Tratamiento endovascular exitoso de aneurisma del arco aórtico en un paciente pediátrico / Successful endovascular treatment of aortic arch aneurysm in a pediatric patient

Resumen Antecedentes: Los aneurismas de la aorta ascendente son patologías poco frecuentes en la infancia, sobre todo en ausencia de enfermedades previas como el síndrome de Marfan. Objetivo: Dar a conocer la posibilidad del manejo endovascular exitoso de los aneurismas de grandes vasos, usando stent y micro catéter con embolización del saco aneurismático. Método: Presentamos el caso de una paciente de 10 años y 2 meses, previamente sana, en quien se documentó un pseudoaneurisma entre el origen de la arteria carótida común izquierda y la arteria subclavia izquierda, que logró manejarse de forma endovascular, inicialmente con un stent cubriendo el cuello del aneurisma con el fin de remodelarlo y posteriormente por medio de microcatéter se realizó embolización del saco del aneurisma con coils, con resultado exitoso. Resultados: Los aneurismas de los grandes vasos, como la arteria carótida común y la arteria subclavia, tienen riesgo de ruptura con complicaciones devastadoras; el manejo endovascular se plantea como una opción poco invasiva de manejo, con resultados favorables. Conclusión: El manejo de aneurismas de grandes vasos, por vía endovascular usando stent y microcatéter con embolización del saco aneurismático, es una opción novedosa de manejo que logra resultados exitosos.

Abstract Background: Ascending aortic aneurysms are rare pathologies in childhood, especially in the absence of previous diseases such as Marfan syndrome. Objective: Present the possibility of successful endovascular management of large vessel aneurysms, using stents and microcatheters with embolization of the aneurysm sac. Method: We present the case of a previously healthy ten-year-old patient, in whom a pseudoaneurysm was documented between the origin of the left common carotid artery and left subclavian artery, successfully managed endovascularly, initially with a stent covering the neck of the aneurysm to remodel it and later with embolization of the aneurysm sac using a microcatheter. Results: Aneurysms of large vessels, such common carotid artery and subclavian artery, are at risk of rupture with devastating complications; endovascular management is considered a minimally invasive management option, with favorable results. Conclusion: The endovascular management of large vessel aneurysms using stents and microcatheters with embolization of the aneurysmal sac is a novel management option that achieves successful results.

A rare case of peripartum cardiomyopathy with takayasu arteritis

29 years old primigravida 38.3 weeks came with complaints of bleeding per vaginum and sudden onset breathlessness. Major finding on examination was that there was no pulse in the left hand along with raised BP, low SPO2 and B/L crepitations. Emergency management was given and patient stabilized temporarily. D/D were discussed with acute LVF due to PPCM most likely and patient was taken up for emergency LSCS. Post section 2D ECHO was performed showing decreased EF. Peripartum cardiomyopathy is an uncommon but potential life-threatening cardiac failure of unknown etiology, encountered late in pregnancy or in the postpartum period. Diagnosis of PPCM should essentially include echocardiographic substantiation of left ventricular dysfunction. TA is a vasculitis that mainly affects women of childbearing age, so it is possible to find pregnant patients with the disease. Women with Takayasu arteritis require multidisciplinary management prior to and during pregnancy. Monitoring for and reducing risk of preeclampsia, FGR and thromboembolic disease are helpful in achieving favorable outcomes.

A Truncus Bicaroticus and an Aberrant Right Subclavian Artery. A Rare Case and Literature Review / Un Tronco Bicarótico y una Arteria Subclavia Derecha Aberrante. Un Caso Raro y Revisión de la Literatura

SUMMARY: An aberrant right subclavian artery described by David Bayford, is rare and one of the aortic arch anomalies. It gives usually incidental findings. We present the case of a 57-year-old woman who was admitted to a neurology outpatient clinic due to a headache and no obvious pathology was detected during a physical examination. In consequence of the brain and chest CT angiography, incidental anomalies of the aortic arch branches were found and asymptomatic aberrant right subclavian artery and bicarotid trunk was diagnosed. The presence of this anomaly is becoming increasingly important in avoiding vascular injuries and cerebral complications in patients undergoing endovascular intervention on the aorta aortic arch surgery, thyroidectomy, or cervicothoracic surgery. So, detailed knowledge of variations of the subclavian artery and bicarotid trunk is paramount for radiologists and surgeons operating on the region of the anterior neck.

