Case Of Pulmonary Artery Sling Research Articles

Anomalous origin of the fetal pulmonary artery.

This study aims to investigate the efficacy of prenatal ultrasonography in diagnosing the anomalous origin of the fetal pulmonary artery (AOFPA). A total of 26 AOFPA cases were retrospectively analyzed from January 2014 to January 2023. The features of the AOFPA were characterized by comparing the prenatal ultrasonic data with the results of anatomical casting after pregnancy termination or postnatal imaging and surgical intervention. Missed diagnoses and misdiagnoses were expounded. Of the 26 AOFPA cases, there were 13 cases of pulmonary artery sling, 8 cases of anomalous origin of the unilateral pulmonary artery, and five cases of unilateral absence of the pulmonary artery; 17 cases received pathological anatomy and casting after pregnancy termination, and nine cases were confirmed by postnatal imaging and surgery. Nineteen cases were accurately prenatally diagnosed (19/26, 73.1%), and seven cases were missed or misdiagnosed (7/26, 26.9%). Prenatal ultrasonography has a favorable diagnostic efficacy for anomalous origin of the fetal pulmonary artery. The absence of either the left or right pulmonary artery from the image of pulmonary artery bifurcation may indicate origin abnormalities of the pulmonary artery in fetuses, which signifies the necessity to detect the abnormal origin of the pulmonary artery on the affected side and other potential intracardiac malformation complications.

A case of pulmonary artery sling, unilateral lung hypoplasia, and congenital heart disease

Vascular rings are rare congenital anomalies that primarily result from an embryological derangement of the paired aortic arches or branching pulmonary arteries. They make up <1% of all cardiac defects. Double aortic arch and right-sided aortic arch with the left ligamentum arteriosum are the most common types, making up 85– 95% of cases, with pulmonary artery slings making up about 10% of cases. The prevalence is estimated as 59 per million children with very few cases reported worldwide. The clinical presentation is variable and this includes severe acute life-threatening episodes, recurrent apnea, severe respiratory distress, feeding difficulties persistent cough, wheezing, or stridor. In some cases, affected individuals may be completely asymptomatic. A high index of suspicion is needed to suspect and diagnose vascular rings as they are uncommon but surgically treatable. We present a case of a 5-day-old term neonate referred to our facility with respiratory distress associated with stridor which started 12 h post-delivery.

Relationship between a Tracheal and Left Pulmonary Artery Stenosis Index and the Prognosis of Pulmonary Artery Sling with Tracheal Stenosis.

Pulmonary artery sling is a rare congenital pulmonary vascular malformation, often associated with tracheal or bronchial stenosis. Surgical treatment of pulmonary artery sling with tracheal stenosis (T) has a high risk of death and a relatively poor prognosis. This study explored the relationship between the T and left pulmonary artery stenosis (P) ratio and the effects of surgery for pulmonary artery sling with tracheal stenosis. Patients undergoing surgery for pulmonary artery sling in our center from January 2010 to December 2018 were retrospectively analyzed. Routine cardiac-enhanced computed tomography (CT) was performed preoperatively, and the P and T diameters were measured on the CT image. The T/P ratio was then calculated and analyzed. Thirty cases of pulmonary artery sling combined with tracheal stenosis were operated under cardiopulmonary bypass. The mean age at operation was 13.8 ± 13.6months (1.2-57.1months, Q1-Q3: 5-17months), and the mean body weight was 8.8 ± 3.5kg (3.8-17.3kg, Q1-Q3: 5.8-11.5kg). Twenty-three patients survived, and seven died, with an overall survival rate of 76.7%. Twenty-four patients underwent left pulmonary artery re-implantation, six patients underwent simultaneous left pulmonary artery re-implantation and slide tracheoplasty, and patients with intracardiac malformations (ventricular septal defect (n = 6) and atrial septal defect (n = 4)) underwent concurrent repair of the intracardiac defect. All cases had different degrees of tracheal stenosis, and the most narrowed trachea occurred with compression by the left pulmonary artery sling. The T/P ratio in the tracheoplasty group was significantly higher than that in the non-tracheoplasty group. The mortality rate in the T/P ≤ 1.15 group was significantly lower than that in the T/P > 1.15 group. Pulmonary artery sling treatment has a high risk of death. Left pulmonary artery re-implantation is an effective and safe surgical method for treating pulmonary artery sling, and slide tracheoplasty is an effective surgical method to correct tracheal stenosis. Children with T > 78.4% should receive active intervention for the tracheal stenosis, and these children have a considerable postoperative survival rate. The T/P ratio can be used to compare the relative superiority of the two compressions. A T/P ratio > 1.15 can be used as a reference index for intervention in tracheal stenosis and is a risk factor for postoperative death.

