A Simple and Feasible Technique for Repair of Anomalous Origin of the Left Pulmonary Artery from the Ascending Aorta

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare congenital cardiac malformation and the direct reimplantation technique has been the strategy of choice, although postoperative restenosis across the anastomosis site is frequently observed. In the described case, a simple and feasible technique was used with satisfactory results at 20-month follow-up.     

A Case of Anomalous Origin of the Pulmonary Arteries: Right Pulmonary Artery from the Descending Aorta and the Left Pulmonary Artery from the Ascending Aorta

A 3-month-old girl classified as having persistant truncus arteriosus underwent surgical correction of the anomalous origin of the pulmonary arteries; the right pulmonary artery from the descending aorta and the left pulmonary artery from the ascending aorta. The patient died on the fourth postoperative day. The definite diagnosis and choice of surgical strategies should be further examined.     

Anomalous Origin of the Left Coronary Artery in Tetralogy of Fallot Associated with Abnormal Mitral Valve Pathology

The association of anomalous left coronary artery with congenital heart disease is a rare occurrence. Seven cases of anomalous left coronary artery associated with tetralogy of Fallot have been reported in the literature. We report a unique case with severe mitral valve abnormality that precluded standard surgical repair.     

Association of Common Origin of the Carotid Arteries with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Common origin of the carotid arteries (COCA) is a common pattern of aortic arch vessels and is the single most common cause of tracheobronchial compression by a congenital cardiovascular anomaly. By no means all affected patients are symptomatic. Symptoms may range from recurrent pulmonary infections and “noisy respiration” to stridor and apneic spells. In our study of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) we found a highly significant association of COCA with ALCAPA (85%), although no patient with ALCAPA in this study had evidence of tracheal stenosis documented in the hospital chart. As COCA is easily correctable, we suggest consideration of COCA during evaluation and surgery of patients with ALCAPA so that, if the patient also has symptoms possibly related to COCA, the artery can be suspended from the posterior wall of the sternum.     

Hoarseness as the Initial Clinical Presentation of Anomalous Left Coronary Artery from the Pulmonary Artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly that, if left untreated, will most often result in severe myocardial ischemia and significant morbidity and mortality. We report an unusual presentation of this defect in a 2-month-old infant who had an initial complaint of a “hoarse cry.” We theorize that impingement of the recurrent laryngeal nerve due to dilatation of the pulmonary artery was the most likely etiology of the patient's symptoms. This case serves as an important reminder that serious congenital heart disease may present with any number of complaints and unusual findings.     

Repair of Tetralogy of Fallot with Anomalous Coronary Arteries Coursing Across the Obstructed Right Ventricular Outflow Tract

Anomalous coronary artery (ACA) anatomy occurs in 2–9% of patients with tetralogy of Fallot (TOF), in which the left anterior descending coronary artery (LAD) originates from the right coronary artery (RCA) crossing the right ventricular outflow tract. The purpose of this study was to review our results of repair for TOF with ACA. Between 1978 and 2001, 43 ACA patients (mean age, 4.8 years; range, 5 months–41 years) underwent repair for TC. The ACA anatomy was classified as the single LAD from the RCA (n = 20), a significant conal branch (dual LAD) from the RCA (n = 13), paired anterior descending arteries originating from the left and right coronary arteries (n = 7), and single RCA from the LAD (n = 3). In cases in which the anomalous LAD crossed the obstructed infundibulum, thinning or coring of the endocardium was done. Patch infundibuloplasty was performed in 39 patients, with 10 needing separate RV and pulmonary artery patches, and the pulmonary valve was preserved. Nine patients had the addition of a monocusp to a transannular incision. Two patients had a main pulmonary arterioplasty alone. There was one early (2.3%) and no late deaths. Mean early and late postoperative gradients were 21.5 ± 10.5 mmHg (4 patients had ≥30 mmHg) and 27.1 ± 13.7 mmHg (5 patients had >30 mmHg; p = 0.12), respectively. There were four reoperations during a mean follow-up of 4.8 years (range, 6 months to 18 years). Actuarial freedom from reoperation was 90% at 5, 10, and 15 years. At the latest follow-up, all patients were in NYHA functional class I. TOF repair for an ACA can be performed without disturbing the native coronary anatomy and without the use of conduits in most cases. Outcomes are similar to those of other patients with TOF. The presence of ACA does not impose increased risk after this surgical strategy. Paper presented in part at the annual meeting of the Southeastern Surgical Congress, Savannah, GA, February 2003     

Tetralogy of Fallot with Left Heart Hypoplasia, Total Anomalous Pulmonary Venous Return, and Right Lung Hypoplasia: Role of Magnetic Resonance Imaging

We report a rare case of tetralogy of Fallot with total anomalous pulmonary venous return, left heart hypoplasia, right lung hypoplasia, and left ocular–mandibular synchinesia (Marcus-Gunn phenomenon), correctly diagnosed by cardiovascular magnetic resonance imaging and successfully operated by modified Glenn anastomosis.     