Una arteria subclavia derecha aberrante descrita por David Bayford, es rara y es una de las anomalías del arco aórtico. Además, suele ser hallazgos accidentales. Presentamos el caso de una mujer de 57 años que ingresó a una consulta de neurología por dolor de cabeza, sin embargo, no se le detectó patología evidente al examen físico. En el angio-TC de cerebro y tórax, se encontraron anomalías incidentales de las ramas del arco aórtico y se diagnosticó arteria subclavia derecha aberrante asintomática y tronco bicarotídeo. Determinar la presencia de esta anomalía es cada vez más importante, para así evitar lesiones vasculares y complicaciones cerebrales en pacientes sometidos a intervención endovascular de la aorta, cirugía del arco aórtico, tiroidectomía o cirugía cervicotorácica. Por lo tanto, el conocimiento detallado de las variaciones de la arteria subclavia y el tronco bicarotídeo es primordial para los radiólogos y cirujanos que operan en la región anterior del cuello.

Williams?Beuren Syndrome with Pseudoaneurysm of Aortic Arch and Infective Vegetations for Modified Broms Procedure: Anesthetic Concerns & Echocardiographic Illustrations

Williams?Beuren syndrome is a rare genetic malformation with predilection for supravalvular aortic stenosis. Apart from cardiovascular malformation, hypocalcemia, developmental delay, and elfin facies, challenging airway make perioperative management more eventful. Association of infective endocarditis within the aortic arch and pseudoaneurysm formation is infrequent. We, hereby report a case of pseudoaneurysm formation and infective vegetation within the aortic arch in a patient with Williams syndrome and the role of transthoracic echocardiography in its perioperative management.

Debranching Thoracic Endovascular Aortic Repair for Kommerell's Diverticulum with Right-Sided Aortic Arch / 日本心臓血管外科学会雑誌

We report a case of debranching thoracic endovascular aortic repair for Kommerell's diverticulum with right-sided aortic arch in 78-year-old women. The computed tomography (CT) demonstrated Kommerell's diverticulum with a right-sided aortic arch and the trachea and esophagus were compressed by the diverticulum. The diverticulum had a maximum diameter of 32 mm, and surgical intervention was chosen because of the aneurysmal change and the possibility of rupture. We performed endovascular aortic repair for Kommerell's diverticulum with a right-sided aortic arch because of low lung function and low frailty. The patient was discharged on the 21st postoperative day. There was no evidence of aortic event during 2 years follow up.

Computational Study of Fenestration and Parallel Grafts Used in Aortic Arch Lesion / 医用生物力学

Objective To explore hemodynamics of the aortic arch and supraarch vessels after thoracic endovascular aortic repair with fenestration and parallel grafts techniques, and compare the differences of these techniques. Methods Four patients with aortic arch lesions whose supraarch vessels were reconstructed by different surgical techniques (fenestration, chimney and periscope) were studied, and three-dimensional (3D) geometric models were established based on postoperative image data. The physiological flow obtained from two dimensional (2D) phase contrast magnetic resonance imaging were imposed on the ascending aorta inlet and the supraarch vessels outlets. The pressure waveform of 3-element Windkessel model was imposed on the descending aorta outlet. Through computational fluid dynamics ( CFD ) simulations, the hemodynamic parameters were obtained, including the pressure of supraarch vessels, the velocity vector of the stent inlet, and the relative residence time. Results The pressure change of the periscope stent was the largest, followed by the fenestration stent, and the pressure change of the chimney stent was the smallest. The velocity of the fenestration and periscope stent inlet was uneven, which might form vortex. The velocity of the chimney stent inlet was even. The high relative residence time concentrated in distal end of the fenestration stent outer wall, the ‘gutter’ part, and the place where the chimney and periscope stent adhered to the vessel wall. Conclusions The pressure difference between the inner and outer walls of the fenestration and periscope stent was high, so it was recommended to use the balloon-expandable stent. The pressure difference between the inner and outer walls of the chimney stent was low, so it was recommended to use the self-expanding stent. The predicted location of thrombosis was consistent with the clinical follow-up data, so it may be used for surgical planning and risk assessment of interventional treatment of aortic arch lesions.

