Arterial Switch Procedure for D-Transposition of the Great Arteries ...

Pediatric Imaging Matthias Gutberlet, MD Thomas Boeckel, MD Norbert Hosten, MD Michael Vogel, MD Titus Ku ¨ hne, MD Hans Oellinger, MD Tilman Ehrenstein, MD Stephan Venz, MD Roland Hetzer, MD Georg Bein, MD Roland Felix, MD Index terms: Aorta, flow dynamics Aorta, MR, 564.12142, 941.129411, 941.129412 Aorta, surgery, 56.459, 941.459 Pulmonary arteries, flow dynamics Pulmonary arteries, MR, 564.121411, 564.121412, 564.12144 Transposition of great vessels, 564.1612, 941.149 Radiology 2000; 214:467–475 Abbreviations: AA 5 ascending aorta LPA 5 left pulmonary artery PA 5 main pulmonary artery RPA 5 right pulmonary artery TTE 5 transthoracic echocardiography 1

From the Departments of Radiology (M.G., N.H., T.K., H.O., T.E., S.V., R.F.) and Pediatric Cardiology (T.B., G.B.), Charite´, Campus Virchow-Klinikum, Medizinische Fakulta¨t der Humboldt-Universita¨t, Augustenburger Platz 1, 13353 Berlin, Germany, and the Departments of Congenital Heart Disease and Pediatric Cardiology (M.V.) and Cardiac, Thoracic, and Vascular Surgery (R.H.), German Heart Institute, Berlin. From the 1998 RSNA scientific assembly. Received December 17, 1998; revision requested February 8, 1999; revision received May 4; accepted August 20. Address reprint requests to M.G. (e-mail: [email protected]).

Arterial Switch Procedure for D-Transposition of the Great Arteries: Quantitative Midterm Evaluation of Hemodynamic Changes with Cine MR Imaging and Phase-Shift Velocity Mapping—Initial Experience1 PURPOSE: To evaluate cine magnetic resonance (MR) imaging and phase-shift velocity mapping for assessment of the hemodynamic relevance of stenotic segments or specific hemodynamic changes in the great vessels after an arterial switch procedure for correction of D-transposition of the great arteries. MATERIALS AND METHODS: Twenty consecutive patients (age range, 2–17 years) with an acoustic window that was insufficient for Doppler transthoracic echocardiography were included in the study. Flow and diameter measurements of the pulmonary arterial trunk and its primary branches were performed with phase-shift velocity mapping and cine MR imaging. RESULTS: There were good correlations between pressure gradients in the pulmonary arteries estimated with MR imaging and those measured with Doppler echocardiography (r 5 0.83, n 5 15) and cardiac catheterization (r 5 0.90, n 5 13). Cine MR imaging revealed that the diameters of the right and left pulmonary arteries decreased with the expansion of the aorta during systole, which increased the peak velocity. This temporary stenosis was more severe in the right than in the left pulmonary artery and was accompanied by a significantly (P , .05) lower volume flow in the right artery. CONCLUSION: The anatomic situation after arterial switch repair tended to produce temporary stenoses in the primary pulmonary arterial branches, with significant changes in hemodynamics. These changes may affect the long-term outcome and go undetected with other imaging modalities.

r RSNA, 2000 Author contributions: Guarantor of integrity of entire study, M.G.; study concepts and design, M.G., T.B., N.H.; definition of intellectual content, M.G., T.B., N.H., M.V., R.H., G.B., R.F.; literature research, M.G., T.K., T.E., H.O., S.V.; clinical studies, M.G., T.B., M.V.; experimental studies, M.G., T.K.; data acquisition, M.G., T.B., H.O., T.K.; data analysis, M.G., T.K., T.E.; statistical analysis, M.G., T.K., T.E.; manuscript preparation, M.G.; manuscript editing, M.G., T.E.; manuscript review, T.B., M.V., N.H., S.V., R.H., G.B., R.F.

