Congenital Left Main Coronary Artery to Main Pulmonary Artery Fistula with Bicuspid Aortic Valve: A Case Report and Review of Literature

Abstract Congenital coronary artery fistulas (CAFs) are uncommon abnormalities. A connection between the left main stem and main pulmonary artery is extremely rare. Congenital CAFs are frequently associated with another congenital heart disease. Associated anomalies include atrial septal defect, tetralogy of Fallot, patent ductus arteriosus, ventricular septal defect, and pulmonary atresia. Association of bicuspid aortic valve with CAF has not been reported in literature to date. We report on a 68‐year‐old man with congenital left main to pulmonary artery fistula associated with bicuspid aortic valve and moderate aortic stenoses, who underwent successful aortic valve replacement with ligation of CAF and also review the natural history, pathophysiology, and management of CAF. (J Card Surg 2010;25:295‐299)     

Transposition of the great arteries with aortic arch obstruction. Anatomical review and report of surgical management

Transposition of the great arteries (S,D,D) is a common congenital cardiovascular malformation that is occasionally associated with ventricular septal defect and left ventricular outflow tract obstruction. Recently right ventricular outflow tract obstruction associated with an aortic arch anomaly has been recognized as an infrequent but important variant of transposition of the great arteries, and this constellation presents a unique surgical challenge. Five infants with this constellation whose systemic circulation was dependent on flow through the ductus arteriosus have undergone definitive surgical treatment with four survivors. An anatomical review of 129 specimens with transposition of the great arteries revealed that 17% had right ventricular outflow tract obstruction and 7% had associated aortic arch obstruction as well. All specimens with aortic arch obstruction and ventricular septal defect except one had a malalignment type ventricular septal defect. Although several treatment options may be considered, recent experience with arterial switch repair in the neonate with transposition of the great arteries (S,D,D) prompted repair in these patients by arterial switch, ventricular septal defect closure, repair of aortic arch obstruction, and augmentation of the right ventricular outflow tract.     

Truncus arteriosus with coarctation of persistent fifth aortic arch

Truncus arteriosus is a rare form of congenital heart defect. Among cases, 10% to 20% are associated with arch anomalies, such as interruption, coarctation, or patent ductus arteriosus. We treated a rare case of combined truncus with complex arch anomaly. The patient was a 2-month-old girl weighing 3.8 kg. Echocardiogram and computed tomogram showed interrupted aortic arch, truncus arteriosus, persistent fifth aortic arch, and its coarctation. One-stage total correction was performed under cardiopulmonary bypass without circulatory arrest. The operation consisted of a Rastelli procedure with a 12-mm valved conduit, coarctoplasty, and intraventricular baffling. The patient recovered well without complications.     

先天性心脏病合并右位主动脉弓的诊断与治疗

目的 探讨先天性心脏病合并右位主动脉弓的诊断与治疗。 方法 回顾性分析2012年1月1日至2013年4月1日北京儿童医院小儿心脏中心27例先天性心脏病合并右位主动脉弓行外科手术治疗患儿的临床资料,其中男20例,女7例;年龄（10.96±12.08） 个月,体重（7.70±3.13） kg。法洛四联症14例,室间隔缺损9例,动脉导管未闭1例;肺动脉吊带1例;单纯双主动脉弓2例;均合并右位主动脉弓。其中13例合并血管环畸形,包括迷走左锁骨下动脉畸形和双主动脉弓畸形。所有患儿均接受手术治疗,在矫治心内畸形的同时,进行左锁骨下动脉移植,矫治迷走左锁骨下动脉畸形,双主动脉弓主要是切断一侧非优势弓。 结果 围术期死亡3例,其中1例术后无法停体外循环死亡,1例未合并血管环的患儿术后7 d死亡,1例合并双主动脉弓患儿术后并发急性呼吸窘迫综合征（ARDS） 死亡;1例放弃治疗。随访23例,随访时间3～17个月,心脏超声心动图提示心内畸形矫治满意,恢复良好,双侧上肢动脉波动良好。 结论 对先天性心脏病合并右位主动脉弓患者行外科矫治时应慎重,术前尽量完善相关检查,选择增强CT、磁共振成像检查,明确诊断,制定手术计划。一期同期矫治血管环畸形及其它先天性心脏病,手术效果满意,近期效果良好。     

Interruption of the aortic arch associated with patent ductus arteriosus and ventricular septal defect. Proposal of a new surgical technique for total correction.

