Two-dimensional echocardiographic features of interruption of the aortic arch

The 2-dimensional echocardiographic features of interruption of the aortic arch are presented based on analysis of the echocardiograms and angiograms from 8 infants: 2 with type A and 6 with type B interruption. Each infant had a patent ductus arteriosus, 6 had a conoventricular septal defect with leftward deviation of the conal septum, 1 had truncus arteriosus with truncal valve stenosis, and 1 had a distal aortopulmonary septal defect with an intact ventricular septum. Echocardiographic images obtained from the suprasternal notch or from a high parasternal approach demonstrated the interruption of the aortic arch and continuation of the patent ductus arteriosus into the descending aorta. These findings were compared with those in infants with aortic atresia and a hypoplastic ascending aorta or discrete coarctation of the aorta with tubular hypoplasia of the aortic arch. Types A and B interruption of the aortic arch were easily differentiated and the caliber of the patent ductus arteriosus was assessed. The characteristic conoventricular septal defect was readily visualized from an apex 2-chamber view or from a subcostal sagittal plane view. With this information subsequent angiography can be more expeditiously performed in this group of critically ill infants.     

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.     

Two-dimensional echocardiographic assessment of the aorta in infants and children with congenital heart disease

To determine the accuracy of two-dimensional echocardiography in the identification of congenital anomalies of the aorta, we compared two-dimensional echocardiographic with angiographic results in 261 consecutive infants and children with congenital heart disease (age 1 day to 20 years, mean 3.3 years). Two-dimensional echocardiography was performed and interpreted without knowledge of angiographic results. Complete visualization of the ascending and descending aorta and aortic arch branches was possible by two-dimensional echocardiographic examination in suprasternal, parasternal, and subcostal views of 255 patients (98%). Identification of the esophagus during swallowing aided the diagnosis of anatomic characteristics of aortic arch. One or more significant aortic arch anomalies were present on angiograms of 116 of 255 patients (46%) and were detected by two-dimensional echocardiography in 110 (sensitivity 95%, 99% specificity). Anomalies detected by two-dimensional echocardiography/angiography were ascending aorta hypoplasia in four/four, truncus arteriosus three/three, right aortic arch 31/31, anomalous subclavian artery 11/16, coarctation 27/29, and patent ductus arteriosus 53/57. We conclude that two-dimensional echocardiography can be used to determine the anatomy of the aorta in most infants and children. In selected patients, two-dimensional echocardiography may eliminate the need for angiographic examination before surgery for congenital heart disease.     

Two-dimensional echocardiographic assessment of left atrial size in children

The ability of 2-dimensional echocardiography (2-D echo) to estimate end-systolic left atrial (LA) size and volume was assessed in 140 infants and children. These subjects were divided into 2 groups. Group A included 91 patients with normal LA volume and Group B included 49 patients with LA volume overload. Five echocardiographic views (left parasternal long-axis, left parasternal short-axis, apical 4-chamber, apical 2-chamber and subcostal 4-chamber) were used. From these views, the LA long-axis and minor-axis lengths were measured and the area was planimetered. These echocardiographically derived measurements were compared with angiographically calculated LA volume. Although all echocardiographic measurements correlated well with angiographic LA volume measurements, the echocardiographic area tracked better than length measurements. Echo LA volume was calculated using 5 single-plane and 3 biplane area-length methods. LA volume calculated from either single- or biplane methods correlated well with angiographically determined LA volume. The degree of correlation depended on the method used. Echocardiographic area and estimated LA volume measured from the parasternal long-axis and apical 2-chamber views best separated patients with LA volume overload from normal. Two-dimensional echo using these views accurately segregated all patients with a LA volume >180% of normal and 15 of 21 patients (71%) with an LA volume between 138% and 179% of normal. Thus, 2-D echo is useful in the evaluation of LA size and volume in Infants and children.     

Prenatal diagnosis of left-sided aortic arch,right descending aorta and right-sided ductus arteriosus associated with anomalous origin of left pulmonary artery from ascending aorta

Left-sided aortic arch (LAA), right descending aorta (rDAo), and right-sided ductus arteriosus (RDA) constitute a rare aortic arch anomaly. Moreover, anomalous origin of the pulmonary artery from the ascending aorta, especially that of the left pulmonary artery, is also a rare anomaly of the pulmonary artery branches. Because of the presence of the ductus arteriosus, prenatal ultrasound is an optimal diagnostic tool for the LAA with rDAo. Four-dimensional color Doppler can clearly demonstrate the spatial relationship between the LAA, rDAo, and RDA and the anomalous origin of the left pulmonary artery from the ascending aorta.     

Dilatation of the descending aorta: a radiologic and echocardiographic diagnostic sign in arteriovenous malformations in neonates and young infants.

