Atypical cardiovocal syndrome associated with right aortic arch and Kommerell diverticulum.

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

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

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

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

Invited Commentary

The purpose of this study was to evaluate the utility of 16-slice multidetector computed tomographic (MDCT) angiography for identifying anatomic features of aberrant subclavian arteries. Seventeen patients with aberrant subclavian arteries were assessed by MDCT angiography. The aortic arch position, the presence of a Kommerell's diverticulum, aneurysm, vascular compression of trachea and oesophagus and associated cardiovascular abnormalities were evaluated. MDCT findings were confirmed by surgery in eight patients but in the other nine patients no further evaluation or management was warranted as the aberrant subclavian artery had no significant clinical consequence. Eleven patients had an aberrant right subclavian artery arising from the left aortic arch and six patients had an aberrant left subclavian artery arising from the right aortic arch. Kommerell's diverticulum was identified in three patients with an aberrant right subclavian artery and in five patients with an aberrant left subclavian artery. In two patients it was aneurysmal. Oesophageal compression was detected in eight patients, and tracheal compression was identified in only one paediatric patient. An aberrant subclavian artery was associated with complex congenital heart disease in one patient, intracardiac defects in two patients, aortic coarctation in two patients, patent ductus arteriosus in two patients and an aberrant vertebral artery in one patient. In conclusion, MDCT angiography is superior to digital subtraction angiography for the assessment of aberrant subclavian arteries since digital subtraction angiography has only a poor ability to visualize adjacent structures completely and is invasive in nature. MDCT angiography or magnetic resonance angiography are the current standard in the initial evaluation of thoracic vascular anomalies.

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.

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KOMMERELL DIVERTICULUM: A RARE BUT POTENTIALLY LIFE THREATENING ANOMALY

Re: Anomalies of the fetal aortic arch.

Unusual cause of chest pain and dysphagia in a middle-aged woman with right aortic arch and aberrant left subclavian artery

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

During the last 6 years we have retrospectively identified 32 patients with aberrant subclavian arteries and related vascular rings. Twenty-seven patients had a left-sided aortic arch with an aberrant right suhclavian artery (ARSA). whereas four had a right-sided aortic arch with an aberrant left subclavian artery. All these patients were asymptoniatic. Aneurysm of an ARSA was seen in one patient. One patient had a double aortic arch and dysphagia. Twenty-one of the subjects were female. and I I were niale. They ranged in age from 5 months to 75 years old (mean age. 54 years old). Barium esophagography was performed in I 3 patients. CT in 20. MR imaging in two. and conventional angiography in three. Discussion The ARSA was first described on necropsy by Hunauld in 1735. In 1787, David Bayford, before the Medical Society of London. used the term “dysphagia lusoria,” or “difficulty in swallowing due to a quirk of nature,” in describing symptoms related to this anomaly. The radiologic findings were first described in 1936 by Kommerell. and surgical management of the problem was discussed by Gross in 1946(1]. The ARSA is the most common of the intrathoracic major arterial anomalies, with an incidence of 0.4-2%. In this anomaly. the ARSA arises as the last great vessel of the aortic arch. The anomaly occurs as a result of interruption between the right common carotid artery and the ARSA in the developing double aortic arch. Normal embryonic development of the aortic arch and great vessels begins as six paired ventral and dorsal arches [I]. Certain segments disappear, and others persist during development of the fetus. The left fourth arch forms the aortic arch. The right fourth arch contributes to the formation ofthe right subclavian artery. as does the seventh intersegmental artery. In the ARSA, the right fourth arch and proximal right dorsal aorta involute. and the right seventh intersegmental artery develops from a persistent dorsal aorta [ I I. An aortic diverticulum may be present at the origin of this vessel. representing the remnant of the distal right aortic arch. The ARSA arises from the dorsal margin of the aorta. It then passes behind the esophagus (Figs. 1-5) in almost all cases; indeed, passage of the ARSA in front of the esophagus or trachea occurs rarely if ever [2[. An aberrant left subclavian artery arising from a right aortic arch is far less common. occurring in one in 1000 people. Most patients with ARSA are asymptomatic. and the anomaly is found incidentally. either by radiography or endoscopy. Rarely. this malformation has been suspected as the cause of dysphagia. Pediatric patients may present with respiratory compromise due to tracheal compression. In adults, the trachea is more resistant to compression. An aneurysmal aberrant subclavian artery may present as a superior mediastinal mass. On barium esophagography. the ARSA consistently produces a characteristic oblique fron-

A right-sided aortic arch with an aberrant left subclavian artery is a congenital vascular anomaly that is easily detectable in utero at the level of the three vessels and trachea view, but which is rarely symptomatic in the neonate. We present a newborn with prenatally diagnosed right-sided aortic arch and aberrant subclavian artery who showed a clinically relevant stenosis of the subclavian artery during the first week of life. An intravascular stent was implanted into the stenosis of the aberrant left subclavian artery by catheterization. This case report demonstrates that a right-sided aortic arch with an aberrant subclavian artery can be diagnosed prenatally, that in these patients a stenosis of the subclavian artery can occur in early infancy and requires awareness of the neonatologist or pediatrician, and that stent implantation represents a minimally invasive therapeutic approach.