An operative case of pseudocoarctation with chronic dissection.

A 47-year-old man with a history of hypertension was found to have a prominent aortic knob on routine chest X-ray and was referred to our hospital. Enhanced computed tomography angiography showed severe flexion at the proximal descending aorta with chronic type B dissection localized to the flexion region. Graft replacement of the distal aortic arch was performed. Surgical management of chronic pseudocoarctation dissection is sparsely reported in the literature because of its rare occurrence. We present an operative case of a patient with chronic dissection of distal aortic arch pseudocoarctation.

Right cervical aortic arch associated with anomaly of epiaortic vessels and haemodynamically significant obstruction between ascending and descending aorta. Extra-anatomic bypass graft between ascending and descending aorta and long-term follow-up.

Right cervical aortic arch is a rare malformation in which a right-sided aortic arch is unusually long, forms a loop, descends, and crosses behind the oesophagus to continue as a left descending aorta. We present a case of right cervical aortic arch associated with anomaly of epiaortic vessels and aortic obstruction, surgically treated with extra-anatomic bypass graft between ascending and descending aorta.

Short-term outcome after the prenatal diagnosis of right aortic arch.

OBJECTIVES: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). METHODS: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. RESULTS: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. CONCLUSIONS: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.

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.

Neonates With Right Aortic Arch Requiring Arch Reconstruction: A Single-Institution Experience.

BACKGROUND: Reconstruction of a right aortic arch is rarely required in the newborn period and has rarely been reported. METHODS: All patients who underwent a right aortic arch repair in the neonatal period from a single institution were retrospectively reviewed. The primary outcome measures included survival, complications, and reintervention. RESULTS: Between 1984 and 2020, 15 patients were identified. Nine patients (60%) presented with an interrupted aortic arch, 5 with a hypoplastic arch (33%), and 1 with anomalous origin of the brachiocephalic vessels (7%). All patients had associated complex congenital heart disease. Median age at surgery was 6 days (range, 2-29 days); median weight was 3.11 kg (range, 2.5-4.18 kg). Genetic syndromes were prevalent and 77% of interrupted aortic arch patients had DiGeorge syndrome. Surgical techniques included end-to-side (27%), end-to-end (27%), or side-to-side anastomosis (13%) and placement of an interposition graft (7%); 65% required patch augmentation. Median intensive care unit and total hospital length of stay were 20 days (range, 7-92 days) and 28 days (range, 10-240 days), respectively. At a median follow-up of 3.97 years (range, 0.19-36 years), 13 of 15 patients were alive (87%). We found vocal cord paralysis in 27%, hemidiaphragm paralysis in 13%, and considerable airway compression in 27%. Overall, 27% patients required reintervention on the aortic arch: 2 surgical and 2 percutaneous balloon dilation. CONCLUSIONS: Right aortic arch reconstruction in the newborn period is rare and associated with complex lesions with an acceptable reintervention rate.

Arterial switch operation for Taussig-Bing anomaly with aortic arch obstruction.

INTRODUCTION: The short-term survival rate after single-stage correction of Taussig-Bing anomaly with aortic arch obstruction remains favorable. However, some cases are encountered occasionally in which single-stage correction was not performed during the neonatal period. Accordingly, we evaluated the midterm outcomes of different surgical strategies. METHODS: Seven patients who underwent an arterial switch operation and intraventricular rerouting as definitive surgery between 2007 and 2017 were investigated. Of these 7 patients, 3 had undergone previous pulmonary artery banding and aortic arch reconstruction. RESULTS: The median body weight at definitive surgery was 3.3 kg (range 2.9-8.3 kg). At definitive surgery, the arrest time for single-stage correction (162.3 ± 21.7 min) was significantly shorter than that of staged repair (206.3 ± 5.1 min, p = 0.020). There was no hospital or late death. One patient in both strategy groups underwent aortic reintervention 54 months and 7.1 months after the definitive operation. Neoaortic valve (perinatal pulmonary valve) diameter decreased significantly from the perinatal valve diameter following definitive surgery (median +4.94z and +2.12z, respectively, p = 0.016) but there was no significant difference in the neopulmonary valve (perinatal aortic valve) diameter. Both single-stage correction and staged repair patients showed a similar trend. At the last follow-up, no patient had greater than mild neoaortic or neopulmonary valve regurgitation. CONCLUSION: The surgical outcomes of both single-stage correction and staged correction for Taussig-Bing anomaly with aortic arch obstruction are excellent. Both strategies produce similar changes in the diameter and regurgitation grade of the neoaortic and neopulmonary valves.

Critical left coronary main trunk stenosis, chronic occluded right coronary artery, left subclavian artery occlusion, severe aortic regurgitation and porcelain aorta in a patient with aortitis.

