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Anomalous origin of right pulmonary artery (RPA) from ascending aorta in a 7-day-old girl who also has double-outlet right ventricle (DORV). a CT angiography oblique sagittal 3-D reconstruction shows both dilated aorta (AO) and smaller main pulmonary artery (MPA) arising from the right ventricle (RV), consistent with DORV. There is a subpulmonic ventricular septal defect (VSD) as well as a smaller, more inferior muscular VSD (arrows). The left pulmonary artery (LPA) is continuous with the MPA. LV left ventricle. b CT angiography coronal oblique 3-D reconstruction shows the RPA arising from the proximal ascending aorta (AO). c CT angiography oblique axial maximum-intensity projection image shows that the RPA and LPA are discontinuous, with the RPA arising from the right/posterior proximal ascending aorta while the LPA arises separately. DAO descending aorta, LA left atrium
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There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range...
Citations
... Criss-cross pulmonary arteries are a type of branch pulmonary artery anomaly, characterized by the left pulmonary artery ostium originating to the right and slightly above the right pulmonary artery ostium [1][2][3]. Although the clinical significance of criss-cross pulmonary artery anomaly when detected in isolation is unclear, it often accompanies complex congenital heart diseases, vascular anomalies, and syndromes. ...
We aimed to assess the frequency of criss-cross pulmonary arteries and associated intracardiac and vascular anomalies in patients who underwent CT angiography due to suspected congenital heart disease or vascular anomaly at our hospital. We retrospectively evaluated the CT angiography images of 355 patients aged 0–18 years between April 2018 and December 2022. The presence of the criss-cross pulmonary artery anomaly was assessed. Additionally, in patients with a criss-cross pulmonary artery anomaly, accompanying branch pulmonary artery anomalies, aortic arch anomalies, and other vascular-cardiac anomalies were also evaluated. A total of 331 patients’ images were evaluated. Criss-cross pulmonary artery anomaly was present in 57 patients (17.2%). Pulmonary artery branch anomaly was present in 16, aortic arch anomaly in 40 patients (70%) with criss-cross pulmonary artery anomaly, while associated intracardiac pathology (by echocardiography) was detected in 43 patients (75.4%). The frequency of criss-cross pulmonary artery was found to be significantly higher in patients with any aortic arch anomaly (p = 0.01). This study represents one of the largest series of patients with criss-cross pulmonary artery anomalies. Our results suggests that it may be more common than previously recognized and potentially overlooked. It is crucial to consider the presence of this anomaly in patients with complex aortic arch anomalies or cardiac pathologies, as it may have implications for surgical approaches and potential complications. Increased awareness of this anomaly among cardiologists and radiologists is necessary for accurate diagnosis and appropriate management.
... The incidence of congenital pulmonary artery (PA) anomalies varies widely: that of isolated pulmonary stenosis (PS) is approximately 1/2000 live births while that of pulmonary agenesis is around 1/100 000 live births [1][2][3] . They are part of a heterogeneous spectrum of diseases and their antenatal diagnosis requires good knowledge of fetal cardiac anatomy because their clinical presentation varies depending on the type and severity of the underlying lesion 4 . ...
Congenital pulmonary vascular anomalies are rare. Antenatal diagnosis of these vascular anomalies requires a good knowledge of fetal cardiac anatomy because clinical presentations are variable. In fact, screening of pulmonary arteries anomalies can be not complex in some cases because of important associated consequences easily detected on ultrasound, but some other anomalies have more discrete features. The severity depends on the underlying lesion and whether or not it is isolated. Moreover, they may be associated with genetic syndromes as well. The aim of this review is to define and review all unusual and abnormal situations of the main pulmonary artery and its branches and to propose, through the identification of "red flags" during routine antenatal heart examination, an optimal screening around the pulmonary pathway. Fourteen different antenatally diagnosed entities of pulmonary artery anomalies were defined. Those entities belonging to the four following family of disorder: 4 anomalies of the pulmonary valvular region with stenosis or atresia of the valve; 4 anomalies related to conotruncal abnormalities; 4 anomalies associated with the abnormal origin or pathway of the pulmonary artery; and finally, 2 anomalies belonging to primitive abnormal development of the pulmonary artery and its branches. We highlight the need to differentiate three‐vessel and three‐vessel and trachea views when assessing a fetus with an abnormality belonging the CPA family spectrum. This article is protected by copyright. All rights reserved.
... This shift produces distortion of cardiomediastinal structures, which is more marked when the right lung is absent because of greater shift of the normally left-side heart, associated vessels and airways. Most cases of unilateral pulmonary agenesis are associated with other anomalies, including congenital heart disease, other vascular abnormalities (pulmonary sling, other PA anomalies, anomalous origin of arch great vessels), esophageal atresia, tracheal stenosis, lung and vertebral anomalies [10,11] (Fig. 2). ...
