Early Resection of Kommerell Diverticulum and Revascularization in Children With Right Aortic Arch and Aberrant Left Subclavian Artery.

The purpose of our study was to describe patterns of airway compression identified on cross-sectional imaging in infants and children with either right aortic arch and aberrant left subclavian artery or left aortic arch with aberrant right subclavian artery. Data from MR imaging and CT performed to evaluate pediatric patients for extrinsic airway compression were reviewed for cases that revealed an aberrant right or left subclavian artery. Clinical, endoscopic, and imaging findings in identified cases were reviewed. Recurrent patterns of extrinsic compression were reviewed among cases. Twelve patients with right aortic arch with aberrant left subclavian artery and nine patients with left aortic arch and aberrant right subclavian artery were identified. All 12 with right aortic arch with aberrant left subclavian artery had airway compression shown, with multiple sites or diffuse compression in six. Of these 12 patients, nine had compression at the level of the arch and aberrant subclavian artery (10 had Kommerell's diverticulum), and nine had compression of the distal airway in association with a midline descending aorta. Five of the nine patients with left aortic arch and aberrant right subclavian artery had airway compression shown, all at the level of the arch and aberrant subclavian artery. None of these compressions was associated with either Kommerell's diverticulum or midline descending aorta. Both right and left aberrant subclavian arteries can be associated with symptomatic airway compression, but the patterns of compression are different. The airway compression in right aortic arch with aberrant left subclavian artery is often associated with either Kommerell's diverticulum or midline descending aorta, whereas compression associated with left aortic arch and aberrant right subclavian artery is not.

Repair of Kommerell's Diverticulum and Aberrant Subclavian Arteries: Classification of the Five Groups in Declining Frequency and Their Operative Approaches

Hybrid Endovascular Treatment of an Anomalous Right Subclavian Artery Dissection in a Patient With Marfan Syndrome

We determined the relationship between aortic arch anatomy in tetralogy of Fallot with pulmonary stenosis and chromosomal or genetic abnormality, by performing analysis of 257 consecutive patients undergoing surgical repair from January, 2003 to March, 2011. Chromosomal or genetic abnormality was identified in 49 of the 257 (19%) patients. These included trisomy 21 (n = 14); chromosome 22q11.2 deletion (n = 16); other chromosomal abnormalities (n = 9); CHARGE (n = 2); Pierre Robin (n = 2); and Kabuki, Alagille, Holt-Oram, Kaufman McKusick, Goldenhar, and PHACE (n = 1 each). Aortic anatomy was classified as left arch with normal branching, right arch with mirror image branching, left arch with aberrant right subclavian artery, or right arch with aberrant left subclavian artery. Associated syndromes occurred in 33 of 203 (16%) patients with left arch and normal branching (odds ratio 1); three of 36 (8%) patients with right arch and mirror image branching (odds ratio 0.4, 95% confidence interval 0.1-1.6); seven of eight (88%) patients with left arch and aberrant right subclavian artery (odds ratio 36, 95% confidence interval 4-302); and six of 10 (60%) patients with right arch and aberrant left subclavian artery (odds ratio 8, 95% confidence interval 2-26). Syndromes were present in 13 of 18 (72%) patients with either right or left aberrant subclavian artery (odds ratio 15, 95% confidence interval 4-45). Syndromes in patients with an aberrant subclavian artery included trisomy 21 (n = 4); chromosome 22q11.2 deletion (n = 5); and Holt-Oram, PHACE, CHARGE, and chromosome 18p deletion (n = 1 each). Aberrant right or left subclavian artery in tetralogy of Fallot with pulmonary stenosis is associated with an increased incidence of chromosomal or genetic abnormality, whereas right aortic arch with mirror image branching is not. The assessment of aortic arch anatomy at prenatal diagnosis can assist counselling.

Objective To investigate the diagnosis, surgical treatment and surgical results of infantile left subclavian artery combined with Kommerell diverticulum. Methods In our hospital from 2014 to 2017, there were 15 cases of left subclavian artery combined with Kommerell diverticulum, 10 males and 5 females, average age 11.8 months(2 to 48 months), average weight of 9.2kg(4 - 24 kg), including 3 cases with ventricular septal defect(VSD), and 1 cases of coarctation of aorta(COA). Children with postoperative respiratory difficulty, recurrent respiratory infection, chronic cough and other respiratory symptoms, preoperative detection of airway and or esophagus compression performance after cardiac computed tomography examination confirmed, general anesthesia, thoracotomy or left chest lateral thoracotomy, cut off arterial ligaments and Kommerell diverticulum Resection and vagal left subclavian artery transplantation were performed in the left common carotid artery combined with cardiac malformation. Results All the children were cured and discharged from hospital. The follow-up and mid-term recovery were good. Conclusion Infants with airway and esophageal compression may have aberrant left subclavian artery combined with Kommerell diverticulum, cardiac CT is an effective means to diagnose this disease. Removal of the associated Kommerell diverticulum and transferring the left subclavian artery to the left common carotid artery, as the main operative method has good early prognosis, and may eliminate residual symptoms and late complications. Key words: Congenital heart disease; Complete vascular ring; Kommerell diverticulum; Children

