Anomalous Left Subclavian Artery Research Articles

Tetralogy of Fallot with vertebral defect and left aberrant subclavian artery: a rare occurrence

BackgroundThe most prevalent cyanotic congenital heart disease is Tetralogy of Fallot (TOF). It has a variety of presentations and is made up of four anatomic abnormalities. Documented literature shows an incidence of 13–20% of a right aortic arch with an anomalous left subclavian artery among individuals diagnosed with TOF. This is the first case that discusses the rare occurrence of overriding of the aortic arch along with the left aberrant subclavian artery and vertebral defect in a 3-week-old girl. Timely identification and management are pivotal in ensuring the best possible outcomes for these young patients.Case presentationA 3-week-old female child came with complaints of dyspnea, dysphagia, fatigue, and cyanosis on extreme crying, feeding, and moderate activity. Echocardiography revealed severe pulmonary stenosis with right ventricular dilatation and ventricular septal defect (VSD); a chest computed tomography was performed that revealed four characteristic features of TOF (pulmonary artery stenosis, VSD, right aortic root deviation, and concentric right ventricular hypertrophy) along with overriding of the aortic arch accompanied with the left aberrant subclavian artery (compressing the trachea and infundibulum) and vertebral defect (butterfly vertebra).ConclusionsThe case of this 3-week-old female infant emphasizes the importance of early and accurate diagnosis in congenital heart diseases, particularly when faced with complex presentations such as the TOF. It serves as a testament to the valuable role of advanced diagnostic imaging techniques in unraveling the complexity of congenital heart conditions.

Cardiovascular Dysphagia as A Rare Symptom of Left Atrial Enlargement: A Case Report

Cardiovascular dysphagia is that which is due to luminal obstruction from extrinsic cardiovascular compression of the esophagus (anomalous left subclavian artery, double aortic arch, right aortic arch, cervical aortic arch, Kommerell’s diverticulum, ligamentum arteriosum, aortic aneurysm or dissection, aorto-oesophageal fistula, post-cardiovascular and left atrial dilatation). The diagnosis is frequently suggested by radiographic findings as a barium swallow, and supported by endoscopic and echocardiographic findings. This article presents a 73-year-old woman with progressive dysphagia and enlarged left atrium secondary to mitral valvulopathy and rheumatic fever.

Prenatal diagnosis of vascular rings and outcome.

Background :Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth.Objectives :The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome.Methods :This is a retrospective observational study between January 2014 and December 2019. Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. Postnatal evaluation by transthoracic echocardiography and computerized tomography angiogram was performed. Surgical repair was done as per standard indications.Results :A total of 35 cases of fetal VRs (median gestational age: 24 weeks [range: 19–35]) were diagnosed during the study period. There were four dichorionic diamniotic twin gestation pregnancies. The right aortic arch (RAA) with anomalous left subclavian artery (ALSA) was suspected in 31 fetuses, double aortic arch (DAA) in 3, and circumflex aorta in 1. Twenty-six (74%) patients had successful deliveries. One patient had a spontaneous miscarriage, 2 underwent termination, and 6 were lost to follow-up. Postnatal assessment showed RAA with ALSA in 18, DAA in 5, circumflex aorta in 2, and no abnormality in 1. Twenty-two (86%) were operated (RAA with ALSA: 17, DAA: 4, and circumflex aorta: 1) and four were waiting for surgery. Two patients died due to prematurity-related complications. All survivors are symptom free during follow-up (median: 2.24; range: 0.2–5.6 years).Conclusions :Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs.

Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Anel vascular: uma causa de desconforto respiratório neonatal frequentemente esquecida

<p>Objetivo: Relatar o caso de um recém-nascido com anel vascular completo causado por artéria subclávia esquerda retroesofágica em arco aórtico à direita, associado a um divertículo de Kommerell. Relato de Caso: Paciente do gênero feminino, prematuro (36 semanas e 3 dias), baixo peso (1670g), nasceu de parto via cesária e com Apgar 7/8. Ao exame físico notava-se dispneia, cianose, estridor inspiratório e sopro sistólico (2/6) em borda esternal esquerda alta. A paciente foi levada para unidade de terapia intensiva neonatal com melhora imediata do padrão respiratório e da cianose após intubação orotraqueal. A radiografia de tórax demonstrou alargamento do mediastino e arco aórtico à direita. O ecocardiograma transtorácico evidenciou: forame oval pérvio, arco aórtico à direita e tortuoso, dilatação da aorta ascendente, canal arterial pérvio grande, shunt esquerda-direita pelo septo interatrial e canal arterial e sinais de obstrução ao fluxo da artéria pulmonar direita por provável compressão vascular. A angiotomografia confirmou o anel vascular e esclareceu sua anatomia: arco aórtico à direita com artéria subclávia esquerda retroesofágica originando-se de um divertículo (Kommerell) associada à um canal arterial patente à esquerda. A artéria subclávia esquerda anômala ocasionava compressão extrínseca esofagotraqueal, do fluxo da artéria pulmonar direita e do brônquio fonte à direita. À esquerda, o grande canal arterial patente completava o anel vascular, o que ocasionou sintomas precoces. Conclusões: O anel vascular é uma malformação congênita que deve ser suspeitada em todos os pacientes com anomalias do arco aórtico que apresentam sintomas respiratórios (dispneia/estridor inspiratório e/ou sibilância crônica).</p>

