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Evaluation of the Single Ventricle

  • Pierre C. WongEmail author
Chapter
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Abstract

The terms “single ventricle” and “functional single ventricle” apply to a broad group of congenital cardiac lesions that, despite their marked anatomic heterogeneity, share a common, unifying characteristic: a biventricular intracardiac repair cannot be performed to separate the systemic and pulmonary circulations. In essence, a single functional ventricular pumping chamber supplies both the systemic and pulmonary vascular beds; oxygenated and deoxygenated blood blend together, and all patients exhibit some degree of systemic arterial desaturation. In the management of these patients, the ultimate goal is to separate the systemic and pulmonary circulations such that deoxygenated systemic venous blood returns passively to the pulmonary arteries without the assistance of a separate pumping chamber. The resultant physiology, known as Fontan physiology, forms the basis for the current management of the single ventricle patient. Transesophageal echocardiography (TEE) can play an important role in the diagnostic evaluation of single ventricle patients. The most common application of TEE is in the intraoperative setting—preoperatively, to verify anatomy and physiology, and postoperatively, to evaluate the results of cardiac surgery. However, there are other non-operative settings in which TEE can be utilized to evaluate the detailed anatomy of the single ventricle patient, and to examine some of the possible problems and complications in operated patients. This chapter explores the use of TEE in the single ventricle patient.

Keywords

Single ventricle Univentricular heart Congenital heart disease Echocardiography Transesophageal echocardiography Fontan operation Pediatric cardiac surgery Palliative surgery Hypoplastic left heart syndrome Tricuspid atresia Heterotaxy 

Supplementary material

Video 10.1

Hypoplastic left heart syndrome, as viewed from the mid esophageal four chamber view. The hypoplastic left-sided cardiac structures and the atrial septum are well seen from this view. Rotation of the imaging plane to approximately 90° affords a long axis visualization of the ascending aorta (MPG 8522 kb)

Video 10.2

Pulmonary atresia/intact ventricular septum, also known as hypoplastic right heart, as viewed from the mid esophageal position, multiplane angle rotated between 0° and 100°. The right ventricular cavity is small, muscular, and non-apex forming; there is an underdeveloped trabecular portion. The pulmonary valve is present but imperforate. By color flow Doppler, no antegrade flow is seen across the pulmonary valve. There is significant tricuspid regurgitation (MPG 14034 kb)

Video 10.3

Tricuspid atresia, normally related great arteries, using a combination of mid and upper esophageal views, multiplane angle 0°. First, the equivalent of the mid esophageal four chamber view is obtained. There is no visible tricuspid valve, only a muscular shelf, and a hypoplastic right ventricular chamber. A small ventricular septal defect or bulboventricular foramen is noted, with turbulent left to right shunting. Withdrawal and slight anteflexion of the TEE probe demonstrates the aortic valve arising from the left ventricle, and with further withdrawal of the probe to the upper esophageal pulmonary artery long axis view, the main and left pulmonary arteries are seen arising from the hypoplastic right ventricle (the right pulmonary artery is not well-seen on this particular study). In this video there is a redundant, mobile atrial septum seen (MPG 20864 kb)

Video 10.4

Tricuspid atresia with D-transposed great arteries, using the mid esophageal long axis view and a multiplane angle of 90°. This shows the aorta arising from a small anterior right ventricular outflow chamber. The bulboventricular foramen/ventricular septal defect (BVF/VSD) is noted by the arrow. LA left atrium, LV left ventricle, PA pulmonary artery, RV right ventricle, Ao Aorta (MPG 12326 kb)

Video 10.5

Double inlet left ventricle, normally related great arteries (Holmes’ heart) as viewed from the equivalent of the mid esophageal four chamber view. A multiplane angle of 30° is used to visualize both atrioventricular valves (mild regurgitation of both valves is identified by color Doppler). Rotation of multiplane angle to approximately 40° and probe tip anteflexion demonstrates the mid esophageal aortic valve short axis view, with the origin of the pulmonary artery from the right ventricle. Withdrawal of the probe (multiplane angle about 0°) and tip anteflexion produce the upper esophageal pulmonary artery long axis view. Aliased flow across a distally placed pulmonary artery band is present, and spectral Doppler interrogation documents a 51 mmHg gradient across this region (MPG 20944 kb)

Video 10.6

Double inlet, double outlet right ventricle, obtained from the equivalent of the mid esophageal four chamber view. Both right and left atrioventricular valves drain into the right ventricle and insert into a large, bizarre papillary muscle in the mid esophageal four chamber view. There is a hypoplastic left sided left ventricle. As the probe is withdrawn into an upper esophageal position, both arterial roots are seen in short axis with the anterior vessel (aorta) giving rise to the left main coronary artery. As the multiplane angle is rotated to about 90°, the mid esophageal long axis view shows the abnormal ventriculo-arterial connection (double outlet) and spatial great artery relationship (parallel). These abnormalities are also confirmed in the deep transgastric sagittal views (MPG 32838 kb)

Video 10.7

Double inlet left ventricle, D-transposed great arteries, following pulmonary artery band. The mid esophageal long axis view (multiplane angle 117°) demonstrates the long axis of both outflow tracts, as well as the ventricular septal defect/bulboventricular foramen. Flow across the pulmonary artery band is identified by color flow Doppler (MPG 10830 kb)