... Congenital tracheal stenosis is associated with several other lesions including tracheal bronchus and other airway/lung anomalies (Fig. 2) as well as pulmonary sling, other structural cardiovascular disease, H-type tracheoesophageal fistula and Down syndrome. Long-segment tracheal stenosis is strongly associated with Type 2 pulmonary sling, occurring in 2/3 of cases [10,12] (Fig. 3). In the PA sling anomaly, the left pulmonary artery arises from the right pulmonary artery and courses to the left hilum between the trachea and esophagus (Fig. 3). ...
... A variety of airway abnormalities accompany the Type 2 sling, including separate right upper lobe bronchus or diverticulum at the normal carinal level resembling a tracheal bronchus, long-segment airway stenosis, low horizontal pseudocarina and bridging right bronchus arising from the left bronchus ( Fig. 3) [13][14][15]. In the Type 1 pulmonary sling, the airway is usually not stenotic, but the right bronchus might be compressed by the sling or malacic, resulting in air trapping in the right lung; a tracheal bronchus might be present; and the carina is at the normal level [10,13]. Congenital cardiac anomalies are often also associated with pulmonary sling (mostly Type 2) including ventricular septal defect (VSD) or more complex heart disease. ...
Airway abnormalities are important but sometimes overlooked problems in children with congenital heart disease. It is often difficult to separate symptoms related to cardiac disease from those associated with airway or lung disease. Some of the lesions are incidental while others cause significant symptoms and are important in overall functional outcome. Congenital and acquired as well as intrinsic and extrinsic lesions occur and can overlap. We review and illustrate these lesions here. Imaging plays a crucial role in diagnosing and assessing the severity of airway abnormalities and guiding medical and surgical management decisions.
... The underlying cause is constriction of the ductal tissue as it extends into the adjacent aorta or proximal left pulmonary artery [15,16]. Flow reversal within the ductus can occur if the ductus connects to an obstructed systemic circulation, as with severe coarctation or interrupted aortic arch [17]. ...
The fetal circulation is characterized by the presence of three physiological vascular shunts — the ductus arteriosus, the foramen ovale and the ductus venosus. Acting in concert, these shunts preferentially stream blood flow in a pattern that maximizes efficiency of blood oxygenation by the maternofetal unit. Shortly following the transition to extrauterine life, a quick and predetermined succession of events results in closure of these embryological structures with consequent establishment of postnatal vascular flow patterns. While this transition is often seamless, the physiological shunts of the fetus occasionally fail to regress. Such failure to regress can occur in isolation or in association with other congenital malformations. This failed regression challenges the circulatory physiology of the neonate and might have implications for the optimum functioning of several organ systems. When symptomatic, these shunts are treated. Interventions, when undertaken, might be medical, endovascular or surgical. The radiologist’s role continues to expand in the assessment of these shunts, in providing a roadmap for treatment and in prompt identification of treatment-related complications. This review is to familiarize radiologists with the embryology, pre- and post-treatment imaging appearances, and associated complications of persistent fetal vascular shunts.
... Main PA and branch PA size are best assessed for enlargement on CT angiography by normalizing measurements to the child's body surface area rather than comparing to the ascending aorta, which could be abnormally small or large in a child with congenital heart disease [5]. CT angiography is superior to echocardiography for assessing the pulmonary arterial tree and is particularly useful for assessing central and peripheral pulmonary arterial stenoses from heritable arteriopathies such as Williams, Alagille and Noonan syndromes [6]. ...
Although rare in the pediatric population, pulmonary hypertension is a significant cause of morbidity and mortality in affected individuals. In addition to evaluating potential causes and severity of parenchymal lung diseases, non-contrast high-resolution CT of the chest can aid in the diagnosis of heritable and acquired causes. In addition to evaluating parenchymal lung disease, CT angiography can help to confirm findings of pulmonary hypertension using criteria similar to echocardiography, and provide detailed assessment of the pulmonary vascularity in specific causes.
... Most of the cases in the literature refer to cava and azygos venous systems coronary sinuses, brachiocephalic veins, umbilical and vitelline veins, and their derivatives and anomalies, and even lesser are the cases involving pulmonary circulation [1,6,9,[13][14][15]. Regarding pulmonary circulation anomalies, veins encounter malposition and atrial connection anomalies [9], while arteries Diagnostics 2021, 11, 2398 2 of 12 are described to run into defects more often, such as caliber and origin malformations, some of which are included in the description of congenital syndromes [16,17]. ...
