Tracheal surgery in children: an 18-year review of four techniques

Objective: Review the short- and long-term outcomes of a single institution experience in infants with congenital tracheal stenosis, comparing four different operative techniques used from 1982 through 2000. Methods: Hospital and clinic records of 50 infants and children who had surgical repair of congenital tracheal stenosis secondary to complete tracheal rings were reviewed. Age at surgery ranged from 7 days to 72 months (median, 5 months, mean 7.8±12 months). Techniques included pericardial patch tracheoplasty (n=28), tracheal autograft (n=12), tracheal resection (n=8), and slide tracheoplasty (n=2). All procedures were done through a median sternotomy with cardiopulmonary bypass. Seventeen patients had a pulmonary artery sling (35%), and 11 had an intracardiac anomaly (22%). Results: There were three early deaths (6% early mortality), two after pericardial tracheoplasty and one after autograft. There were six late deaths (12% late mortality), five after pericardial tracheoplasty and one after slide tracheoplasty. Length of stay (median) was 60 days (pericardial tracheoplasty), 28 days (autograft), 14 days (resection), and 18 days (slide). Reoperation and/or stent placement was required in seven patients (25%) after pericardial tracheoplasty, in two patients (17%) after autograft, in no patients after resection, and in one patient (50%) after slide tracheoplasty. Conclusions: Our current procedures of choice for infants with congenital tracheal stenosis are resection with end-to-end anastomosis for short-segment stenoses (up to eight rings) and the autograft technique for long-segment stenoses. Associated pulmonary artery sling and intracardiac anomalies should be repaired simultaneously.     

采用双冠状动脉导丝法建立先天性心脏病介入治疗复杂路径轨道

目的探讨以双冠状动脉导丝法建立先天性心脏病(简称先心病)介入治疗复杂路径轨道的价值。方法回顾性分析12例应用双冠状动脉导丝法建立介入治疗轨道的先心病患儿的临床资料,其中肺动脉闭锁伴室间隔缺损(PA/VSD)矫治术后肺动脉狭窄5例,法洛四联症(TOF)矫治术后肺动脉狭窄1例,大动脉转位(TGA)术后肺动脉狭窄1例,粗大体肺侧支(MAPCAs)3例,复杂型肺动静脉瘘1例及左冠状动脉回旋支-右心房瘘1例;分析先心病介入治疗复杂路径特点。结果采用双冠状动脉导丝均成功建立输送轨道。对PA/VSD矫治术后肺动脉狭窄、TOF术后肺动脉狭窄、TGA术后肺动脉狭窄患儿成功进行球囊扩张,对MAPCAs、复杂型肺动静脉瘘及左冠状动脉回旋支-右心房瘘患儿均封堵成功,未出现瓣膜损伤、血管损伤、心包填塞及死亡等严重并发症。结论以双冠状动脉导丝法建立先心病介入治疗复杂路径轨道方法安全、有效,可调控性强,能提供足够支撑力通过纡曲段,有利于提高介入治疗成功率,值得临床推广应用。     

Results of the Ross operation in a pediatric population

Objective: To analyse the results of the mid-term clinical and echocardiographic follow-up of the pediatric Ross operation. Methods: Echo-Doppler follow-up of 53 consecutive pediatric Ross procedures performed between 1994 and 2003. Median age was 9.7 years at time of operation (2 weeks–17.7 years). Six patients were younger than 3 months. Median age at follow-up was 15.6 years. Aortic valve/left ventricular outflow tract (LVOT) anomalies were congenital in 49 (92%). Seventy percent had previous surgery or balloon valvuloplasty. Root replacement was used in all. Thirteen patients (25%) had LVOT enlargement. Mean cross-clamp time was 113 (69–189) minutes. Results: Early mortality occurred in 3 patients after emergency surgery following balloon failure (n=1) and extended Ross following interrupted arch/VSD repair (n=2). Late mortality was due to LV fibroelastosis in 2 patients and complicated pulmonary artery stenting in another. RVOT reoperations were required because of late homograft obstruction in 2 patients and because of pulmonary artery stenosis in another. Five patients (9.4%) were reoperated for pulmonary autograft dilatation (n=3) and for leaflet fibrosis or perforation (n=2). Autografts were repaired in two patients, while a mechanical valve was inserted in 3 cases. At 9 years the actuarial survival and event free survival were 89 and 74%, respectively. At last follow-up 90% of autograft diameters indexed to body surface area was above the 90th percentile of normal aortic root diameters. LVOT and RVOT gradients were low and autograft insufficiency was trivial to mild in 84% and mild to moderate in 16%. Autograft stenosis was not noticed. Conclusions: The pediatric Ross procedure remains an important tool but autograft dilatation also occurs in the pediatric population. The significance of this finding has yet to be determined.     