Kommerell's diverticulum, right-sided aortic arch, and a hypoplastic left vertebral artery arising from the left common carotid artery

A 68-year-old man was admitted with sudden onset of headache chest and neck pain, Q waves in the V1-V6 leads on his electrocardiogram, and elevated troponin levels. He previously had hypertension, ischemic heart disease, and bilateral cardiovascular events. The primary diagnosis was subendocardial infarction, and he was treated with systemic thrombolysis. A coronary angiogram showed three-vessel disease with 75% stenosis. Carotid duplex imaging showed >80% stenosis of the internal carotid artery (ICA) bilaterally, a normal right vertebral artery (VA), and high resistance flow in the left VA. A three-dimensional contrast-enhanced computed tomography aortogram showed right-sided aortic arch (AA) (A and B), anomalous retroesophageal left subclavian artery (SA) (C), a diverticulumof Kommerell (DOK) at its origin (Cover) and a hypoplastic left VA (D) arising from left common carotid artery (CCA). He underwent right carotid endarterectomy and triple coronary artery bypass grafting a month later. Patient consent was obtained.

Syncope as Initial Presentation of Kommerell Diverticulum

Kommerell diverticulum (KD) is an aortic arch diverticulum at the origin of an aberrant subclavian artery. It is a rare anatomical anomaly that can be associated with a double aortic arc, a left aortic arch, and anomalous origin of the right subclavian artery or a right aortic arch with anomalous left subclavian artery. We present a case of KD presenting initially as syncope, as well as a review of the literature of this rare syndrome, including diagnosis, imaging techniques, and current surgical treatments.

Right Aortic Arch with Anomalous Left Subclavian Artery and Left-Sided Patent Ductus Arteriosus (Vascular Ring) in an Extremely Low-Birth-Weight Infant

We present three-dimensional reconstructed images of the vascular ring of a 4-day-old extremely premature neonate which were obtained utilizing multidetector computer tomography. This is a unique case of early detection of Kommerell's diverticulum and vascular ring in an extremely low-birth-weight infant using this noninvasive technology.

Poster 166: Bilateral thigh pain after unilateral deep brain stimulator placement: a case report.

Setting: Rehabilitation hospital. Patient: An 81-year-old right-handed woman with bilateral essential tremor. Case Description: The patient underwent deep brain stimulator placement in the ventral intermediate nucleus of the left thalamus at another institution to treat right-sided tremor. The procedure was complicated by injury to the anomalous left subclavian artery. Left upper-extremity arterial emboli resulted and required open thrombectomies. The postoperative course was notable for confusion attributed to anesthesia and urinary tract infection. Because of ongoing functional deficits (patient required moderate assistance with dressing and bathing and minimum assistance to ambulate 75ft with an assistive device), the patient was admitted to inpatient rehabilitation on postoperative day 10 from stimulator placement. On admission to rehabilitation, she complained of bilateral thigh pain, which had developed during hospitalization. The aching thigh pain disrupted sleep and therapies and did not respond to anti-inflammatory, narcotic, or neuropathic pain medications. Lumbar spine imaging revealed degenerative disease without foraminal stenosis. Electromyography of the lower extremities was normal. Head computed tomography confirmed stimulator placement in the left thalamus. Assessment/Results: Bilateral thigh pain resolved after the deep brain stimulator frequency was increased to control upper-extremity tremor. Pain medications were subsequently discontinued successfully. The patient was discharged to home at a modified independence level and could ambulate >1000ft after 22 days of inpatient rehabilitation. Discussion: Complications associated with deep brain stimulator placement can cause functional impairments requiring rehabilitation, and physiatrists should be familiar with side effects associated with deep brain stimulation (DBS). Although parasthesias are a known side effect of deep brain stimulator placement, discomfort is typically treated by decreasing stimulation. This is the first reported case, to our knowledge, of bilateral pain from unilateral DBS that responded to increased frequency of stimulation. Conclusions: Unilateral DBS can cause bilateral pain; increasing DBS frequency can relieve pain.