Two Cases of Descending Aortic Diverticulum Associated with the Right-Sided Aortic Arch / 日本心臓血管外科学会雑誌

Case 1 is a 70-year-old male. He has a history of cholelithiasis and left inguinal hernia. A preoperative examination of the inguinal hernia showed the enlargement of the mediastinal shadow, and he was referred to our department. A close examination revealed a right-sided aortic arch, a right descending aorta, and a descending aortic diverticulum. No subjective symptoms, intracardiac malformations, or other cardiovascular diseases were observed. The surgery was scheduled for descending aorta replacement including a diverticulum with right posterior lateral 4th intercostal thoracotomy and lower body partial extracorporeal circulation. However, due to aortic intima injury at the proximal end, hypothermic cerebral circulatory arrest and proximal anastomosis were performed by the open proximal method. There was no problem with the postoperative course, and he was discharged 19 days after surgery. Case 2 is a 51-year-old female. Born in China, she has lived in Japan for 15 years. No notable history. An abnormal shadow was shown on chest Xp performed in a medical examination, and aortic malformation was suspected on chest CT. She was referred to our department. The diagnosis was right-sided aortic arch, right descending aorta, aberrant left subclavian artery, and Kommerell diverticulum. There were no subjective symptoms and no intracardiac malformations. The operation was a two-stage operation. As the initial surgery, median sternotomy was performed, total arch replacement with intrathoracic reconstruction of the left subclavian artery, and open stent graft insertion, and the Kommerell diverticulum was covered with an open stent graft. We did not treat the diverticulum because it was located on the dorsal side. At 15 days after surgery, we performed embolization of the origin of the left subclavian artery from the Kommerell diverticulum. There was no problem with the postoperative course, and she was discharged 19 days after the initial surgery.

Surgical repair of type Ⅱ right-sided aortic arch with Kommerell diverticulum / 中华胸心血管外科杂志

Objective:To summarize our experience and outcomes of surgical repair of type Ⅱ right-sided aortic arch(RAA) with Kommerell's diverticulum(KD).Methods:From May 2010 to August 2020, a total of 13 patients with type Ⅱ RAA and KD underwent surgery at our center. Mean age was(50.46±10.31) years, 10 were male, and 3 were female. All patients had an aneurysmal KD and aberrant left subclavian artery(ALSA). Preoperative comorbidities included type B aortic dissection in 1 case, aortic arch pseudoaneurysm in 2 cases, and type B intramural hematoma in 2 cases, respectively. Eight(61.5%) patients underwent stented elephant trunk procedures under moderate hypothermic circulatory arrest combined with selective antegrade cerebral perfusion via median sternotomy, and all of them had ALSA reconstruction. Five(38.5%) patients underwent distal arch and descending thoracic aortic replacement through a right posterolateral thoracotomy, the ALSA was reconstructed or ligated in 1 each, and ALSA embolization was performed before surgery in the other 3 cases.Results:No operation deaths occurred. Recurrent laryngeal nerve injury occurred in 2 cases. There was 1(7.69%) in-hospital death. Follow-up was complete in 100 % at mean(5.28±3.84) years. No late death occurred. A persistent anastomotic leak of the proximal arch was detected in a patient who underwent stented elephant trunk procedure, but no aortic dilatation or tracheal and esophageal compression was observed during follow-up. Meanwhile, aortic events, limb ischemia, or symptoms of tracheal and esophageal compression were not observed in the remaining 11 patients.Conclusion:Surgical repair of type Ⅱ RAA with KD can achieve favorable early and midterm outcomes. Surgical strategies should be chosen based on the anatomy of the aorta and whether it is combined with compression symptoms.

Diagnostic method and value of echocardiography for screening right patent ductus arteriosus in infants / 中华超声影像学杂志