Primary repair of aortic arch obstruction with ventricular septal defect in preterm and low birth weight infants

Objective: Previous reports have suggested that prematurity and low birth weight are risk factors for definitive surgical intervention in congenital cardiac malformations. The following data review our experience with primary repair of the complex malformation of aortic arch obstruction with ventricular septal defect (VSD) in this patient population. Methods: Since 1988, 21 consecutive preterm (≤36 weeks) and/or low birth weight (<3000 g) infants with interrupted aortic arch (IAA; n=10), or aortic coarctation (n=11) with VSD, underwent primary arch repair and VSD closure. The mean weight at operation was 2310 g (range, 1200–2900 g), including 12 patients at ≤2500 g. The gestational age ranged from 30 to 41 weeks (mean, 36.4 weeks). Five patients with interrupted arch and two patients with coarctation also had severe subaortic stenosis, which was relieved by transatrial incision of the infundibular septum. Results: The overall hospital mortality was 14% (3/21). Death was related to low cardiac output in association with severe subaortic stenosis (n=2) and sepsis (n=1). Late mortality occurred in three patients, two of which were non-cardiac. The mean follow-up was 33 months. Two patients had significant recurrent arch obstruction, which was successfully relieved by balloon angioplasty and surgical correction in one each. The survival at 30 days, and at 1 and 3 years was 86, 76 and 70%, respectively. Conclusions: Complete primary repair of aortic arch obstruction with VSD can be achieved with good results, even in the preterm and low birth weight infant. Therefore, early surgical repair of this congenital malformation is recommended.     

Double-outlet right ventricle with non-committed ventricular septal defect

Objective: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. Methods: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n=21) and arterial switch operation with VSD to pulmonary artery baffle (n=2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. Results: There were two hospital deaths (9%, 70% CL: 3–19%). Eight patients (35%, 70% CL: 23–48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. Conclusions: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.     

Surgical relief of tracheobronchial obstruction in infants and children

Objective: Congenital tracheobronchial obstruction (TBO) presents a complex problem both in terms of diverse aetiology, presence of associated anomalies and the operative strategy to be adopted. We report a single centre experience in managing this difficult problem. Methods: Twenty-four infants and children with TBO referred to our unit over a 12-year period are reviewed. Aetiology of TBO included vascular rings (n=9), anomalous innominate artery (n=6), congenital tracheal stenosis (n=5), segmental bronchial stenosis (n=2) and pulmonary artery compression of the main bronchi (n=2). Seven patients had concurrent cardiac anomalies. Stridor was the commonest presenting symptom (67%). Mean delay from onset of symptoms to referral was 19 months. One patient died preoperatively due to acute airway obstruction. Mean age at operation was 33.1±42 months (range 4 days–156 months) and 11 children were under 1 year at the time of surgery. In cases of TBO secondary to vascular rings, division of the ring resulted in relief of symptoms in seven cases, with two requiring further surgery for resultant tracheomalacia. Four of the five patients having tracheal resection were operated on with the use of cardiopulmonary bypass; three of these patients had concurrent correction of cardiac lesions, with two survivors. Tracheobronchial anastomoses were carried out using continuous polydioxanone (PDS). Patients with anomalous innominate arteries required aortopexy in five and innominate artery suspension in one, while those with pulmonary artery compression of the main bronchi had correction of their intracardiac defects (n=2). Results: Hospital mortality was 8.7% and there has been one late death due to Eisenmenger syndrome secondary to pulmonary regurgitation, atrial septal defect (ASD) and patent ductus arteriosus (PDA). On follow-up (mean 40±31 months), 19 patients are alive and symptom free. There have been no anastomotic strictures following tracheobronchial resection. The single most important predictor of mortality was the presence of associated cardiac anomalies. Conclusions: TBO can be managed effectively by a single operation in both infants and children without a detrimental effect on tracheal growth. We advocate consideration of concurrent repair of the tracheal and cardiac lesions. Cardiopulmonary bypass (CPB) allows this concurrent correction of cardiac lesions and also facilitates tracheal resection.     