BACKGROUND: Coronary involvement is rare but can be critical in patients with aortitis. Although cardiac ischemia can be resolved by coronary artery bypass grafting (CABG), patients complicated with cardiac ischemia, calcified aorta, and valve insufficiency pose difficult problems for surgeons. CASE PRESENTATION: A 71-year-old woman was referred to our institution because of unstable angina. She had been previously diagnosed with aortitis and left subclavian artery occlusion. Contrast-enhanced computed tomography revealed severe left coronary main trunk stenosis, right coronary artery occlusion, and porcelain aorta. Ultrasonic echocardiogram showed severe aortic regurgitation. We performed emergent coronary artery bypass grafting, aortic valve replacement and ascending aorta replacement under hypothermic circulatory arrest. CONCLUSIONS: The technique of circumferential calcified intimal removal and reinforcement with felt strips was effective for secure anastomosis. Unilateral cerebral perfusion from the right subclavian artery enabled good visualization and sufficient time to perform distal anastomosis.

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.

Type B Interrupted Right Aortic Arch: Diagnostic and Surgical Approaches.

Interrupted right aortic arch is a rare congenital cardiovascular anomaly typically associated with other forms of congenital heart disease. We report two cases of interrupted right aortic arch associated with isolated left pulmonary artery and bilateral ductus arteriosus in the first case and with truncus arteriosus in the second case. Computed tomography was complementary to echocardiography in delineating the anatomic details in both cases, informing surgical repair with reconstruction of a left aortic arch in the first case and right arch in the second case.

Cervical aortic arch: an unusual cause of a pulsatile neck mass.

A 72-year-old man presented with epistaxis on two occasions requiring admission. Prior to performing a bilateral sphenopalatine artery ligation diathermy, anaesthetic concern was raised regarding what the patient described as a 'congenital aortic aneurysm', with an overlying scar secondary to explorative surgery as a child. The abnormality was a cervical aortic arch (CAA). CAA is a rare vascular anomaly, which most commonly manifests as a pulsatile neck mass. In this case, we discuss the differential diagnosis for a pulsatile neck mass and considerations to be made in the workup. We also highlight the importance of cardiovascular risk factor management in patients with CAA.

Left aortic arch with right descending aorta and severe coarctation: an unusual "vascular clamp" with airway compression.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

Successful Resection of Esophageal Carcinoma With a Double Aortic Arch.

We report a patient with esophageal carcinoma accompanied by a double aortic arch. The preoperative computed tomography scan only detected the right aortic arch, but we found the double aortic arch during the operation. The patient underwent an esophagectomy through a left thoracotomy. Preoperative three-dimensional reconstruction of computed tomography images should be conducted to completely understand the anomalies of the great vessels.

Surgical Relief of Left Ventricular Outflow Tract Obstruction in Adults With Congenital Aortic Stenosis and Associated Aortic Annulus Hypoplasia and/or Subaortic Obstruction.

BACKGROUND: In children and adolescents, a Ross/Konno operation is commonly done to both enlarge the aortic root and provide a competent aortic valve with relief of left ventricular outflow tract obstruction (LVOTO). Optimum management is not so straightforward in adults. METHODS: Between 1995 and 2014, 16 patients of mean age 39.4 years (18-57 years) with hypoplastic aortic annulus (AA) measuring 20mm and less, and mean aortic valve/LVOT gradient of 61mmHg (30-70mmHg) presented for surgery. RESULTS: Eight patients with mean LVOT/AA diameter 19.6mm (18-20mm) underwent an "inclusion-cylinder" type Ross procedure (RP). Eight patients with more severe LVOT/AA obstruction, with mean diameter of 17.4mm (16-19mm) underwent mechanical aortic valve replacement (AVR) with standard Konno-type aortoventriculoplasty. There was zero early and late mortality; with mean follow-up of 11.6 years (3-21 years) in the Ross group and 6 years (2-10 years) in the Konno-AVR group. One patient in the Konno-AVR group had reoperation after 2 years for RVOT obstruction. The postoperative echocardiograms of these patients at last follow-up show residual mean gradient across LVOT/AA of 4.4mmHg (2-6mmHg) after RP, and 11.9mmHg (8-17mmHg) after Konno-AVR. CONCLUSIONS: In adults, the "inclusion-cylinder" Ross-procedure is a good alternative for mild to moderate aortic root hypoplasia. However, for cases with severe LVOT obstruction, a Ross-Konno is not possible with the same method of autologous support used in a non-Konno RP, and this could be expected to have an impact on late durability and the need for further intervention, in a group that has already undergone multiple procedures in childhood. Both methods of RP and Konno-AVR lead to excellent early and late results.