Eleven infants with arteriovenous malformations were admitted to St. Christopher's Hospital for Children between 1970 and 1978. Dilatation of the descending aorta was seen in the chest roentgenogram in 8 of the 11 patients. Echocardiography used in the two most recent cases revealed approximation of the descending aorta to the posterior left atrial and left ventricular wall in association with generalized cardiomegaly. The most frequently reported radiologic findings in infants with arteriovenous malformations are cardiomegaly, increased pulmonary vasculature and a widened superior mediastinum. These findings can accompany other congenital malformations of the heart, but the radiographic and echocardiographic findings of a dilated descending aorta appear to be more specifically associated with arteriovenous malformations in the neonate and infant.     

Echocardiographic features of arterial tortuosity: A new syndrome?

Twenty-one consecutive children belonging to one extended family, nine girls and twelve boys, with a median age of 3 years (1 day to 16 years), with similar clinical features of velvety, hyperextensible skin, hypermobility of joints, and characteristic facial features, were studied prospectively with echocardiography. Cross-sectional measurements included the aortic annulus, sinus of Valsalva, and the ascending aorta in the parasternal long axis view. In the short axis parasternal view, the pulmonary trunk was measured midway between the pulmonary valve and bifurcation. The right and left pulmonary arteries were measured at their bifurcation points. The appearance of a bifid pulmonary artery, an elongation of the aortic arch and tortuosity and elongation of the brachiocephalic arteries, using high parasternal short axis view and short and long axis suprasternal views, were assessed qualitatively. The descending aorta was visualized in its long axis in a sagittal subcostal view. The control group consisted of 21 normal age and weight matched children who were evaluated with echocardiography for possible heart disease. No statistically significant difference was found in the echocardiographic measurements between the two groups. However, all children in the study group had a bifid pulmonary artery, an elongation of the aortic arch and tortuosity and elongation of the brachiocephalic arteries. Seven of these children also had tortuosity of the descending aorta, three had dilatation of the aortic annulus, sinus of Valsalva and ascending aorta, and five had multiple peripheral pulmonary stenoses. The consistent findings of a bifid pulmonary artery, an elongation of the aortic arch and tortuosity and elongation of the brachiocephalic arteries, can be reliably evaluated with echocardiography, thus identifying children with arterial tortuosity even early in life when characteristic facial features may not be present.     

Interrupted aortic arch in an adult single-stage extra-anatomic repair

Interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment. To our knowledge, the world medical literature contains only 12 reports of isolated interrupted aortic arch diagnosed in adults. Nine of these patients underwent successful surgical repair, but 1 died during the early postoperative period. We describe a 10th successful surgical repair, which involved a 42-year-old woman who had an asymptomatic type B interrupted aortic arch (characterized by interruption between the left subclavian and left carotid arteries). We performed a single-stage extra-anatomic repair by placing a 16-mm extra-anatomic Dacron graft between the ascending and descending portions of the thoracic aorta and by interposing a 7-mm extra-anatomic Dacron graft between the 16-mm graft and the left subclavian artery. The patient recovered uneventfully and continued to do well 6 months later.     

Echocardiographic Examination of the Aortic Arch: Anomalies Presenting in the Neonatal Period

Congenital anomalies of the aortic arch that present in the first 30 days of life include clinically significant lesions such as coarctation of the aorta and interruption of the aortic arch. Other anomalies of aortic arch position may present as part of a larger congenital complex. Knowledge of the embryology and anatomy of these structures is essential to the understanding of this class of defects. A systematic and careful two-dimensional echocardiographic examination of the arch structures from suprasternal and high parasternal views should reveal the anatomy. A careful Doppler examination using pulsed- and continuous-wave Doppler, as well as color flow imaging, is used to delineate the presence and severity of obstructive lesions. This review discusses the anatomy and echocardiographic techniques that may be used to demonstrate this group of lesions. Noninvasive echocardiographic examination of the aortic arch complex in the neonate should be sufficient to make the diagnosis of most aortic arch anomalies. (ECHOCARDIOGRAPHY, Volume 8, July 1991)     

Aortic valve replacement in an adult 20 years after a single-stage extra-anatomic repair of an interrupted aortic arch

Interrupted aortic arch is a rare congenital malformation, which is defined as a loss of luminal continuity between the ascending and descending aorta. Usually, there is a considerable distance between the ascending and descending parts of the aorta. According to the classification system of Celoria and Patton, three subtypes have to be differentiated. We describe a single-stage, extra-anatomic repair in an adolescent patient with a rare type C (the interruption is proximal to the left common carotid artery) interrupted aortic arch and his subsequent aortic valve replacement 19 years later.     