Anesthesia for high‐risk procedures in the catheterization laboratory

Recent advances in catheterization and imaging technology allow for more complex procedures to be performed in the catheterization laboratory. A number of lesions are now amenable to a percutaneous procedure, eliminating or at least postponing the need for a surgical intervention. Due to the increase in the complexity of the procedures performed, the involvement of anesthesiologists and their close collaboration with the interventional cardiologists have increased. It is important to understand the physiology and pathophysiology of the patients and to anticipate the plans and the potential complications in order to manage them. We are witnessing a rise in the number of complex interventions in newborns and infants, such as balloon valvotomy (critical aortic stenosis, pulmonary stenosis), radio frequency perforation (of pulmonary atresia and intact ventricular septum), right ventricular outflow tract stenting (in Tetralogy of Fallot), ductal stenting (in some ductus‐dependent pulmonary circulation), and combined with a surgical procedure (hybrid procedure for hypoplastic left heart syndrome). Multiple registries have been created in order to understand and improve outcomes of patients with congenital heart disease undergoing catheterization procedures and to develop performance and quality metrics, from which data regarding anesthetic‐related risks can be extrapolated. Experienced personnel and a multidisciplinary team approach with direct communication among the team members is a must to ensure anticipation and management of critical events when they occur.     

心脏外科手术与介入性心导管术镶嵌治疗小儿先天性心脏病

目的 报道23例介入性心导管术与心脏外科手术镶嵌治疗小儿先天性心脏病(CHD)的经验。方法外科术后镶嵌治疗：12例动脉导管未闭结扎术后残余分流及2例心脏术后留置室间隔缺损(VSD)的复杂CHD行经导管封堵术。2例法洛四联症(TOF)根治术后分支肺动脉狭窄分别行球囊血管成形术及支架置入术，1例肺动脉闭锁合并VSD根治术后出现体肺侧支血管(APCAs)破裂行介入栓塞止血术。外科术前介入治疗：5例室间隔完整的大血管转位(TCA)病例行大动脉调转术前行球囊房隔造口术(BAS)，1例有巨大APCAs的重症。TOF在根治术前予以侧支血管堵塞术。结果动脉导管未闭术后残余分流及复杂CHD残留VSD病例均封堵成功，分支肺动脉狭窄病例介入治疗后狭窄减轻或治愈；侧支血管破裂病例栓塞止血后出血停止。BAS病例介入术后低氧及酸中毒改善，大动脉调转术后3例治愈，2例死亡。TOF病例APCAs堵塞后成功行根治术。结论心脏外科手术与介入性心导管术镶嵌治疗是治疗小儿CHD术后残余分流及部分复杂疑难CHD的有效方法。     

Mechanical Aortic Valve Replacement in Children and Adolescents After Previous Repair of Congenital Heart Disease

Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown‐ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart defects. Between July 2002 and November 2008, 15 (10 male and 5 female) consecutive patients (mean age 14.5 ± 10.5 years) underwent mechanical AVR. Hemodynamic indications for AVR were aortic stenosis in four (27%), aortic insufficiency in eight (53%), and mixed disease in three (20%) after previous repair of congenital heart defects. All patients had undergone one or more previous cardiovascular operations due to any congenital heart disease. Concomitant cardiac procedures were performed in all of them. In addition to AVR, in two patients, a mitral valve exchange was performed. One patient received a right ventricle‐pulmonary artery conduit replacement as concomitant procedure. The mean size of implanted valves was 23 mm (range 17–29 mm). There were neither early deaths nor late mortality until December 2008. Reoperations were necessary in five (33%) and included implantation of a permanent pacemaker due to complete atrioventricular block in two (15%), mitral valve replacement with a mechanical prosthesis due to moderate to severe mitral regurgitation in one (7%), aortocoronary bypass grafting due to stenosis of a coronary artery in one (7%), and in one (7%), a redo subaortic stenosis resection was performed because of a secondary subaortic stenosis. At the latest clinical evaluation, all patients were in good clinical condition without a pathological increased gradient across the aortic valve prosthesis or paravalvular leakage in echocardiography. Mechanical AVR has excellent results in patients after previous repair of congenital heart defects in childhood, even in combination with complex concomitant procedures. Previous operations do not significantly affect postoperative outcome.     

