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(BQ) Part 2 book “A practical guide to fetal echocardiography normal and abnormal hearts” has contents: Aortic stenosis and bicuspid aortic valve, common arterial trunk, fetal arrhythmias, fetal cardiomyopathies and fetal heart tumors,… and other contents.
● UNIVENTRICULAR ATRIOVENTRICULAR CONNECTION Univentricular atrioventricular connection describes a group of cardiac malformations where the atrioventricular connection is completely or predominantly to a single ventricular chamber Embryologically, this malformation is thought to result from failure of the development of the bulboventricular loop stage Much debate still exists today on the various subclassifications of cardiac anomalies within this group and what should be included or excluded (1–4) From a clinical point of view, a congenital heart defect with a univentricular atrioventricular connection, single ventricular physiology, describes a heart with one functioning ventricle with inflow from one or both atria Numerous terms were used to describe this malformation, including univentricular heart, primitive ventricle, common ventricle, single ventricle, cor triloculare biatriatum, cor biloculare, dominant ventricle, and double inlet ventricle (DIV) (3) The classic Van Praagh classification (5), which was later modified by Hallermann et al (6), described one or two atrioventricular valves that empty into a single ventricle and excluded mitral or tricuspid atresia (TA) Anderson’s simpler classification described a single ventricular mass with or without a rudimentary chamber and allowed for the inclusion of mitral or TA (7, 8) In Anderson’s classification, the rudimentary chamber, if present, should not have an inlet but may have an outlet (7, 8) Within univentricular atrioventricular connection, three subgroups can be identified: double inlet, where two atria connect to a single ventricle through two patent atrioventricular valves; single inlet, where one atrium connects to a single ventricle through a single atrioventricular valve; and common inlet, where both atria connect to a single ventricle through a single atrioventricular valve (1) The morphology of the ventricle is generally a left ventricular morphology with a rudimentary right chamber On rare occasions, a right ventricular morphology with a rudimentary left chamber, or a ventricle of indeterminate morphology without a rudimentary chamber, can be seen A single ventricle heart, which results from a surgical repair of a congenital heart anomaly, should not be classified as univentricular atrioventricular connection Table 19.1 lists several cardiac anomalies that may show a single ventricle on fetal echocardiography Of those, DIV and TA with ventricular septal defect (VSD) have been commonly classified in the univentricular atrioventricular connection and will be discussed in this chapter Figure 19.1 represents four-chamber views in fetuses with different cardiac defects and a single ventricle anatomy Figure 19.1: Spectrum of univentricular atrioventricular connection: four different fetal heart defects showing a “single ventricle” (V) in the four-chamber view The detection of one ventricle on fetal echocardiography is not synonymous with a single ventricle A: Fetus with a hypoplastic left heart with absent left ventricle in mitral and aortic atresia B: Fetus with a hypoplastic right ventricle in pulmonary atresia with intact septum C: Common inlet single ventricle in a fetus with right isomerism and other complex anomalies and (D) double inlet ventricle See text and Table 19.1 for details TABLE Cardiac Anomalies That May Show a Single 19.1 Ventricle on Fetal Echocardiography • Hypoplastic left heart syndrome • Pulmonary atresia with intact septum • Atrioventricular septal defect (large or unbalanced) • Single ventricle in right and left isomerism • Corrected transposition with tricuspid atresia • Mitral atresia with ventricular septal defect • Double inlet