Ultrasonic Imaging of Vascular Rings and Other Anomalies Causing Tracheobronchial Compression

Display of aortic arch anatomy using single plane views does not readily allow for a three-dimensional interpretation of structural relationships. A method that includes a sweep consisting of multiple contiguous frontal planes has been developed for the evaluation of the arch sidedness and number, brachiocephalic vessel pattern, upper descending thoracic aorta sidedness, ductus arteriosi site(s), and proximal pulmonary arteries. The addition of Doppler color flow mapping aids in the tracing of multiple vascular structures in complex cases, and in assessing ductus arteriosi and arch patency. Posterior angulation can display encirclement of the air-filled trachea in cases of double aortic arch. Vascular ring segments without lumens cannot be displayed.     

Feasibility and utility of three-dimensional color flow echocardiography of the aortic arch: The "echocardiographic angiogram"

Two-dimensional transthoracic echocardiography (2DE) is the most commonly used diagnostic modality to evaluate congenital and acquired abnormalities of the aortic arch. However, 2DE is frequently limited in this ability due to the three-dimensional character of the arch and its interrelationships with other vascular structures. Recently, three-dimensional echocardiography (3DE) with 3D color flow Doppler became commercially available. We examined the feasibility and utility of 3DE with 3D color Doppler in the evaluation of patients with congenital (native and postoperative) abnormalities of the aortic arch. We found that 3DE color flow provides important diagnostic information in patients with aortic arch anomalies, in a manner that is quick and user-friendly. In addition, 17 of the 26 patients had their 3DE findings confirmed by additional modalities, providing reasonably significant validation for our findings with 3DE.     

Coarctation and related aortic arch anomalies in hypoplastic left heart syndrome

The aortic arches of 34 specimens with hypoplastic left heart syndrome were studied in order to establish the frequency, the nature and the clinical implications of aortic arch anomalies. A localized aortic coarctation was present in 23 specimens. The coarcation was located preductally in 2 and paraductally in 21 cases. The degree of obstruction caused by the coarctation varied considerably. In only 6 cases (1 preductal and 5 paraductal) was the obstruction judged to be severe. One of these cases had an additional aneurysm of the aortic wall proximal to the coarctation. An aberrant relation of the ductus arteriosus and the aortic arch was found in 2 specimens without localized coarctation. In the remaining 9 specimens the aortic arch appeared normal. The aortic arch anomalies were mostly present in specimens with a severely hypoplastic ascending aorta. Clinicians, when preparing infants with hypoplastic left heart syndrome for surgical palliation, should always suspect associated aortic arch anomalies, especially when there is severe hypoplasia of the ascending aorta. Coarctation of the aorta will require additional surgical treatment.     

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.     

Transesophageal Echocardiographic Diagnosis of Right-Sided Aortic Arch

We present the transesophageal echocardiographic findings in two adult patients with right-sided aortic arch: one without dissection and the other with traumatic aortic injury (dissection). In both patients, the branching pattern was the left common carotid artery and then the right common carotid artery, followed by the right and left subclavian arteries. The technique for the diagnosis of this anomaly and the identification of adjacent vascular structures using contrast echocardiography is described. Three-dimensional reconstruction of the aortic arch also was performed in both patients.     

Coarctation of the aorta in prenatal life: an echocardiographic, anatomical, and functional study.

In a prospective echocardiographic study of over 2000 pregnancies, 24 fetuses were found to have dilatation of the right ventricle and pulmonary artery when these structures were compared with those of the left heart. In 18 of these cases the diagnosis of coarctation or interruption of the aorta was correctly inferred from these findings. On direct echocardiographic examination of the aortic arch, arch hypoplasia or interruption of the aortic arch was recognisable prenatally in 10 of these 18 cases, most readily between 20-30 weeks' gestation. In 11 of 12 cases of aortic arch anomaly studied by Doppler echocardiography, a reduction in blood flow in the ascending aorta was demonstrated. The intracardiac appearances were suggestive of coarctation but the Doppler findings were within normal limits in two further cases which later proved to be normal. A diaphragmatic hernia was found in four fetuses and the intracardiac echocardiography and Doppler findings were the same as those found in fetuses with aortic coarctation. Five cases of coarctation were overlooked on the prenatal echocardiogram but these were found at follow up of the infants. Thus coarctation of the aorta can be diagnosed prenatally, although the condition may be missed or incorrectly predicted by the criteria examined in the present study. Reduced aortic blood flow is a demonstrable feature of aortic arch abnormalities but it is found in other conditions that are not associated with coarctation.     

