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.

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.

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.

Long-term outcomes of complete vascular ring division in children: a 36-year experience from a single institution.

Objectives: Complete vascular rings are rare and cause tracheoesophageal compression. Following surgical division, some patients have persisting tracheomalacia. We aim to assess the long-term outcomes of complete vascular ring division. Methods: All patients (n = 132) who underwent surgical division of a complete vascular ring between 1978 and 2014 were identified from the hospital database and retrospectively reviewed. Results: Complete vascular rings consisted of a double aortic arch (n = 80), right aortic arch with an aberrant subclavian artery and left ligamentum arteriosum (n = 50), right aortic arch with mirror image branching and left ligamentum arteriosum (n = 1), and a left aortic arch with right descending aorta and right ligamentum arteriosum (n = 1). Kommerell's diverticulum was identified in 10 patients. Preoperative tracheomalacia was identified via bronchoscopy in 25 patients. Concomitant tracheal reconstruction was not performed in any patient. Kommerell's diverticulum was resected in 1 patient. The hospital mortality rate was 1.5% (2/132). There were no late deaths. The overall survival rate was 98.3 ± 1.2% (95% CI: 93.4, 99.6) at 20 years. Postoperatively, persistent tracheal compression was reported in 3 patients, and tracheomalacia in 16 patients. The rate of freedom from reoperation was 88.6 ± 4.0% (95% CI: 77.9, 94.3) at 20 years. No patient required tracheal surgery during the follow-up period. Follow-up was 92% (121/132) complete, with a median follow-up of 11.4 years (range 44 days to 36 years). At the last follow-up, 7 patients had mild tracheomalacia. Conclusions: Outcomes of division of a complete vascular ring are excellent. Tracheomalacia often improves following division of the vascular ring. Respiratory symptoms following complete vascular ring division are uncommon.

The modified 'no touch' technique in the antegrade endovascular approach for left common carotid artery ostial stenosis stenting.

BACKGROUND: Open surgery and the retrograde endovascular approach via the distal left common carotid artery (LCCA) have some limitations in LCCA ostial stenosis treatment. The 'no touch' technique used in the renal artery was modified for this situation. METHODS: Fifteen selective LCCA stenosis patients were treated by the modified 'no touch' technique in the antegrade endovascular approach from March 2013 to March 2016. Thirteen underwent the transfemoral approach and the other two had the transbrachial approach due to a 'bovine aortic arch'. Distal embolic protection devices were used in all cases. Follow-up included a neurological examination, carotid duplex scan, and office interview. Mean follow-up time was 18.2±11.5 months. RESULTS: The initial technical success rate was 100%. The average procedure time was 84.0±16.3 min. There were no procedure-related deaths. No clinical neurological complications occurred during the in-hospital stay. No incidence of death or major stroke occurred during the follow-up period; 6.7% (1/15) of patients had a contralateral minor stroke, 66.7% (4/6) of symptomatic patients were relieved of initial symptoms, and the rest showed improvement. No patient developed new ipsilateral neurological symptoms and no in-stent restenosis occurred during the follow-up period. These results were confirmed by ultrasound. CONCLUSIONS: The modified 'no touch' antegrade endovascular technique is a feasible method for treating LCCA ostial lesions with a satisfactory initial success rate, acceptable procedure time, and comparable mid- and long-term results. This technique could be considered as a complementary option for LCCA ostial stenosis in addition to open surgery and the retrograde endovascular approach.

Palliation Outcomes of Neonates Born With Single-Ventricle Anomalies Associated With Aortic Arch Obstruction.

BACKGROUND: The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution. METHODS: Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed. RESULTS: Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p = 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p = 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p = 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p = 0.012). CONCLUSIONS: Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients.

A novel procedure for reconstructing an extensive hypoplastic aortic arch in older children.

Aortic arch reconstruction is the key to successfully repairing an interrupted aortic arch (IAA) with tubular hypoplasia of the aortic arch (THAA), especially in older children. We report a novel reconstruction technique involving aortapulmonary fusion that was used to treat THAA in a 9 year-old patient with IAA. In this procedure, the underside of the aortic arch and the upside of the main pulmonary artery were fused to reconstruct the aortic arch. The short-term outcome of the procedure has been promising. This procedure may represent an alternative for repairing extensive THAA in older children.

Aortic Arch Advancement and Ascending Sliding Arch Aortoplasty for Repair of Complex Primary and Recurrent Aortic Arch Obstruction.

The optimal treatment of patients with complex coarctation of the aorta and hypoplastic aortic arch is controversial. Children undergoing repair of obstructive arch lesions are at lifelong risk of recurrence. Multiple techniques have been described to address complex primary and recurrent coarctation. Our approach at Texas Children's Hospital (Houston, TX) has been to address these lesions in an anatomic fashion via median sternotomy, under cardiopulmonary bypass using the techniques of aortic arch advancement and ascending sliding arch aortoplasty. Anatomic repair of complex primary or recurrent aortic obstruction is safe and produces a favorable repair with a low rate of recurrence. Both operations restore normal arch contour using native aortic tissue. The decision of which particular surgical strategy to use depends on the patient's age, anatomy, and the elasticity of the tissue.

Modified Norwood Procedure for Tricuspid Atresia, Transposition of Great Arteries, and Hypoplastic Right Arch With Complete Vascular Ring.

We report a modified Norwood stage I procedure for tricuspid atresia, transposition of great arteries, and hypoplastic right aortic arch with complete vascular ring. In this technique, we applied dual arterial cannulation to avoid circulation arrest during neoaortic reconstruction, and also corrected the arch laterality during the Norwood stage I palliation procedure. Pulmonary flow was supplied by the Blalock-Taussig shunt. Postoperative imaging revealed the patent left neoaortic arch, and the vascular ring was relieved with a patent tracheobronchial tree.