Residuae, sequelae, and complications of surgery for congenital heart disease

Definitive, if not curative surgery is available for the eight most common congenital cardiac defects—ductus arteriosus, ASD, VSD, coarctation, pulmonary valve stenosis, aortic valve stenosis, tetralogy of Fallot, and transposition.The results of surgery for uncomplicated cases of DA, ASD, VSD, and coarctation usually can be determined by clinical means (including chest radiogram and ECG). Postoperative heart catheterization is recommended for evaluation of the patient who has had surgery for pulmonary valve stenosis or aortic stenosis and is necessary after tetralogy of Fallot or transposition of the great arteries repair to identify the important postoperative residua and sequelae.The term “curative” surgery probably should be reserved for operation for division of ductus arteriosus unassociated with pulmonary hypertension and performed in childhood.After closure of ASD, patients should continue to be observed for late development of arrhythmias and persistent cardiac enlargement, although the incidence of these problems is low.After VSD closure the patient is still followed at intervals for possible ill effects of the ventriculotomy scar, manifest as arrhythmias, ventricular aneurysm or myocardial insufficiency.The patient with coarctation repair must be observed for a possible late complication from one of the several clinically silent cardiovascular or cerebrovascular anomalies as well as for the chance of restenosis or unrelieved hypertension.Patients who have had an effective relief of the pressure gradient from pulmonary valve stenosis have a good outlook but should be observed for signs of myocardial insufficiency or the possible effects of increased load on the right heart resulting from pulmonary insufficiency.The high incidence of some degree of aortic insufficiency after valvotomy for aortic valve stenosis as well as the slight chance of restenosis necessitates follow-up examinations for these patients.After tetralogy of Fallot repair partially unrelieved infundibular stenosis or pulmonary annular stenosis and/or persistent VSD may require some limitation of physical activities even though the patient feels much improved because of disappearance of the cyanosis. Whether there are residual defects or not, patients should continue to be re-examined annually for an indefinite period of time because of possible myocardial insufficiency or arrhythmias.The problems after transposition repair include arrhythmias, right ventricular myocardial insufficiency, tricuspid insufficiency, persistent communications around the atrial baffle, and vena caval and/or pulmonary vein stenosis. Despite the frequent residua and sequelae of surgery, most patients with transposition obtain remarkable relief of symptoms.The use of exercise stress testing should increase the reliability of clinic assessment of the operative result. In those patients at risk for arrhythmias after surgery, electrophysiological studies at the time of postop cath may contribute to the management of the patient. The addition of ultrasound in the pre and postoperative evaluation of patients will aid the identification of certain clinically silent associated defects and some of the postoperative sequelae.The risk of subacute bacterial endocarditis remains in most patients after intracardiac repair of defects and, with few exceptions, patients who have had definitive surgery require long-term follow-up despite relief of symptoms and the disappearance of clinical manifestations of cardiovascular dysfunction.It is reasonable to expect that with improvement in diagnostic and surgical technique, many of the currently acceptable residua and sequelae of surgery for congenital heart disease will, in the future, be designated as avoidable complications in these patients. With the current healthy interest in long-term postoperative results there is a good chance that more precise information will become available to guide in the selection for surgery of the patient with congenital heart disease.     

Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy

The ascending aorta or pulmonary trunk in congenital heart disease may dilate out of proportion to hemodynamic or morphogenetic expectations, may become aneurysmal, and may rupture. A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Congenital heart diseases such as single ventricle, truncus arteriosus, transposition of the great arteries and tetralogy of Fallot are also associated with aortic medial abnormalities. Aortic regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation which may lead to regurgitation and predispose to dissection and rupture which can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities such as pulmonary atresia, right aortic arch and a history of an aorto-pulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker administration for limiting progressive dilatation of the aortic root in patients with congenital heart disease and repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and or prevent the risk of aortic dissection. Meticulous follow-up of the aortic root after repair, tetralogy of Fallot are thus recommended.     