Coarctation of the aorta in the adolescent and adult: echocardiographic evaluation prior to and following surgical repair

The combination of two-dimensional and continuous-wave (CW) Doppler echocardiographic imaging forms the cornerstone of diagnostic imaging in pre- or postoperative coarctation of the aorta. Because of the frequent association of other congenital heart defects, e.g., bicuspid aortic valve, a segmental imaging approach with multiple image planes should be used. CW Doppler examination from the suprasternal notch should be utilized to assess the degree of obstruction at the coarctation site in all patients. This enhances diagnostic sensitivity. CW Doppler examination can also be applied throughout exercise. Such application allows detection of relative degrees of aortic obstruction following surgical repair of coarctation that may only manifest at elevated levels of cardiac output, e.g., exercise. It aids in the identification of individuals with exercise related systolic hypertension following "successful" coarctectomy and provides a rationale for treatment with beta blockade. The methodology for applying this relatively new technique is discussed.     

Congenital Anomalies of the Aortic Arch

Congenital anomalies of the aortic arch can occur either in isolation or in conjunction with other intracardiac defects. This review will examine congenital aortic arch anomalies as if they occur in isolation, with the awareness that intracardiac defects often coexist. The three general classes of abnormalities to be reviewed are: (1) abnormal connections between the aorta and pulmonary arterial system; (2) aortic arch obstructions; and (3) defects in the formation of the aortic arch typified by right aortic arches, vascular rings, and cervical aortic arches.     

Echocardiography in the diagnosis of thoracic aortic pathology

Transthoracic two-dimensional and Doppler echocardiography has been well established as a useful technique for evaluating many pathologic processes affecting the thoracic aorta. However, the distance of the aortic arch and descending thoracic aorta from the chest wall and the interposition of highly attenuating lung and highly reflective mediastinal structures between the transducer and the aorta present unavoidable limitations. Transesophageal echocardiography is a relatively new technology that overcomes many of the inherent limitations with transthoracic imaging. Complete echocardiographic evaluation of the entire thoracic aorta can now be achieved in nearly all patients. This article will review the continually expanding role of echocardiography in the evaluation of thoracic aortic pathology, including the dramatic impact of transesophageal imaging on the diagnosis of life-threatening disorders such as aortic dissection.     

Postmortem echocardiography and tomographic anatomy of hypoplastic left heart syndrome after palliative surgery

A study was undertaken to improve the understanding of the 3-dimensional (3-D) topology of a complex surgical reconstruction. The pathologic anatomy was investigated by first fixing postmortem heart specimens in such a way as to preserve the 3-D relations. Next, a technique for postmortem 2-D echocardiography was developed to aid in selection of tomographic planes for sectioning the specimens. Subsequent adjustment of planes of section was made to better show particular facets of the anatomy. The material for this investigation was drawn from cases of hypoplastic left heart syndrome after surgical palliation by the Norwood procedure. Three potential hemodynamic sequelae—restrictive inter atrial communication, aortic obstruction at any level and distortion of the pulmonary artery confluence—served as the anatomic focus for this study. Careful preservation of 3-D topology and postmortem 2-D echocardiographic imaging coupled with tomographic sectioning of specimens led to development of new, clinically relevant echocardiographic views for imaging specific atrial septal and aortic arch anomalies. These techniques offer insight into the spectrum of anatomic sequelae of this type of surgery and may be applied to echocardiographic imaging of patients and refinement of surgical technique for other forms of complex congenital heart disease.     

Pseudocoarctation

Pseudocoarctation, also known as kinking or buckling of the aorta, is an uncommon anomaly. Its recognition is important, because it may be mistaken for true coarctation, aneurysm or mediastinal neoplasm. A case of pseudocoarctation associated with left cervical aorta is reported. The present case is unique in the demonstration of obvious tortuosity and kink formation of the cervical aorta and main branches without frank aneurysm formation. Magnetic resonance angiography as a noninvasive imaging modality was suggested for the definitive diagnosis of cervical aortic arch and its accompanying anomalies.     

3-D echocardiographic imaging of double aortic arch.

Delineation of arch abnormalities is difficult by conventional 2-D echocardiography and MRI has been the investigation of choice. 3-D echocardiography is increasingly used in congenital heart disease for both functional anatomy and morphology. This case report demonstrates that 3-D echocardiography can be used in delineating arch anomalies, which can avoid further imaging that needs a general anaesthetic.     

Aortic Arch Interruption and Persistent Fifth Aortic Arch in Phace Syndrome: Prenatal Diagnosis and Postnatal Course

PHACE is a rare congenital neurocutaneous syndrome where posterior fossa malformations, hemangiomas, cerebrovascular anomalies, aortic arch anomalies, cardiac defects, and eye abnormalities are variably associated. We describe the prenatal detection and the postnatal course of a child with PHACE syndrome with a unique type of aortic arch anomaly consisting of proximal interruption of the aortic arch and persistence of the fifth aortic arch. The fifth aortic arch represented in this case a vital systemic‐to‐systemic connection between the ascending aorta and the transverse portion of the aortic arch allowing adequate forward flow through the aortic arch without surgical treatment.     

