Primary resection of Kommerell's diverticulum in a 37-year-old patient

A previously non-operated 37-year-old patient presented with esophageal dysphagia for solid food and stridorous breathing. Computed tomography and magnetic resonance showed a 33-mm wide Kommerell's diverticulum. The diverticulum and the obliterated ductus arteriosus arising from it towards the pulmonary artery were resected. The left subclavian artery, which originated from the diverticulum, was reimplanted into the descending aorta. In addition to dissecting any tissue which is making vascular ring-like structures around the trachea and the esophagus, it is important to resect the diverticulum itself and reimplant the left subclavian artery arising from it, in this way leaving the aortic arch free from any additional dislocating forces.     

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.     

Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.     

Surgical treatment of an aneurysm originating from a Kommerell's diverticulum in the right-sided aortic arch with retroesophageal component.

We report the case of a 41-year-old man presenting with a large Kommerell's diverticulum aneurysm in the right-sided aortic arch with retroesophageal component and moderate thoracic aortic dilation. Surgical treatment was performed through left thoracotomy and consisted of aneurysmectomy, closure of the distal aortic arch defect and aorta-left subclavian artery bypass. After 2 years computed tomography showed no modifications in the thoracic aortic morphology and the patency of the graft to the subclavian artery.     

Aberrant left subclavian artery associated with Kommerell's diverticulum: chance finding in a 75-year-old patient

The most common anomaly of the aortic arch is the occurrence of a left aortic arch with an aberrant right subclavian artery. Other, less common anomalies have also been described. These include the occurrence of a right aortic arch with an aberrant left subclavian artery that, in addition, has a diverticulum at its site of origin known as Kommerell's diverticulum. All cases described in the literature have occurred in individuals younger than 35 years of age, generally with symptoms related to tracheal or esophageal compression. We present the case of a patient diagnosed by chance with this rare anomaly at 75 years of age.     

Vascular ring and sling in a fetus who developed esophageal and airway compression after birth

Vascular ring and sling are congenital anomalies of the vascular structure in the thorax with a prevalence of 2.4/10,000 live births. Double aortic arch (DAA), right aortic arch with left ductus arteriosus and aberrant left subclavian artery (RAA-ALSA), and pulmonary artery sling (PAS) are the three common types of vascular ring and sling. These anomalies can be isolated or accompanied by intracardiac malformation. The presence of both vascular ring and PAS is extremely rare. Here, we report a fetus who was prenatally diagnosed with PAS and RAA-ALS, and developed symptoms due to esophageal and airway compression after birth.     

Isolated origin of the left subclavian artery from the left pulmonary artery

We describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.     

Unusual vascular ring in infant with pulmonary atresia and ventricular septal defect.

A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.     

Digital subtraction angiography in the diagnosis of vascular rings

Digital subtraction angiography was performed in six patients suspected of having a vascular ring. There were four males and two females. Two patients were less than 6 months of age, two were between 14 and 20 months, and two were ages 4 and 53 years. Four of the six patients had abnormal esophagrams. Five patients underwent peripheral intravenous digital subtraction angiography and a sixth patient underwent digital subtraction angiography via arterial catheterization. In two patients (ages 20 months and 4 years), the presence of a vascular ring was excluded by demonstrating an anatomically normal arch with an anomalous right subclavian artery. In three patients, a right aortic arch was present. Two of these patients had mirror image branching and a left ligamentum arteriosum. The third patient had an aberrant left subclavian artery and a left ligamentum. One patient had an anomalous right subclavian artery and a systemic collateral vessel arising from the aorta, supplying a confluence of vessels in the right lung hilum. In all patients, anatomic definition was accurate with the intravenous injection and there were no complications. In the patient who underwent intra-arterial injection, the dose of contrast was reduced 50% from the dose usually administered. Digital subtraction angiography appears to be a good adjunctive diagnostic method in patients in whom the presence of a vascular ring needs to be confirmed angiographically and obviates the need for arterial catheterization.     

Management options for Kommerell's diverticulum

An aberrant right subclavian artery (ARSA) arising from a left-sided aortic arch (LAA) is an uncommon aortic arch anomaly. Aneurysms of ARSA arising from different degrees of Kommerell's diverticulum may modify surgical approach. We describe a safe technique for the surgical management of ARSA aneurysm in a 67-year-old gentleman. We performed exclusion of an ARSA aneurysm using left heart bypass, with concomitant R axillary to RSCA bypass. This obviated the need for any major procedure such as an aortic arch replacement.     