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

Surgical treatment and early-mid follow-up results of complete atrioventricular septal

Objective: To discuss the surgical treatment and early-mid follow-up results of complete atrioventricular septal defect (CAVSD). Methods: The clinic data of 235 cases of CAVSD underwent surgical treatment at Department of Cardiac Surgery, Guangdong General Hospital from June 2009 to June 2016 was analyzed retrospectively (104 male and 131 female patients). The patients were aged 18 days to 58 years (mid age 8.9 months) at surgery, and 2 cases of newborns (0.9%), 24 cases of 1 to 3 months (10.2%), 56 cases of 4 to 6 months (23.8%), 66 cases of 7 to 12 months (28.1%), 36 cases of 1 to 3 years (15.3%), 37 cases of 4 to 18 years (15.7%), and 14 cases above 18 years (6.0%). There were 129 cases of Rastelli type A (54.89%), 16 cases of type B (6.8%), 25 cases of type C (10.6%) and 65 cases of transitional type (27.7%). The patients were combined with 7 cases of unroofed coronary sinus syndrome, 5 cases of coarctation of aorta, 4 cases of tetralogy of Fallot, 3 cases of double outlet right ventricle, 1 case of transposition of the great arteries, 1 case of multiple ventricular septal defect, 1 case of pulmonary artery sling. The CAVSD correction operations were performed in 199 cases with modified single patch technique (84.7%), 26 cases with two patch technique (11.1%), 10 cases with conventional single patch technique (4.3%), and the corrective surgeries of other anomalies were performed in the meantime. Generalized Estimated Equation was used to statistical analysis of postoperative regurgitation level of mitral valve and tricuspid valve. Results: Followed-up for 1 to 7 years, 18 cases (7.7%) died overall and 15 cases (6.4%) died within 30 days post-operation.The mortality of little infant (operation age ≤3 months) was much highest(26.9%). The mortality of different operation age had significant difference(P=0.007). There was no difference among the mortality with three techniques. Seven cases (3.0%) suffered from the re-operation for severe mitral regurgitation. A total of 217 patients survived more than 1 year after initial surgery, and there were 11 cases (5.1%) with severe mitral regurgitation and 7 cases (3.2%) with severe tricuspid valve regurgitation at 1 year post-operation.The degree of mitral regurgitation after operation decreased at 1 week to 6 months, and slightly higher after 1 year postoperatively.The ratios of severe mitral regurgitation in different operative age group was statistically significant difference(P=0.017), mainly from 3 months group and 1 to 3 years group.The degree of tricuspid regurgitation decreased significantly after operation, and decreased gradually in the follow-up period. The ratio of postoperative residual severe tricuspid regurgitation was the highest in surgical aged >18 years old, less in 3 years group, the lowest in 3 to 18 years group. Conclusions: The mortality and the ratio of patients with serious mitral regurgitation were higher in infants less 3 months than elder patients. The infants need more frequently follow-up and medicine treatment after operation. The modified one patch technique is effective for all age groups.

A case of pulmonary artery sling where onset Was induced by therapeutic hypothermia

A neonate with severe neonatal asphyxia was treated with therapeutic hypothermia. He developed hypothermia-induced respiratory deterioration, after which congenital tracheal stenosis and pulmonary artery sling were diagnosed. Even low-grade hypothermia is likely to induce bronchial narrowing in neonates, especially in neonates with congenital respiratory tract anomalies. Congenital tracheal stenosis represents a potential pitfall in differential diagnosis and should be carefully ruled out in cases of bronchial narrowing episode induced by therapeutic hypothermia. Pediatr Pulmonol. 2017;52:E7-E10. © 2016 Wiley Periodicals, Inc.