Objective:To investigate the diagnostic method and value of echocardiography in screening right patent ductus arteriosus(PDA) of infants.Methods:This was a prospective study.Thirty-one infants with right PDA diagnosed by ultrasound and confirmed by prenatal ultrasonography, electronic computed tomography angiography, angiocardiography and/or surgery in Hebei Children′s Hospital from April 2014 to May 2022 were collected as research subjects, and the association of right ductus arteriosus with aortic arch anomalies and complex cardiac malformations were summarized. The diagnostic method and value of ultrasonic screening were summed up.Results:Of the 31 cases, 30 cases were correctly diagnosed by ultrasound and 1 case was misdiagnosed, who was a left aortic arch descending to the right, a crossover variation of the right and left pulmonary arteries, and a rightward displacement of the ductus arteriosus. Among these cases diagnosed correctly, 27 cases (including 24 cases with right aortic arch and 3 cases with left aortic arch) presented that ductus arteriosus was open and its ostium of pulmonary artery end was located in the proximal right pulmonary artery in views of parasternal short-axis view of great vessels at cardiac base with the combination of two dimensions and color Doppler flow imaging. Other 3 cases of right aortic arch were all single ventricle with transposition of the great artery. Due to the parallel relationship of the two great arteries, the standard parasternal short-axis view of great vessels could not be obtained, and the right ductus arteriosus was found in the high parasternal views.In all of the 27 cases with right aortic arch and right ductus arteriosus, high parasternal views showed that one end of the ductus arteriosus was connected to the right aortic arch isthmus and the other end was connected to the right pulmonary artery. In all of the 3 cases with left aortic arch and right ductus arteriosus, the high parasternal views showed that one end of the ductus arteriosus was connected to the right subclavian artery and the other end was connected to the right pulmonary artery. Among the 27 cases with right aortic arch, 16 cases were accompanied with mirror image branches, 9 cases of which had complex cardiac malformations; 10 cases were associated with aberrant left subclavian artery, 1 case of which had complex cardiac malformations; 1 case was with isolated left subclavian artery, and without complex cardiac malformations. All 3 cases of left aortic arch were accompanied with isolated right subclavian artery and none of them were associated with complex cardiac malformations. Clinical outcomes of 30 cases with right PDA: 14 cases underwent ductus arteriosus ligation due to thick ductus or other heart malformations. In other 16 cases, 4 cases were closed spontaneously, 9 cases had persistent small ductus arteriosus, and 3 cases were lost to follow-up.Conclusions:Right ductus arteriosus is mostly related to the right aortic arch, and those with mirror image branches are prone to complex cardiac malformations; cases of left aortic arch with right ductus arteriosus are tend to accompany isolated right subclavian artery. Ultrasound has an important application in the screening and diagnosis of right PDA.

Midterm clinical results of Sun's procedure with median resternotomy / 中华胸心血管外科杂志

Objective:To summarize the clinical data of Sun's procedure(total aortic arch replacement with frozen elephant trunk technique) in the treatment of complex aortic arch disease after previous cardiac surgery.Methods:From January 1, 2018 to December 31, 2020, a total of 55 patients underwent resternotomy Sun's procedure in the Aortic Surgery Center of Beijing Anzhen Hospital, including 41 males and 14 females, with a mean age of(45.4±12.7) years old, were retrospectively analyzed. The indications of primary cardiac surgery included type A aortic dissection, aortic root or ascending aortic aneurysm, heart valve surgery, and coronary heart disease. Indications for reoperation included residual aortic dissection larger than 55 mm in diameter, aortic aneurysm dilation, new type A aortic dissection, anastomotic leakage with symptoms, and pseudoaneurysm. All the operations were performed under general anesthesia and median resternotomy, total aortic arch replacement with the stented elephant trunk implantation and were performed by anterograde unilateral or bilateral cerebral perfusion.Results:There was no intraoperative death, and the postoperative mortality was 9.1%(5/55). The causes of death were 2 cases of low cardiac output, 1 case of respiratory failure, 1 case of cerebral complications, and 1 case of gastrointestinal bleeding. Except death, there were 2 cases of postoperative cerebral complications(2/50, 4%), 5 cases of spinal cord injury(transient paraplegia)(5/50, 10%), the median duration of ventilator use was 17 hours(14-42 h). Other postoperative complications included respiratory insufficiency requiring ventilatory support longer than 48 hours(8/50, 16%), renal insufficiency requiring temporary dialysis(2/50, 4%). The follow-up time was(25.9±11.2) months(10-47 months), during which 1 case died due to cerebral complication, 4 cases underwent total thoracoabdominal aorta replacement, and 1 case underwent anastomotic leakage repair.Conclusion:It is safe and effec to perform Sun's procedure(total aortic arch replacement with frozen elephant trunk technique) in the treatment of complex arch disease after previous cardiac surgery.

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.