Surgical Treatment of Vascular Ring Including Right Cervical Aortic Arch

Abstract Cervical aortic arch is a developmental entity consisting of persistence of the right or left third branchial arch and regression of the fourth branchial arches. In most cases, the aorta is redundant and crosses behind the esophagus to the opposite side. In the presence of an aberrant subclavian artery contralateral to the side of the aortic arch and a ligamentum arteriosum, a vascular ring is formed around the trachea and esophagus. Two young patients with right-sided cervical aortic arch, aberrant left subclavian artery, and ligamentum arteriosum presented with dys-phagia and exertional dyspnea. In one patient, through a left thoracotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were dissected thoroughly above and below the level of the ring. In addition, the aberrant left subclavian artery was divided at its origin from a large diverticulum and implanted into the left common carotid artery; the aortic di-verticulum was resected. In the other patient, who had associated 22q11 chromosomal deletion, in addition to left-sided compression of the trachea and esophagus, there was additional marked compression of the right anterolateral trachea by the redundant ascending aorta. Through a median sternotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were widely mobilized; an additional aortopexy of the ascending aorta to the right of the sternum resulted in the absence of tracheal compression. The cases of the two reported patients illustrate the clinical variability of vascular ring, including a right cervical aortic arch and the consequently versatile surgical approach that is needed to successfully address this combination of vascular anomalies.     

Aberrant right subclavian artery-esophageal fistula and severe gastrointestinal bleeding after surgical correction of scimitar syndrome

Scimitar syndrome and gastrointestinal bleeding from an aberrant right subclavian artery-esophageal fistula are each extremely rare. Although scimitar syndrome and aberrant right subclavian artery are typically asymptomatic in adults, fistulous connection between the aberrant artery and the esophagus is associated with a poor prognosis. Outcomes are contingent upon timely diagnosis and prompt surgical repair. Prolonged nasogastric and endotracheal intubation can lead to gastrointestinal bleeding in patients who have an aberrant right subclavian artery or other vascular ring. We recommend neither embolization nor the use of endovascular stents as anything other than a temporizing measure in the management of aberrant right subclavian artery injury. These methods can stop acute hemorrhage; however, sentinel bleeding will eventually occur and require definitive ligation.We report the case of a 57-year-old woman in whom an aberrant right subclavian artery-esophageal fistula developed after surgical correction of symptomatic scimitar syndrome. Massive gastrointestinal bleeding resulted from prolonged nasogastric and endotracheal intubation. To our knowledge, this is the first report of aberrant right subclavian artery and scimitar syndrome in the same patient, and the 4th report of a patient's surviving a fistula between the aberrant artery and the esophagus.     

Repair of Vascular Ring with Resection of Kommerell Diverticulum and Transposition of Aberrant Left Subclavian Artery

A 32-year-old female presented with dysphagia. Radiographic studies revealed external compression of esophagus by a vascular ring. The anatomy was a right-sided aortic arch with aberrant retroesophageal left subclavian artery, emanating from a large Kommerell diverticulum (KD). Traditional repair with ligamentum division and adhesiolysis leaves a large KD still adjacent to the esophagus with the potential for persistent or recurrent symptoms. The objective of this study was the modification of operative technique to minimize the potential for persistent or recurrent symptoms. The operative repair included resection of KD with transposition of the left subclavian artery into the left carotid artery, in addition to the division of the ligamentum arteriosum and mobilization of the esophagus. The patient''s dysphagia resolved and postoperative barium studies showed no residual compression. There were no significant perioperative complications. Resection of KD is a potential adjunct to traditional repair of vascular rings and might offer better long-term palliation by minimizing residual vascular compression of the esophagus.     

Developmental defects of the aortic arch and the right subclavian artery

The study included 10 patients with anomalous development of the aortic arch, of whom 5 had a double aortic arch, 5 had anomalous branching of the right subclavian artery with the formation of a vascular ring. 5 patients were operated with good long-term results during a 15-year follow-up period.     

Double aortic arch with atretic left arch distal to the origin of left subclavian artery accompanied by dyspnea: A case report and literature review

Double aortic arch with atretic left arch distal to the origin of left subclavian artery is a rare type of vascular ring, and it can be easily confused with the right aortic arch with mirror branching. We provided a rare case of a 10‐month‐old infant with dyspnea. Echocardiography showed a suspicious double aortic arch with atretic left arch distal to the origin of left subclavian artery, which was confirmed intra‐operatively. We summarize ultrasonic image characteristics of the disease and combine it with computed tomography angiography, bronchoscopy, and clinical symptoms in order to improve the detection rate and treatment strategy.     