A four-year-old girl underwent complete correction of the association of interruption of the aortic arch (Type A, Celoria and Patton's classification), patent ductus arteriosus, and ventricular septal defect. The surgical procedure consists of establishment of a continuity between the ascending and descending aortas utilizing the patent ductus arteriosus and the anterior wall of the pulmonary arterial trunk, reconstruction of the rest of pulmonary arterial trunk with pericardium, and closure of the ventricular septal defect. Though the patient expired from cerebral complications, the hemodynamic result after repair was quite satisfactory. The procedure described makes total correction of this complex anomaly feasible at one operation through a median sternotomy and seems to be a method of choice for most patients with this association of anomalies, unless there is severe narrowing of the patent ductus arteriosus.     

Rare presentation of subclavian artery isolation in a neonate with a family history of aortic arch anomalies

Isolation of the left subclavian artery is a rare aortic arch anomaly in which the artery originates from the pulmonary artery through a ductus arteriosus rather than the aorta. In neonates it is usually diagnosed incidentally with other aortic or cardiac anomalies and can be associated with chromosomal deletions. We describe an extremely rare presentation whereby subclavian artery isolation was presented with left arm ischemia in a 6-day-old child. There were also a concurrent right aortic arch, ventricular septal defect, persistent left superior vena cava, and both radial and ulnar artery hypoplasia. A family history of aortic arch anomalies with no known chromosomal aberration was also present, whereby all the male family members on the maternal side had undergone aorto-cardiac surgery as neonates. Diagnosis and surgical management are discussed.     

Neonatal repair of left atrial diverticulum with gigantic thrombus without cardiopulmonary bypass

A 5-day-old neonate with coarctation of the aorta, hypoplastic aortic arch, large apical muscular ventricular septal defect, and patent ductus arteriosus developed pulmonary over-circulation and systemic hypoperfusion underwent bilateral pulmonary artery banding through median sternotomy as a part of hybrid stage I palliation. At operation, left atrial diverticulum with gigantic thrombus formation at the base of the left atrial appendage was incidentally detected by intraoperative direct echocardiography, and therefore, was successfully resected with the whole thrombus inside it without use of cardiopulmonary bypass. Histopathological finding was compatible with diverticulum. The patient was free from atrial arrhythmia and recurrent thrombus formation.     

Bilateral PDA in a patient with VSD and pulmonary atresia

Patent ductus arteriosus is one of the most common congenital cardiac pathologies, besides patency of ductus may be somewhat vital for various congenital cardiac defects, otherwise death is inevitable. Anatomically, ductus is single and located between the descending aorta and the pulmonary artery. The review of the literature reveals presence of more than one ductus arteriosi in sporadic cases, most commonly associated with aortic arch anomalies. In this report, we present a nine-month-old baby with the diagnosis of ventricular septal defect (VSD), pulmonary atresia (PA), nonconfluent pulmonary arteries, and bilateral patent ductus arteriosi. He underwent a successful pulmonary reconstruction and central-shunt operation with modified aortopulmonary window technique without cardiopulmonary bypass. This is a very rare case with double ductus arteriosi associated with VSD, PA, and nonconfluent pulmonary arteries.     

Pediatric lung transplantation for pulmonary hypertension and congenital heart disease.

Five children underwent lung transplantation for end-stage pulmonary hypertension and respiratory insufficiency associated with congenital heart disease. One (17 mo) had pulmonary hypertension with a patent ductus arteriosus and required two periods of preoperative extracorporeal membrane oxygenation before successful bilateral sequential lung transplantation. One (21 mo) required bilateral lung transplantation for pulmonary hypertension and bronchopulmonary dysplasia associated with iatrogenic injury to the left pulmonary artery. This child also had patent ductus arteriosus ligation and preoperative catheter closure of an atrial septal defect. Extracorporeal membrane oxygenation was required for early postoperative pulmonary support. One child underwent right single-lung transplantation and closure of an atrial septal defect for pulmonary hypertension. Two patients had single-lung transplantation for Eisenmenger's syndrome: 1 with muscular inlet ventricular septal defect closure, atrial septal defect closure, and right single-lung transplantation; 1 with ventricular septal defect closure, patent ductus arteriosus ligation, right ventricular outflow tract patch repair, and single-lung transplantation. All patients survived operation, with one late death (lymphoproliferative disease). The 4 survivors are all ambulatory without oxygen and have evidence of normal pulmonary artery pressure 9 to 12 months after transplantation.     