Surgical treatment of persistent esophageal compression by an unusual form of right aortic arch

A 32-year-old woman with dysphagia due to an unusual form of right aortic arch and anomalous left subclavian artery had successful repair after two previous failures. The definitive repair was accomplished by resection of the retroesophageal portion of the right aortic arch. The continuity of the aorta was established with a prosthetic graft. The operation was performed through a median sternotomy with cardiopulmonary bypass and circulatory arrest.

Surgical Correction of Vascular Ring Anomalies

Between 1981 and 1997, 25 patients underwent operations for relief of tracheoesophageal compression due to vascular rings. Seventeen patients (68%) had a double aortic arch, 6 (24%) had a right aortic arch with an anomalous left subclavian artery and ligamentum arteriosum, and 2 (8%) had a left aortic arch with an anomalous right subclavian artery arising from a Kommerell's diverticulum. Preoperative symptoms consisted of stridor and dysphagia. Four patients (16%) were ventilator-dependent prior to surgery. After division of the vascular ring, wide dissection of the superior mediastinal structures was performed to prevent any compression of the trachea or esophagus. There were no early deaths. Ventilatory support was necessary for a mean of 48 ± 8 hours. One patient required reintubation for 24 hours due to persistent left lower lobe atelectasis. During late follow-up, 2 patients (8%) had recurrent pneumonia, and there were 2 deaths. Surgical repair of vascular rings provided excellent early and late results. The index of suspicion of these aortic arch anomalies should increase if an infant or young child presents with a history of recurrent stridor, dysphagia, or respiratory distress that is not easily explained.

Aortic arch complex anomalies: 20-year experience with symptoms, diagnosis, associated cardiac defects, and surgical repair.

To better understand the clinical presentation and surgical management of children with aortic arch complex anomalies ("vascular rings"), a retrospective study was performed on patients who presented to Children's National Medical Center between the years 1969 and 1989. A total of 59 patients were identified ranging in age at admission from birth to 14 years, of whom 35 (59%) were male and 24 (41%) female. Patients were classified into four major subtypes based on their surgical anatomy, with 29 (49%) patients having right aortic arch and left ductus/ligamentum arteriosus, 21 (36%) double aortic arch, 6 (10%) anomalous left pulmonary artery, and 3 (5%) arch artery anomaly (no ring); 19 patients (32%) had associated cardiac defects. The mean (+/-SD) age at onset of symptoms was 4.6 +/- 14.0 months, and the age at surgical repair was 18 +/- 34 months. There were no intraoperative mortalities, but two late deaths occurred. Three (5%) patients had a surgical complication. In contrast to previous studies, the incidence of nonring lesions was lower and associated cardiac defects higher. Forty-nine percent of patients had symptoms present at birth, and patients with associated cardiac disease did not present earlier than those without. In patients with right aortic arch and left ductus/ligamentum arteriosus, few had an anomalous left subclavian artery. Finally, equal dominance of the arches was most frequent in patients with double aortic arch. Aortic arch complex anomalies present symptomatically in a variety of ways, and noninvasive methods are used to identify the specific lesion and associated cardiac defects. Surgical repair is associated with low or no mortality in patients with uncomplicated aortic arch complex anomalies.

Endoscopic, radiographic, and manometric findings associated with cardiovascular dysphagia

The roentgenographic, echocardiographic, endoscopic, and manometric findings were studied in five consecutive patients with cardiovascular dysphagia, including four with a dilated left atrium and one with an anomalous left subclavian artery. Common and different manometric findings were found in the two types of cardiovascular dysphagia. The major manometric abnormality in all cases was an elevated baseline pressure, with superimposed large rhythmic pressure waves occurring at the same frequency as the electrocardiogram in the mid-esophagus. This manometric abnormality, produced by pulsatile cardiovascular compression, provides direct evidence that cardiovascular dysphagia is caused by esophageal luminal obstruction from cardiovascular compression. Indirect evidence supporting this mechanism includes smooth extrinsic compression and hang-up of ingested barium in the mid-esophagus on esophagogram and transmitted mural pulsations and a compressed lumen in the mid-esophagus at panendoscopy. Two of the five patients had deranged esophageal peristalsis within the high-pressure zone, which also contributed to the dysphagia. Autopsy in one patient with deranged peristalsis revealed a band of ischemic esophageal mucosa in the zone compressed by the dilated left atrium. A novel manometric maneuver might distinguish dysphagia due to an anomalous left subclavian artery from dysphagia due to a dilated left atrium. Left arm elevation during manometry in the single patient with the anomalous artery significantly increased the mean mid-esophageal baseline pressure by 92% (N = 10 trials), and mean pressure wave amplitude by 93% (N = 10 trials, P < 0.002 for each, nonparametric signed rank test). Left arm elevation in this patient also increased the observed luminal obstruction during endoscopy. These manometric and endoscopic findings may be explained by increased arterial compression of the esophagus produced by arterial stretch and anterior displacement with arm elevation.