ventricle • Tricuspid atresia with ventricular septal defect ● DOUBLE INLET VENTRICLE Definition, Spectrum of Disease, and Incidence DIV is considered a classic and most common form of univentricular atrioventricular connection (1) It is characterized by two normally developed right and left atria that connect via separate right and left atrioventricular valves to a common ventricle (Fig 19.2) The most common form of DIV is a double inlet to a morphologic left ventricle, representing about 80%, and the anomaly is also called double inlet left ventricle (DILV) (5) In DILV, a small underdeveloped right ventricle (not shown in Fig 19.2) is commonly present and connects to the single ventricle with a VSD This “remnant” ventricle is a small outlet chamber and the septal defect is usually called bulboventricular foramen The aorta and pulmonary arteries usually arise in D- or L-malposition, and depending on the looping, one or both vessels (double outlet) may commonly arise from the small outlet chamber In cases where the bulboventricular foramen (septal defect) is restrictive, the corresponding arising vessel(s) from the remnant chamber may be diminutive (pulmonary stenosis or aortic coarctation) Other forms of DIV include a double inlet right ventricle, a DIV of mixed morphology, and a DIV of undetermined or undifferentiated morphology (5) DIV is rare and is found in 0.1 per 1,000 live births (9) The prevalence is more common in fetal series due to the easy detection of DIV on the four-chamber view of the heart Figure 19.2: Schematic drawing of double inlet ventricle Note the presence of right (RA) and left (LA) atria, two patent atrioventricular valves, and both atria drain into a single ventricle In most cases, the single ventricle is morphologically a left ventricle A rudimentary ventricle can occasionally be seen (not shown in this scheme) Ultrasound Findings Gray Scale The four-chamber view is abnormal in DIV as it shows a single ventricle with a missing ventricular septum (Fig 19.3) Identifying the morphology of the single ventricle on ultrasound is based on the anatomic characteristic of the morphologic right and left ventricles as discussed in Chapter The left ventricular myocardium appears smooth with fine trabeculations, whereas the right ventricular myocardium is coarse with an irregular surface Assessment of atrioventricular valve anatomy and/or insertion of papillary muscles cannot be used to determine ventricular morphology in univentricular atrioventricular connection Occasionally, the rudimentary right ventricle is seen in the fourchamber plane (Fig 19.4) but in most cases the septal defect (bulboventricular foramen) and the rudimentary right ventricle in DILV are often not visualized in the four-chamber plane but in a more cranial plane, when an attempt to visualize the great vessels is made (Fig 19.5) The rudimentary outlet chamber in DILV is more commonly located on the left side of the main ventricle (L-looping) but can be located on the right side (D-looping) (2) The great arteries are generally in L-malposition if the small outlet chamber is on the left side of the ventricle When the small outlet chamber is localized on the right side, the great arteries arise either in D-malposition or are normally related with the pulmonary artery arising from the small outlet chamber (2) Outflow tract obstructions are recognized due to size discrepancy rather than flow disturbances, which may be absent A narrow pulmonary artery suggests the presence of pulmonary stenosis or atresia, whereas a narrow ascending aorta may be associated with coarctation of the aorta or tubular aortic arch hypoplasia Figure 19.3: Four-chamber views in gray scale (A) and color Doppler (B) in a fetus with a double inlet ventricle Note the presence of right (RA) and left (LA) atria and a single ventricle (SV) in A B shows, in color Doppler, blood flow from the RA and LA through two respective atrioventricular valves into the SV L, left Figure 19.4: Four-chamber view in gray scale in a fetus with a double inlet ventricle Note that the right (RA) and left (LA) atria drain through two distinct atrioventricular valves into the left ventricle (LV) There is a rudimentary right ventricle (RV) as an outlet chamber drained from the LV L, left Color Doppler Color Doppler may be misleading since two atrioventricular valves are patent and two color stripes are visualized, thus mimicking the virtual presence of a separation or septum (10) (Figs 19.3 and 19.6) Diagnosis is typically made on grayscale ultrasound, and color Doppler provides additional information on the patency of the left and right atrioventricular valves, flow across the VSD, and great vessels (Fig 19.5), especially to detect stenosis or atresia (Fig 19.7) Restrictive VSD, which may occur in this condition, is better evaluated using color Doppler Figure 19.5: Long-axis views in gray scale (A) and color Doppler (B) in the same fetus shown in Figure 19.4 with a double inlet ventricle (SV) and a rudimentary outlet ventricle The rudimentary outlet ventricle is connected with the SV through a ventricular septal defect (asterisk), called bulboventricular foramen Aorta (Ao) and pulmonary artery (PA) arise in parallel orientation Note that the Ao is smaller than the PA, due to the small size of the ventricular septal defect Aortic coarctation was diagnosed after birth Inf., inferior Figure 19.6: Fetus at 15 weeks’ gestation with a double inlet ventricle, with both right (RA) and left (LA) atria draining through two respective atrioventricular valves into a single ventricle (SV) A is in gray scale and B is in color Doppler L, left Early Gestation DIV can be detected in early gestation (Figs 19.6 and 19.7) by detecting the absence of a ventricular septum on the four-chamber view as well as abnormally arising great vessels Three-Dimensional Ultrasound The combination of three-dimensional (3D) ultrasound with tomographic imaging permits the simultaneous visualization of the abnormality in the fourchamber plane and the demonstration of the rudimentary ventricle with the course of the great vessels Navigating through the volume in an offline setting may facilitate the evaluation of the spatial orientation of the great arteries Surface rendering shows the large ventricle with inflow from two atrioventricular valves and a rudimentary outlet chamber (Fig 19.8) and may help in identifying the spatial relationship of the great vessels Figure 19.7: Four-chamber (A) and longitudinal (B) views in color Doppler in a fetus at 15 weeks’ gestation with a double inlet ventricle (same fetus as in Fig 19.6) Note in A that the right (RA) and left (LA) atria drain through two respective atrioventricular valves into a single ventricle (SV) The longitudinal plane in B reveals the presence of pulmonary atresia The pulmonary artery (PA) is hypoplastic, demonstrates retrograde flow (arrow), and is located posterior to the aorta (Ao) Inf., inferior; L, left Figure 19.8: Surface-rendering mode of the four-chamber view in a fetus with double inlet ventricle showing the right (RA) and left (LA) atria as well as the single ventricle (SV) A small rudimentary ventricle can also be identified (arrows) L, left; AO, descending aorta Associated Cardiac and Extracardiac Findings Associated malformations in DIV are atresia, hypoplasia or straddling of the atrioventricular valves, pulmonary (or subpulmonic) outflow obstruction, (sub)aortic outflow obstruction, and conduction abnormalities, primarily due to the anatomic disruption of the conduction system (1) The most important extracardiac abnormality to rule out is the presence of right or left isomerism (see Chapter 30), especially in the presence of a common inlet ventricle (11) The sequential approach to the ultrasound examination of the heart may permit detection of corresponding abnormalities Chromosome anomalies and other extracardiac anomalies than isomerism are possible but rather unusual Differential