A Case of Staged Hybrid Repair for a Distal Aortic Arch Aneurysm with Shaggy Aorta / 日本心臓血管外科学会雑誌

We report on a case of a distal aortic arch aneurysm with severe shaggy aorta treated by two-stage hybrid surgery without complications. The patient was a 67-year-old man. The thoracic aortic aneurysm was identified on computed tomography imaging by his treating physician during routine follow-up for lung cancer. The patient was referred to our hospital for further investigation and treatment. The aneurysm had a maximum diameter of 68 mm with severe shaggy aorta. Based on these findings, the risk of cerebral infarction and spinal cord ischemia was considered very high. The patient underwent total arch replacement with elephant trunk, using a brain isolation technique for this initial surgery. The postoperative course was uneventful and thoracic endovascular aortic repair（TEVAR) was performed 26 days after the initial surgery. The patient was discharged on post-operative day 38, without complications.

Minimally invasive surgical technique for the treatment of congenital double aortic arch / 中国胸心血管外科临床杂志

@#Objective    To summarize the treatment results of double aortic arch (DAA) by minimally invasive surgical technique. Methods    We retrospectively analyzed the clinical data of DAA patients who underwent minimally invasive surgeries in our center between October 2016 and August 2021. Results    There were 11 males and 4 females with a mean age of 3-61 (20.00±18.80) years. There were 8 patients of DAA and 7 patients of DAA complicated with distal left-sided aortic arch atresia and ligamentum connection. All patients received operations through minimal subaxillary incision, 13 patients were through left side and 2 patients were through right side. One patient with ventricular septal defect was performed operations concurrently under the cardiopulmonary bypass through right minimal subaxillary incision. All patients had symptom improvement without surgery related complications or death in postoperative period. The duration of operation was 30-192 (61.93±40.19) min and mechanical ventilation time was 2-9 (5.33±2.53) h. The length of ICU stay was 18-124 (51.00±38.07) h and hospital stay time was 8-21 (12.67±3.42) d. All patients had sympto-matic relief with good growth and exercise tolerance during the follow-up of 6 (3, 9) months. Conclusion    Minimally invasive surgical technique is a safe, effective and cosmetic approach with good results for DAA treatment.

Long-term outcomes after endovascular aortic treatment in patients with thoracic aortic diseases / Resultados a longo prazo após tratamento endovascular da aorta em pacientes com doenças da aorta torácica

Abstract Background Endovascular treatments for thoracic aortic diseases have been adopted rapidly, and long-term studies are relevant for durability evaluation. Objective To evaluate the long-term results of a prospective observational study of endovascular treatment in patients with thoracic aortic diseases who underwent percutaneous implantation of self-expandable endoprostheses. Methods Procedural success was defined as the absence of endoleak into the aneurysm or dissection-induced false lumen, no migration, and no conversion to open surgery. Intraoperative, postoperative, and late postoperative outcomes were evaluated in terms of complications, mortality, and evolution of the endoprosthesis over a follow-up of up to 179 months (median: 46 months). Results A total of 150 endoprostheses were implanted in 112 patients. Primary success was observed in 100 (82.14%) patients. Immediate mortality occurred in 7 patients (6.25%). Late mortality occurred in 31 patients (27.68%), 10 (8.93%) of whom died from cardiovascular causes, 12 (10.71%) from non-cardiovascular causes, and 2 (1.78%) from natural causes, while 7 (6.25%) had no diagnosis for cause of death. Types I, II, and IV endoleaks occurred during hospitalization in 4 (3.57%), 5 (4.46%), and 3 (2.68%) patients, respectively. Late types I and IV endoleaks occurred in 5 (4.46%) and 3 (2.68%) patients respectively. Twenty-two patients (19.64%) had clinical complications in the immediate postoperative period. Actuarial survival free from death from cardiovascular causes was 79.3% (95% confidence interval, 67.0-91.7%) at 132 months. Conclusions The low levels of intraoperative and postoperative complications demonstrate that endovascular treatment is safe and effective. The high rate of late survival for these critically ill patients indicates that the endovascular technique is beneficial for treatment of thoracic aortic diseases in terms of long-term outcomes.

