Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery

A case of tetralogy of Fallot associated with anomalous origin of the right pulmonary artery from the ascending aorta and hypoplastic left pulmonary artery was successfully repaired. Following the simultaneous procedure with banding of the right pulmonary artery and replacement of modified Blalock-Taussig shunt to the left pulmonary artery at 34 days of age, the intracardiac repair was performed successfully at 1 year and 10 months of age. The patient was well 6 months postoperatively, without unilateral pulmonary hypertension.     

Complete Agenesis of the Right and Left Pulmonary Arteries, with Main Pulmonary Artery Originating from the Right Ventricle, Presenting as Primary Pulmonary Hypertension of the Newborn: Case Report

A female infant who had been misdiagnosed as having primary pulmonary hypertension of the newborn was found to have agenesis of the branch pulmonary arteries with collateral-dependent pulmonary circulation. The intracardiac anatomy was completely normal. Unifocalization of branch pulmonary artery was done, followed by stenting of the ductus arteriosus.     

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.     

Bilateral Coronary Artery Dilatation and Supravalvular Pulmonary Stenosis in a Child with Noonan Syndrome

Noonan syndrome is the second most frequent congenital malformation syndrome, after Down syndrome, associated with cardiovascular abnormalities. The most prevalent cardiovascular abnormalities in Noonan syndrome are pulmonary stenosis and hypertrophic cardiomyopathy. We report the case of a 12-year-old girl with Noonan syndrome who had multiple cardiovascular abnormalities, including extensive bilateral coronary artery dilatation, valvular and supravalvular pulmonary stenosis, atrial septal defect, and mitral valve prolapse. Both coronary artery dilatation and supravalvular pulmonary stenosis, although rarely reported, are abnormalities of the cardiovascular system that may occur in Noonan syndrome.     

Right Ventricular Myocardial Sinusoidal–Coronary Artery Connections in Critical Pulmonary Valve Stenosis

Ventriculo-coronary arterial connections are frequently observed in pulmonary atresia with intact interventricular septum. Sustained right ventricular hypertension during cardiac morphogenesis is thought to be responsible for persistence of myocardial sinusoidal–coronary artery connections. Considering an unusual observation of ventriculo-coronary arterial connections in a patient with critical pulmonary valve stenosis, we speculate that sinusoidal connections in pulmonary atresia with intact ventricular septum occur not only because it is the only exit for blood from the blind right ventricle, but also because of intrinsic altered morphogenesis of the right ventricle myocardium.     

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.     

Valvuloplasty with Large Trefoil Balloons for the Treatment of Congenital Pulmonary Stenosis

ABSTRACT. Percutaneous balloon valvuloplasty was performed in 16 children, 1 1/2 to 14 years old, with congenital pulmonary stenosis with the use or trefoil balloons. These balloons, which consist of 3 identical angioplasty balloons, do not interrupt completely the blood flow during inflation. The size of the trefoil balloons used was 30 to 50 % larger than the valve anulus. A dual balloon technique employing a large trefoil and a smaller single balloon was used to obtain an effective pulmonary valvuloplasty in two patients with large valve anulus. The mean prevalvuloplasty systolic gradient of 78.2 28.9 mmHg (range 40 to 140 mmHg) was reduced to 20.3±5.7 (range 10 to 30 mmHg) after valvuloplasty. No patient developed significant hypotension or bradycardia or other complications as a result of the procedure. The findings demonstrate that percutaneous balloon valvuloplasty with oversized trefoil balloons effectively treated congenital pulmonary stenosis without complications in the cases studied. The use of large trefoil instead of single balloons of a similar diameter is advantageous since they cause no significant compromise of the cardiac output during valvuloplasty.     

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。     

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.     

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.     

Origin of the Main Pulmonary Artery from the Left Coronary Artery in Complex Pulmonary Atresia

We describe a 22-year-old woman with a history of unrepaired pulmonary atresia with ventricular septal defect. This woman was interesting in that her main pulmonary artery and right pulmonary artery arose from the left main coronary artery. She developed significant pulmonary hypertension in addition to isolation of the left pulmonary artery following ductal closure, subsequent to which the majority of her pulmonary blood flow was coronary dependent.     

The Role of Ductal Constriction in Transient Stenosis of the Left Pulmonary Artery

To confirm the hypothesis that narrowing of the left pulmonary artery is produced by ductal closure, Doppler velocities in the pulmonary arteries from 20 premature infants with a patent duct were compared to velocities from 20 with a closed duct. In infants with a patent duct, the mean Doppler velocity in the main, right, and left pulmonary arteries were, respectively, 86 cm/sec ± 5 standard error (SE), 120 ± 7, and 125 ± 8. With closed duct, the comparable velocities were 102 cm/sec ± 7, 129 ± 8, and 190 ± 11. The only significant difference was the higher velocity in the left pulmonary artery associated with a closed duct (p= 0.00003). This physiologic origin of left-branch stenosis associated with ductal constriction should be considered when a murmur is detected after administration of indomethacin (in addition to possible persistent ductal flow), and as an explanation for some murmurs in the normal newborn. No anatomic or physiologic support was found for the theories that murmurs in the neonate were due to ductal flow, or to acute angulation of the branch pulmonary arteries.     

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。     

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.     

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.     

An Isolated Left Common Carotid Artery from the Main Pulmonary Artery: Possible Malseptation of the Truncoaortic Sac

An isolated left common carotid artery (LCA) is an extremely rare condition with only four reported cases. In each case, the isolated carotid artery connects to the right or left pulmonary artery via the ductus arteriosus and the embryologic basis for the abnormalities is believed to reflect an error in the development of the branchial arches. We present a case of an isolated LCA connecting to the main pulmonary artery in association with a right aortic arch and an anomalous origin of the left subclavian artery from the descending aorta. The left ligamentus arteriosus was identified separately. This may represent a disturbance in the septation of the truncoaortic sac secondary to abnormal migration of neural crest cells rather than a pure developmental anomaly of the branchial arches.     

Successful Dilatation of Left Pulmonary Artery Stenosis by Stent Implantation Through a Modified Blalock-Taussig Shunt in an Infant with Pulmonary Atresia and Ventricular Septal Defect

We successfully implanted a coronary stent via a modified Blalock-Taussig shunt to relieve stenosis of the left pulmonary artery in an infant with pulmonary atresia and ventricular septal defect. The placement of a flexible coronary stent via a modified Blalock-Taussig shunt is a safe and feasible catheter intervention for the treatment of infants with hypoplastic pulmonary arteries.     

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.     

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 the Right Coronary Artery from the Left Sinus of Valsalva

A 1-month-old boy without precedent clinical signs of heart disease died unexpectedly. At autopsy, the child was found to have origin of the right coronary artery from the left sinus of Valsalva immediately leftward to the thickening of the aortic wall at the cuspal commissure and adjacent to the ostium of the left main coronary artery. The association of this coronary anomaly with otherwise unexplained prenatal or early postnatal death has only recently been appreciated as a potential cause of sudden infant death and mimic of sepsis, dysrhythmias, congestive heart failure, or metabolic storage diseases.