Anomalous Left Pulmonary Artery Research Articles

A case of resected lung cancer with displaced anomalous left upper division bronchus and anomalous left pulmonary artery

A case of resected lung cancer with displaced anomalous left upper division bronchus and anomalous left pulmonary artery

Partial Anomalous Left Pulmonary Artery Anterior Versus Posterior Types: A Systematic Review.

The aim of this study was to investigate the features of partial anomalous left pulmonary artery (PALPA) and differences between cases with posterior versus anterior a nomalous vessels in relation to the tracheobronchial tree. We hypothesized that statistical significance was dependent on the course of the anomalous vessel due to airway compression in the posterior type. This study included cases obtained from the literature (n = 33) and an institution teaching file (n = 2). Information collected: age, sex, medical history, additional anomalies, anomalous vessel course, and respiratory symptoms. Data were analyzed with independent samples t-test and Fisher’s exact test. PALPAs were more commonly anterior than posterior. Mean age: 5.3 years (SD = 12.4) for anterior and 6.8 years (SD = 18.5) for posterior (p = 0.77). Respiratory symptoms: 20% of anterior and 60% of posterior cases (p = 0.032). Tracheobronchial anomalies: 35% of anterior and 60% of posterior cases (p = 0.182). Non-cardiac and non-tracheobronchial anomalies: 30% of anterior and 47% of posterior cases (p = 0.511). Kabuki syndrome: 25% of anterior and 6.7% of posterior cases (p = 0.207). In conclusion, respiratory symptoms were the only significant difference between anterior and posterior PALPA types.

Partial Anomalous Left Pulmonary Artery: Pseudo-Pulmonary Sling.

Partial Anomalous Left Pulmonary Artery: Pseudo-Pulmonary Sling.

One patient with an anomalous origin of the left pulmonary artery directly from the right ventricle: a case report.

BackgroundThe anomalous origin of one pulmonary artery is a rare malformation, which so far has mainly been found as an anomalous origin from a different site of the aorta, accounting for 0.12% of all congenital heart diseases. This case report introduced a very rare case of the anomalous origin of one pulmonary artery which had never reported in the clinic.Case summaryA 2-year-old boy with a 6-month history of shortness of breath and recurrent respiratory infection, was diagnosed left pulmonary artery (LPA) directly arising from the right ventricle by transthoracic echocardiography and multidetector computed tomography without a deletion in the region of 22q11. Eventually, the LPA was further conformed that arised from the right ventricle during the operation, and was corrected with a well clinical outcome.DiscussionThe surgical technique for repair of this anomalous LPA was not difficult in our case. However, the embryonic development of the present case still could not be completely explained by the current embryologic postulates since it was a new malformation that never reported. Due to its rarity, there is still much to learn about the origin and development of the pulmonary arteries that possibly develop prenatally.

Prenatal sonographic diagnosis of partial left pulmonary artery sling: A rare case report

Pulmonary artery sling is a rare congenital vascular anomaly. Partial anomalous left pulmonary artery is even rarer and no in utero observation has yet been reported. Here, we present the ultrasonographic findings of a 38-year-old woman at 32 weeks of gestation whose fetus showed a normal bifurcation of the pulmonary trunk into the right and left pulmonary arteries, but an anomalous origin of the left lower lobe pulmonary artery from the right pulmonary artery. These findings were confirmed by postnatal echocardiography and thoracic computed tomography.

Partial Anomalous Left Pulmonary Artery: A Case Report and Literature Review

: Hereby, the case of an 8-month-old girl diagnosed with partial anomalous left pulmonary artery (PLPA) is presented. Echocardiography and computed tomography (CT) examination demonstrated that the anomalous artery originated from the right pulmonary artery supplying the left upper lobe. PLPA is sometimes associated with tracheobronchial anomaly and congenital cardiovascular defects. A relationship between PLPA and Kabuki syndrome has also been suggested. Echocardiography and CT angiography can be used as accurate tools for identifying an anomalous left pulmonary artery and adjacent abnormal anatomic structures.