54 cases of infectious endocarditis seen in 32 years in a population of 2038 congenital heart diseases

A longitudinal study of a 32-year period (1954-1987) involving 2038 patients with congenital heart defects followed by the same physician yielded 54 cases of infective endocarditis (IE). Complex cyanogenic cardiopathies were particularly exposed to the risk of infection (8.2 IE for 1000 patient-years), then came ventricular septal defects (2.4), tetralogy of Fallot (2.3), aortic stenosis (2.0) and atrioventricular canal (1.7). The risk was smaller in patients with Eisenmerger complex (1.2), persistent arterial canal (1.4) and coarctation (0.7). Patients under 10 years of age (16.7%) were less affected than young adults in the 20-29 years age-group (33.4%). The organisms most frequently isolated were streptococci (42%); staphylococci ranked second (23%). Less common organisms were found in 14% of the cases, and blood cultures were negative in 21%. Systemic prophylaxis with penicillin V, introduced 16 years ago, seems to have almost halved the incidence of infective endocarditis due to penicillin-sensitive organisms.     

Cardiac arrest in patient with significant pulmonary regurgitation after surgical valvulotomy at 10 years of age for isolated pulmonary stenosis

The number of adult patients with a congenital heart defect has increased. They are 2–3 times more numerous than children suffering from congenital heart defects, therefore, it is important to be aware of the most frequent congenital heart defects in adulthood – atrial septal defect, ventricular septal defect, aortic coarctation, tetralogy of Fallot and pulmonary stenosis. These patients either underwent one or more operations in childhood, or were not operated at all (defect was not significant or inoperable), or the heart defect was not diagnosed (mostly atrial septal defect). Some of the patients for various periods of time stop attending regular follow-ups and being asymptomatic (even in cases of hemodynamically significant defects), they do not seek medical attention. We present the case of a 46-year-old man with gradually progressing pulmonary regurgitation after surgical valvulotomy at 10 years of age. Despite regular follow-ups by the cardiologist, the patient was never referred to a specialized centre and cardiac arrest caused by ventricular fibrillation occurred after physical exercise.     

Left ventricular non‐compaction: Prevalence in congenital heart disease

Introduction

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease.

Methods

From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects.

Results

From a total of 202 patients with LVNC, 24 patients (12%; mean age 32 ± 11 years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni- or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni- or bicuspid aortic valves (7/963, 1%).

Conclusion

In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.     

Surgical experience with congenital heart disease in Down's syndrome

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.     

Management of ventricular arrhythmias in adults with congenital heart disease

The management of ventricular arrhythmias in adults with congenital heart disease is becoming increasingly more important. Ventricular arrhythmias are associated with a number of congenital heart defects (particularly tetralogy of Fallot), and sudden death. A number of invasive and noninvasive methods have been applied to identify those patients at risk, although indications and the prognostic value of these tests are unclear. Treatment of ventricular arrhythmias in this population include antiarrhythmic medications, catheter ablation, surgery, and implantable cardiac defibrillators.     

Congenital heart surgery

In reviewing selected publications on congenital heart disease management for 1990 and 1991, some topics have been chosen for discussion, including isolated patent ductus arteriosus, coarctation of the aorta, critical aortic stenosis in early infancy, hypoplastic left-heart syndrome, pulmonary artery banding, Fontan procedures, pulmonary atresia and intact ventricular septum, Ebstein's anomaly, tetralogy of Fallot, transposition of the great vessels, and others.     

心内直视下镶嵌治疗肌部室间隔缺损

目的 探讨心内直视下镶嵌治疗肌部室间隔缺损（mVSD）的手术方法及临床疗效.方法 2008年1月至2013年7月,在体外循环心内直视下镶嵌治疗肌部室间隔缺损29例,男19例,女10例,年龄2个月～7岁;其中单个肌部室间隔缺损7例,多发室间隔缺损22例.合并法洛四联症5例,完全性大血管转位1例,肺动脉瓣狭窄3例,房间隔缺损6例,主动脉缩窄3例.合并心脏畸形均同期手术纠治.结果 死亡2例,死亡率6.9％.术后随访3个月～3年,无远期死亡,封堵器边缘少量残余分流3例,无封堵器偏移、二尖瓣反流、主动脉瓣反流、Ⅲ度房室传导阻滞.结论 心内直视下镶嵌治疗肌部室间隔缺损是一种安全、简便、有效的方法.     