Surgical Repair of Tetralogy of Fallot with Large Conus Artery

The large conus artery transversing the right ventricular outflow tract may cause more confusion and concern in the surgery of tetralogy of Fallot with a small pulmonary annulus than other well-known coronary anomalies. We experienced an infantile case that precipitated into a critical left ventricular failure caused by transection of a large conus artery for the transannular right ventricular outflow tract reconstruction and rescued the patient with an extracorporeal lung–heart-assist system. Preoperative precise diagnosis of coronary anatomy should serve to protect against fatal mistakes, and various techniques for repair must be chosen individually for this subset of anomalies.     

Tetralogy of Fallot with severe pulmonary valvar stenosis and pulmonary vascular sling (anomalous origin of the left pulmonary artery from the right pulmonary artery)

Summary A patient with the rare combination of tetralogy of Fallot with severe pulmonary valvar stenosis and pulmonary vascular sling is presented. The limitations imposed by pulmonary artery hypoplasia on the display of pulmonary vascular sling by conventional imaging techniques are discussed.     

Double-Outlet Right Ventricle (Tetralogy of Fallot Type) Associated with Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery: Report of Successful Total Repair in a 2-Month-Old Infant

The case reported herein demonstrates the rare association of double-outlet right ventricle (tetralogy of Fallot type) with anomalous origin of the left coronary artery from the pulmonary artery. It is the first reported successful total surgical repair in an infant.     

左冠状动脉起源于肺动脉37例患儿各年龄段的临床特点及诊治

目的分析各年龄段左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特点和诊治情况,提高对此病的认识,减少误诊,提高救治率。方法 1999年4月-2011年3月本院共收治37例ALCAPA患儿。男21例,女16例;年龄(5.0±4.8)岁。其中婴儿组(≤1岁)8例,幼儿组(>1～3岁)10例,大年龄组(>3岁)19例。37例中并二尖瓣关闭不全31例,室壁瘤2例,法洛四联症/ASD、重度肺动脉瓣狭窄/部分型肺静脉异位引流、ASD各1例。对各年龄段临床特点和诊治结果进行回顾性分析。随访结果依据门诊复查结果及电话随访。结果婴幼儿组发病年龄均早于大年龄组;ECG出现Q波及ST-T改变13例(35.1%,13/37例),其中婴儿组5例(62.5%)、幼儿组3例(30.0%)、大年龄组5例(26.3%),3组差异无统计学意义;胸部X片示心脏均明显增大,心胸比婴儿组大于幼儿组和大年龄组,幼儿组大于大年龄组;超声心动图示左心室舒张末径(LVED)3组均增大,以婴儿组增大最明显;左心室射血分数(LVEF)<50%者婴儿组多于幼儿组和大年龄组,幼儿组与大年龄组无明显差异。首次诊断时误诊或漏诊18例,占48.6%(18/37例)。婴儿组1例由于核素扫描提示存活心肌很少,等待心脏移植,另2例家长放弃手术。34例患儿行冠状动脉移植术23例,肺动脉内隧道术8例,冠状动脉搭桥2例,单纯结扎冠状动脉左主干1例。无手术早期死亡。术后有近期随访资料26例(76.5%,26/34例),随访6～130(13.6±18.4)个月,晚期死亡1例。患儿术后LVED均较术前缩小,仍有二尖瓣轻度关闭不全8例。有随访资料患儿中,8例术前LVEF<50%,术后3～6个月LVEF均恢复正常。结论 ALCAPA婴儿患儿临床表现较幼儿及年长儿患儿重。小儿ALCAPA由于缺乏特征性表现,各年龄段均易误诊。对于小儿各年龄段表现为心脏扩大、心功能不全、二尖瓣关闭不全等患儿,要注意除外小儿ALCAPA。可以结合其ECG表现,反复超声ECG探查冠状动脉,必要时加行多层螺旋CT或心血管造影以明确诊断,确诊后应尽快手术治疗。     