Diagnosis Table 19.1 lists several cardiac malformations in the differential diagnosis of DIV DIV may be missed on prenatal ultrasound in a lateral view of the heart in diastole because the papillary muscles may mimic a ventricular septum in a single ventricle Prognosis and Outcome DIV with patent atrioventricular valves is well tolerated in the fetus Follow-up ultrasound is important prenatally as outflow tract obstruction may develop or worsen due to reduced flow and lack of vessel growth The neonatal course of DIV is dependent on the presence of associated malformations, such as obstruction of the great vessels or atrioventricular valve abnormalities Surgical treatment corresponds to a single ventricular repair The type of surgical repair (pulmonary artery banding, Fontan procedure, or other) mainly depends on detailed evaluation of the great vessel arrangement and perfusion An overall mortality rate of 29% with follow-up up to 25 years of age was noted in an outcome study on 105 patients with DILV and transposed arteries (12) Multivariate analysis showed the presence of arrhythmia and pacemaker requirement as independent risk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality (12) Gender, era of birth, aortic arch anomaly, and systemic outflow obstruction were not risk factors for long-term outcome (12) Similar findings were reported on eight fetuses with DILV with L-transposition of the great vessels (13) Of these, four fetuses (50%) had pulmonary atresia, one fetus (12.5%) also had TA and coarctation of the aorta (died), and one fetus had Pulsed Doppler aortic stenosis, 322f aortic valve, 167, 168f approach to, 162t cardiac rhythms, 547, 549, 549–551f ductus arteriosus constriction, 393–394 ductus venosus, 172–173, 172f principles of, 160–162 pulmonary atresia with intact ventricular septum, 388, 388f pulmonary stenosis, 378–379, 379f pulmonary valve, 167, 168f tetralogy of Fallot, 399, 400f total anomalous pulmonary venous connection, 523, 526f, 528 tricuspid regurgitation, 301–302f, 309, 309–310f ventricular septal defects, 265f Pulsed Doppler sonography, 22t RASopathies, 41–43 Real-time four-dimensional echocardiography, 204–205 Region of interest box, 201, 201f Resistance index (RI), 163t Retinoic acid, 5t, 6 Rhabdomyomas, 543–545, 543f complications of, 544 echodensity, 543 outcome of, 544 Right and left lungs, 50 Right aortic arch in absent pulmonary valve syndrome, 414 cardiac anomalies in, 474, 476–477, 478t color Doppler findings, 469–471 conotruncal anomaly with, 476 definition of, 469 differential diagnosis, 478–479 double aortic arch vs., 478–479 in double outlet right ventricle, 438–439 early gestation detection of, 472–473, 476–477f embryology of, 468f, 469 four-chamber view of, 471f gray-scale findings, 469–471, 471f incidence of, 469 with left ductus arteriosus, 468f, 469, 470, 472–474f, 479 with mirror image branching, 468f, 469 with right ductus arteriosus, 469–470, 471–472f in tetralogy of Fallot, 401, 404 three-dimensional ultrasound of, 473–474, 477–478f three-vessel-trachea view of, 472f Right atrial appendage, 105f, 489f, 495 Right atrial isomerism anatomy of, 489f cardiac anomalies in, 495, 498–499 color Doppler findings, 497f description of, 287 differential diagnosis, 499 early gestation detection of, 496–498, 498f extracardiac abnormalities in, 498–499 features and findings in, 494t, 495 four-chamber view of, 490f gray-scale findings, 497–498f incidence of, 485–486 left persistent superior vena cava in, 495 outcome of, 499–500 prognosis for, 499–500 suspicion of, 486–487 total anomalous pulmonary venous connection and, 495 Right atrium, dilation of, in Ebstein anomaly, 299 Right cardiac output (RCO), 186 Right pulmonary artery, 107f Right subclavian artery, aberrant, 479–483, 480–482f Right ventricle in congenitally corrected transposition of the great arteries, 458, 461f double outlet see Double outlet right ventricle hypertrophy of, in pulmonary stenosis, 376, 376f hypoplastic, 282f, 