Double Outlet Right Ventricle with Anomalous Left Pulmonary Artery Arising from Arterial Duct : Multimodality Imaging for Prepairing A Double-Root Translocation in A Rare Association

Double Outlet Right Ventricle with Anomalous Left Pulmonary Artery Arising from Arterial Duct : Multimodality Imaging for Prepairing A Double-Root Translocation in A Rare Association

Abnormal arterial anatomy and an unexpected intracranial haemorrhage

A baby boy was born at term by spontaneous vaginal delivery to non-consanguineous parents following an unremarkable pregnancy. He was admitted to his local neonatal intensive care unit shortly after...

Pulmonary Artery Sling And Exercise Capacity

INTRODUCTION: Pulmonary artery sling is a rare congenital vascular malformation that consists of an anomalous origin of the left pulmonary artery from the posterior aspect of the right pulmonary artery. The anomalous left pulmonary artery crosses the mediastinum posterior to the trachea or carina and anterior to the esophagus, compressing both organs. Respiratory complaints predominates over esophageal symptoms. In asymptomatic patients, surgical repair is controversial. Data regarding exercise capacity and other cardiopulmonary parameters is scarce. AIM: To evaluate exercise parameters in an early pubertal girl with uncorrected pulmonary artery sling at baseline and 3 years later (late puberty). METHODS: Maximal cardiopulmonary exercise testing (CPET) was completed on a cycle ergometer at age 11 y/o and 14 y/o by a girl with uncorrected pulmonary artery sling. Data was processed and analyzed retrospectively. RESULTS: Peak oxygen uptake (VO2) was higher in the first test (41.9 ml/kg/min, 119% predicted) than in the second test (30.6 ml/kg/min, 87% of predicted). Maximal and submaximal pulmonary exercise parameters improved in the second test (breathing reserve; 9.7 liters, 15% predicted in the first test vs. 31.5 liters, 39% predicted in the second test and for lowest VE/VCO2 and VE/VCO2 slope; 28.7 and 34.9 vs 25.7 and 27.7 respectively), with no similar expected improvement in cardiovascular parameters (oxygen pulse (VO2/HR) 9.2 ml/beat, 125% predicted in the first test vs. 9.3 ml/beat, 92% of predicted respectively and for VO2/HR slope; 14.1 ml/beat vs. 14.9 ml/beat). CONCLUSIONS: In this case of uncorrected pulmonary sling a deterioration in exercise capacity and cardiovascular exercise parameters was observed over puberty inspite of improvement in pulmonary parameters. Possible mechanisms are maturation dependent abnormal pulmonary sling flow, deconditioning and increased % body fat. To the best of our knowledge this is the first report of exercise evaluation in this unique population. More studies are needed with larger number of patients, including accurate measurement of body composition and characterizing exercise capacity evaluation following surgical repair.

Multislice Computed Tomography Assessment of Tracheobronchial Patterns in Partial Anomalous Left Pulmonary Artery.

The aim of this study was to present relationship between partial anomalous left pulmonary artery (PALPA) and the tracheobronchial tree and patterns of the tracheobronchial tree assessed by multislice computed tomography (MSCT). Nine patients were assessed by MSCT. The relationships between the tracheobronchial tree and PALPA and different tracheobronchial patterns, location of tracheobronchial stenosis, severity of stenosis, and associated cardiac defects were evaluated. The results of MSCT for these patients were compared with the operative findings. The anatomy of PALPA was clearly identified by MSCT in all 9 patients. Three relationships between PALPA and the tracheobronchial tree were noted. In addition, 3 patterns of tracheobronchial tree anatomy were also demonstrated. The PALPA arose from the right pulmonary artery, forming a pulmonary sling (n = 2). The PALPA, which arose from the proximal right pulmonary artery, went below the tracheal bifurcation and passed anterior (n = 1) or inferior-anterior (n = 6) to the proximal left main bronchus. Three patterns of the tracheobronchial tree were presented with normal (n = 5), normal pattern with right tracheal bronchus (n = 3), and bridging bronchus (n = 1). The rate of tracheobronchial stenosis was 56% (5/9).Five patients underwent operation, and at that time, the relationship between PALPA and the tracheobronchial tree defined on MSCT was confirmed. The PALPA can be associated with tracheobronchial anomalies and airway compression depending on its orientation to the airway. Noninvasive imaging modalities such as MSCT will be helpful for making further management decisions.