左冠状动脉起源于肺动脉6例患儿的诊断与治疗

目的分析左冠状动脉起源于肺动脉(ALCAPA)的诊断特点,评估其手术效果。方法本院2006年3月-2007年10月共收治ALCAPA患儿6例。男4例,女2例;年龄6个月～10岁,平均43.5个月。对这6例患儿的临床表现、心电图(ECG)特征、心脏超声、64排螺旋CT及心导管检查资料、手术治疗及转归进行分析。结果ALCAPA5例患儿有不同程度的多汗、烦躁、气促、拒食等临床表现,临床检查示心功能不全。ECG特异表现:4例患儿ECG存在前侧壁异常Q波及ST段改变,1例患儿存在ST段改变,1例患儿ECG大致正常。典型彩色超声心动图表现为:4例患儿出现左室扩大伴左室收缩功能减低;2例患儿存在二尖瓣轻-中量返流,2例患儿为大量二尖瓣返流;2例患儿表现为右冠状动脉扩张。增强CT及心导管检查有特征性改变。其中5例患儿行手术治疗:2例直接移植,3例行左冠状动脉重建术;2例重度二尖瓣返流患儿同时行二尖瓣成形术。1例无症状患儿未行手术治疗。术后患儿症状改善,无死亡病例。结论了解ALCAPA的病变特点及相关辅助检查,可提高ALCAPA的检出率,尽早手术治疗,改善预后。     

Isolated Left Pulmonary Artery in Absent Pulmonary Valve Syndrome

A very rare combination including tetralogy of Fallot (TOF), absent pulmonary valve, and isolated left pulmonary artery is described. A 4-month-old girl presented with mild cyanosis and severe dyspnea. Echocardiography established the diagnosis of TOF and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve site. A right ventricle angiogram showed the severe aneurismal dilation of the proximal right pulmonary artery and the remarkable pulmonary annular stenosis but failed to show the left pulmonary artery (LPA). An angiography after pulmonary vein wedge injection demonstrated the isolated and hypoplastic LPA.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Ventricular Septal Defect and Mitral Stenosis

We report a case of a child with ventricular septal defect, mitral stenosis, and patent ductus arteriosus, who was also found to have anomalous origin of the left coronary artery from the pulmonary artery. Preoperative diagnosis allowed successful surgical correction.     

Congenital Pulmonary Steal Phenomenon Associated with Tetralogy of Fallot, Right Aortic Arch, and Isolation of the Left Subclavian Artery

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).     

Anomalous Origin of Left Pulmonary Artery from Aorta with Atrial Septal Defect

Anomalous origin of left pulmonary artery (ALPA) from aorta with atrial septal defect (ASD) is a rare congenital cardiac malformation. We report a case of ALPA from the aorta with ASD in a 1.8-kg baby. Repair was needed at an early age due to progressive congestive heart failure. Surgical correction was performed with cardiopulmonary bypass for closure of ASD and direct anastomosis of the ALPA onto the main pulmonary artery.     

Anomalous Left Coronary Artery from the Right Sinus of Valsalva and Noncompaction of the Left Ventricle

Anomalous origin of the left coronary artery is a well-known cause of sudden death. Noncompaction of the ventricular myocardium is a cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses. Both anomalies are rare. We report the case of a child with both anomalous origin of the left coronary artery from the right sinus of Valsalva and noncompaction of the left ventricular myocardium found during an evaluation for Kawasaki’s disease.     

Abnormal Flow in the Posterior Descending Artery: An Echo Doppler Clue to the Anomalous Origin of Left Anterior Descending Coronary Artery from the Pulmonary Trunk

Anomalous origin of the left anterior descending coronary (LAD) from the pulmonary trunk is a rare lesion. We describe a neonate with anomalous origin of the LAD from the pulmonary artery and the circumflex artery arising normally from the left-facing sinus. The finding of a tortuous and dilated posterior descending coronary artery suggested increased intercoronary collateral flow and provided a clue to the identification of the anomaly in an otherwise asymptomatic neonate. Interrogation of the posterior descending coronary artery should be included in the routine echocardiographic examination of children, as unusual flow patterns may help identify aberrant coronary artery connections. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Tetralogy of Fallot with Levoatrial Cardinal Vein

A levoatrial cardinal vein is a rare congenital anomaly of the systemic veins. It is frequently associated with left-side obstructive anomalies. We report a case of tetralogy of Fallot with a levoatrial cardinal vein. The innominate vein was absent and two left upper pulmonary veins connected to this vein. There were no left-side obstructive anomalies.     

Double-Outlet Technique for Tetralogy of Fallot-Type Disease with an Anomalous Coronary Artery

To reduce the right ventricular (RV) pressure and the pressure gradient between the RV and the pulmonary artery (PA) in Tetralogy of Fallot (TOF) with small pulmonary annulus, it is inevitable to enlarge the small annulus by incising and patching from RV to PA via PA annulus. If the anomalous coronary artery exists in the RV outflow tract, the procedure can not be done.