383f, 384, 390f left ventricle asymmetry with, 365t speckle tracking applied to, 194f volume overload, tricuspid regurgitation caused by, 311, 313f Right ventricular outflow tract view, 103–104, 103f Scimitar syndrome, 530–531, 533f Screening, nuchal translucency thickness, 3 S/D ratio, 163t Selective serotonin reuptake inhibitors (SSRIs), 5t, 6–7 Semilunar valves aortic see Aortic valve clinical application and abnormalities, 168 Doppler technique for, 167, 168f Doppler waveforms across, 167 pulmonary see Pulmonary valve Septal defects see Atrial septal defects; Atrioventricular septal defect; Ventricular septal defects Septum primum atrial septal defect, 253, 253–254f Septum secundum atrial septal defect, 253, 253f Sequential segmental analysis in fetal cardiac evaluation, 66 in fetus, 66t Serotonin, 6–7 “Shelf sign,” 358f, 359f, 360, 361f Shone complex, 322 Shone syndrome, 362 Short-axis view, of fetal heart color Doppler, 155, 156f common arterial trunk, 424, 425f D-transposition of the great arteries, 450, 450f evaluation of, 94 scanning technique, 92–94 Single ventricle description of, 281 four-chamber view of, 282f incidence of, 2t Sinus bradycardia, 555 Sinus tachycardia, 558 Sinus venosus atrial septal defect, 253–254, 253f Situs ambiguous, 485, 487–489f Situs inversus color Doppler, 501 in congenitally corrected transposition of the great arteries, 457 definition of, 485, 500–501, 501f differential diagnosis, 502 early gestation detection of, 502 findings in, 494t gray-scale findings, 501, 502f incidence of, 500–501 with levocardia, 500–501 outcome of, 502 prognosis for, 502 three-dimensional ultrasound, 502 Situs solitus, 485, 486f Sodium valproate, 5, 5t Sonographic cardiac screening, 14–16 Sotalol, 561 Spatial temporal image correlation (STIC), 237, 238f, 320 description of, 201, 202f, 203f, 204, 208f for fetal cardiac function, 195–196 pulmonary stenosis, 381f ventricular septal defects, 266 Speckle reduction imaging, grayscale image optimization, 138, 138f Speckle tracking techniques for fetal cardiac function, 192, 193–194f, 195, 195t strain and strain rate, 192, 195 Spectral Doppler ultrasonography, 161, 161f Spectral tissue Doppler imaging (S-TDI), for fetal cardiac function, 188, 189f, 189t Sternum, 50 Storage diseases, 540–542 Strain, 192 Stroke volume (SV), 163t Superior cavopulmonary connection, 350t Superior vena cava (SVC), 63, 63f, 64, 131–132, 132f embryology of, 505 left see Left superior vena cava retrograde flow in, 549 three-vessel-trachea view of, 100f, 101 tissue Doppler imaging of, 551f transverse view of, 101, 101–102f Supraventricular tachycardia, 558–559, 559f Surface-rendering mode, 212–213, 213–214f Systemic veins, development of, 29 Systolic aortic flow, 349t Systolic function, for fetal cardiac function, 182t Tachyarrhythmias, 558–561, 559–560f TBX5, 43 Teratogens, 4–7 Tetralogy of Fallot, 46t absent pulmonary valve syndrome in, 415 anatomy of, 396f atrioventricular septal defect in, 399f cardiac anomalies in, 401, 403–404, 404t cardiac axis measurements in, 243f chromosomal abnormalities, 401, 403–404 color Doppler findings, 399–401, 399–400f, 401t common arterial trunk vs., 430–431, 431t definition of, 396 diagnosis of, 398, 398f differential diagnosis, 405, 405t, 431t early gestation detection of, 40f, 401 extracardiac abnormalities in, 401, 403–404, 404t five-chamber view of, 397–399f four-chamber view of, 397 genes associated with, 42t gray-scale findings, 397–398, 397–398f incidence of, 2t, 396 outcome of, 405 prognosis for, 405, 406t pulmonary atresia with ventricular septal defect vs., 401t, 406, 409–410 pulmonary stenosis associated with, 398 pulsed Doppler findings, 399, 400f spectrum of, 396 three-dimensional ultrasound of, 401, 403–404f Thebesian valve, 64 Thoracic aorta, 62 Thoracic cavity, 50–52 heart, 50, 51f posterior mediastinum, 50–52 right and left lungs, 50 sternum, 50 thymus gland, 50, 51f Three-dimensional ultrasound aberrant right subclavian artery detection in, 