主动脉右弓右降合并Stanford B型主动脉夹层的外科治疗

目的:总结主动脉右弓右降合并Stanford B型主动脉夹层的外科治疗经验。方法:3例右位主动脉弓、右位降主动脉、迷走左锁骨下动脉(迷走左锁骨下动脉型)合并Stanford B型主动脉夹层的患者经胸部右后外切口行胸降主动脉置换术、迷走左锁骨下动脉缝扎术。结果:3例患者均痊愈出院,住院天数7～10 d,无左上肢缺血症状及神经系统并发症。结论:主动脉右弓右降合并Stanford B型主动脉夹层患者行胸降主动脉置换术方法可行,临床疗效满意,术中判断后行迷走左锁骨下动脉缝扎术,可简化手术方式,但应避免术后左上肢缺血坏死。     

Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion.We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion.     

Extraanatomic correction of interrupted aortic arch--an alternative method

Interruption of the aortic arch is a rare but lethal congenital malformation, usually presenting as a surgical emergency during the first 2 weeks of life. Presented is a case of interruption between the left subclavian and left carotid arteries (type B) with origin of the right subclavian artery from the descending aorta. The extraanatomic arch reconstruction consisted of performing an end-to-side anastomosis between the right subclavian artery and right carotid artery with a good functional result.     

Right atrial appendage function in patients with chronic nonvalvular atrial fibrillation

To assess right atrial appendage (RAA) flow and its possible relationship to left atrial appendage (LAA) flow in chronic nonvalvular atrial fibrillation (AF), transesophageal echocardiography (TEE) was performed in 26 patients with chronic nonvalvular AF (group I). For the purpose of comparison, an additional group of 27 patients with chronic valvular AF due to mitral stenosis (group II) was analyzed. The clinically estimated duration of AF in group I was significantly longer than that of group II (8.7+/-3.4 versus 2.7+/-1.1 years). Although right atrial size and RAA maximal area were larger in group I than those in group II, left atrial size was larger in group II than that in group I. Group II had larger LAA maximal areas than group I, but this difference did not reach statistical significance. The two groups were not different with respect to the RAA or LAA emptying velocities. Significant correlations were observed between echocardiographic parameters of the two atria in patients with nonvalvular AF (r range, 0.4 to 0.7). In contrast, in patients with valvular AF, no correlation was observed between the echocardiographic parameters of the two atria (appendage emptying velocity, r = 0.38, p = 0.051; atrial size, r = -0.03, p = 0.89; maximal appendage area, r = 0.07, p = 0.75, respectively). There were no significant differences in the presence of right and left atrial spontaneous echo contrast and thrombus between the groups. All of the right and left atrial thrombi were confined to their respective appendages and were found in the atria with spontaneous echo contrast. Both RAA and LAA thrombi were present in one patient. In conclusion, our findings suggest that AF could affect both atria equally in nonvalvular AF, in contrast to valvular AF. Therefore, the assessment of RAA function as well as LAA may be important in patients with chronic nonvalvular AF.     

Stent-graft treatment of type B aortic dissection involving the right aortic arch: case report

A 56-year-old man suffered from type B aortic dissection associated with the right aortic arch and right descending aorta. The patient was successfully treated by implantation of 2 stent-grafts (a 40-mm Z-stent covered with a 37.5-mm woven Dacron graft) and ligation of the dissected left subclavian artery. A postoperative computed tomographic scan revealed thrombosis of the false lumen and a reduction in the descending aortic diameter.     

Obstructive membrane in arch of aorta in a case of Shone's complex

Shone''s complex is a rare congenital heart disease consisting of multisite obstruction on the left side of the heart. The obstructive membrane in the arch of aorta is never described among these obstructions. We report echocardiographic findings in a patient with Shone''s complex with the obstructive membrane in the arch of aorta.     

A case of Standford type B aortic dissection involving a right-sided aortic arch with mirror-image branching and right-sided descending aorta

Isolated right aortic arch with mirror-image branching is a rare congenital anomaly. To date, no case has been reported for aortic dissection involving a right aortic arch with mirror-image branching. We report here on a case involving a 58-year-old man in whom expanding type B aortic dissection was demonstrated in the right aortic arch with mirror-image branching and a right descending aorta. The patient was successfully treated by interposition of a prosthetic graft via a right posterolateral thoracotomy approach. We also reviewed the literature.     

Interrupted right aortic arch in DiGeorge syndrome

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.     

Dissecting aneurysm of a right-sided descending aorta with a left-sided aortic arch

A 66-year-old man presented with myocardial infarction. Chest X-ray showed a large mediastinal mass. Aortic dissection was suggested by a past history of chest trauma, but the mass was in a very atypical site. Dissection of an abnormally placed right-sided descending aorta was confirmed by computed tomography. The aortic arch lay on the left side. This rare combination of congenital and acquired heart disease led to diagnostic difficulty.     

Interrupted right aortic arch in DiGeorge syndrome.

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.