The adult with congenital heart disease: born to be bad?

The population of patients with adult congenital heart disease is approximately 800,000 in the U.S. Those with prior cardiac surgery often consider themselves "cured," although the majority faces a lifetime of problems including arrhythmias, ventricular dysfunction, and one or more re-operations. Even patients with repaired "simple" lesions such as an atrial septal defect may not have normal survival if they are repaired in adulthood. Patients with repaired coarctation may have premature cardiovascular complications including sudden cardiac death, myocardial infarction, and stroke. They also have aortic complications such as aneurysm and dissection, which result from a diffuse arteriopathy and continued hypertension that may be caused by underlying endothelial dysfunction. In addition, bicuspid aortic valve occurs in more than one-half of the patients with coarctation, so continued surveillance for significant aortic valvular heart disease is necessary. More complex lesions also pose problems after "total correction." Patients with repaired tetralogy of Fallot often have pulmonary regurgitation, which is frequently overlooked on clinical exam and echocardiography. Pulmonary valve replacement should be performed before the development of irreversible right ventricular dysfunction and an increased risk of ventricular tachycardia or sudden cardiac death. Because they are vulnerable to deterioration of systemic ventricular function, those with congenitally corrected transposition require special vigilance, usually with concomitant atrio-ventricular valve regurgitation. Late referral is common with a deleterious effect on long-term survival. These patients need lifelong follow-up and the residua and sequelae of their complex anomalies must be understood in order to provide optimum care.     

Ventricular late potentials and spontaneous ventricular arrhythmias after surgical repair of tetralogy of Fallot: do they have prognostic value?

OBJECTIVE--To determine the prognostic significance of ventricular late potentials and spontaneous ventricular arrhythmias detected early after surgical repair of tetralogy of Fallot. DESIGN--Prospective observational. SETTING--Cardiology department of a teaching hospital. METHODS--Between June 1984 and June 1991, 104 children (63 boys, 41 girls) were studied by signal averaging and 24 hour ambulatory monitoring after surgical repair of tetralogy of Fallot. Mean (SD) age at operation was 6 (3) years, mean interval between operation and evaluation was 8 (25) months, and the follow up after evaluation was one to 88 (mean 30) months. RESULTS--Ventricular late potentials were detected in 24/104 patients (23%) and spontaneous ventricular arrhythmias in 39/96 patients (38%); usually (in 81%) these were unifocal and rare. Patients with ventricular late potentials were older at operation than patients without late potentials (9 (3) v 6 (3) years, P = 0.002). No correlation was found between the presence of ventricular late potentials and the presence or complexity of spontaneous ventricular arrhythmias early after operation. During the mean follow up of 2.5 years no case of sudden death or sustained ventricular tachycardia was found. CONCLUSION--Short-term prognosis after surgical repair of tetralogy of Fallot is good; ventricular late potentials and spontaneous ventricular arrhythmias are often detected shortly after operation, but in the medium term follow up they do not predict sudden death or serious ventricular arrhythmias.     

Primitive ventricle with normally related great vessels and stenotic subpulmonary outlet chamber. Angiographic differentiation from tetralogy of Fallot

Four patients with primitive ventricle and normally related great vessels with stenotic subpulmonary outlet chamber (Holmes' heart with pulmonary stenosis) are reported. The history, physical examination, and chest x-ray film are not helpful in distinguishing Holmes' heart with pulmonary stenosis from tetralogy of Fallot. Electrocardiogram often provides the first clue to the presence of Holmes' heart; left axis deviation with or without left ventricular hypertrophy is an unusual finding in tetralogy of Fallot, but common in Holmes' heart. Selective ventriculography is diagnostic: the right ventricular outflow chamber overlies the aortic root and aortic valve in the frontal view in Holmes' heart with pulmonary stenosis, but is to the left of the aortic valve in tetralogy of Fallot; no ventricular septum can be identified in Holmes' heart. The diagnosis can be suspected in a child with clinical features of tetralogy of Fallot but atypical electrocardiogram, and can be established by angiography.     