Pulmonary artery sling—a case report

Vascular sling/pulmonary artery sling is a congenital anomaly in which the left pulmonary artery arises from the right pulmonary artery extrapericardially (anomalous left pulmonary artery), courses to the left behind the tracheal bifurcation and in front of the esophagus to reach the left lung hilum, and forms a sling around the trachea. The prevalence is 1 in every 17,000 school-aged children. We present a 1.5-year-old male child of consanguineous parentage with history of recurrent chest infection and stridor leading to repeated hospitalization. Chest X-ray showed right upper lobe collapse with hyper inflated right middle and lower lobes. Echocardiography revealed left pulmonary artery arising from the right pulmonary artery with no intracardiac shunt. Cardiac computed tomography diagnosed the pulmonary artery sling—left pulmonary artery arising from the right pulmonary artery and compromising the lumen of proximal portion of right bronchus leading to right upper lobe collapse. The surgery included complete mobilization of the left pulmonary artery from its origin and along its course behind the trachea. The left pulmonary artery was disconnected from the right pulmonary artery and was then brought out from behind the trachea and aorta and reimplanted to the main pulmonary artery in an end to side fashion. Child developed chest infection postoperatively, which was treated with intravenous antibiotics and was subsequently discharged on postop day 10. Child is on regular follow-up for the past 8 months and is doing well.

Радикальная коррекция двойного отхождения сосудов от правого желудочка с пластикой трахеи при ринслинг комплексе в условиях искусственного кровообращения и экстракорпоральной мембранной оксигенации у 3-месячного ребенка

В описании клинического случая представлена коррекция редкой патологии – ринг-слинг комплекса в сочетании с двойным отхождением сосудов от правого желудочка у ребенка 3-месячного возраста. Составляющая ринг-слинг комплекса – это неразделенные хрящевые кольца вместе с аномальным отхождением левой ветви легочной артерии от правой ветви с ретротрахеальным ходом и формированием сосудистой петли или так называемого слинга. В мировой литературе данное сочетание, включающее скользящую пластику трахеи и радикальную коррекцию двойного отхождения сосудов от правого желудочка, не описано. Особенностью представленного наблюдения является также ранний возраст пациента. Для клинициста интерес представляют также внезапные изменения клинического состояния больного в результате течения заболевания, что потребовало не только реимплантации аберрантной левой легочной артерии, но и выполнения жизнеспасающей трахеопластики с необходимостью продленного сеанса экстракорпоральной мембранной оксигенации. Следует также отметить, что активная кардиохирургическая помощь больным с сопутствующей патологией трахеи в условиях искусственного кровообращения в нашей стране осуществляется только в последние несколько лет. В связи с этим каждое подобное наблюдение является ценным для накопления опыта, пока еще ограниченного единичными случаями.

PT232 Origin Anomalous Left Coronary Trunk Pulmonary Artery (ALCAPA) in Adult: Case Report

PT232 Origin Anomalous Left Coronary Trunk Pulmonary Artery (ALCAPA) in Adult: Case Report

Anomalous branch of pulmonary artery from the aorta and tetralogy of Fallot: morphology, surgical techniques and results