Over the past 2 decades, after the description in 1975 by Jatene et al (1), primary arterial switch repair—rather than atrial repair—for D-transposition of the great arteries has become the surgical treatment of choice in newborns (2,3). Left ventricular impairment and complications involving the reimplanted coronary vessels (4,5) are among the most common early postoperative complications of this procedure, whereas supravalvular pulmonary stenosis is widely recognized as the most frequent midterm complication after arterial switch repair (6–10). The need for repeated intervention after an arterial switch operation ranges from 5% to 30% (6–10). Besides the hemodynamic burden to the right ventricle, there have been isolated cases of hemolysis (10), false aneurysm, and endocarditis due to supravalvular pulmonary obstruction after anatomic correction. Postoperative obstruction may occur at different levels. In recent years, echocardiography has become the diagnostic method of choice for preoperative evaluation of patients 467

ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND NA NA 6 5 240 6 10 13 10 6 8 1 8 6 6 240 11 3 10 5 15 8 7.6 3.6 1/3 2/4 3/10 4/3 5/7 6/9 7/8 8/3 9/2 10/4 11/10 12/8 13/3 14/11 15/3 16/17 17/9 18/3 19/9 20/5 Mean SD

Note.—NA 5 not applicable, ND 5 no data. * Number is ratio of pulmonary flow (QP) to systemic flow (QS). † Vessel was occluded. ‡ Stent was placed in vessel.

LPA RPA

ND ND ND ND ND ND ND ND ND ND ND 45 ND 64 ND ND ND ND ND ND NA NA 32 49 0 ND 4 4 19 18 50 13 36 ND 16 ND 6 64 25 64 12 8 NA NA

PA LPA

ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND NA NA ND ND ND ND ND ND ND ND ND ND ND 45 ND 50 ND ND ND ND ND ND NA NA

RPA PA

60 40 ND 65 15 11 37 20 50 15 30 ND 15 ND 16 45 ND ND 20 ND NA NA ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND NA NA

LPA RPA

ND ND ND ND ND ND ND ND ND ND ND 45 ND 50 ND ND ND ND ND ND NA NA 30 ND 10 115 3 2 5 ND ND 24 10 ND 21 ND ND 60 ND 50 ND 14 NA NA 9 8 10 3 10 10 8 5 6 8 11 NA 6 10 9 20 8 7 6 10 8.8 3.8 8 6 11 4 9 10 8 4 5 7 9 NA 5 4 8 19 10 6 8 7 8.0 3.9 5 8 7 12 5 8 8 5 9 11 10 9 8 7 10 16 8 11 10 15 9.0 2.8 3 4 5 11 5 6 6 4 5 9 9 5 6 2 7 8 9 6 9 10 6.5 2.3 13 11 20 11 13 21 14 12 15 14 19 26 13 28 13 18 14 18 17 14 16.2 5.1 15 12 22 14 15 23 15 16 19 16 24 28 15 32 15 23 19 20 19 18 19.0 5.6 10 11 19 11 15 11 16 8 11 12 15 13 12 23 14 19 10 NA 13 15 13.7 4.0 9 12 19 12 16 12 17 11 13 12 16 15 11 25 12 25 13 NA 11 11 14.4 4.7 32 31 38 35 44 53 57 40 18 45 37 33 58 35 41 102 48 22 73 66 48.2 18.6 16 11 30 10 30 45 40 32 12 22 19 NA† 30 25 17 68 15 18 40 43 27.4 16.3 16 20 8 25 14 8 17 8 6 23 18 33 28 10 24 34 33 4 33 23 20.8 9.9 1.31 0.86 0.91 0.88 1.07 0.86 0.75 0.92 1.00 0.96 0.88 1.08 0.97 0.93 0.79 0.99 0.98 NA 0.94 1.00 1.00 0.10 26 37 44 40 45 70 80 50 50 46 51 51 62 55 47 109 48 40 70 77 52.1 19.4 34 32 40 35 48 60 60 46 50 44 45 55 60 51 37 108 47 NA‡ 66 77 52.5 19.3

End Systole End Diastole End Systole End Diastole RPA QP/QS* Day of Surgery Patient No./ Age (y)

Body Surface Area (m2)

PA

AA

Flow Rate (mL/sec)

LPA

RPA 1 LPA

End Systole

PA

End Systole

End Diastole

LPA RPA Diameter (mm) AA

End Diastole

PA 468 • Radiology • February 2000

Patient Data and Flow, Diameter, and Pressure Gradient Measurements in 20 Children

with transposition of the great arteries. Subcostal imaging provides a flexible acoustic window that allows wide angulation and rotation of the transducer beam to optimize simultaneous demonstration of the great arteries (ascending aorta [AA] and main pulmonary artery [PA] with its primary branches) and their respective ventricular connections in the neonate (11). The growth of the child complicates postoperative assessment, however, because it becomes more difficult to find an adequate acoustic window owing to the typical retrosternal location of the pulmonary trunk and its primary branches after the Lecompte maneuver (Fig 1). In such cases, transesophageal echocardiography may by helpful (12–14). Systematic studies of the value of transesophageal echocardiography in such patients have not been performed, to our knowledge, and were not performed in the present study. The aim of the present study was to evaluate whether magnetic resonance (MR) imaging, especially phase-shift velocity mapping and cine MR imaging, could provide information for the quantitative evaluation of stenosis in addition to anatomic information. Further, we investigated whether morphologic and/or hemodynamic changes after an arterial switch operation would provide some indication of the cause of stenosis in