The adult with congenital heart disease

Internal medicine cardiovascular specialists and internists are going to be seeing increasing numbers of adult patients with congenital heart malformations, and most of them will have had one or more surgical or therapeutic catheter procedures. The nonsurgical cases will have either benign defects or important but inoperable anomalies, or the physician may encounter the rare case which, though amenable to surgery, has escaped operation until adult life. Among patients with postoperative congenital heart disease, a few are totally cured and require no special follow-up or any special precautions, e.g., those with certain cases of atrial septal defect of ostium secundum types, patent ductus arteriosus without pulmonary hypertension, and fully repaired total anomaly of pulmonary venous return. Others who are very much improved by definitive repair have residual defect for which surgical treatment was not attempted, e.g., bicuspid aortic valve in the patient who had surgical repair of coarctation or the patient with persistent cleft of a mitral valve leaflet for which no repair was attempted at the time of closure of the ostium primum type of atrial septal defect. Some patients have had incomplete and unsuccessful repair of a defect but yet are symptomatically improved compared with their preoperative status, e.g., those with tetralogy of Fallot with loss of right-to-left shunting by closure of the ventricular septal defect but persistent right ventricular outflow tract or pulmonary artery branch obstruction. Patients with persistently high intracardiac pressure from unrelieved obstruction are at higher risk for sudden death than they were preoperatively, especially those with a high grade of ventricular ectopy. Despite symptomatic improvement compared with the preoperative status, such cases must have reoperation, if feasible, as a means to prevent a sudden catastrophe. Patients with congenital malformations have symptoms that are distinctly different from those with acquired heart disease. Furthermore, their reactions to their problems and to their lives and work in relation to others are often colored by their restricted and overprotected childhoods, which were often filled with doctor visits, several admissions to the hospital, one or more operations, and usually several heart catheterizations. Otherwise, these patients are capable of the same level of achievement intellectually, and only a few have important defects of other systems that would interfere with their roles as independent and productive adults. The problem arises as to what group of physicians will care for these patients in the future.(ABSTRACT TRUNCATED AT 400 WORDS)     

Congenital diverticulum of the right ventricle associated with coarctation of aorta, atrial and ventricular septal defect and ductus.

Congenital right ventricular muscular diverticula are extremely rare and are usually associated with other congenital cardiac anomalies, (in half of the cases tetralogy of Fallot). They functionally behave like an accessory ventricular chamber which contracts synchronously with the normal ventricles.Less than 30 patients with a right ventricular diverticulum have been reported in literature. An apical right ventricular diverticulum occurs in patients with thoraco-abdominal midline defects or abnormalities of the cardiac position([1]). However, an antero-superior diverticulum is usually associated with other congenital cardiac defects, such as a ventricular septal defect, tetralogy of Fallot, double outlet right ventricle and pulmonary stenosis([2--9]).We report an 11-year-old boy with an antero-superior diverticulum of the right ventricle associated with a coarctation of aorta, ductus arteriosus, and atrial and ventricular septum defects. To the best of our knowledge, such an association has not been reported before.     

Primitive ventricle with normally related great vessels and stenotic subpulmonary outlet chamber. Angiographic differentiation from tetralogy of Fallot.

Four patients with primitive ventricle and normally related great vessels with stenotic subpulmonary outlet chamber (Holmes' heart with pulmonary stenosis) are reported. The history, physical examination, and chest x-ray film are not helpful in distinguishing Holmes' heart with pulmonary stenosis from tetralogy of Fallot. Electrocardiogram often provides the first clue to the presence of Holmes' heart; left axis deviation with or without left ventricular hypertrophy is an unusual finding in tetralogy of Fallot, but common in Holmes' heart. Selective ventriculography is diagnostic: the right ventricular outflow chamber overlies the aortic root and aortic valve in the frontal view in Holmes' heart with pulmonary stenosis, but is to the left of the aortic valve in tetralogy of Fallot; no ventricular septum can be identified in Holmes' heart. The diagnosis can be suspected in a child with clinical features of tetralogy of Fallot but atypical electrocardiogram, and can be established by angiography.     

Actualización 2013 en cardiopatías congénitas,cardiología clínica e insuficiencia cardiaca y trasplante

This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices.     