Tratamiento híbrido de los aneurismas del arco aórtico. Nuestra experiencia / Hybrid approach to aortic arch aneurysm. Experience in a single centre

Introducción: El tratamiento de la disfunción del arco aórtico sigue siendo un reto. El reemplazo quirúrgico del arco aórtico se asocia a una elevada morbimortalidad. Se propone el tratamiento híbrido como una alternativa menos invasiva. Objetivos: Presentamos la experiencia de nuestro centro en el tratamiento híbrido de aneurismas del cayado aórtico. Se describe la morbimortalidad a 30 días y a medio plazo; endofugas, reintervenciones y permeabilidad de los bypasses. Material y métodos: Realizamos un estudio descriptivo y retrospectivo de los aneurismas de aorta con afectación del arco que se sometieron a un procedimiento híbrido entre enero del 2009 y diciembre del 2014. Incluimos pacientes tratados tanto de forma urgente como electiva. Resultados: De un total de 38 pacientes tratados por disfunción aneurismática del cayado aórtico en dicho periodo, 12 (31,5%) recibieron tratamiento híbrido. Se realizaron 2 bypasses aortotronco branquiocefálico-carotídeo izquierdo y carótido-subclavio izquierdo, 5 bypasses carótido-carótido-subclavio izquierdo, un bypass carótido-carotídeo y 5 bypasses carótido-subclavio izquierdo, seguidos de la colocación de una endoprótesis torácica mediante técnica habitual. Se consiguió éxito técnico del 92%. La mortalidad a 30 días fue del 16% (n = 2). El seguimiento medio fue de 29,3 meses (±15 meses). Dos pacientes (16%) cursaron con endofuga en el seguimiento, ambas de tipo Ia, por lo cual precisaron de reintervenciones mediante bypass proximal y nueva endoprótesis. La mortalidad global fue del 25% (n = 3). La permeabilidad de los bypasses fue del 92%. No apareció ningún caso de isquemia medular ni ictus en el postoperatorio ni durante el seguimiento. Conclusión: Los resultados obtenidos en nuestro centro son similares a los publicados en grandes series en la literatura. El debranching del cayado aórtico es una alternativa válida en la cirugía electiva y una gran herramienta en el caso urgente, con una aceptable morbimortalidad en el medio plazo (AU)

Introduction: Aortic arch aneurysm treatment is still considered a challenge for surgeons. Total aortic arch replacement is associated with high morbidity and mortality. Hybrid treatment is considered an alternative and a less invasive treatment. Objective: To present our results for the treatment of aortic arch aneurysms with hybrid procedures. An analysis was made of the 30-day and mid-term morbidity and mortality, endoleak rate, re-intervention rate, and bypass patency. Material and methods: An extensive review was carried out on all patients treated by hybrid procedures, from January 2009 to December 2014. Elective and urgent cases were included. Results: A total of 38 patients with aortic arch and thoracic aneurysm were reviewed. Of these, 12 (31.5%) required hybrid treatment. Two complete revascularisations of supra-aortic vessels were performed, as well as 5 carotid-carotid bypass plus left subclavian artery bypass, one carotid-carotid bypass alone, and 4 left carotid-to-left subclavian artery bypass, all followed by endovascular aortic aneurysm exclusion. Technical success was 92%. The 30-day mortality rate was 16% (n = 2). Mean follow-up was 29.3 months (±15 months). Two patients (16%) developed a type Ia endoleak in the follow-up that required further proximal revascularisation plus proximal endograft extension. Overall mortality was 25%. Patency of supra-aortic vessels revascularisation was 92% during follow-up. No neurological complications were found. Conclusion: These findings support the assumption that the hybrid approach in the management of aortic arch aneurysm is a reliable alternative to open surgery in elective surgery. This technique is also valid for patients in the urgent setting. Our results are comparable to other published series. There is low morbidity and mortality in the mid-term (AU)

Abordaje híbrido de la disfunción aórtica: a propósito de 2 casos y revisión de la literatura / Hybrid approach to aortic disease: Report of 2cases and literature review

La corrección de los aneurismas de la aorta toracoabdominal y del arco aórtico es sumamente compleja debido a su relación con las arterias viscerales y con los troncos supraaórticos respectivamente. El desarrollo progresivo de las técnicas endovasculares ha permitido realizar abordajes híbridos, en los que estos procedimientos se han combinado con intervenciones quirúrgicas abiertas. La aplicación de la cirugía híbrida ha mejorado las tasas de morbimortalidad en los últimos años, y es la primera opción en pacientes de edad avanzada o que presentan comorbilidades que contraindican el abordaje quirúrgico abierto. Ello es debido a que se pueden evitar los tiempos quirúrgicos prolongados, el pinzamiento aórtico o la circulación extracorpórea. En este artículo describimos 2 casos de tratamiento híbrido, uno de aorta toracoabdominal y otro de arco aórtico y realizamos una revisión de la literatura al respecto (AU)