481, 482f absent pulmonary valve syndrome, 419, 419f advantages of, 202, 204 aortic stenosis, 320–321, 324f, 325f atrioventricular septal defect, 274, 277f B-flow rendering, 218–220, 219f chest, 206t coarctation of the aorta, 362, 363–364f color Doppler, 217–218 common arterial trunk, 429, 430–431f double inlet ventricle, 285–286, 285–286f double outlet right ventricle, 442, 442–443f Ebstein anomaly, 302, 303f glass body mode, 217–218, 217–219f, 453, 454f inferior vena cava, 518–519, 520f interrupted aortic arch, 371 inversion mode, 215–217, 216f, 453, 455f, 477–478f left atrial isomerism, 498, 499f left superior vena cava, 511 multiplanar orthogonal display, 205–208, 206t, 207t optimization of, 200–202, 201t pulmonary atresia with intact ventricular septum, 388, 390f with ventricular septal defect, 413, 414f pulmonary stenosis, 380, 381–382f reference plane, 200, 201f region of interest box, 201, 201f right aortic arch, 473–474, 477–478f summary of, 223–224 surface-rendering mode, 212–213, 213–214f tetralogy of Fallot, 401, 403–404f tomographic ultrasound imaging, 209–211, 209f total anomalous pulmonary venous connection, 532–533, 533f transparent minimum mode, 215, 215f transposition of the great arteries congenitally corrected, 459–460, 462–463f D-, 453, 454–455f tricuspid atresia with ventricular septal defect, 290, 292, 293f ventricular septal defects, 265–267, 266–268f volume acquisition angle, 201, 201f considerations for, 200–201 real-time 4D, 204–205 spatiotemporal image correlation, 201, 203f, 204 static 3D, 202–204, 203f volume display, 205–212 volume rendering, 212–220, 213–219f, 460 Three-vessel-trachea (3VT) view, 109–121 aberrant right subclavian artery, 480, 480–481f of aortic arch, 98, 98f, 115 coarctation of the aorta, 356–357, 357f, 360f in color Doppler, 156f, 156–157, 157t common arterial trunk, 425, 426f connection between great vessels, 116 dilated pulmonary artery, 113, 114f dilated SVC, 117 dilated transverse aortic arch, 113 double aortic arch, 475f double outlet right ventricle, 438, 438f ductus arteriosus, 392–393f fetal heart, 98, 100f, 101 findings in, 111–117 four vessels, demonstration of, 117 gray scale and color Doppler assessment, 111 great artery, course of, 115–116 great vessel of enlarged size, 115 great vessel of normal size, 114–115 interrupted aortic arch, 369f, 370 left brachiocephalic vein, 118–120, 119f, 120f left superior vena cava, 506, 508, 508–510f, 510 narrow or absent aortic arch, 112–113, 112f, 113f narrow or absent pulmonary artery, 113, 114f pulmonary artery, 98, 100f, 101 pulmonary atresia with intact ventricular septum, 387f pulmonary stenosis, 377–378f right aortic arch, 472f scanning technique for, 110–111, 110f, 111f superior vena cava, 100f, 101 left, 505, 506, 508–510f, 510 three vessels, without SVC, 117 thymus gland, 117f, 118 total anomalous pulmonary venous connection, 527–528 transposition of the great arteries D-, 449–450, 451f tricuspid atresia with ventricular septal defect, 290, 291f Thymic–thoracic ratio (TT ratio), 119f Thymus gland, 38, 50, 51f, 117f, 118, 425, 426f Time-to-peak velocity, description of, 163f, 164t, 167 Time-velocity integral (TVI), 163t, 165t Tint maps, 140, 141f see also Color maps Tissue Doppler imaging, 188, 549, 551–552f, 553 Tomographic ultrasound imaging common arterial trunk, 429 description of, 209–211, 209f right aortic arch, 473–474 ventricular septal defects, 265–267 Total anomalous pulmonary venous connection anatomy of, 522f blood flow in, 527–528 cardiac (type II), 522f, 528, 529f cardiac anomalies in, 533–534 color Doppler findings, 523, 528f definition of, 522 differential diagnosis, 534 early gestation detection of, 531 extracardiac abnormalities in, 534 four-chamber view of, 523, 524f, 529f gray-scale findings, 523, 524f in heterotaxy, 523, 533–534 incidence of, 2t, 522–523 infracardiac (type III), 522f, 528, 530f, 534 mixed (type IV), 529 outcome of, 534 prognosis for, 534 pulsed Doppler findings, 523, 526f, 528 right