Syndromal associations of common origin of the carotid arteries.

The term "common origin of the carotid arteries" (COCA) has been proposed to replace the older terms "origin of the left carotid artery from the innominate stem" and "bicarotid trunk with anomalous right or left subclavian artery." These anatomic patterns are usually reported to occur in about 11% of whites and 20-25% of blacks and have been reported to have increased frequency in patients with esophageal atresia-tracheoesophageal fistula, DiGeorge anomaly, and anomalous origin of the left coronary artery from the pulmonary artery. COCA is a significant, if not invariant, feature of the great arteries in the condition usually called in the more recent literature "anomalous origin of the innominate artery," the most frequent cause of symptomatic tracheal compression by anomalous systemic arteries. Analysis of associations of COCA with various other congenital cardiovascular lesions showed, in addition, significant association with congenital polyvalvular disease, truncus arteriosus, aorticopulmonary window, trisomy 13, 18, and 21 syndromes, acrocephalosyndactyly (especially Apert syndrome), tetralogy of Fallot not associated with DiGeorge anomaly, and clinical Noonan phenotype. Pentalogy of Cantrell was associated with no increase in incidence of COCA.

Coarctation of aorta with right aortic arch. Report of surgical correction in 2 cases: one with associated anomalous origin of left circumflex coronary artery from the right pulmonary artery.

Two patients with right aortic arch, anomalous left subclavian artery, and coarctation of theaorta, have been operations for the relief of coarctation, successful in one but only partially successful in the other: in one case, a 'dacron' patch was inserted into the narrowed segment, but a mild recurrence of coarctation was found a year later; in the other, the proximal segment of the anomalous left subclavian artery was used for the repair, with complete relief of the coarctation, both immediately and at late follow-up. The first case had multiple associated congenital abnormalities, including anomalous origin of the left circumflex coronary artery from the right pulmonary artery, which was also corrected surgically: this anomaly has only been recorded once hitherto. The second had facial haemangiomata and a small ventricular septal defect. Four previously reported cases of coarctation and right arch are reviewed. These 6 cases are considered in the light of current views on the development and classification of aortic arch anomalies.

Right aortic arch with ruptured aneurysm of anomalous left subclavian artery

An aneurysm in the anomalous left subclavian artery developed in an elderly woman with a right aortic arch and an aberrant left subclavian artery (left ligamentum arteriosum connected to the left common carotid artery). The aneurysm gradually enlarged, eventually rupturing with exsanguination. A progressive increase in the size of this aneurysm was associated with symptoms and anatomic findings consistent with the subclavian “steal” syndrome.

Tracheoesophageal constriction produced by an unusual combination of anomalies of the great vessels: A case report

A case which demonstrates an unusual combination of anomalies of the great vessels is described. These include three of the more commonly recognized vascular rings, namely, right aortic arch with left ligamentum arteriosum, anomalous left common carotid artery, and anomalous left subclavian artery. An additional anomaly was the insertion of the ligamentum arteriosum to the site of coarctation of the anomalous left subclavian artery and a ventricular septal defect. The importance of considering the simultaneous occurrence of multiple anomalies of the heart and great vessels is emphasized.

Combination of Ventricular Septal Defect and Aortic Insufficiency

(1) Two cases of ventricular septal defect with aortic insufficiency were presented.(2) The diagnosis was confirmed at autopsy in both cases. In one case, an associated right aortic arch and anomalous left subclavian artery were also noticed.(3) Differentiation of this lesion from similar conditions, especially those of the patent ductus arteriosus, ruptured aneurysm of the sinus of Valsalva and aortic septal defect, was discussed.

Kartagener's syndrome with an anomalous left subclavian artery.

1. Two cases in one family of Kartagener's syndrome are described, and the radiological findings are illustrated. 2. One case presented with an anomalous left subclavian artery passing behind the œsophagus. This diagnosis is based on radiographic and fluoroscopic findings. 3. As far as can be ascertained an anomalous left subclavian artery in a case of dextrocardia has not been recorded previously. 4. No symptoms accompanied this anomalous great vessel development. 5. The two cases described appear to support the theory that the syndrome described by Kartagener is a triad of congenital origin.