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Great Artery and Other Vascular Abnormalities

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Transesophageal Echocardiography for Congenital Heart Disease

Abstract

Echocardiography is the diagnostic modality of choice in the initial evaluation and serial assessment of most types of pediatric heart disease. The anomalies of a congenital nature that most often affect the great arteries and related vascular structures are frequently first suspected and identified by high-resolution imaging provided by transthoracic echocardiography. Technological advances made during the last several decades allow for complementary noninvasive imaging modalities to be applied when further characterization of these congenital cardiovascular malformations or variants is required. Transesophageal echocardiography (TEE) plays a limited diagnostic role in these anomalies but nonetheless provides major contributions in the care of affected patients. In the intraoperative setting, TEE is able to confirm the presence of selected abnormal vascular structure(s) or connection(s). More importantly, TEE facilitates the detailed evaluation of associated defects, provides for intraoperative monitoring, and allows for assessment of the surgical intervention(s). This chapter discusses the most frequently encountered great artery and vascular anomalies of a congenital nature, focusing on the applications of TEE in the evaluation of these lesions.

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Authors and Affiliations

  1. Divisions of Pediatric Anesthesiology and Pediatric Cardiology, Departments of Pediatrics and Anesthesiology, Texas Children’s Hospital, Baylor College of Medicine, 6621 Fannin, Suite W17417, Houston, TX, 77030, USA

    Wanda C. Miller-Hance MD, FACC, FASE

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  1. Wanda C. Miller-Hance MD, FACC, FASE
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Correspondence to Wanda C. Miller-Hance MD, FACC, FASE .

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Editors and Affiliations

  1. Childrens Hospital Los Angeles Department of Cardiology, University of Southern California Keck School of Medicine, Los Angeles, California, USA

    Pierre C. Wong

  2. Pediatric Cardiovascular Anesthesiology, Texas Children's Hospital, Houston, USA

    Wanda C. Miller-Hance

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Patent ductus arteriosus. Upper esophageal pulmonary artery long axis video demonstrating left-to-right shunting across a patent ductus arteriosus (PDA, arrow). AO aorta, PA main pulmonary artery (MOV 564 kb)

Patent ductus arteriosus. Mid esophageal right ventricular inflow-outflow view depicts flow (blue signal) across a patent ductus arteriosus (PDA) into main pulmonary artery (MPA). AO aorta (MOV 2137 kb)

125052_1_En_13_MOESM3_ESM.mov

Patent ductus arteriosus. Upper esophageal aortic arch short axis view demonstrating aliased flow (between the arrows) corresponding to a restrictive patent ductus arteriosus. Ao aorta, PA pulmonary artery (Reproduced with permission from Russell et al. [131]) (MOV 254 kb)

Tricuspid regurgitation. Color Doppler interrogation of the tricuspid valve in two different planes demonstrates moderate regurgitation and a peak velocity of the regurgitant jet that reaches 4 m per second. This jet velocity predicts an elevated right ventricular and pulmonary artery systolic pressure in this infant. Ao aorta, LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 5116 kb)

Left heart dilation resulting from a patent ductus arteriosus. Mid esophageal four chamber view obtained in a patient with a ductus arteriosus and left to right shunting. The left sided structures are dilated, particularly the left atrium, due to the volume overload. A tricuspid valve aneurysm is seen without evidence of ventricular level shunting. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 1821 kb)

Aortopulmonary window. Mid esophageal aortic valve short axis view in infant with an aortopulmonary (AP) window. Note the echocardiographic drop out in the region between the arterial roots (arrow), corresponding to faulty aortopulmonary septation, and the shunting by color Doppler. AO aorta, PA pulmonary artery (MOV 2696 kb)

Aortopulmonary window. View of the ascending aorta obtained at the upper esophageal level depicting an aortopulmonary (AP) window (arrow). The spectral Doppler tracing across the right pulmonary artery displays continuous forward flow during diastole as a result of the abnormal aortopulmonary connection. AO aorta, PA pulmonary artery, RV right ventricle (MOV 1311 kb)

Aortopulmonary window. Postoperative transesophageal echocardiogram in the mid esophageal ascending aortic long axis view depicts the bright region along the wall of the ascending aorta (arrow) corresponding to the pericardial patch placed to obliterate the abnormal communication. Color Doppler interrogation across the right pulmonary artery does not suggest concerning obstruction. Ao aorta, MPA main pulmonary artery, RPA right pulmonary artery (MOV 404 kb)

Pulmonary artery sling. Upper esophageal pulmonary artery long axis view of a pulmonary artery sling in an infant. The abnormal takeoff of the left pulmonary artery from the right pulmonary artery is seen. Note the absence of the normal main pulmonary artery bifurcation, which should be seen in a more proximal relationship to the pulmonary valve. The course of the anomalous vessel with respect to the trachea (asterisk) is noted. AO aorta, LPA left pulmonary artery, MPA main pulmonary artery, RPA right pulmonary artery (MOV 1548 kb)

Pulmonary artery sling. Color Doppler image of the same cross-section depicted in Video 13.9 obtained in a zoom mode. Note flow into the left pulmonary artery (LPA) as it arises from the right pulmonary artery (RPA). AO aorta, MPA main pulmonary artery (MOV 1163 kb)

Main pulmonary artery bifurcation. Normal branching of the main pulmonary artery (MPA) into the right (RPA) and left (LPA) pulmonary arteries as imaged from the upper esophageal pulmonary artery long axis view. AO aorta (MOV 241 kb)