One stage combined repair of intracardiac defects with vascular ring employing sternotomy approach

We present an unusual case of atrial septal defect and ventricular septal defect with a vascular ring formed by a right-sided aortic arch with an aberrant left subclavian artery that gave rise to a patent ductus arteriosus connecting to the main pulmonary artery. We performed a single-stage repair of the intracardiac defects and division of vascular ring with a sternotomy instead of the traditionally practiced dual approach. This included division and reimplantation of the aberrant left subclavian artery to the left carotid artery after transection. This approach has not been described so far.     

A vascular ring: right aortic arch and descending aorta with left ductus arteriosus

A case is presented of vascular ring caused by right aortic arch with mirror-image branching and left ductus arteriosus. In this case, the descending aorta was located right of the tracheoesophagus and the left ductus arteriosus connected to the descending aorta far below the arch, producing compression of the esophagus only. Through median sternotomy, the ligation and division of the ductus was performed with concomitant repair of ventricular septal defect.     

Spontaneous closure of ductus arteriosus in interrupted aortic arch with ventricular septal defect

A 2-month-old boy diagnosed with interrupted aortic arch type B was treated with a two-stage procedure. His ductus arteriosus had closed spontaneously. Collaterals via both vertebral arteries developed. A 15-mm stenotic segment existed between the left subclavian artery and the descending aorta. The direct anastomosis between the common carotid artery and the descending aorta was performed as a first palliation at the age of 3 months. The left subclavian artery was reconstructed by end-to-side anastomosis to the descending aorta. The postoperative course was uneventful. The closure of ventricular septal defect and pulmonary artery debanding were performed as a second operation 4 months after the first palliation. The patient is alive and well 7 months after the second operation.     

Adult aortic arch atresia.

Atresia in the aortic arch is a rare and severe congenital cardiovascular anomaly. Without surgical therapy, only a few patients can survive to adulthood. A 29-year-old woman with atresia of the aortic arch (Celoria-Patton Type A) without any intracardiac shunt underwent primary surgical correction involving reconstruction of the aortic arch with prosthetic interposition between the transverse aortic arch and the descending aorta and division of the persistent ductus arteriosus. Her postoperative course was uneventful, and she is now doing well at three years to date after surgical treatment.     

Surgical correction of congenital heart defects in infants and young children]

The results of surgical treatment of congenital heart diseases in 595 children under 3 years of age are generalized. There were no fatal outcomes after correction of coarctation of the aorta (9 cases), patent ductus arteriosus (312), pulmonary and aortic stenoses (7). Among 62 patients who underwent removal of an atrial septal defect 3 (4.8%) died. Operations were performed on 140 patients for a ventricular septal defect with high pulmonary hypertension, 31 (22.1%) of them died from various causes. Operations for complicated heart diseases were acts of despair in children whose condition was critical.     

External compression of bronchus by aneurysm from divided major aortopulmonary collateral artery after unifocalization.

The right ventricle to pulmonary artery connection with an extracardiac conduit, left pulmonary artery reconstruction, ligation of patent ductus arteriosus, and take-down of right Blalock--Taussig shunt were performed on a 1-year-8-month-old boy who had pulmonary atresia, ventricular septal defect, patent ductus arteriosus, and major aortopulmonary collateral arteries. He previously underwent the unifocalization and right modified Blalock--Taussig shunt at 9 months of age. He repeatedly had a difficulty in weaning from the mechanical ventilator. After removing the aneurysm from the divided major aortopulmonary collateral artery that compressed the left main bronchus externally, it was possible to wean him from the mechanical ventilator.     

Primary repair of interrupted aortic arch and severe aortic stenosis in neonates

Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.     

