Origin Of Pulmonary Artery Research Articles

Anomalous origin of right pulmonary artery from ascending aorta - A case report

Anomalous origin of right pulmonary artery from ascending aorta - A case report

Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.

The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic imaging technology have led to better delineation of the vasculature anatomy and the identification of previously unrecognized and unclassified anomalies. In this manuscript, we review the existing literature and propose a series of modifications to the original Stewart classification of congenital aortic arch abnormalities to incorporate this new knowledge. In brief, we propose the following modifications: (1) In Group I, we further divide subgroup B into left arch atretic and right arch atretic; (2) In Group II, we add three more subgroups, including aberrant right innominate artery, "isolated" right innominate artery (RIA), "isolated" right carotid artery with aberrant right subclavian artery; (3) In Groups I, II, and III, we add a subgroup of absence of both ductus arteriosus; and (4) In Group IV, we add three subgroups, including circumflex retro-esophageal aorta arch, persistent V aortic arch, and anomalous origin of pulmonary artery from ascending aorta.

Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis

Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis

Fetal aortic arch and its branches anomalies: prenatal ultrasound, vascular cast and gene detection

Objective To explore the application values of prenatal ultrasound, vascular cast in the diagnosis of fetal aortic arch and its branches anomalies and to analyze the genetic characteristics by gene detection. Methods Twenty-two cases of the vascular cast specimens of the fetal aortic arch and its branches anomalies were analyzed and studied by comparing with their prenatal ultrasonography. Then the characteristics of each type of fetal aortic arch and its branches anomalies, the missed diagnosis and misdiagnosis were summarized and the results of their gene detection were also analyzed. Results The 22 cases of fetal aortic arch and its branches anomalies were as follows: 2 cases of double aortic arch showed the ascending aorta was divided into two branches after converging as the descending aorta. Three cases were left aortic arch with aberrant right subclavian artery. Twelve cases were right aortic arch: 8 cases were right aortic arch with mirror-image branching, 3 cases were right aortic arch with aberrant left subclavian artery, 1 case was right aortic arch with isolated left subclavian artery. Of the 8 right aortic arch with mirror-image branching, 3 cases of left arterial duct showed the vertical walking between the fusion site of the left innominate artery and the pulmonary artery. Right aortic arch with aberrant left subclavian artery with arterial duct showed U shaped vascular ring. Five cases were other types, including 2 cases of the coarctation of aortic arch, 1 case of interrupted aortic arch, 1 case of pulmonary artery sling, and 1 case of abnormal origin of right pulmonary artery. The ultrasonic missed diagnosis were the 6 deformities: 3 cases of arterial duct and 3 cases of aberrant subclavian artery. The ultrasonic misdiagnosis were the 5 deformities: 2 cases of arterial duct location, 1 case of aberrant subclavian artery, 1 case of isolated left subclavian artery, and 1 case of the coarctation of aortic arch. Genetic test results: In the 18 cases of the genetic detection, 2 cases were positive, 1 case was 22q11.2 microdeletion syndrome and 1 case was carrying KMT2D gene variant. Conclusions There are various kinds of fetal aortic arch and its branches anomalies, which are often associated with intracardiac malformations and venous branches variation. And prenatal ultrasound is of great value in diagnosing them.Vascular cast can visually display their characteristic changes, which is helpful to improve the differential diagnosis of the different aortic arch and its branches anomalies. The detailed genetic detection can improve the further understanding of its etiology. Key words: Ultrasonography, prenatal; Fetus; Aortic arch anomalies; Vascular cast; Gene detection

Late Presentation of Anomalous Origin of the Right Pulmonary Artery

Late Presentation of Anomalous Origin of the Right Pulmonary Artery

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.

Prenatal diagnosis of anomalous origin of pulmonary artery.

Anomalous origin of the pulmonary arteries is a rare congenital pulmonary vascular malformation that includes unilateral absence of the pulmonary artery (UAPA), anomalous origin of unilateral pulmonary artery (AOPA), and left pulmonary artery sling (LPAS). We analyze 15 cases of fetal pulmonary artery abnormalities from 2011 to 2017, detected via prenatal ultrasound at our center. The 15 cases include UAPA (5), AOPA (6), and LPAS (4). Of the UAPA cases, 2 had pulmonary atresia and 3 had pulmonary artery stenosis. In 5 cases, the descending aorta issued collateral vessels to the left lung. Of the AOPA cases, 2 had left pulmonary artery abnormalities originating in the ascending aorta and 4 showed right pulmonary artery abnormalities originating in the ascending aorta. Of the LPAS cases, 2 were type IA, 1 was type IIA, and 1 was type IIB. Two of the LPAS were associated with right lung dysplasia. Abnormal origin of the pulmonary artery has a characteristic ultrasonographic appearance. The branch of the pulmonary artery cross section can provide important clues to the diagnosis of abnormal pulmonary artery origin. Pulmonary artery abnormalities are often associated with pulmonary atresia or stenosis.