Resumo Contexto Os tratamentos endovasculares para doenças da aorta torácica têm sido adotados rapidamente e estudos de longo prazo são relevantes para avaliação de durabilidade. Objetivo Avaliar através de estudo observacional e prospectivo, os resultados a longo prazo do tratamento endovascular em pacientes com doenças da aorta torácica submetidos a implante percutâneo de endoprótese autoexpansível. Métodos O sucesso do procedimento foi definido como ausência de vazamento interno para o aneurisma ou falso lúmen induzido por dissecção, ausência de migração e sem necessidade de conversão para cirurgia aberta. Os resultados intraoperatórios, pós-operatórios e tardios foram avaliados quanto a complicações, mortalidade e evolução da endoprótese em um seguimento de até 179 meses (mediana de 46 meses). Resultados Um total de 150 endopróteses foram implantadas em 112 pacientes. Sucesso primário foi observado em 100 (82,14%) pacientes. Mortalidade imediata ocorreu em sete pacientes (6,25%). A mortalidade tardia ocorreu em 31 pacientes (27,68%), dos quais 10 (8,93%) morreram por causas cardiovasculares; 12 (10,71%), por causas não cardiovasculares; dois (1,78%), por causas naturais e sete (6,25%) não tiveram a causa da morte diagnosticada. Vazamentos tipo I, II e IV ocorreram durante a internação em quatro (3,57%), cinco (4,46%) e três (2,68%) pacientes, respectivamente. Vazamentos tardios tipo I e IV ocorreram em cinco (4,46%) e três (2,68%) pacientes, respectivamente. Complicações clínicas no pós-operatório imediato foram observadas em 19,64% dos pacientes. A sobrevida atuarial por causas cardiovasculares foi de 79,3% em 132 meses. Conclusões Os baixos índices de complicações intra e pós-operatórias demonstram que o tratamento endovascular é seguro e eficaz. A alta taxa de sobrevida em 132 meses em pacientes críticos indica que a técnica é benéfica para o tratamento de doenças da aorta torácica em resultados a longo prazo.

Treatment-refractory heart failure as a manifestation of aortic arch atresia / Falla cardiaca refractaria a tratamiento como manifestación de atresia de arco aórtico

Abstract 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.

Resumen 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.

Serie de casos de arco aórtico derecho: aproximación diagnóstica prenatal / Case series of right aortic arch: prenatal diagnostic approach

RESUMEN El arco aórtico derecho es producto de la involución anormal de los arcos vasculares embrionarios. En los últimos años, el diagnóstico fetal se ha hecho más frecuente con la utilización de la ultrasonografía rutinaria del corazón y los grandes vasos. El hallazgo de un arco aórtico derecho involucra muchos aspectos que pueden afectar el pronóstico del feto; por ello, el estudio exhaustivo debe ser sistematizado. A continuación, presentamos una serie de seis casos de diagnóstico prenatal y sugerimos un algoritmo de evaluación.

ABSTRACT The right aortic arch is a product of abnormal involution of the embryonic vascular arches. In recent years, fetal diagnosis has become more frequent with the use of routine ultrasonography of the heart and great vessels. The finding of a right aortic arch involves many aspects that may affect the prognosis of the fetus; therefore, the exhaustive study must be systematized. Below, we present a series of six cases of prenatal diagnosis and suggest an evaluation algorithm.

Hemorrhagic shock caused by double aortic arch and aortoesophageal fistula in a newborn: a case report and literature review / 中国新生儿科杂志

Objective:To study the clinical characteristics of double aortic arch (DAA) combined with aortoesophageal fistula (AEF), and summarize the diagnosis and treatment experience.Methods:Retrospective analysis was performed on the diagnosis and treatment of a newborn with hemorrhagic shock caused by DAA combined with AEF in the Guangzhou Women and Children's Medical Center. The key searching words included "double aortic arch", "aortoesophageal fistula", "vascular ring", "newborn or neonate", and "infant, newborn". The relevant reports were retrieved from databases of CNKI, Wanfang, VIP, PubMed, Springer Link, Google Scholar, Web of Science, Embase, Cochrane Library and OVID, to summarize the clinical features, diagnosis and treatment experience of neonates with DAA and AEF. The retrieval deadline was December 31, 2020.Results:A full-term female newborn was hospitalized for dyspnea immediately after birth, and failed to evacuate from the ventilator for several times. The patient was fed with nasogastric tube and transferred to our hospital because of hemorrhagic shock occurring in 32 days after birth, and gastrointestinal bleeding occurred repeatedly with the maximum bleeding volume reaching 200 ml/time. DAA was diagnosed by cardiac ultrasound and CT, AEF hemorrhage was finally confirmed by gastroscopy, aortography and operation. DAA correction and esophagus repair were successfully performed, and the infant recovered well after the operation. At 9-month old, the infant grew and developed well. At present, no reports of DAA combined with AEF neonates have yet to be published in medical literatures in China. Seven English language literatures included 7 cases of AEF complicated with DAA in neonatal period, 5 cases survived and 2 cases died have so far been reported. All patients have a long history of gastric tube indwelling.Conclusions:The incidence of DAA combined with AEF is rare in the newborn with respiratory and swallowing difficulties as the first manifestation. The disease symptoms progressed rapidly, and life-threatening digestive tract hemorrhage may occur, which often requires surgical treatment. Prolonged gastric tube retention should be avoided in DAA children to prevent the occurrence of AEF.