0.6 0.7 1.0 0.5 0.9 1.0 1.0 0.5 0.5 0.7 1.0 1.0 0.6 0.9 0.5 1.6 1.1 0.5 1.0 0.8 0.8 0.3

MR Imaging (n 5 19) Doppler Echocardiography (n 5 16) Catheterization (n 5 14)

Pressure Gradient (mm Hg)

Figure 1. Three-dimensional reconstruction (right lateral view) of a two-dimensional electrocardiographically triggered in-flow MR angiogram (11/5.7; section thickness, 4 mm with 2-mm overlap; matrix, 128 3 256) shows the typical nonphysiologic anatomic arrangement of the AA (AAO) surrounded by the pulmonary branches after the arterial switch operation performed with the Lecompte maneuver. This situation causes changes in hemodynamics in the right pulmonary artery (RPA) and the left pulmonary artery (LPA). No stenoses of the AA, PA, RPA, and LPA were found in this patient. DAO 5 descending aorta.

Gutberlet et al

means of oral administration of chloral hydrate (60 mg/kg).

Imaging

a.

b. Figure 2. (a) Transverse T1-weighted turbo spin-echo MR image (706/11 [effective]) of the pulmonary bifurcation obtained during diastole shows occlusion of the LPA (*) and highgrade stenosis of the RPA (arrow) in an 8-yearold boy after an arterial switch operation with the Lecompte maneuver. (b) Corresponding transverse modulus image derived from flowsensitive gradient-echo MR image (20/2.4; flip angle, 30°) obtained during systole shows occlusion of the LPA (*) and stenosis of the RPA (arrow). (c) Graph shows results of quantitative MR imaging flow measurements obtained during the cardiac cycle. The peak flow velocity was 360 cm/sec. The estimated pressure gradient was about 52 mm Hg, which correlated well with the pressure gradient of 45 mm Hg measured at cardiac catheterization.

c.

the pulmonary trunk and its primary branches that may affect the long-term outcome for these patients.

MATERIALS AND METHODS Patients Twenty consecutive patients (13 boys, seven girls) aged 2–17 years (mean age 6 SD, 6.6 years 6 3.9) were examined. The mean age at surgery (18 patients) had been 8 days 6 3.6. In two patients, a secondary arterial switch procedure had been performed at age 6 months. The examinations were performed at a mean of 6 years after surgery (Table). All patients had undergone preoperative cardiac catheterization, balloon atrial septostomy, and alprostadil infusion before the primary arterial switch procedure. The Volume 214 • Number 2

Lecompte maneuver (15,16), which causes displacement of the anterior aorta to a position posterior to the bifurcation of the pulmonary artery (Fig 1), had been performed in all 20 patients. In one patient, the pulmonary arterial connection was established by means of an interposed prosthetic tubular conduit. Doppler transthoracic echocardiography (TTE) and electrocardiography were performed before MR imaging in all patients. In fourteen patients, cardiac catheterization was performed to evaluate the coronary anatomy or to investigate a possible valvular or supravalvular pulmonary stenosis. All patients had an insufficient acoustic window at Doppler TTE, which was an indication for the use of MR imaging. Our institutional ethics committee approved the use of MR imaging for flow measurement and ventriculometry in patients with congenital heart disease. Informed consent was obtained from the parents of each patient prior to MR imaging. Before MR imaging, sedation for about 45 minutes was instituted in nine patients by