Concomitant repair of congenital tracheal stenosis and complex cardiac anomaly in small children

Severe obstructive lesion of the trachea combined with complex congenital cardiac anomaly has generally been regarded as a fatal disease. Herein we report the successful concomitant repair of severe tracheal stenosis and complex cardiac anomaly with the use of cardiopulmonary bypass in two cases. The first patient was a 5-year-old boy with tetralogy of Fallot associated with a localized intrathoracic tracheal stenosis caused by complete cartilaginous rings. Tracheal resection and end-to-end anastomosis combined with total correction of tetralogy of Fallot was performed with the aid of cardiopulmonary bypass. The second patient was a 5-month-old girl with a pulmonary artery sling, scimitar syndrome, and extensive tracheal stenosis. The patient underwent definitive correction of cardiac lesions and complete tracheal reconstruction with a cartilaginous graft with the aid of cardiopulmonary bypass. Utmost care was paid to avoid contamination during the operation. Both of the patients are doing well at present without any signs of complication, 2 years 5 months and 1 year 10 months after the operation, respectively. We advocate concomitant repair of both lesions, with cardiopulmonary bypass, in the surgical managements of infants and small children who have a difficult and otherwise fatal combination of complex congenital cardiac anomaly and severe intrathoracic tracheal stenosis.     

Cardiac malformations associated with the congenital nephrotic syndrome

The association of cardiac malformation with the congenital nephrotic syndrome (CNS) has been previously reported in only one family. We report four patients with CNS: three with pulmonary valve stenosis (one requiring valvuloplasty) and one with discrete sub-aortic stenosis requiring surgical resection. We conclude that the cardiac status of all patients with CNS should be reviewed regularly by a paediatrician, with a low threshold for referral to a cardiologist, as flow murmurs due to chronic anaemia may obscure cardiac pathology. It is important to diagnose any associated cardiac lesions as these may require intervention, and may also predispose to the development of bacterial endocarditis if surgical or dental procedures are undertaken without appropriate antibiotic prophylaxis. Received: 3 May 1999 / Revised: 8 March 2000 / Accepted: 9 March 2000     

Long-term clinical results of percutaneous transluminal angioplasty in transplant renal artery stenosis.

Twenty-five patients with transplant artery stenosis were identified among 1141 renal graft recipients. Impaired graft function (9 patients), hypertension (4 patients) or both (12 patients) were the indications for arteriography. All were treated by percutaneous angioplasty (PTA). The immediate technical success rate was 88% and actuarial graft survival was 88% and 80% at 2 and 5 years respectively. The long-term success rate on graft function was 67% (median observation time 24 months) and on hypertension 63% (median observation time 23 months). Six patients needed rePTA (8 procedures) and in only one patient was surgical repair performed. No case of graft loss due to PTA was recorded and in only one case did occlusion of a segmental artery lead to impairment of graft function. Minor complications were recorded in four other cases and in no case was surgical intervention necessary. Based on these results we favour PTA as a first-line interventional procedure in transplant renal artery stenosis, and the need for surgical repair has been low.     

Koronararterielle Stentimplantation vor nichtkardiochirurgischen Eingriffen Wiegen wir uns fälschlich in Sicherheit?