Prenatal ultrasound diagnosis of fetal right aortic arch / 中国医师进修杂志

Objective:To explore the prenatal ultrasound manifestations of fetal congenital right aortic arch and its diagnostic significance.Methods:The characteristics of prenatal ultrasound images (focusing on the three vessel trachea section) and clinical data of 128 cases of fetal congenital right aortic arch diagnosed in Dalian Women and Children′s Medical Center(Group) from January 2014 to January 2019 were analyzed retrospectively.Results:Among 128 cases of fetal congenital right aortic arch, 111 cases were right aortic arch and leftductus arteriosus with aberrant left subclavian artery (RAA-LDA-ALSA), 1 case was diagnosed as left aortic arch atresia of double aortic arch by operation after birth; 9 cases were right aortic arch and right ductus arteriosus (RAA-RDA); 6 cases were mirror-image right active aortic arch and left ductus arteriosus connected to descending aorta (RAA-BAMB-LDA-DAO); 2 cases were mirror-image right aortic arch and left ductus arteriosus connected to the left innominate artery (RAA-BAMB-LDA-LINA). The three vessels and trachea view (3VT) had characteristic sonographic features.If necessary, coronary section of the upper thoracic aorta and / or stic were added - HD live flow technology assisted the diagnosis of 3vt section.Conclusions:Three vessel trachea view is a sensitive and effective view for prenatal detection and diagnosis of congenital right aortic arch. Upper thoracic aorta coronal section and combined stic - HD live flow technology can make up for its shortcomings. Prenatal ultrasound diagnosis of right aortic arch hasguiding significance for prenatal prognosis consultation.

Influences of Morphological Characteristics of Modular Inner Branched Stent Graft on Hemodynamic Performance of Stent / 医用生物力学

Objective To study the effect of morphological characteristics of modular inner branched stent graft (MIBSG) on hemodynamic performance of postoperative aortic arch based on parameterized MIBSG model. Methods The fluid-structure interaction model of blood-MIBSG coupling performance was solved, and the effects of stent branch angles, stent diameters on hemodynamic characteristics were analyzed. Results With the increase of angles between branch stent and aortic arch stent, blood flow within the branch decreased, but the stress and displacement increased. With the decrease of stent diameters, blood flow perfusion decreased significantly, but the stress and displacement increased first, and then decreased. Conclusions The morphological changes of MIBSG not only have an impact on blood perfusion rate of branch stent, but also affect the stress exerted on stent and the corresponding displacement. Before application in clinic treatment of aortic arch diseases, the movement and torsion of MIBSG should been taken into full account in operation plan according to the actual situation.

Saccular Aortic Arch Aneurysm with Kommerell's Diverticulum / 日本心臓血管外科学会雑誌

The patient was a 73-year-old man who was referred to our hospital due to an abnormal thoracic shadow. CT scans revealed Kommerell's diverticulum and saccular aortic arch aneurysm accompanied by abnormal origins of the right aortic arch and the left subclavian artery. Although there were no subjective symptoms, a surgical operation was planned considering the risk of a rupture of the saccular aneurysm. For the surgery, a median sternotomy approach was employed. Under cardiopulmonary bypass, the aortic arch was detached using the open distal method. Further, an open stent graft was inserted, and the aortic arch was replaced with a four-branched artificial blood vessel. After weaning off the cardiopulmonary bypass, coil embolization was performed on the left subclavian artery, and the site was checked to ensure that there was no endoleak. Although hoarseness was noted postoperatively due to paralysis of the right vocal cord, the patient progressed without any other major complications and was discharged 30 days after the operation.