Tetralogy of Fallot (TOF) with hemitruncus (HT) is a rare entity. In this report, we present our experience with this condition over the last 20 years. Between January 1994 and June 2013, 11 patients with HT and TOF underwent surgery at the All India Institute of Medical Sciences, New Delhi, India. All available clinical, radiographic, echocardiographic, cardiac catheterization, operative and follow-up data were reviewed. The mean age was 73 ± 7.1 months (range 7 months to 18 years) and the mean weight was 15.7 ± 1.2 kg. The mean preoperative saturation was 79.3 ± 11.7% (range 62-92%). Six patients had anomalous left pulmonary artery (PA), whereas 5 had an anomalous right PA arising from the aorta. Surgical procedures consisted of complete intracardiac repair of TOF with direct implantation of the anomalous PA into the main PA (n = 7), intracardiac repair of TOF with an interposition saphenous vein graft between the right PA and main PA (n = 1), and reconstruction of the left PA with autologous pericardium with intracardiac repair of TOF (n = 1), direct implantation of the anomalous PA into the main PA with an innominate to right pulmonary artery shunt (n = 1) and a right PA banding with innominate to left PA shunt (n = 1). There were two early deaths. Follow-up ranged from 3 to 73 months. All survivors are in NYHA Class I and follow-up echocardiograms did not show any residual lesions. Surgical repair of HT with TOF results in acceptable early outcomes. The surgical strategy needs to be individualized to the anatomy of the patient.

Computational fluid dynamics simulation of airflow in the trachea and main bronchi for the subjects with left pulmonary artery sling.

BackgroundLeft pulmonary artery sling (LPAS) is a rare but severe congenital anomaly, in which the stenoses are formed in the trachea and/or main bronchi. Multi-detector computed tomography (MDCT) provides useful anatomical images, but does not offer functional information. The objective of the present study is to quantitatively analyze the airflow in the trachea and main bronchi of LPAS subjects through computational fluid dynamics (CFD) simulation.MethodsFive subjects (four LPAS patients, one normal control) aging 6-19 months are analyzed. The geometric model of the trachea and the two main bronchi is extracted from the MDCT images. The inlet velocity is determined based on the body weight and the inlet area. Both the geometric model and personalized inflow conditions are imported into CFD software, ANSYS. The pressure drop, mass flow ratio through two bronchi, wall pressure, flow velocity and wall shear stress (WSS) are obtained, and compared to the normal control.ResultsDue to the tracheal and/or bronchial stenosis, the pressure drop for the LPAS patients ranges 78.9 - 914.5 Pa, much higher than for the normal control (0.7 Pa). The mass flow ratio through the two bronchi does not correlate with the sectional area ratio if the anomalous left pulmonary artery compresses the trachea or bronchi. It is suggested that the C-shaped trachea plays an important role on facilitating the air flow into the left bronchus with the inertia force. For LPAS subjects, the distributions of velocities, wall pressure and WSS are less regular than for the normal control. At the stenotic site, high velocity, low wall pressure and high WSS are observed.ConclusionsUsing geometric models extracted from CT images and the patient-specified inlet boundary conditions, CFD simulation can provide vital quantitative flow information for LPAS. Due to the stenosis, high pressure drops, inconsistent distributions of velocities, wall pressure and WSS are observed. The C-shaped trachea may facilitate a larger flow of air into the left bronchus under the inertial force, and decrease the ventilation of the right lung. Quantitative and personalized information may help understand the mechanism of LPAS and the correlations between stenosis and dyspnea, and facilitate the structural and functional assessment of LPAS.

Anomalous Origin of the Left Pulmonary Artery from the Right Pulmonary Artery (Pulmonary Artery Sling)

PULMONARY artery (PA) sling is created by anomalous origin of the left PA from the posterior aspect of the right PA. The anomalous left PA courses over the right mainstem bronchus and then from right to left, posterior to the trachea or carina and anterior to the esophagus, to reach the hilum of the left lung. This compresses the esophagus and the lower trachea and right mainstem bronchus, producing feeding and respiratory problems, such as recurrent cough and respiratory infection, stridor and wheezing. In addition, associated anomalies of the tracheobronchial tree and/or of the cardiovascular system are present in about 50% of the patients.1,2In addition, bronchoscopy can show a pulsating stricture in the trachea. Medical care is supportive until the patient can undergo definitive surgical correction. Anesthetic management is the same as with any tracheal obstruction; spontaneous ventilation for bronchoscopy and endotracheal intubation bypassing the tracheal restriction for other surgeries. Hypoxemia and respiratory distress are treated with supplemental oxygen and endotracheal intubation. Surgical repair consists of transection of the left PA at its origin and implantation into the main PA anterior to the trachea. Tracheoplasty utilizing end-to-end anastomosis is often needed if the sling is not repaired early. In this case, cardiopulmonary bypass or extracorporeal membrane oxygenation may be required. The anatomy could be correctly diagnosed by magnetic resonance imaging in 97% and by angiography in 90% of patients3 (angiogram shows pulmonary trunk, right PA, and left PA).