Patients undergoing non-cardiac surgical procedures who carry coronary artery stents have to be classified as high risk patients. Perioperative myocardial infarction and severe bleeding are possible. Therefore, anaesthetic management directed by invasive monitoring, ECG ST analysis, transesophageal echocardiography and referral to an intensive care unit are absolutely justified. The urgency of the surgical procedure, perioperative risk and an antiplatelet regimen have to be discussed with the patient and the surgeon in advance. In the case of cardiac complications, rapid therapy by an interventional cardiologist must be available.     

Congenital heart disease in infancy--surgery for specific defects

The evolution of surgery for congenital cardiac malformations in infancy in the past decade warrants an analysis of the indications, technical aspects, advantages, complications and results of the treatment of specific defects. Considerable variations in approach are found, and every surgeon should attempt to evaluate the most appropriate current management in each particular case. Primary closure is usually preferred to pulmonary artery banding in patients with large ventricular septal defects. But will the same approach produce a high late survival rate in patients with other left-to-right shunts, e.g. atrioventricular canal and truncus arteriosus? Primary repair of tetralogy of Fallot, for example, especially in patients who might require a pulmonary outflow tract patch, has been challenged by many. In which patients, if any, is an initial systemic-pulmonary anastomosis the preferable treatment? Which is the best surgical approach for other cyanotic congenital cardiac malformations such as transposition of the great arteries and pulmonary and tricuspid atresia? The multitude of procedures available for 'complete correction' of some malformations is an indication of their limitations, while other defects, such as some forms of univentricular heart and hypoplastic left heart, are not yet fully correctable.     

Primary repair of pulmonary artery sling with double outlet right ventricle and distal tracheal stenosis

The fate of patients with pulmonary artery sling depends on associated tracheal lesions. Distal tracheal stenosis involving the carina frequently results in lethal obstruction due to secretion or inflammatory edema. Further-more, associated complex cardiac anomalies with excessive pulmonary flow make the situation more complicated. We present a case of successful simultaneous one-stage repair of pulmonary artery sling, double outlet right ventricle and distal tracheal obstruction. Pulmonary artery sling was relieved by relocation of the left pulmonary artery (left pulmonary artery to main pulmonary artery) and a tracheoplasty (resection and end-to-end anastomosis). We advocate early aggressive simultaneous repair of pulmonary artery sling with tracheal stenosis and concomitant repair of intracardiac anomalies whenever possible.     

Intraoperative stents to rehabilitate severely stenotic pulmonary vessels

BACKGROUND: Patch enlargement of severe branch pulmonary artery stenosis (PAS) or pulmonary vein ostial stenosis (PVS) can be technically challenging. Recurrences are common and exposure may require long periods of cardiopulmonary bypass (CPB). METHODS: Since 1993, we performed 31 procedures on 27 patients with endovascular stents placed intraoperatively under direct surgical vision: 22 patients with tight PAS and 5 patients with PVS. Selection for intraoperative (vs catheterization laboratory) stent placement was prompted by: (1) the need for a concomitant cardiac surgical procedure (16 cases); (2) limited vascular access for catheterization laboratory stent placement (11 cases); or (3) "rescue" of patients with complications after attempted placement of stents (4 cases). RESULTS: In this group of very complex and challenging patients there were 5 hospital deaths (hospital survival, 81%). Follow-up of survivors has ranged from 1 month to 7 years (mean 2.8 +/- 1.7 years). There have been 3 late deaths (late "series" survival, 70%). No complication or death was related to stent placement. Surviving patients have had significant clinical improvement. Mean pulmonary gradient (postoperative vs preoperative echo) has fallen in all survivors and has decreased from a mean of 66 mm Hg preoperatively to 28 mm Hg postoperatively (p = 0.01). All pulmonary arteries are appreciably enlarged and will be easier to deal with at a later date if necessary. One patient (DORV, HLHS ) with pulmonary vein stents has gone on to a successful Glenn procedure. The other two surviving patients with PV stents have occlusion of their proximal PVs on follow-up catheterization; thus only 1 of 5 patients with stents for PVS has had a successful outcome. Four patients have had repeat surgery. Stents have produced no impediment to subsequent surgical procedures, and the pulmonary arteries were easy to work with. CONCLUSIONS: Intraoperative stenting provides an attractive option for "rehabilitation" of pulmonary vessels. Direct vision insertion on CPB is extremely quick and immediately effective, limiting the CPB exposure required to treat this problem. Once stented, vessels remain open and are amenable to future surgical intervention as necessary. Outcome is better for patients with PAS versus those with PVS.     