Video 10.8

Pre-Fontan study in a patient with right isomerism (asplenia) and double outlet right ventricle/pulmonary atresia and unbalanced atrioventricular canal, right dominant. Withdrawal of the probe from lower esophageal situs short axis view to mid esophageal four chamber position (multiplane angle 0°) demonstrates that the inferior vena cava receives the right hepatic veins and returns to the right atrium. The pulmonary veins are also seen to return to the right sided atrium (MPG 23660 kb)

Video 10.9

Separate entrances of the right and left hepatic veins (patient from Video 10.8). Withdrawal of the probe from lower esophageal situs short axis view to mid esophageal four chamber position (multiplane angle 0°) demonstrates that the inferior vena cava receives the right hepatic veins and returns to the right atrium in the normal manner; however, the left sided hepatic veins return separately and directly to the left sided atrium (MPG 13222 kb)

Video 10.10

Right modified Blalock-Taussig shunt, visualized from upper esophageal pulmonary artery long axis view, multiplane angle 0°, showing continuous flow entering the small right pulmonary artery (MPG 3240 kb)

Video 10.11

Evaluation of pulmonary artery band in a patient with D-transposed great arteries and a ventricular septal defect. The band is well-seen using two-dimensional imaging and color flow Doppler, as seen from the mid esophageal long axis view (multiplane angle 80–110°) and deep transgastric long axis view (multiplane angle approximately 0°). The deep transgastric window provides for an excellent angle for Doppler interrogation across the pulmonary artery band, with a peak gradient of approximately 84 mmHg. Ao aorta, PV pulmonary valve (MPG 9454 kb)

Video 10.12

Damus-Kaye-Stansel anastomosis as viewed from mid to upper esophageal view, multiplane angle 80–90°. The widely patent anastomosis (shown by the arrow) lies just above the semilunar valves. Ao native aorta, PA native pulmonary artery (MPG 9388 kb)

Video 10.13

Problematic Damus-Kaye-Stansel (DKS) repair in a patient with situs inversus, congenitally corrected transposition of the great arteries and hypoplastic right ventricle, as viewed from the mid esophageal long axis view. The creation of the DKS anastomosis resulted in distortion of the native pulmonary valve, producing significant pulmonary regurgitation (posterior great artery) and trace aortic regurgitation (anterior great artery). The ventricular function was severely compromised. A large subepicardial hematoma occurred as the result of external cardiac massage (MPG 10384 kb)

Video 10.14

Patient with hypoplastic left heart syndrome variant, following Norwood-Sano procedure. The anastomosis between a relatively good size native aorta (Ao) and native pulmonary artery (PA) is well seen from the mid esophageal view long axis view. A few frames showing pulsatile Sano conduit flow are seen toward the end of the study. Though not well-shown in this video, the Sano conduit is positioned in the typical location along the anterior aspect of the right ventricle (RV), arising from the RV outflow tract just below the neo-aortic valve (old pulmonary valve). Ao native aorta, PA pulmonary artery (MPG 9550 kb)

Video 10.15

The Sano shunt (right ventricle to pulmonary artery conduit) shown from the upper esophageal pulmonary artery long axis view, multiplane angle approximately 0°. Color Doppler shows aliased to and fro flow seen in the Sano conduit (also shown on the spectral Doppler tracing); this flow is also seen in both branch pulmonary arteries (MPG 6356 kb)

Video 10.16

Right bidirectional cavopulmonary (Glenn) anastomosis as viewed from the mid esophageal ascending aortic long axis view with rightward probe rotation, multiplane angle 95°. The anastomosis is well seen by imaging and color flow Doppler. RPA right pulmonary artery, SVC superior vena cava (MPG 5242 kb)

Video 10.17

Atriopulmonary Fontan, as seen from the mid esophageal four chamber view in a patient with double inlet left ventricle and L-malposed great arteries. Note the severely dilated right atrium with sluggish flow demonstrated as swirling (also known as spontaneous echo contrast). The atriopulmonary connection is shown by two-dimensional imaging and color flow Doppler, as the probe is rotated leftwards (MPG 19302 kb)

Video 10.18

Atriopulmonary Fontan in a patient with tricuspid atresia, showing a severely dilated right atrium (RA) completely filled with thrombus. Note the dilated coronary sinus (CS). LA left atrium, LV left ventricle (MPG 2468 kb)

Video 10.19

Lateral tunnel Fontan connection in a patient with double inlet left ventricle, as seen from the transgastric, lower and mid esophageal positions. With a multiplane angle of 0°, the lateral tunnel is seen in cross-section as a sweep is performed inferiorly to superiorly (a catheter is seen in the upper portion of the tunnel). Rotation of the multiplane angle to 90° shows the entire length of the tunnel and the Glenn anastomosis more superiorly. LV left ventricle (MPG 20676 kb)

Video 10.20

Extracardiac Fontan with a patent fenestration, as viewed from 0° mid esophageal four chamber view. Right to left shunting is clearly seen by color flow Doppler into the physiologic “left” atrium (MPG 5756 kb)

Video 10.21

Extracardiac Fontan conduit placed between a right sided IVC and the left pulmonary artery in a patient with heterotaxy and dextrocardia. TEE evaluation was performed as a sweep from lower to mid to upper esophageal positions, using a multiplane angle of 0°. As it passes to the left, the conduit compresses the pulmonary veins, causing pulmonary venous obstruction (mean gradient 8 mmHg) that had previously led to a low cardiac output state, and may have contributed to the development of thrombus within the conduit (MPG 31622 kb)

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Copyright information

© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Division of Cardiology, Children’s Hospital Los Angeles, Department of Pediatrics, Keck School of MedicineUniversity of Southern CaliforniaLos AngelesUSA

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