自体肺动脉组织一期矫治主动脉弓病变合并心内畸形

目的 探讨自体肺动脉组织一期矫治主动脉弓病变合并心内畸形的手术方法和治疗效果.方法 1993年4月至2009年11月,共25例主动脉弓病变合并心内畸形的患儿接受了一期矫治手术.男性17例,女性8例;年龄26 d～6.5岁,平均11个月;体质量3.0～14.5 kg,平均12.4 kg.其中6例患儿为主动脉弓缩窄,6例为主动脉弓中断,13例为主动脉弓发育不良.手术技术包括切开肺动脉前壁,切除动脉导管,应用部分自体肺动脉壁重建主动脉弓,重建肺动脉,并同时矫治合并心内畸形.结果 24例患儿术后恢复顺利,1例患儿死于围手术期肺动脉高压危象.随访心脏超声及心脏CT检查证实主动脉弓重建及心内畸形矫治满意.术后未发生神经系统及其他并发症.随访过程中,患儿生长发育正常,主动脉弓无再狭窄.结论 自体肺动脉具有生长潜力且柔韧性好,是一种理想的重建主动脉弓的材料.一期矫治手术治疗先天性主动脉弓病变合并心内畸形可以得到满意的结果.     

多层螺旋CT在诊断法洛四联症中的应用

目的探讨多层螺旋CT对法洛四联症的诊断价值及其临床意义。方法46例经手术病理证实的法洛四联症患者,术前均接受MSCT检查,分析其临床资料及MSCT表现,并与术后结果进行对照。结果MSCT对46例法洛四联症患者均能做出定性诊断。法洛四联症室缺类型:嵴下型室缺44例,嵴上型室缺2例。主动脉骑跨率:骑跨50%为44例,骑跨40%为2例。肺动脉狭窄:46例均有右室流出道狭窄。合并肺动脉瓣二瓣化畸形36例。合并房间隔缺损或卵圆孔未闭14例,动脉导管未闭2例,右位主动脉弓14例,永存左上腔4例,另2例左头臂静脉异常走行。结论MSCT对心外畸形的检出率,肺动脉及段级分支的变异和发育情况有较高的价值。MSCT可以清楚显示冠状动脉起源、走行以及心内畸形。     

Thymoma concomitant with a right aortic arch

A 63-year-old woman was referred to our hospital because screening had detected an anterior mediastinal tumor with a right aortic arch. She underwent typical total thymectomy via a median sternotomy but developed left recurrent laryngeal nerve (RLN) palsy postoperatively. The pathology examination revealed that the tumor was a stage I thymoma. This is the first report of a thymoma with a right aortic arch. The left RLN goes around the left ductus arteriosus, which connects the origin of the left subclavian artery to the left pulmonary artery. The RLN was likely to be injured in the neighborhood of the left ductus arteriosus when the tumor and thymus were dissected over the pulmonary artery. During operations for an anterior mediastinal tumor with a right aortic arch, we should be attentive to the location of the tumor, the left ductus arteriosus, and the left RLN.     

A case report of ventricular septal defect accompanied by winded and elongated malformation of the aortic arch

A three-year-old boy, surgically treated for a ventricular septal defect, had a winded and elongated aortic arch between the left common carotid artery and the left subclavian artery. Angiography revealed that the arch was positioned more caudalward than normally, and that it was in contact with the left pulmonary artery at a point near the central portion of the arch (i.e., near the position of the ductus arteriosus). The embryological cause of this malformation was speculated. It seemed unlikely that malformations was caused by involution, abnormal growth, etc., of the components of the great vessels in the coursed of embryonic development, but rather to have been caused by an abnormal elongation of the artery at the fourth arch and of the ventral artery between the 6th and 7th intersegmental artery, which had occurred after normal embryonic development. Under extracorporeal circulation, where blood was returned to the patient via the femoral artery, the patient underwent open heart surgery to close the ventricular septal defect. Pressure monitoring during the operation revealed no pressure loss in the winded and elongated portion of the artery; hence, the malformation seemed to cause no hemodynamic problems at present. Considering that the patient is only three years old and that the aorta will continue to grow, we have decided to refrain from any surgical treatment of the winded and alongated part of the aorta for the time being. However, the patient might develop aortic aneurysm in the future because of the relative coactation of the aorta and the insufficient mechanical strength of the winded and elongated area.(ABSTRACT TRUNCATED AT 250 WORDS)