diafragmática congénita izquierda y se hace revisión del diagnóstico, tratamiento y pronóstico de la enfermedad.

The late-presenting congenital diaphragmatic hernia is a rare entity that corresponds to the 5 - 20% of all congenital diaphragmatic hernia cases. The clinical manifestations ,diagnosis and treatment differ from the neonatal presentation in the absence of pulmonary hypoplasia and pulmonary hypertension. The wide clinical spectrum of the late-presenting diaphragmatic hernia or its incidental finding on radiological images can lead to a challenging diagnosis. The delay in the detection and approach increases the morbidity and mortality, and darkens the prognosis. We are presenting a case of an eighth year-old scholar who is diagnosed with a congenital left diaphragmatic hernia, as well as a review of the diagnosis, treatment and prognosis of this pathology.

Video-Assisted Thoracoscopic Division of Vascular Rings

This study reports our early experience with thoracoscopic division of vascular rings. Three patients were reviewed; their ages at surgery were 25 months, 4 years, and 57 years. All patients were suffering from complete vascular rings involving combinations of the right aortic arch, left ligamentum arteriosum, Kommerell's diverticulum, and retroesophageal left subclavian artery. The median surgical time was 180.5 minutes, and the patients showed immediate recovery. Three complications, namely chylothorax, transient supraventricular tachycardia, and left vocal cord palsy, were observed. Our early experience indicates that thoracoscopic division of a vascular ring may provide early recovery and could be a promising operative choice.

Surgical management of aortic arch obstruction associated with intracardiac anomalies in children / 中国医师杂志

Objective To summarize the clinical experience in repair of aortic arch obstruction associated with intracardiac anomalies in children retrospectively.Methods From March 2010 to March 2014,73 children diagnosed as coarctation of the aorta (CoA,n =68),interrupted aortic arch (IAA,n =3),and double aortic arch with CoA (n =2) underwent surgical management.Six of them were complicated with complex intracardiac anmalies,including tetralogy of Fallot (TOF,n =2),transposition of great arteries (TGA,n =1),total anomalous pulmonary venous connection (TAPVC,n =1),double outlet of right ventricle (DORV,n =1),and Shone's syndrome (n =1) ; the rest 67 patients were associated with ventricular septal defect (VSD) and other simple anomalies.Twenty eight cases had hypoplasia of the aortic arch.All the patients had one-stage repair except for one.The aortic arch reconstruction was end to end anastomosis between the descending aorta and the arch in 42 patients,end to side anastomosis in 22,and the aortic arch were enlarged using autologous pulmonary artery patch in 9.The associated intracardiac anomalies were repaired in the same stage.Results There were 2 deaths.The operative mortality was 2.7％.Renal failure was occurred in 2 cases who were cured afterwards by peritoneal dialysis.All survivors were followed up for 3 ～ 36 months,anastomotic restenosis was found in 1 case who underwent reoperation 14 months after the first operation.No neurological complications were occurred.Conclusions One-stage complete correction of CoA and IAA with intracardiac anomalies through median sternotomy can achieve excellent short-and mid-term surgical results.