Treatment strategy for patients with concomitant thoracic or abdominal aortic aneurysm and noncardiovascular disease

Therapeutic planning for patients with concomitant thoracic aortic aneurysm (TAA) or abdominal aortic aneurysm (AAA) and noncardiovascular disease such as cerebral aneurysm, carotid artery stenosis, or lung, intraabdominal or urologic tumor should be considered based on the combination of the two different conditions, the size of aneurysm, or the severity of noncardiovascular disease. The aims of this paper are to review the therapeutic plans for concomitant TAA or AAA and noncardiovascular disease. In patients with concomitant TAA or AAA and cerebral aneurysm, carotid artery stenosis, and concomitant TAA and intraabdominal or urologic tumor, the surgical procedures have usually been staged with the repair of cerebral aneurysm, or carotid artery stenosis, the resection of intraabdominal or urologic tumor performed first, followed by the repair of TAA or AAA. Simultaneous surgical treatment has been performed for most patients with concomitant TAA and lung tumor, and concomitant AAA and intraabdominal or urologic tumor. The issue of performing simultaneous pulmonary resection and repair of AAA in patients with concomitant lung tumor and AAA remains controversial. Endovascular grafting of TAA and AAA can be performed with relatively low procedure-related morbidity and mortality rates in selected patients.     

Hybridoperationssaal aus Sicht der Herzchirurgie

Nowadays, increasing numbers of procedures jointly conducted by cardiac surgeons and cardiologists are performed as minimally invasive surgical procedures or interventions. Transcatheter aortic valve implantation, endovascular aortic aneurysm repair and a large variety of hybrid procedures for congenital heart disease have become current standards. Some of these hybrid procedures were shown to improve the therapeutic safety and efficacy, effects particularly true for high-risk patients and complex interventions. Hybrid procedures require indirect imaging, commonly provided by an angiography system in the hybrid operation theatre. This article describes the technical prerequisites required for a hybrid operation theatre as well as indications and rationales for hybrid procedures conducted in this environment. It is likely that the indications for cardiovascular hybrid procedures will continue to be expanded and that the hybrid operation theatre may become a laboratory for developing innovative approaches in the cardiovascular field. Therefore, the hybrid operation theatre will not only be the working environment for hybrid surgeons and interventionalists but also help to evolve their future.     

Successful early surgical recruitment of the congenitally disconnected pulmonary artery

BACKGROUND: Our purpose was to document our experience with early recruitment of congenitally disconnected pulmonary arteries and to assess subsequent pulmonary artery growth and function. METHODS: Patients born in the 10-year period from 1989 to 1999 with a disconnected pulmonary artery diagnosed in infancy and treated in our unit were studied. To be included patients had nonconfluent pulmonary arteries with one or both completely disconnected from the main pulmonary artery. This series did not include patients with acquired stenosis causing occlusion of a pulmonary artery. Echocardiography, cardiac catheterization, MRI, lung perfusion scans, and intraoperative assessment were used to gauge pulmonary artery growth and function. RESULTS: Seven patients with a disconnected pulmonary artery associated with intracardiac conotruncal congenital cardiac disease underwent successful early surgical recruitment of the affected pulmonary artery at 3 months of age or younger. Median follow-up from date of first operation was 4.2 years (range, 1.6 to 13.4). All 7 patients had postrecruitment lung perfusion scans showing a mean of 44% (range, 27% to 78%) of total pulmonary flow through the affected lung. Significant growth in the diameter of the recruited native pulmonary artery was demonstrated in all patients. There were no deaths reported in our series to date. CONCLUSIONS: The rare possibility of a congenitally disconnected pulmonary artery needs to be considered in all patients with a conotruncal cardiac anomaly. To facilitate surgical correction, ensure subsequent growth of the pulmonary artery, and optimize associated lung development, early diagnosis and surgical recruitment is recommended.