Prenatal imaging diagnosis and prognosis assessment of vascular ring

Objective By comparing the results of prenatal diagnosis of vascular rings by ultrasound and MRI, chromosome screening and postnatal follow-ups, to explore the clinical significance of prenatal diagnostic technology for the diagnosis of vascular rings and prognostic assessment. Methods Ninety-five fetuses that were diagnosed by fetal echocardiography were followed up regularly, of which 72 cases had chromosome screening, 31 cases had MRI examination. These results were compared with the postnatal follow-up outcome. Results Ninety-five cases of vascular rings included 85 cases of right aortic arch(RAA) and left ductus arteriosus(LDA), 4 cases of pulmonary artery sling(LPAS), 4 cases of double aortic arch(DAA), 1 case of RAA and double ductus arteriosus(DDA), 1 case of left aortic arch(LAA) and aberrant right subclavian artery(ARSA). Sixty-six cases of isolated vascular rings and 29 cases of complex vascular rings(combined with other cardiovascular abnormalities) were detecded. Fifty-four cases were born(2 infants died), 8 cases were ongoing pregnancy, 6 cases were lost to follow-up, 27 cases were aborted. Prenatal ultrasound and MRI examination indicated 6 cases of trachea compression. However, only 3 infants showed clinical symptoms due to trachea compression from born 5 cases. Conclusions Isolated vascular ring is the most common format and its diagnosis and type can be determined by prenatal ultrasound. Dynamic follow-up using ultrasound in pregnancy combined with MRI is helpful to assess the compression degree of trachea induced by vascular ring and prognosis. Key words: Ultrasonography, prenatal; Magnetic resonance imaging; Fetal heart; Vascular ring

Assessment of congenital vascular rings with MDCT on children

To evaluate the value of MDCT on diagnosis of congenital vascular rings on children. Retrospective analysis on 43 cases of congenital vascular rings, which underwent MDCT during Oct 2008 to Dec 2014 in Beijing Anzhen hospital affiliated to capital medical university. 21 males, 22 females; age from 29 days to 8 years, mean age 1.46 years, 33 cases are not beyond 1 year. All the results were compared with that of the echocardiogram or record of the surgery. The CT data were read and reconstructed with multiplanar reconstruction (MPR), maximum intensity projection (MPR), minimum intensity projection (MinIP), volume rendering (VR). The image quality was evaluated and the diagnostic value and the standard diagnostic program were discussed. Of 43 cases of vascular rings:there were 6 cases of pulmonary artery sling (13.95%), 9 cases of right aortic arch /aberrant left subclavian artery(20.93%), 18 cases of left aortic arch/aberrant right subclavian artery (41.86%), 10 cases of double aortic arch (23.26%). Forty cases (93.02%) were combined with other cardiovascular or pulmonary malformations. Every malformation was revealed clearly and proved by echocardiogram. Of 3 cases (6.98%) without any other malformation, 2 cases were combined tracheal stenosis. A pulmonary artery sling was proved by surgery; the other 2 cases were double aortic arch. All the images of 43 cases could be reconstructed well. MPR and VR showed the origin, shape, and whole course of vascular rings directly; MinIP and VR could display the shape, width and development of trachea, revealed the relationship between vascular rings, trachea and esophagus. It was important to show and measure the component vascular of the ring. Attention should be paid to the whole course of trachea and esophagus, especially those segments which were close to the ring vascular. The tracheal stenosis as well as intra-cardio anatomy malformations should be measured on MPR images if existed. According to the segmental analysis method, comes the overall final diagnosis. A standard diagnostic program on vascular ring was proposed. MDCT axis images with various 3D post processing methods could reveal the compose of vascular rings and the relationship between vascular rings, trachea and esophagus.