atrial isomerism and, 495 spectrum of, 522–523 supracardiac (type I), 522f, 525, 526–528f, 527–528 three-dimensional ultrasound of, 532–533, 533f three-vessel-trachea view of, 527–528 Transabdominal transducers, 228, 232f Transducer 3D and 4D, 220–223, 221–223f choice of, 136, 137f in compound imaging, 138, 138f focal zone distance and, 138 in harmonic imaging, 136–138, 138f linear, 136, 137f in transabdominal obstetric scanning, 136 Transparent minimum mode, 215, 215f Transposition of the great arteries, 46t congenitally corrected anatomy of, 457f atrioventricular discordance associated with, 457, 458, 460f cardiac anomalies in, 457, 458t, 460 color Doppler findings, 458, 462f D-transposition of the great arteries vs., 463t definition of, 457 dextrocardia in, 457 differential diagnosis, 461, 463, 463t early gestation detection of, 459 four-chamber view of, 458, 459–460f gray-scale findings, 458, 459–461f heart block in, 464, 556 incidence of, 457 left ventricle in, 458, 461f outcome of, 463–464 outflow tracts in, 458, 461f prognosis for, 463–464 right ventricle in, 458, 461f situs inversus in, 457 three-dimensional ultrasound of, 459–460, 462–463f D anatomy of, 447–448f cardiac anomalies in, 453, 455 color Doppler findings, 451, 452f congenitally corrected transposition of the great arteries vs., 463t definition of, 447–448 diagnosis of, 448 differential diagnosis, 455–456 early gestation detection of, 452–453, 453f extracardiac abnormalities in, 453, 455 false-positive diagnosis of, 456 four-chamber view of, 448, 449f gray-scale findings, 448–450, 449–451f incidence of, 2t, 447–448 outcome of, 456 prognosis for, 456 short-axis view of, 450, 450f spectrum of, 447–448 three-dimensional ultrasound of, 453, 454–455f three-vessel-trachea view of, 449–450, 451f ventricular septal defects associated with, 453 Transvaginal transducers, 228 Transvaginal ultrasound, 228f, 230f, 235f, 413f Transverse view of aortic arch, 98, 98f, 102, 102f of arterial duct, 101, 101f of superior vena cava, 101, 101–102f Tricuspid atresia with ventricular septal defect anatomy of, 288, 289f cardiac anomalies in, 293–294 color Doppler findings, 290, 291–292f definition of, 288 early gestation detection of, 290, 292f extracardiac abnormalities in, 294 four-chamber view of, 288, 288–289f, 292–293f gray-scale findings, 288–290, 289–290f incidence of, 2t, 288 outcome of, 295 prognosis for, 295 pulmonary atresia with intact ventricular septum vs., 294t, 390 spectrum of, 288 surgical correction of, 295 three-dimensional ultrasound of, 290, 292, 293f three-vessel-trachea view of, 290, 291f Tricuspid regurgitation cardiac anomalies in, 311 chromosomal abnormalities in, 314 color Doppler findings, 301f, 309, 309–310f definition of, 309 differential diagnosis, 312t in dilative cardiomyopathy, 304 in Down syndrome, 314, 314f duration of, 310 early gestation detection of, 380 in Ebstein anomaly, 300, 301f holosystolic, 310, 311f myocardial function impairment, 312 peak velocity of, 310, 311f pulmonary atresia with intact ventricular septum and, 391 in pulmonary stenosis, 379, 379f pulsed Doppler findings, 301–302f, 309, 309–310f right ventricular volume overload as cause of, 311, 313f spatial expansion, 310 tricuspid valve dysplasia and, 311 trivial, 310–311, 310f Tricuspid valve dysplasia of 3D ultrasound, fetus, 308f anatomy of, 306f cardiac anomalies in, 305–306 color Doppler findings, 306, 307f definition of, 305 Ebstein anomaly vs., 299, 304 outcome of, 307 postnatal chest x-ray of, 308f short-axis view of fetus, 308f spectrum of, 305–306 tricuspid regurgitation and, 311 E/A ratio across, 166f long-axis displacement of, 191, 193f spectral Doppler waveforms across, 184f Trigeminy, 553, 555–556, 555f Trimethadione, 5, 5t Trisomy 8 mosaicism, 37t Trisomy 9, 37t Trisomy 13, 35, 37t, 361–362 Trisomy 18, 35, 37t Trisomy 21 aberrant right subclavian artery in, 481–483, 482f, 483t atrioventricular septal defect in, 276–277 description of, 34–35, 37 tricuspid regurgitation