Right aortic arch with retroesophageal left ligamentum arteriosum

A 49-year-old woman with a 2-year history of severe dysphagia, claudication of the right arm, and persistent pulsatile back pain presented at our institution. An esophagogram showed 2 marked indentations of the upper esophagus. Computed axial tomography showed a right-sided aortic arch with mirror image branching and an aortic diverticulum; the thoracic aorta distal to the origin of the right subclavian artery was elongated and tortuous, ascending to the base of the neck to form the "2nd arch", which compressed the right-arm plexus nerve and the upper vertebral column. Through a right thoracotomy, a segment of thoracic aorta around the Kommerell's diverticulum was resected, and the proximal and distal ends of the thoracic aorta were reapproximated. The patient tolerated the procedure well and remained symptom free.     

Countercurrent aortography by contrast injection into a peripheral artery

Countercurrent aortography performed through left brachial artery in 8 children, aging 27 days to 42 months. Systemic-pulmonary anastomosis was possible to be assessed in 3 out of 4 cases, even in one aging 42 months. Aortic coarctation could be evaluated in another 3 patients. In the last case with a clinical suspection of vascular ring, countercurrent aortography disclosed an anomalous right subclavian artery. We concluded that the procedure is safe and useful to assess some conditions involving thoracic aorta, even in older children.     

Video‐assisted Thoracoscopic Surgery: Is It a Superior Technique for the Division of Vascular Rings in Children?

Objective. The use of video‐assisted thoracoscopic surgery (VATS) is becoming increasingly common in the treatment of congenital heart defects, particularly for the division of vascular rings. We compare the short‐term outcomes of vascular ring division by VATS as opposed to open thoracotomy and discuss new issues raised by VATS division of vascular rings. Methods. Over a 3‐year period, 47 pediatric patients underwent isolated division of a vascular ring. We reviewed preoperative, operative, and postoperative data. For patients whose vascular ring contained a ligamentous or atretic segment, comparisons were made between operations performed by open thoracotomy and by VATS. Results. For the 47 patients, diagnoses included right aortic arch/aberrant left subclavian artery/left ligamentum arteriosum (25), double aortic arch/right dominant (19), and double aortic arch/left dominant (3). At the time of repair, ages ranged from 1.4 to 207 months (median 15.0) and weights ranged from 2.8 to 54.0 kg (median 11.0). The 29 patients with a vascular ring containing a ligamentous or atretic segment were included in the comparison between open thoracotomy and VATS. This included 25 patients with a right aortic arch, aberrant left subclavian artery, and ligamentum arteriosum, and 4 patients with a right dominant double aortic arch in which the left arch was atretic. Patients with patent rings requiring clamping and oversewing of the vessels were not included in the comparison. Thoracoscopic division was successful in 14/16 (88%) patients. There was no significant difference in the procedure time (P = .48) with the thoracoscopic approach. There were also similar times for intensive care unit stay (P = .25) and total length of hospital stay (P = .22). Conclusions. The division of vascular rings by VATS shows similar outcomes compared with the open thoracotomy approach. It is expected that, with an increase in comfort level and further improvements in technology, the use of VATS can be extended to include patients with other congenital heart defects.     

Two cases of 'asthma' revealing a diverticulum of Kommerell

Among embryonic aortic vascular malformations, persistence of a right aortic arch and aberrant left subclavian artery associated with a diverticulum of Kommerell is rare, and is estimated to occur in 0.1% of the general population. We report two cases of diverticulum of Kommerell in which tracheal compression due to the vascular ring induced respiratory symptoms such as asthmatic dyspnoea. This reminds us that at least a chest X-ray should be performed in newly diagnosed asthmatic patients, focusing on the trachea and upper mediastinum. In case of vascular abnormality, angiography and magnetic resonance are the best investigations to define the abnormal anatomy and guide surgery.     

Non-penetrating subclavian artery trauma: management by selective transluminally placed stent device.

Non-penetrating injury to the subclavian artery has not often been reported. The limited experience of surgeons with this type of trauma and the difficult vascular control required for its management make it a surgical challenge. We report on two cases, one after blunt trauma and the other with a subclavian artery aneurysm following anterior dislocation of the shoulder. Percutaneous stent implantation in the subclavian artery was successfully performed with, in the second case, coil embolization of the aneurysm. Follow-up Doppler sonography and angiogram demonstrated patency and luminal integrity of the involved artery. This less invasive procedure may be a significant advance and a new approach in the conservative management of traumatic subclavian injury for selected cases.