Value of combination of three-vessel views in ultrasound diagnosis of fetal aortic arch and pulmonary arterial branch anomalies

Objective To evaluate the role of combination of three-vessel views in ultrasound diagnosis of fetal aortic arch and pulmonary arterial branch anomalies. Methods The data of 66 cases of fetal aortic arch and pulmonary arterial branch anomalies were retrospectively analysed, including echocardiographic data, autopsy and operation records and postnatal follow-up results. Echocardiogaphic features and display frequencies on three vessels and trachea view(3VT), three vessels view(3VV) and three vessels and pulmonary arterial branches view(3VP) were summarized. Results There were 52 cases of aortic arch abnormalities, including 32 cases of right aortic arch with left ductus arteriosus, 4 cases of double aortic arch, 7 cases of aberrant right subclavian artery, 6 cases of coarctation of aorta and 3 cases of interruption of aortic arch. Fourteen cases were diagnosed pulmonary aterial branch abnormalities, including 10 cases of crossed pulmonary arteries, 2 cases of anomalous origin of left pulmonary artery from aortic arch and 2 cases of pulmonary artery sling. The display frequencies of fetal aortic arch and pulmonary arterial branch anomalies on 3VT, 3VV and 3VP were 80.3%, 19.7% and 39.4%. Conclusions The combination of three-vessel views is of great value in prenatal diagnosis of fetal aortic arch and pulmonary arterial branch anomalies. Key words: Ultrasonography, prenatal; Heart defects, congenital; Three-vessel view; Aortic arch; Pulmonary arterial branch

Two Adult Cases of Pulmonary Artery Sling

Two Adult Cases of Pulmonary Artery Sling

The diagnostic value of MSCT multi-dimensional reconstructions for congenital vascular ring with tracheal stenosis

To evaluate the diagnostic value of multi-slice CT (MSCT) reconstructions for congenital vascular rings together with tracheal stenosis. 9 cases of children with congenital vascular ring and tracheal stenosis confirmed by surgery were collected in the study, all cases had undergone thin slice CT contrast enhancement, the MSCT data were transmitted to the workstation for multiplanar reconstruction (MPR), volume rendering technique (VRT) and VR transparency reconstruction. With the surgical results as the gold standard, the imaging characteristics of echocardiography (UCG) and MSCT were comparatively analyzed. In 9 cases, there were 4 cases of pulmonary artery sling, 3 cases of right aortic arch combination with left aberrant subclavian artery, 1 case of double aortic arch, 1 case of innominate artery compression syndrome. In this group, 5 cases were accompanied with other cardiac malformations (tetralogy of Fallot in 2 cases, double outlet right ventricle with patent ductus arteriosus and ventricular septal defect in 1 case, ventricular septal defect in 1 case, double superior vena cava in 1 case), 1 case of tetralogy of Fallot demonstrated many tortuous collateral arteries around aorta. All malformations were well displayed by VRT, MPR. VR transparency reconstruction can stereoscopically display trachea and bronchial compression condition, the main trachea was compressed in 6 cases, the main trachea and left main bronchus was compressed in 2 cases, the main trachea and left main bronchus was compressed in 1 cases, UCG detected all intracardiac malformations, 1 case of pulmonary artery sling was misdiagnosed as patent ductus arteriosus, 8 cases of vascular rings, tracheal and bronchial stenosis were missed. MSCT reconstruction technology is a noninvasive, rapid diagnostic method, it can clearly show the congenital vascular rings abnormalities and the degree of tracheal stenosis, it has important significance for clinic treatment.