Treatment of congenital heart disease: risk-reducing measures in young adults

Adults with congenital heart disease form a new and relatively young population, since surgical treatment of heart defects became available three to four decades ago. Owing to improved survival this population is steadily growing in number and age. Little is known regarding long-term survival; however, late complications occur frequently. During adulthood, almost half of the patients have one or more complication, such as endocarditis, stroke, systemic or pulmonary hypertension, aortic aneurysm or dissection and arrhythmias. Heart failure and sudden cardiac death are the main causes of death. Treatment of adults with congenital heart disease is aimed at the reduction of symptoms, but also at minimizing the risk and severity of late complications. In this article the most recent advances in the treatment of congenital heart disease will be discussed. The main focus of the article will be on pharmacological, interventional and surgical interventions that reduce the risk of heart failure, arrhythmias, vascular complications, pulmonary hypertension and endocarditis.     

Two cases of pheochromocytoma associated with tetralogy of Fallot.

We report two cases of pheochromocytoma combined with tetralogy of Fallot who showed different clinical courses. Case 1 was a 45-year-old woman with a history of radical operation for tetralogy of Fallot at 20 years of age. She presented with sudden hypertensive attack, and was diagnosed with pheochromocytoma of the left adrenal gland. She was treated surgically, and her high plasma noradrenaline level normalized. Case 2 was a 41-year-old woman who had been suffering from severe cyanosis due to tetralogy of Fallot throughout her life. A palliative operation had been performed at 7 years of age, but a radical operation had not been performed. She has had resistant hypertension since 38 years of age. She was diagnosed as having pheochromocytoma of the left adrenal gland at 41 years of age, but surgery was not performed. She was pharmacologically treated with doxazosin, followed by bisoprolol. Her symptoms somewhat improved, although she continued to have high plasma levels of noradrenaline and adrenomedullin. The combination of pheochromocytoma with tetralogy of Fallot or cyanotic congenital heart disease is rare; however, pheochromocytoma and congenital heart disease might be related through chronic hypoxia and/or gene abnormalities. The presence of pheochromocytoma worsens the hemodynamic state in patients with congenital heart disease regardless of whether radical surgery for congenital heart disease had been performed. Differential diagnosis of pheochromocytoma could be paramount in congenital heart disease patients who show unexpected or unusual symptoms.     

Echocardiographic diagnosis alone for the complete repair of major congenital heart defects

Objectives. The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair.Background. Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects.Methods. To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery.Results. Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality.Conclusions. This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.     

Frequency of aberrant subclavian artery, arch laterality, and associated intracardiac anomalies detected by echocardiography

Tetralogy of Fallot is generally considered to be the most common congenital heart defect associated with an aberrant subclavian artery (ASA), but the prevalence of ASA in patients with other cardiac anomalies is not well described. The pediatric echocardiography database, with 15,871 initial echocardiograms, was searched for all patients with ASA. Arch laterality and associated intracardiac anomalies were documented for each patient. ASA was found in 226 patients, of whom 171 had a left aortic arch (LAA) and 55 had a right aortic arch (RAA). The occurrence of ASA was 1% in patients with LAA (171 of 15,650) and 25% in patients with RAA (55 of 221; p = 0.001). Intracardiac anatomy was normal in 32% of patients with ASA/LAA and 25% with ASA/RAA. Conotruncal anomalies occurred more frequently with ASA/RAA than ASA/LAA (36% vs 18%; p = 0.01). Atrioventricular canal defects accounted for 10% and left-sided cardiac obstructive lesions accounted for 11% of subjects with ASA/LAA. ASA was rarely associated with d-transposition of the great arteries (1 of 226) and double-outlet right ventricle (5 of 226). The prevalence of ASA was highest in patients with interrupted aortic arch (11 of 38; 29%). In patients with tetralogy of Fallot, the overall prevalence of ASA was 8% (34 of 447), but was higher with RAA (16 of 103; 16%). The highest prevalence of ASA occurred in the subgroup of patients with tetralogy of Fallot with pulmonary atresia and RAA (6 of 25; 24%). In conclusion, ASA was more common in patients with RAA, especially with conotruncal anomalies. In patients with LAA, hypoplastic left heart syndrome, aortic coarctation, and atrioventricular canal defects were commonly associated with ASA.