Doppler TTE was performed (T.B., M.V.) first. An ultrasonographic (US) unit (128 XP; Acuson, Mountain View, Calif) with a 3.5- or 5.0-MHz transducer was used. MR imaging was then performed (M.G., H.O.) by using a 1.5-T unit (Gyroscan ACS-NT; Philips Medical Systems, Best, the Netherlands). A body or surface coil was used, depending on the chest measurement and heart axis of the patient. For the anatomic evaluation, electrocardiographically gated turbo spin-echo MR images (706/11 [repetition time msec/effective echo time msec]; echo train length of five; section thickness, 3–5 mm; matrix, 256 3 256; trigger delay, 460 msec) were obtained during diastole in the transverse, sagittal, and coronal planes. In one patient (patient 10), a two-dimensional electrocardiographically triggered in-flow MR angiogram (11/5.7 [repetition time msec/echo time msec]; section thickness, 4 mm with 2-mm overlap; matrix, 128 3 256) was obtained for use in threedimensional reconstruction of the typical nonphysiologic anatomic arrangement of the AA and PA after the arterial switch operation (Fig 1). Quantitative flow measurements were performed with a flowsensitive gradient-echo sequence by using phase-shift velocity mapping (20/2.4, flip-angle, 30°; section thickness, 3–6 mm; field of view, 150–300 mm; matrix, 96 3 128). Retrospective gating was used to cover the whole cardiac cycle. The temporal resolution was 30–60 msec, depending on the number of signals acquired during the cardiac cycle, which in turn was dependent on the patient’s heart rate. An in-plane measurement was obtained parallel to the course of the vessel to assess the geometry of the main flow vector (Figs 2a, 3a). Next, a through-plane flow measurement was obtained perpendicular to the course of the vessel or to the course of the jet of a stenosis or regurgitation (Figs 2b, 2c, 3b). In cases of stenosis or valvular incompetence, measurements were performed at the area of the peak velocity on the in-plane flow measurement images. In other words, we performed phase-shift velocity mapping in a manner analogous to continuous-wave and pulsed-wave Doppler echocardiography. The advantages of both Doppler methods could be combined in MR imaging: High velocities and the exact localization could be measured on phase-shift velocity maps.

MR Imaging Follow-up after Arterial Switch Operation • 469

Figure 3. (a) Transverse modulus images derived from flow-sensitive gradient-echo MR image (20/2.4; flip angle, 30°) of the pulmonary bifurcation obtained during systole. Left: In most patients after the Lecompte maneuver, the LPA courses from the pulmonary trunk at a steep angle and is not as close to the AA as is the RPA, the course of which is, in most cases, at a flat angle close to the AA. This causes a temporary, hemodynamically significant stenosis in the RPA during systole, with a reduction in diameter and an increase in peak velocity. Right: During diastole, the pulmonary bifurcation shows no substantial reduction in the diameters of either the LPA or the RPA. (b) LPA during systole. Through-plane modulus image (left) and corresponding phase-shift velocity map (right) with the region of interest (9500, circle) for peak velocity measurements show normal expansion of the vessel and the peak velocity. (c) LPA during diastole. Throughplane modulus image (left) and corresponding phase-shift velocity map (right) with the region of interest (9500, circle) show decreases in vessel diameter and peak velocity. The latter is indicated by an increase in signal intensity in the LPA relative to that in b.

Measurements were performed for the PA, AA, RPA, and LPA. In addition to peak velocity, the volume flow rate was measured in milliliters per second for all four vessels. For estimation of the pressure gradients at the site of a stenosis, the modified Bernoulli equation (17,18) was used. For volume flow rate measurements, the cross-sectional area of each vessel was manually outlined on images obtained during the whole cardiac cycle. The MR imager software was used to calculate mean volume flow rates from the mean velocities within the manually outlined cross-sectional areas (19). Furthermore, the maximum and minimum diameters of the PA, AA, RPA, and LPA during the cardiac cycle were measured on modulus images (Fig 3b) derived from the flowsensitive cine gradient-echo MR images. All in vivo measurements were performed once. The results of the flow measurements were compared with the results of cardiac catheterization and/or Doppler TTE measurements. Furthermore, the volume flow rates in the PA and AA were assessed to exclude the effects of important left-to-right or right-to-left shunting. To monitor the accuracy of the in vivo flow rate measurements, the ratio of pulmonary flow to systemic (aortic) flow was calculated. The closer this ratio was to 1, the more accurate the measurement was.

a.

b.

c.