in, 314, 314f ventricular septal defects in, 268 Trivial tricuspid regurgitation, 310–311, 310f Tuberin gene, 544 Tuberous sclerosis, 544–545, 544t, 545f complex, 44–45 Tubular hypoplasia of the aortic arch, 354 Turner syndrome, 35, 37, 37t, 361–362 22q11 deletion syndromes D-transposition of the great arteries, 455 description of, 38–39, 40t interrupted aortic arch in, 371, 372 pulmonary atresia with ventricular septal defect, 414 tetralogy of Fallot, 404 Twin–twin transfusion syndrome, 3 Two-dimensional (2D) grayscale ultrasound image optimization, 136–143, 141t choice of transducer, 136, 137f compound imaging, 138, 138f dynamic range, 139–140, 140f focal zone(s), 138–139, 139f harmonic imaging, 136–138, 138f high frame rate, 140 image preset, 136–140 speckle reduction imaging, 138, 138f tint/color maps, 140, 141f U-sign, 470, 473–474f, 477f Uhl anomaly, 311 Umbilical arteries, 162 Umbilical vein, 153f Umbilical–portal system, 127–128, 128f Unbalanced atrioventricular septal defect, 272f, 275–276 Unguarded tricuspid orifice, 311 Univentricular atrioventricular connection, 281–282 Upper mediastinum, 109–121 V-sign, 469–470, 471f, 476 Valve see specific valve Valvular aortic stenosis, 317 Vein of Galen aneurysm, 311, 313f Veins, of heart, 63–64 Velocardiofacial syndrome, 38, 40t Velocity scale, 147, 148f Vena cavae see Inferior vena cava; Superior vena cava Venous system see also Fetal venous system anatomy of, 123 systematic evaluation of, 123–135 Ventricle see Left ventricle; Right ventricle; Single ventricle Ventricles septation, 25, 26f, 28, 28f Ventricular septal defects, 46t, 349t anatomy of, 259–260f, 261f, 416f atrioventricular septal defect vs., 277 cardiac anomalies in, 260t, 268 classification of, 259–260, 260t color Doppler findings, 263–264, 264–265f in common arterial trunk, 422, 429 in D-transposition of the great arteries, 453 definition of, 259 differential diagnosis, 267, 268–269 in double outlet right ventricle, 435, 436t in Down syndrome, 268 dropout artifact vs., 267 early gestation detection of, 264–265 extracardiac abnormalities in, 268 five-chamber views of, 264–265f four-chamber views of, 261–262f gray-scale findings, 261–263, 261–263f incidence of, 1, 2t, 259 inlet, 259, 260t, 262f, 277, 288 interrupted aortic arch and, 368–369, 368f muscular, 259, 260t, 263f, 266f, 268 outcome of, 268–269 perimembranous, 259, 260t, 263, 264–265f prognosis for, 268–269 pulmonary atresia with see Pulmonary atresia, with ventricular septal defect pulsed Doppler findings, 265f three-dimensional ultrasound of, 265–267, 266–268f in transposition of the great arteries, 453 tricuspid atresia with see Tricuspid atresia with ventricular septal defect in trisomy 21, 268 Ventricular septum anatomy of, 98, 259 inlet, 259 intact, pulmonary atresia with see Pulmonary atresia, with intact ventricular septum long-axis view of, 104, 104f membranous, 259 outlet, 259 trabecular, 259 Ventricular tachycardia, 559–560 Ventriculo-coronary arterial communications, 383f, 387, 387f Vertical vein, 527–528, 528f Volume rendering, 212–220, 213–219f A Wave, 164, 166 definition of, 164t Williams-Beuren syndrome description of, 40t Williams–Beuren syndrome, 322 Wolf–Hirschhorn syndrome, 40t Wolff–Parkinson–White syndrome, 559 Z-scores, 248–250, 249t ... A narrow pulmonary artery suggests the presence of pulmonary stenosis or atresia, whereas a narrow ascending aorta may be associated with coarctation of the aorta or tubular aortic arch hypoplasia Figure 19.3: Four-chamber views in gray scale (A) and color Doppler (B) in a. .. fetuses had patent great vessels, 16 had pulmonary stenosis, 11 had pulmonary atresia, had aortic stenosis, had coarctation of the aorta, had aortic hypoplasia, had interrupted aorta, and had a common... in a multicenter series of TA diagnosed prenatally (23 ) Postnatal outcome is dependent on associated cardiac and extracardiac findings An outcome study of prenatally diagnosed TA estimated an 83% survival at year of age