Cardiac and cardiovascular malformations are of real interest in terms of definition, epidemiology, and means of early diagnosis by imaging. Although ultrasound examination reaches exceptional performance nowadays, unusual pathologies are still exposed to the risk of either incorrect acquired image or misinterpretation by the specialist in a routine scan. Herein, we present a case of a 20-week-old fetus (from an apparently low-risk pregnancy) with complex cardiac and vascular abnormalities, including an arteriovenous malformation along with ventricular septal defect, ductal coarctation of the aorta, aneurysm of a brachiocephalic vein, and dilation of the entire neck and upper mediastinum venous system, and the limitations that were encountered in the process of diagnosis and management of the case.
... Dear Editors, We would like to thank the authors Newman and Alkhori [1,2] for the excellent two-part review concerning congenital anomalies of the central pulmonary arteries. The articles synthesize and illustrate a wide range of pulmonary artery abnormalities. ...
... Malposition of the branch pulmonary arteries is usually accompanied by congenital heart disease, of which ventricular septal defects (48.8% of cases; Online Supplementary Material 1), atrial septal defects (43.2%) and conotruncal defects (38.6%) are the most recurrent. Newman and Alkhori [2] claimed that malposition of the branch pulmonary arteries is commonly associated with left pulmonary artery stenosis; however, such stenosis was described in only one case [5,8]. A significant portion is associated with some syndrome or mutation (27.3% of cases), among which the main one is DiGeorge syndrome (22q11 deletion) [5,8]. ...
... malposition of the branch pulmonary arteries without crossing (left pulmonary artery ostium superior to the right pulmonary artery ostium, and pulmonary artery path directed to the respective lungs, without crossing); (c) malposition of the branch pulmonary arteries with typical crossing or crossed pulmonary arteries (left pulmonary artery ostium superior and rightward to the right pulmonary artery ostium, and pulmonary artery path directed to the respective lungs, with crossing); and (d) malposition of the branch pulmonary arteries with atypical crossing (left pulmonary artery ostium inferior and rightward to the right pulmonary artery ostium, and pulmonary artery path directed to the respective lungs, with crossing) Fig. 2 Imaging in a 2-month-old boy with malposition of the branch pulmonary arteries and typical crossing. a, b Superior (a) and lateral (b) CT volume-rendered images depict the origin of the left pulmonary artery (1) (superior and rightward of the contralateral artery); the intersection of pulmonary arteries (2) anterior to the trachea (3); the superior vena cava (4); persistent left superior vena cava (5); and pulmonary ductus diverticulum (6) ...
... Pulmonary atresia is classified into pulmonary atresia with ventricular septal defect (PA-VSD) and pulmonary atresia with intact interventricular septum (PA-IVS) [25]. ...
... The van Praagh classification divides truncus arteriosus with VSD (Type A) into four types. In Type A1, a common arterial trunk for aorta and main pulmonary artery ; in Type A2, no MPA present, and separate origin of right and left central branch PAs from the posterior or posterolateral proximal common trunk; in Type A3, origin of one central PA branch (usually right) from the proximal common trunk while the opposite lung is supplied by a central branch PA originate from the aortic arch (ductal origin) or aortopulmonary collateral supply; in Type A4, there is interrupted aortic arch with Types 1 or 2 PA branching and a ductus arteriosus supplying the descending aorta [25]. ...
... Pulmonary artery sling is a rare vascular anomaly causing respiratory distress in which the left pulmonary artery originates from the right pulmonary artery which courses between the trachea and esophagus toward the left hilum [25]. ...
Congenital pulmonary arteries anomalies included a wide-ranged spectrum of pathology that is usually associated with other congenital heart diseases. These anomalies can be classified into several major categories, which show considerable overlap among those categories. The aim of this study is to evaluate the role of multi-slice computed tomography (MSCT) angiography in assessment of pulmonary arteries anomalies. This study included 76 patients (42 males and 34 females) with male to female ratio 55.2% to 44.7%. The age of the patients ranged from 4 days to 15 years. The studied cases for pulmonary arteries anomalies were subclassified to anomalies of the caliber, origin and development (conotruncal anomalies). We compared MSCT findings with the data collected by cardiac catheterization and/or operation in 38 patients. 320-MSCT diagnosed cases of pulmonary arterial anomalies with 99% sensitivity, 99.8% specificity, 99% PPV, 99.8% NPV and 93.4% accuracy. This study concluded that MSCT is a non-invasive imaging modality that has a significant value in the evaluation of the congenital pulmonary arteries anomalies and its associated extracardiac anomalies in pediatric patients as well as assessment of post-operative complications. It is superior to ECHO in evaluating the pulmonary artery anomalies specially the pulmonary artery branches which are obscured by aerated lung. Also, there is good outcome of the cases owing to early and accurate diagnosis of the cases and post-operative follow up.