Left pulmonary artery sling: Difference in monochorionic monoamniotic twin presentation

Pulmonary artery sling (PAS) is a rare congenital vascular anomaly where the left pulmonary artery (LPA) originates from the right pulmonary artery and passes between the trachea and esophagus. Limited literature has been published about PAS in twins. This report demonstrates two different outcomes in mono-mono twins presenting with PAS. Patients with PAS can experience sequelae including wheezing, stridor, atelectasis, or emphysema. Vascular rings occur when the anomalous LPA encircles the airway and esophagus, causing direct compression and respiratory distress. In the case of LPA sling, the sling does not encircle the airway and esophagus but acts as a cradle so the airway which is adjacent to the vessel is not directly compressed. Unfortunately, patients with LPA sling may become severely ill due to airway narrowing even when there is no compression of the adjacent airway. This is the more common presentation in PAS and was seen in Twin A whereas direct airway compression was seen in Twin B. PAS usually presents in patients during their first year of life and high clinical suspicion is important for diagnosis. As this case report demonstrates, presentations and outcomes can be variable in mono-mono twins with LPS and as a result, appropriate investigations should be offered with monitoring of respiratory compromise.

Anomalous Left Pulmonary Artery Without Sling Formation

A case of aberrant left pulmonary artery originating from the right pulmonary artery resembling pulmonary artery sling malformation, but without true sling formation, is presented. Anomalous left pulmonary artery was accompanied with long-segment proximal tracheal stenosis and other congenital malformations typically associated with PAS complex. The anomalous pulmonary artery passed anterior to the trachea; therefore, no true sling was formed. Because no airway compression by the aberrant left pulmonary artery was detected, the proximal tracheal stenosis was thought to be primary.

Partial anomalous left pulmonary artery with associated bronchial anomalies in a patient with repaired tetralogy of Fallot

We present a 28 year old man with repaired Tetralogy of Fallot who underwent CT imaging prior to pulmonary valve replacement. CT demonstrated a partial anomalous LPA with associated bronchial anomalies and normal variant pulmonary venous drainage. The case demonstrates the utility of multislice CT in delineating 3-dimensional vascular and bronchial anatomy in these complex anomalies.

The anomalous origin of the branch pulmonary artery from the ascending aorta

The anomalous origin of one pulmonary artery branch from the aorta (AOPA) is rare. We report our single-institution surgical experience with this condition. Between January 1994 and February 2011, 17 patients (age: 1 month-25 years) with AOPA underwent surgery at our institute. Thirteen patients had an anomalous origin of the right pulmonary artery (RPA) while four had an anomalous origin of the left pulmonary artery (LPA) from the aorta. In patients with anomalous RPA, 11 patients had the proximal type and two patients had the distal type of AOPA. Four patients had associated Tetralogy of Fallot (TOF). In 14 patients, direct implantation into the main pulmonary artery was performed, while three patients required interpositon of a graft. There was one operative death due to persistent hypoxia in a 7-month old child with TOF and an anomalous LPA from the aorta. At a median follow-up of 36.5 months (range: 2-192 months), all 16 survivors were asymptomatic. On echocardiography, two patients showed a gradient of 25 and 30 mmHg across the anastomosis and are being followed up. In our experience, early repair of AOPA results in acceptable haemodynamic and anatomic results. Long-term survival can be expected with a low incidence of re-operation or re-intervention.