The correction of aneurysms of the thoraco-abdominal aorta and aortic arch is extremely complex, due to their relationship with the visceral arteries and the supra-aortic trunks, respectively. The progressive development of endovascular techniques has allowed hybrid approaches to be made, in which these procedures have been combined with open surgical procedures. The application of hybrid surgery has improved morbidity and mortality rates in recent years. It is the first choice in elderly patients or those with comorbidities that contraindicate the open surgery approach. This is due to preventable prolonged surgical time, aortic clamping, and/or extracorporeal circulation. In this paper we describe 2 cases of hybrid treatment are described in this work; one of the thoraco-abdominal aorta, and the other of the aortic arch. A review of the literature is also presented (AU)

Surgical Repair of Cervical Aortic Arch With Brain Circulation Anomaly Through Clamshell Incision.

We report the successful surgical repair of a cervical aortic arch and diverticulum with a brain circulation anomaly through a clamshell incision. Because of the reliability of selective antegrade cerebral perfusion and superior exposure, we chose an approach through a clamshell incision. We describe the utility of this approach for treating a cervical aortic arch with a diverticulum.

Single-Stage Correction for Taussig-Bing Anomaly Associated With Aortic Arch Obstruction.

Taussig-Bing anomaly and aortic arch obstruction are two types of complex congenital cardiac malformations. Almost 50% of patients with Taussig-Bing anomaly have aortic arch obstruction. This report assesses the surgical outcomes of single-stage correction in neonates with both defects. Between November 2006 and November 2015, 39 neonates with Taussig-Bing anomaly and aortic arch obstruction (28 patients with coarctation of the aorta and 11 patients with interrupted aortic arch) underwent a one-stage arterial switch operation and aortic reconstruction. There were three in-hospital deaths and one late death (8 months after the surgery). The short-term survival rate was 92.3% (36/39), and the mid-term survival rate was 89.7% (35/39). Follow-up data were available for all patients who survived the operation (range 6-92 months). Echocardiology showed six cases of recoarctation, three cases of left ventricular outflow tract obstruction, three cases of right ventricular outflow tract obstruction, four cases of pulmonary artery stenosis, five cases of aortic regurgitation, and eight cases of pulmonary regurgitation. Eight patients required a reoperation during the follow-up period with no mortality. All survivors remained in good condition (New York Heart association functional class I or II). Single-stage correction of Taussig-Bing anomaly with aortic arch obstruction in neonates had favorable short- and mid-term outcomes in terms of mobility and reoperation rate. The optimal operative procedure should be chosen according to the position of the coronary arteries and the type of aortic anomaly.

Extra-anatomical bypass in complex and recurrent aortic coarctation and hypoplastic arch.

OBJECTIVES: Our goal was to report the selection schemes, technical variations and long-term outcome of extra-anatomical bypass to correct complex, recurrent aortic coarctation and hypoplastic aortic arch. METHODS: Between 1989 and 2012, 53 patients (mean age 13.2 ± 4.3, median 11.6, range 9-23 years) with complex aortic coarctation (n = 33; long-segment hypoplastic aortic arch in 15), recurrent coarctation (n = 20; anastomosic pseudoaneurysm in 10), underwent correction using extra-anatomical bypass, either with (n = 18: femoral bypass = 13, left heart bypass = 5) or without (n = 35) extracorporeal circulation via a left lateral thoracotomy (n= 48) and combined median sternotomy and median laparotomy (n = 5). The decision to use extracorporeal circulation was based on the anatomical location of the coarctation, the length of the hypoplasia and a history of previous repair. Preoperatively, mean systolic blood pressure was 130 ± 30 mmHg at rest and 180 ± 40 mmHg during exercise, with a mean pressure gradient of 80 ± 11.6 (range 40-120) mmHg. RESULTS: Various extra-anatomical bypass strategies included left subclavian artery to descending aorta (n = 38), ascending aorta to left subclavian artery (n = 3), ascending aorta to descending aorta (n = 4), aortic arch to descending aorta (n = 3) and ascending aorta to abdominal aorta (n = 5). Graft size (median 18, range 10-26, mm) was chosen according to the diameter of the vessel proximal and distal to the planned graft. No operative deaths, paraplegia or abdominal malperfusion occurred. The mean reduction in systolic blood pressure was 60 ± 25 mmHg without pressure gradients. During a mean follow-up of 18.3 ± 3.7 years, there were no reoperations, graft complications or pseudoaneurysm formation on anastomotic sites. Seven (11.6%) patients are on antihypertensive medications. No patient presented with claudication nor did anyone experience orthostatic problems from the steal phenomenon. CONCLUSIONS: Extra-anatomical bypass is safe, an effective technique, and achieves satisfactory long-term results.