Restoring Fetal Circulation as a Means of Bridging Treatment Prior to Surgical Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta with Persistent Pulmonary Hypertension of the Newborn.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario.

Two Cases of Staged Repair of Anomalous Origin of Right Pulmonary Artery from the Ascending Aorta

Two Cases of Staged Repair of Anomalous Origin of Right Pulmonary Artery from the Ascending Aorta

Аберрантное отхождение левой ветви легочной артерии, стеноз трахеи и ринг-слинг комплекс (Часть I)

В публикации представлено обзорное описание проблем, связанных с крайне редкой патологией, – ринг-слинг (англ. «ring-sling») комплексом, включающим аномальное отхождение левой ветви легочной артерии от ее правой ветви и наличие неразделенных колец трахеи. Достаточно полно обобщены как базисные, так и современные литературные сведения, касающиеся терминов, истории описания, классификаций, теорий и основ эмбриогенеза порока. Указаны также трудности, не позволяющие дать общепринятое определение патологии, объясняется, можно ли считать понятия «слинг легочной артерии» и «аномальное отхождение левой легочной артерии от правой» равнозначными. Раскрыт термин «ринг-слинг комплекс», описаны причины рассмотрения данной патологии под более частым названием слинга в обширном разделе полных и неполных сосудистых колец и сосудистых петель. Это, в свою очередь, целый ряд различных пороков развития, к которым относят аномалии дуги аорты и/или ее расположения, двойную дугу аорты, персистирование пятой дуги аорты, перерывы дуги аорты и др. Подобное разнообразие объясняется особенностями эмбриологического развития артериальных сосудистых дуг, в результате нарушения развития которых и появляется та или иная патология. Однако по многим из этих нозологий в нашей стране и в мире в целом имеется лишь ограниченный опыт как хирургического лечения, так и общего описания количества наблюдений. Слинг легочной артерии в этом плане не исключение и считается одной из наиболее редких и в то же время типичных разновидностей сосудистых петель. Слинг также является одной из «классических» причин развития респираторного дистресс-синдрома у детей, вызванного врожденной сосудистой патологией. Термин «слинг легочной артерии», являясь достаточно распространенным, используется, в свою очередь, в отношении неоднородной группы больных. По меньшей мере, выделяется два варианта течения или две группы, существенно отличающихся по прогнозу и тактике лечения. В одних случаях нарушения трахеобронхиальной проходимости связывают в основном с компрессией и трахеобронхомаляцией, в других же субстрат обструкции дыхательных путей связан с наличием врожденного стеноза трахеи или полных колец трахеи (собственно ринг- слинг комплекс). Определить, к какому из этих типов следует отнести патологию, является одной из основных задач специалиста, установившего диагноз сосудистой петли. Помимо этого, частое наличие сопутствующих пороков сердца, встречающихся при слинге легочной артерии приблизительно в половине случаев, также значительно усложняет выбор тактики и прогноз в целом. Обзор литературы вполне может иметь продолжение в части диагностики, тактики, методов хирургического лечения и результатов

Aberrant origin of the left pulmonary artery, congenital tracheal stenosis and ring-sling complex. Part II.

Aberrant origin of the left pulmonary artery, congenital tracheal stenosis and ring-sling complex. Part II.

Double Aortic Arch Presenting with Neonatal Respiratory Distress But not Due to Airway Obstruction: Anatomic and Embryologic Explanation

Double aortic arch resulting from embryonic persistence of bilateral fourth branchial arches often leads to neonatal stridor due to tracheal compression. Two neonates presented with dyspnea without evidence of tracheal compression. An aneurysmal ductal aneurysm from the left-sided arch in the first neonate led to symptoms. Spontaneous duct closure and regression of ductal aneurysm led to relief of symptoms. Second neonate had severe neonatal pulmonary hypertension due to aortic origin of left pulmonary artery (LPA) that was disconnected from main PA. Surgical reanastomosis of the LPA and division of the smaller right arch corrected the anatomy. These two associations show that airway obstruction alone is not the cause of respiratory symptoms in neonates with double aortic arch.