Phantom Studies Phantom studies were performed (M.G., T.K.) with a pulsatile flow phantom 470 • Radiology • February 2000

(18,20) by using flow-sensitive gradientecho MR imaging and phase-shift veloc-

ity mapping (same sequence parameters as for imaging in patients) to evaluate the Gutberlet et al

Demonstration of Arteries

Figure 4. Box plots of pressure gradients estimated with phase-shift velocity maps and Doppler TTE as compared with invasively measured pressure gradients obtained at cardiac catheterization in the PA, LPA, and RPA in 10 patients. A tendency toward overestimation with Doppler TTE and a slight tendency toward overestimation with MR imaging are present.

accuracy of the volume flow rate measurements obtained with that MR sequence. The echocardiographic trigger was produced by means of an artificial pneumatic heart (Berlin Heart; Mediport Berlin, Germany), which was used to maintain the ‘‘circulation’’ (18,20). Variable frequencies of 60–100 beats per minute and different volume flow rates of 100–1,500 mL/min were used. Measurements were performed three times with both pulsatile and constant flow. The mean of the three measurements was used for comparison. The volume flow rate in the circulatory model was monitored by means of an integrated (18) flowmeter with a 7.5-MHz probe (model T109R; Transonic Systems, Ithaca, NY) at a constant angle of 30°.

Statistical Analyses The Wilcoxon rank sum test for unpaired data was used for comparisons. Results were considered to be significantly different for P values of less than .05. Furthermore, regression analyses were performed, and the data were presented by using box plots, regression curves, or both.

RESULTS The pressure gradient, flow velocity, flow rate, and vessel diameter results obtained with MR imaging, Doppler TTE, and cardiac catheterization are summarized in Figures 4–7. Volume 214 • Number 2

The PA, RPA, LPA, and AA were visualized at both turbo spin-echo and flowsensitive cine gradient-echo MR imaging in all patients. The PA could not be visualized at Doppler TTE in four (20%) of 20 patients, and the LPA and RPA could not be seen at TTE in 13 (65%) patients. As expected, cine MR imaging was more susceptible than turbo spin-echo MR imaging with regard to flow artifacts (21) such as a signal void due to turbulence in the area of stenosis or regurgitation and susceptibility artifacts (22). Occlusion of the LPA was found in one patient. Stenosis (.50% reduction in diameter and/or pressure gradient . 40 mm Hg) of the LPA, RPA, PA, or pulmonary valve was suspected on the basis of spin-echo MR imaging findings in seven patients and was quantitatively verified at cine gradient-echo MR imaging and flow measurements in six patients.

Flow Measurements In vivo peak velocity.—The mean peak velocity was slightly increased in the PA (2.1 m/sec 6 1.1), as compared with normal values (23). The mean peak velocity was almost within the normal range in the AA (1.2 m/sec 6 0.4). The mean peak velocity in the RPA was significantly higher than that in the LPA (1.7 m/sec 6 1.1 vs 1.0 m/sec 6 0.9; P , .05; Table). Volume flow rate in phantoms.—The volume flow rates measured in the phantom at flow-sensitive gradient-echo MR imaging showed a good correlation with in vivo volume flow rates (r 5 0.999, P , .001). The correlation of peak velocities showed a slightly weaker correlation (r 5 0.95, P , .001). An in vivo velocity greater than the ‘‘encoded velocity’’ results in aliasing, as occurs at Doppler US. An encoded velocity of up to 100% greater than the in vivo flow velocity did not result in a measurable loss of signal intensity. Therefore, the standard adjustment for the encoded velocity was 2 m/sec. In the case of aliasing or slower flow, the encoded velocity was changed as necessary. In vivo volume flow rate.—The mean volume flow rate was 52.5 mL/sec 6 19.3 in the PA and 52.1 mL/sec 6 19.4 in the AA, and the sum of the mean volume flow rates in the RPA and LPA was 48.2 mL/sec 6 18.6 (Table). These results demonstrate good internal consistency for the volume flow rate measurements. Al-

most no difference was observed in the cardiac output of about 3,000 mL/min in the PA and AA, and the mean ratio of pulmonary flow to systemic flow in 19 patients was 1.0 6 0.1. No patient had a persistent atrial or ventricular septal defect or any other form of shunt. In one patient with high-grade pulmonary valvular incompetence and a stent in the PA, there was not good agreement between the results of the volume flow rates in the AA and PA, so the results in this patient were excluded from this comparison. The mean volume flow rate in the LPA was significantly higher than that in the RPA (27.1 mL/sec 6 16.3 vs 20.8 mL/sec 6 9.9; P , .05; Fig 6).