A Kommerell's diverticulum in patients with a right aortic arch may become aneurysmal and be an independent cause of tracheoesophageal compression, even after ligation and division of a left ligamentum. We review the indications for and results of Kommerell's diverticulum resection and left subclavian artery transfer in children with a right aortic arch who previously underwent vascular ring (ligamentum) division. From 1998 through 2001, eight children have been referred with recurrent respiratory symptoms (n=8) and/or recurrent dysphagia (n=4) after vascular ring division. Each child had a right aortic arch with a left ligamentum and had undergone division of the ligamentum elsewhere. All had a Kommerell's diverticulum that was not addressed at the initial operation. All patients had a repeat left thoracotomy with resection of the diverticulum. Five patients had division and reimplantation of the left subclavian artery into the left carotid artery to relieve the sling-like effect of the retroesophageal left subclavian artery on the right aortic arch. One other patient had primary Kommerell's diverticulum resection and transfer of the left subclavian artery to the left carotid artery. The mean age at the initial operation was 1.7+/-0.9 years, and the mean age at reoperation was 8.0+/-3.7 years. In all patients postoperative bronchoscopy confirmed relief of the tracheal compression. There were no complications related to the subclavian artery transfer. Two patients developed postoperative chylothorax, one requiring thoracic duct ligation. The median hospital stay was 5 days. All patients had dramatic resolution of their preoperative symptoms. Kommerell's diverticulum is an important anatomic structure that can cause recurrent symptoms in patients with a right aortic arch after ligamentum division. In selected patients, reoperation with resection of the Kommerell's diverticulum and transfer of a retroesophageal left subclavian artery results in relief of symptoms. This technique has become our procedure of choice as a primary operation for children with a right aortic arch and a significant Kommerell's diverticulum.

NOT ALL THAT WHEEZES IS ASTHMA: A CASE OF KOMMERELL DIVERTICULUM

Reoperative vascular ring surgery is uncommon. Standard redo ipsilateral thoracotomy may present technical challenges and risks. We describe a patient with right aortic arch, aberrant left subclavian artery, and a Kommerell diverticulum in whom previous vascular ring division via left thoracotomy did not relieve dysphagia. Three years after the unsuccessful operation, left subclavian-carotid transposition via supraclavicular incision followed by resection of the Kommerell diverticulum via right thoracotomy with extracorporeal circulation relieved symptoms. Contralateral thoracotomy with extracorporeal circulation provides a safe, alternative approach to redo ipsilateral thoracotomy for resection of a symptomatic Kommerell diverticulum. We review the literature on the incidence, surgical indications, and operative approaches to manage symptoms from a Kommerell diverticulum.

Hybrid Treatment for Ruptured Diverticulum of Kommerell: A Minimally Invasive Option

Condensed Contents

Type B Aortic Dissection Involving an Isolated Right-Sided Aortic Arch

Structural aortic arch shift and supraaortic angle configuration changes after subclavian to carotid transposition as a proposed mechanism for relief of severe esophageal compression in aberrant subclavian artery anatomy

KOMMERELL DIVERTICULUM: A RARE BUT POTENTIALLY LIFE THREATENING ANOMALY

Kommerell diverticulum (KD) with aberrant left subclavian artery (ALSA) is a rare congenital variation of vascular structure. We reported a case of 3-year-old boy with KD and ALSA, which was preliminarily diagnosed by transthoracic echocardiography and verified by computed tomography angiography (CTA). The patient was treated successfully with KD resection and anastomosed the left subclavian artery directly to the left carotid artery under cardiopulmonary bypass. Our case illustrates that echocardiography and CTA should be used to comprehensively assess all the internal and external cardiac structures in order to determine the appropriate surgical plan.

Objective To explore the value of MRI in the diagnosis of fetal aortic arch anomalies. Methods We retrospectively collected 10 fetuses with aortic arch anomalies indicated by prenatal ultrasound and underwent MR examination and were subsequently proven by autopsy or post-birth follow-up from 320 pregnant women. We focused on the observations of the location of the aortic arch and brachiocephalic artery anomalies, the locations of the liver and stomach in the abdominal cavity, and the large vessels in abdomen. The above-mentioned finding were compared with prenatal ultrasound and follow-up findings. Results Of 10 cases, right aortic arch with aberrant left subclavian artery was seen in 7 cases, right aortic arch with the mirror branch, left aortic arch with aberrant right subclavian artery, right aortic arch with aberrant left subclavian artery combined with cervical aortic arch and double aortic arch was seen in 1 case, respectively. All aortic arch anomalies detected by MRI were consistent with post-birth or autopsy findings. Ultrasound misdiagnosed aortic branch malformation in 5 places, which included right aortic arch but misdiagnosed aberrant left subclavian artery in 2 cases, right aortic arch never diagnosed mirror branch anomaly in 1 case, right aortic arch with left subclavian artery misdiagnosed cervical aortic arch in 1 case, left atrial isomerism but misdiagnosed left aortic arch with aberrant right subclavian artery in 1 case; One double aortic arch was misdiagnosed as right aortic arch with aberrant left subclavian artery in ultrasound. Conclusion Fetal cardiovascular MRI is an effective and supplementary examination to complement ultrasound in diagnosis of fetal aortic arch anomalies. Key words: Fetus; Aorta,thoracic; Vascular malformations; Magnetic resonance imaging