The Management of Left Pulmonary Artery Sling in Senegal: Our Experience from One Case

Pulmonary Artery Sling (PAS) is a rare congenital heart disease due to abnormal origin of Left Pulmonary Artery (LPA) from the Right Pulmonary Artery (RPA), then encircling the distal part of the trachea. The treatment is a surgical repair consisting of reimplantation of the LPA on the main pulmonary artery over cardiopulmonary bypass. The authors report a clinical case of PAS in a 5.5 years old boy diagnosed in Senegal then successfully operated in France.Cardiovasc. j. 2017; 10(1): 94-96

Paediatric case of ductal origin of right pulmonary artery presenting with exertional dyspnoea and mimicking asthma and primary ciliary dyskinesia

Ductal origin of pulmonary artery is a rare anomaly that is frequently misdiagnosed. Patients may present with exertional dyspnoea, recurrent respiratory infections and pulmonary hypertension. The presence of pulmonary hypertension...

Main pulmonary artery trifurcation in association with persistent right common pulmonary vein: a piece of embryological puzzle.

Wide variety of congenital anomalies of pulmonary arteries like pulmonary stenosis, pulmonary atresia, proximal interruption, anomalous origin of left pulmonary artery (LPA) and idiopathic dilatation of pulmonary trunk are described in the literature (1,2). Few studies have also described the variations in pulmonary artery branching pattern, mainly describing anomalies of lobar and segmental branching pattern (3,4). Combination of ipsilateral arterial and venous anomaly is relatively uncommon. We present a unique case of main pulmonary artery (MPA) trifurcation into LPA, right upper and lower lobar pulmonary arteries with associated persistent right common pulmonary vein (rCPV). We review the embryology and propose an embryological explanation to this rare anatomical variation.

Prenatal Diagnosis of Anomalous Origin of the Right Pulmonary Artery

Prenatal Diagnosis of Anomalous Origin of the Right Pulmonary Artery

Congenital anomalies of the pulmonary arteries: Spectrum of findings on computed tomography

ObjectiveCongenital anomalies of the pulmonary arteries are uncommon. They can occur in isolation or in association with congenital heart defects. Isolated congenital anomalies remain undiscovered until they are reported as incidental findings on imaging tests, usually not until adolescence. We review the embryological development and normal anatomy of the pulmonary arteries as well as the spectrum of computed tomography findings for various congenital anomalies: unilateral interruption of the pulmonary artery, anomalous origin of the left pulmonary artery (pulmonary artery sling), idiopathic aneurysm of the pulmonary artery, and other anomalies associated with congenital heart defects. ConclusionCongenital anomalies of the pulmonary arteries represent a diagnostic challenge for clinicians and radiologists. Computed tomography is useful for their diagnosis, and general radiologists need to be familiar with their imaging appearance because they are often discovered incidentally.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Anomalous origin of right pulmonary artery from innominate artery: Repair using pulmonary artery pedicled flap plasty.

Origin of the right pulmonary artery from innominate artery is an exceedingly rare anomaly. We report two cases with this anomaly that underwent surgical repair. The surgical technique described achieves tissue-to-tissue anastomosis using a pedicled flap from the main pulmonary artery.

Rare Case of Membranous Obstruction at the Origin of Left Pulmonary Artery: Role of Perioperative Echocardiography to Evaluate the Anatomy of Pulmonary Artery

ABSTRACT Branch pulmonary artery (PA) stenosis may occur as an isolated finding or in conjunction with other cardiac malformations; it may be congenital or acquired postoperatively. Branch PAs need to be assessed individually to determine the adequacy of their size, whether they are confluent or nonconfluent, any stenosis at origin or distally, and severity of stenosis. Certain lesions, such as supravalvar pulmonary stenosis, or stenosis at the branch PAs bifurcation, are better dealt with at surgery. Thus, perioperative transthoracic and transesophageal echocardiography (TEE) assume a very important role in precisely evaluating the anatomy of main and branch PAs. How to cite this article Bhat IH, Damodaran S, Mandal B. Rare Case of Membranous Obstruction at the Origin of Left Pulmonary Artery: Role of Perioperative Echocardiography to Evaluate the Anatomy of Pulmonary Artery. J Perioper Echocardiogr 2017;5(2):74-76.