Aberrant left pulmonary artery with tracheal stenosis without vascular sling

Summary We report a case of abnormal origin of the left pulmoanry artery from the right pulmonary artery, associated with tracheal stenosis but with no vascular sling. This is the first such case, to our knowledge, without vascular sling.     

Congenital tracheal stenosis associated with aberrant left pulmonary artery. Apropos of a severe form with neonatal disclosure

The case report is presented of a newborn infant afflicted with extensive congenital tracheal stenosis associated with anomalous left pulmonary artery, proving rapidly fatal. The possibility of tracheal anomaly must be envisaged in the face of any neonatal respiratory distress unexplained by pulmonary parenchymatous pathology. Diagnosis of this complex malformation relies on endoscopy and oesophagogram and tracheo-bronchogram contrasts.     

Aberrant right subclavian artery with left aortic arch: Associated cardiac anomalies

Summary Anomalous origin of the right subclavian artery (ARSA) from the aorta distal to the normally positioned left subclavian artery is a relatively frequent congenital anomaly in subjects with left aortic arch. The purpose of this study was to determine the relative frequency of associated cardiovascular anomalies in individuals with this anomaly. From the records of approximately 11,000 pathologic specimens in the Registry of Cardiovascular Disease of United Hospital (St. Paul, MN, USA), we found 128 (1.2%) with ARSA.Of the 128 ARSA, 117 (2.9%) occurred among 4102 instances of congenital heart disease.The 117 cases with congenital heart disease and ARSA were conotruncal anomalies in 38%, septal defects in 28%, obstructive anomalies of the left side of the heart in 21%, right heart anomalies in 5%, and miscellaneous conditions in the other 8%. Down syndrome existed in 14 (12%) of the 117 specimens with ARSA and some congenital cardiac anomaly; nine of the latter had an atrioventricular canal (AVC) malformation.     

MRI diagnosis of isolated origin of the left subclavian artery from the left pulmonary artery

Right aortic arch with isolation of the left subclavian artery is a rare disorder. In this entity, the left subclavian artery has its origin from the pulmonary artery via the ductus arteriosus. We report an infant with an unbalanced atrioventricular septal defect, right aortic arch, bilateral ductus arteriosi and an isolated left subclavian artery. This infant also had chromosome 22q11 deletion. The origin and course of the isolated subclavian artery were well-demonstrated using contrast-enhanced 3-D magnetic resonance angiography. This non-invasive diagnostic modality can accurately reveal detailed mediastinal vascular anatomy in a young infant and allows diagnosis of aberrant vascular connections.     

Anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCA-PA) is a rare form of congenital heart disease. In this report, three cases with this anomaly are described; two patients presented in infancy with heart failure from myocardial ischemia and infarction, while the third was asymptomatic and ALCA-PA was diagnosed during evaluation of a residual murmur after surgery for associated cardiac defects (ventricular septal defect and patent arterial duct). All three cases underwent aorto-pulmonary tunnel repair (Tukeuchi procedure), and to our knowledge two of them are the first infantile cases reported in Turkey.     

Aberrant right subclavian artery

Summary Two cases of aberrant right subclavian artery are presented, with a brief discussion of the clinical features, diagnosis and treatment of dysphagia lusoria. From the Main Hospital, Bhilai Steel Plant, Bhilai, M. P.     

Isolated origin of the left internal carotid artery from the pulmonary artery

The authors describe what is, to their knowledge, the first reported case of the anomalous origin of an internal carotid artery from the pulmonary artery. An otherwise asymptomatic 6-year-old girl, who presented with headaches and hypertension, underwent a comprehensive workup that revealed extensive meningeal and cerebral artery anastomoses to the left internal carotid artery--itself arising from the origin of the left pulmonary artery. This unique anatomical anomaly, caused by a disturbed pattern of aortic arch regression, resulted in a right-to-left vascular shunt into the pulmonary artery and a disturbance of intracranial artery flow patterns, complicating the management options.     

左冠状动脉起源于肺动脉的诊治特点

目的探讨左冠状动脉起源于肺动脉的临床诊治特点。方法对16例左冠状动脉起源于肺动脉患者的体表心电图、超声心动图、心血管造影检查及手术治疗结果进行分析。结果①心电图:Ⅰ、Ⅱ、aVL、V5、V6导联T波倒置且ST段压低8例,其中6例伴左胸导联异常Q波;T波倒置4例。②超声心动图:16例左冠状动脉起源于肺动脉患儿中合并房间隔缺损2例,左室侧壁运动减弱及左室射血分数(LVEF)降低6例。③15例心导管造影提示左冠状动脉起源于肺动脉,合并房间隔缺损2例,合并动脉导管未闭1例。④治疗结果:所有患儿均进行手术治疗,死亡2例,余14例随访1个月～6 a,冠状动脉血流通畅。结论对于左冠状动脉起源于肺动脉的诊断,应结合其特征性心电图表现有助于诊断,超声心动图具重要价值,结合心导管造影检查可确诊,一经诊断应尽早手术治疗。     

Anomalous left pulmonary artery with an unusual barium swallow

An atypical finding on barium swallow in a child with an anomalous left pulmonary artery causing obstructive emphysema in the right lung is described. The value of bronchography and need for early surgical intervention is stressed.     

Tracheobronchography in the evaluation of anomalous left pulmonary artery

In three patients the diagnosis of an anomalous left pulmonary artery was confirmed when tracheobronchography demonstrated a discrete extrinsic impression on the proximal right bronchus. The diagnosis was unsuspected clinically in one patient with a hyperlucent left lung on conventional chest radiographs. In the other two patients there was a suspicion of a pulmonary sling, but findings on plain chest or airway radiographs were atypical. The tracheobronchogram can be a valuable procedure for diagnosing a pulmonary sling, detecting additional developmental anomalies of the bronchi, and predicting the post-operative clinical course.     

Bridging bronchus and posterior left pulmonary artery: a unique association

A 6-month-old female with a lifelong history of respiratory distress became increasingly difficult to manage and required right upper and middle pulmonary lobectomies for worsening emphysema and mediastinal shift. The postoperative course was stormy and confusing and the patient died despite emergency tracheostomy. The autopsy disclosed an anomalous bronchus to the right lower lobe, originating from the left mainstem bronchus. In addition, the left main pulmonary artery was positioned posterior to the left mainstem bronchus. Two other cases of bridging bronchus have been reported, but the association with posterior left pulmonary artery has not been described.     

Adult type anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a clinical entity characterized by myocardial necrosis which becomes symptomatic shortly after birth; survival beyond infancy is uncommon because of severe left heart failure. To our knowledge, it is rare for an ALCAPA patient to survive to adulthood. Here we present a case of a 17-year-old girl with ALCAPA who was referred to our hospital because of palpitation and dyspnea.