Clinical analysis on 3 cases of pulmonary artery sling

目的 总结3例肺动脉吊带畸形患儿的临床表现和诊治经验,减少其误诊率.方法 回顾分析3例肺动脉吊带畸形病例诊治的临床资料及实验室检查资料的特点.结果 (1)临床表现:3例患儿的初次发病年龄<3个月,主要临床表现均为反复咳嗽、喘息,均因呼吸困难加剧急诊入院,均有多次门诊及住院治疗史,确诊前均被诊断为重症肺炎、先天性喉喘鸣,例2还诊断为急性喉炎,例3合并先天性心脏病(房间隔缺损).(2)CT检查:3例患儿均表现为左肺动脉起自右肺动脉,绕行于气管后方进入肺门,气管都有明显狭窄.心脏超声及纤维支气管镜对本病的诊断有提示作用.(3)治疗:3例均行手术治疗,2例于术后7～10 d成功脱离呼吸机,控制肺部感染后出院,1例患儿因气管内肉芽组织形成,不能脱离呼吸机而放弃治疗.结论 肺动脉吊带畸形婴幼儿可表现为反复咳喘,因呼吸困难急诊入PICU.肺动脉吊带畸形的诊断主要依靠CT、超声心动图及纤维支气管镜检查.早期诊断、最佳时机手术和围手术期监护是成功治疗的关键。

Alça da artéria pulmonar: relato de dois casos e revisão da literatura

Neste estudo são relatados dois casos de artéria pulmonar esquerda anômala, diagnosticados através da reconstrução tridimensional das imagens da árvore traqueobrônquica e esôfago obtidas por exame de imagem por ressonância magnética ou tomografia computadorizada helicoidal. Os achados clínicos típicos desta anormalidade congênita, que pode não ser tão infreqüente quanto se pensava, incluem aqueles relacionados à obstrução traqueobrônquica grave, embora casos assintomáticos ou pouco sintomáticos tenham sido descritos. A importância do diagnóstico precoce desta anomalia é extrema, já que existe a necessidade, na maioria dos casos, de uma correção cirúrgica imediata devido à sua alta letalidade. A técnica da reconstrução tridimensional permite a identificação simultânea das malformações cardiovasculares e traqueobrônquicas associadas, e demonstra com clareza a anomalia vascular. Assim, o diagnóstico pode ser feito usualmente de maneira não invasiva, evitando o uso da angiocardiografia seletiva, considerada padrão-ouro.

Pulmonary Artery Sling Associated with Tetralogy of Fallot

We describe a rare case of pulmonary artery sling occurring simultaneously with tetralogy of Fallot. This report describes the successful concomitant repair of both intracardiac anomalies.

A Case of Pulmonary Artery Sling Associated With Long-Segment Funnel Trachea and Bronchus Suis

An infant with pulmonary sling syndrome associated with an abnormal branching of the trachea, bronchus suis, complicated by long segment intrathoracic tracheal stenosis (ie, “funnel trachea”) and concomitant tracheomalacia underwent reoperation at 5 months of age. We review the anatomy of this rare entity and describe the surgical technique to avoid the residual stenosis.

術中，気管支狭窄が増悪したＰｕｌｍｏｎａｒｙ Ａｒｔｅｒｙ Ｓｌｉｎｇの１例

術中，気管支狭窄が増悪したＰｕｌｍｏｎａｒｙ Ａｒｔｅｒｙ Ｓｌｉｎｇの１例

Air Way Instability due to Pulmonary Artery Sling combined with Diffuse Tracheal Stenosis

Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery arises anomalously fmm the right pulmonary artery extrapericardially and encirdes the right main stem bronchus and passes between the trachea and esophaghus to reach the hilum of the left lung. Respiratory obstruction vesults from compression of the lower area of trachea and right main stem bronchus. It is rare cause of respiratory distress in the new bom and may be associated with significant morbidity and mortality. We experienced the case of pulmonary artery sling with diffuse trachea stenosis and congenital heart disease (pulmonary atresia and PDA) and confirmed the anomaly by bronchogram and MRI. The presumed cause of death was respiratory obsruction due to pulmonary artery sling.

Pulmonary Artery Sling

A case of pulmonary artery sling in a 1-year-old boy, treated by resection of the left pulmonary artery (LPA) at its origin from the right pulmonary artery (RPA) and reanastomosis of the LPA to the main pulmonary artery (MPA), is reported. In this patient the LPA-MPA anastomosis was demonstrated to be patent in a follow-up pulmonary angiogram six months after the operation. Pulmonary artery sling should be kept in mind when assessing respiratory distress syndrome in children.