Main pulmonary artery bifurcation. Modified deep transgastric long axis image obtained in an infant with double outlet right ventricle and a subaortic ventricular septal defect to demonstrate the normal pulmonary artery bifurcation as seen in cross-section from this window. AO aorta, LA left atrium, LPA left pulmonary artery, RA right atrium, RPA right pulmonary artery (MOV 1685 kb)

Aortic origin of right pulmonary artery. Upper esophageal pulmonary artery long axis view displaying only the left pulmonary artery (LPA) as it arises from the main pulmonary artery (MPA). Note the absence of the normal pulmonary artery confluence in this patient, due to anomalous origin of the right pulmonary artery from the aorta (AO) (MOV 806 kb)

Aortic origin of right pulmonary artery. Long axis plane imaging using a biplane transesophageal probe displaying anomalous origin of the right pulmonary artery (RPA) from the aorta (AO). LA left atrium, RA right atrium, RV right ventricle (MOV 3526 kb)

Aortic origin of right pulmonary artery. Transverse plane sweep in same patient as depicted in Video 13.14 demonstrating anomalous origin of the right pulmonary artery (RPA) from the aorta (AO) by two-dimensional and color Doppler imaging. A catheter is seen in the superior vena cava. MPA main pulmonary artery, RA right atrium (MOV 4886 kb)

Anomalous origin of right pulmonary artery. Mid esophageal ascending aortic long axis view displaying anomalous origin of right pulmonary artery from the ascending aorta (AO). Note the more distal origin of the anomalous vessel (arrow) as compared to that seen in the patient shown in Videos 13.14 and 13.15. Flow into the anomalous pulmonary artery is demonstrated by color and spectral Doppler interrogation. A perimembranous ventricular septal defect is briefly seen in this video. PA main pulmonary artery (MOV 2438 kb)

Coarctation of the aorta. Mid esophageal descending aorta long axis view depicting narrowing at the level of the thoracic descending aorta (arrow) consistent with coarctation of the aorta. (Reproduced with permission from Russell et al. [131]) (MOV 468 kb)

Coarctation of the aorta. Sweep obtained from the upper esophageal window (upper esophageal short axis view) demonstrating the aortic arch in short axis (Arch), a large patent ductus arteriosus (PDA) and the left pulmonary artery (LPA). As the probe is slightly advanced and rotated to the left, the descending aorta (AoDT) is displayed longitudinally; the LPA is again seen in this view. A discrete area of narrowing is noted (arrow) corresponding to a coarctation. Color Doppler imaging displays turbulent flow across the region of obstruction (MPG 10322 kb)

Interrupted aortic arch. Two-dimensional mid esophageal four-chamber view and corresponding color flow mapping in an infant with interrupted aortic arch demonstrating a large posteriorly malaligned ventricular septal defect, hypoplastic subaortic region, aortic annulus, and ascending aorta. AO aorta, LA left atrium, RV right ventricle, LV left ventricle (MOV 1549 kb)

Interrupted aortic arch. Mid esophageal aortic valve long axis view obtained from the same infant as shown in Video 13.19, demonstrating the ventricular septal defect and marked discrepancy in the sizes of the arterial roots. AO aorta, PA main pulmonary artery (MOV 875 kb)

Interrupted aortic arch. Deep transgastric long axis image from the same patient as shown in Videos 13.19 and 13.20 demonstrating the extent of the ventricular septal defect and subaortic narrowing. AO aorta, LA left ventricle, LV left ventricle RA right atrium, RV right ventricle (MOV 2006 kb)

Interrupted aortic arch. Mid esophageal ascending aortic short axis view demonstrates a severely hypoplastic ascending aorta (AO). Note the dilated pulmonary artery (PA) (MOV 1522 kb)

Interrupted aortic arch. Intraoperative images from the mid esophageal four-chamber view following aortic arch advancement, aortic (AO) augmentation, subaortic resection, and closure of the ventricular septal defect (VSD) in the infant with interrupted aortic arch shown in previous videos. Note the large pericardial VSD patch and the relatively small subaortic area, aortic annulus, and aortic root. In the presence of moderately decreased left ventricular systolic function, no significant gradient was recorded across the left ventricular outflow tract. Note that an adequate spectral Doppler tracing could be obtained of the outflow tract in this particular TEE view. LA left atrium, LV left ventricle, RV right ventricle (MOV 3414 kb)

Interrupted aortic arch. The same findings noted in Video 13.23 following surgical intervention are confirmed in the mid esophageal long axis view. Trace intermittent aortic regurgitation is seen. AO aorta, LA left atrium, LV left ventricle, PA main pulmonary artery, RV right ventricle, VSD ventricular septal defect (MOV 1514 kb)

Interrupted aortic arch. Postoperative, mid esophageal, four-chamber view demonstrating a dilated left ventricle (LV) with markedly decreased systolic function in the same infant depicted in prior videos. Moderate mitral valve regurgitation is also seen. AO aorta, LA left atrium, VSD ventricular septal defect (MOV 1408 kb)

Video 13.3

Patent ductus arteriosus. Upper esophageal aortic arch short axis view demonstrating aliased flow (between the arrows) corresponding to a restrictive patent ductus arteriosus. Ao aorta, PA pulmonary artery (Reproduced with permission from Russell et al. [131]) (MOV 254 kb)

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Miller-Hance, W.C. (2014). Great Artery and Other Vascular Abnormalities. In: Wong, P., Miller-Hance, W. (eds) Transesophageal Echocardiography for Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-84800-064-3_13

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  • DOI: https://doi.org/10.1007/978-1-84800-064-3_13

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  • Online ISBN: 978-1-84800-064-3

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