Noninvasive Evaluation of Aortic Valve Anatomy

Although aortic valve anatomy is three-dimensionally complex, careful echocardiographic investigation can yield surgically relevant information. The promise of intravascular and transesophageal imaging is in yielding the raw data of sufficient resolution to make the three-dimensional reconstruction clinically meaningful. (ECHOCARDIOGRAPHY, Volume 13, May 1996)     

A unique case of “double‐orifice aortic valve”—comprehensive assessment by 2‐, 3‐dimensional,and color Doppler echocardiography

Transesophageal echocardiography (TEE) is a powerful imaging tool for the comprehensive assessment of valvular structure and function. TEE may be of added benefit when anatomy is difficult to delineate accurately by transthoracic echocardiography. In this article, we present 2‐, 3‐dimensional, and color Doppler TEE images from a male patient with aortic stenosis. A highly unusual and complex pattern of valvular calcification created a functionally “double‐orifice” valve. Such an abnormality may have implications for the accuracy of continuous‐wave Doppler echocardiography, which assumes a single orifice valve in native aortic valves.     

胎儿右主动脉弓畸形合并多脏器畸形尸解病理分析

目的:通过研究胎儿右主动脉弓畸形的血管走行及其合并多脏器畸形,并进行文献复习,提高对此畸形的认识。方法:在我院近10年胎儿尸解1 400余例中,发现4例右主动脉弓发育畸形,对其进行详细解剖和观察,同时复习文献。结果:4例右主动脉弓病例中,男性2例,女性2例;I型2例,Ⅱ型2例,4例均有复杂先天性心脏病,3例合并心外多脏器畸形。文献所见,右主动脉弓存活者不少,有症状者多系血管环压迫所致。结论:右主动脉弓并不罕见,可为单发畸形,亦常合并多种心脏及或心外畸形,其非致死性畸形,伴血管环者可通过手术矫正。B超、MIR、CT等可在孕中期发现此畸形。染色体检查亦多有异常。加深对其认识具有重要的临床实践意义。     

Imaging the ascending aorta and aortic arch using transesophageal echocardiography: the expanded aortic view

Transesophageal echocardiography (TEE) imaging of the aorta requires multiple views and is limited by its inability to visualize the distal ascending aorta and proximal aortic arch ("blind spot"). Recent advances in ultrasound technology allow improved imaging of cardiac structures. In this study we describe the ability to image the aortic valve, entire ascending aorta, and aortic arch in one view, the "expanded aortic view" obtained from a novel proximal transgastric window. In 22 patients, an expanded aortic view was acquired with the transducer positioned in a proximal transgastric position using new generation ultrasound system with a TEE probe that incorporates increased range of mobility. Fluoroscopy imaging was performed to confirm the position of transducer in relation to the cardiac anatomy. In 7 of 22 patients, the ability to achieve this view was also tested using an older echocardiography system. The novel proximal transgastric plane allowed reliable images of the aortic valve, ascending aorta including the "blind spot," and aortic arch in a single view-the expanded aortic view.     

Mobility of the Intimal Flap and Thrombus Formation in Aortic Dissection: A Transesophageal Echocardiographic Study

The mechanisms of the motion of the intimal flap and of thrombus formation in acute or chronic aortic dissection are not definitively elucidated. Transesophageal echocardiography seems to be a technique of choice to analyze the flow in the true and false lumina. Twenty-one consecutive patients were studied in order to define the mobility of the intimal flap, the color Doppler flow patterns, the presence of spontaneous echocardiographic contrast, and thrombus formation at different levels of the aorta. The results suggest that clotted false lumen is more often seen in chronic aortic dissection at the level of the descending thoracic aorta. However, thrombosed false lumen in the aortic arch is suggestive of a retrograde aortic dissection. In cases of complete obliteration of the false lumen, the differentiation between aortic dissection and aortic ectasia with mural thrombus may be extremely difficult.     

A Right‐Sided Aortic Arch Misdiagnosed as Asthma Since Childhood

Congenital malformation of the aortic arch complex occurs in 3% of humans. These vascular aberrations result from embryonic structures that persist when there is incomplete atrophy or regression during normal development. Typically, anomalies of the aortic arch do not cause respiratory symptoms. However, in some individuals, the unusual position of the remnant vessels may compress the airway and cause airflow limitation. As might be expected, a symptomatic vascular anomaly is most often clinically apparent in childhood. We report an adult male with exertional dyspnea who had been diagnosed with asthma since childhood. After his symptoms proved refractory to newer asthmatic therapy, he was referred for further evaluation. Bronchial hyperactivity was not present with objective testing, and compression of his trachea by a right‐sided aortic arch was confirmed with appropriate imaging studies. This report illustrates the need to confirm the diagnosis of asthma with objective measures of airflow and reminds the clinician that a congenital vascular aberrancy may initially elude diagnosis.