Vessel Diameter In all patients, the maximum vessel diameters in the PA and AA were achieved during systole, and the minimum diameters were achieved during diastole, as expected. The mean maximum vessel diameter of the PA was 14.1 mm 6 4.7 (area, 156.1 mm2), and the mean minimum diameter was 13.7 mm 6 4.0 (area, 147.4 mm2). During systole, therefore, the mean increase in diameter was 3%, and the mean increase in area was 6%. The mean maximum diameter of the AA was 19.0 mm 6 5.6 (area, 283.5 mm2), and the mean minimum diameter was 16.2 mm 6 5.1 (area, 206 mm2). During systole, therefore, the mean increase in diameter was 17.3%, and the mean increase in area was 37.6%. The AA bulges into the pulmonary arteries during systole (Fig 3a). Therefore, the maximum diameter in the RPA at the region closest to the AA was achieved in all patients during diastole, and the minimum diameter was achieved during systole. In the LPA, however, the maximum diameter was achieved during systole in some patients (Fig 3b) and during diastole in others. The mean maximum diameter of the RPA was 9.0 mm 6 2.8 (area, 63.6 mm2), and the minimum diameter was 6.5 mm 6 2.3 (area, 33.2 mm2). During systole, therefore, the mean reduction in diameter was 31.4%, and the mean reduction in area was 54%. In the LPA, the mean maximum diameter was 8.8 mm 6 3.8 (area, 61 mm2), and the mean minimum diameter was 8.0 mm 6 3.9 (area, 50.3 mm2). During systole, therefore, the mean reduction in diameter was 9.1%, and the mean reduction in area was 17.6%. The difference between the systolic diameter in the RPA and that in the LPA was not significant (P 5 .18) (Fig 7).


a.

b. Figure 5. Regression lines for pressure gradients measured in the PA, LPA, and RPA. (a) Gradients in 11 patients as estimated with Doppler TTE show a good correlation with invasively measured gradients obtained at cardiac catheterization. The equation for the regression line is y 5 21.016 1 0.43x (r2 5 0.56). (b) Gradients in 15 patients as estimated with Doppler TTE and MR imaging show a good correlation. The equation for the regression line is y 5 13.053 1 0.632x (r2 5 0.69). (c) Gradients in 13 patients as estimated with MR imaging phase-shift velocity mapping show the best correlation with invasively measured gradients obtained at cardiac catheterization. The equation for the regression line is y 5 1.618 1 1.074x (r2 5 0.81). (Only 12 measurements are indicated in the graph owing to overlapping data.)

c.

Stenosis and Valvular Incompetence Morphology.—In the AA and the descending aorta, no vessel or valvular stenosis could be detected on turbo spinecho or flow-sensitive cine gradient-echo MR images in any patient. This was confirmed in all patients at Doppler TTE, cardiac catheterization, or both, for the aortic valve and the AA, which was visualized at TTE in all patients. The Doppler TTE and cardiac catheterization criterion for no vessel or valvular stenosis in the AA or descending aorta was a peak flow 472 • Radiology • February 2000

rate of less than 1.6 m/sec or a peak pressure gradient of less than 10 mm Hg. None of the patients examined had a ‘‘neocoarctation’’ such as has been described by others (23) after use of the Lecompte maneuver (in which the anterior aspect of the AA manifests posterior displacement caused by the stretched, anteriorly placed PA and bifurcation). Pressure gradients.—Pressure gradients were calculated from MR images only for the PA, LPA, and RPA and were compared with gradients estimated with Doppler TTE or measured at cardiac catheteriza-

tion, if available. The pressure gradients calculated with results from phase-shift velocity mapping showed a good correlation with the invasive (catheterization) data, which were available for 14 patients. In one patient with a severe stenosis of the pulmonary valve and an additional stenosis of the LPA, quantification of the pressure gradient with phase-shift velocity mapping was impossible, owing to the presence of turbulence; therefore, results in this patient were excluded from the comparison (Fig 5c). Cardiac catheterization revealed a pressure gradient of 115 mm Hg at the pulmonary valve, but Doppler TTE resulted in underestimation of the gradient (Table). Despite the inability to quantify, a high-grade stenosis was suspected on the basis of findings from turbo spin-echo MR imaging and the decreased signal intensity in the area of the pulmonary valve and the LPA on the modulus image derived from flow-sensitive gradient-echo MR images. Pressure gradients estimated at Doppler TTE in 11 patients also showed a good Gutberlet et al

strated at all at echocardiography (18,27), owing to the changed anatomy after an arterial switch operation.

Flow Measurements

Figure 6. A, Box plots show comparison of volume flow rate measurements in the RPA with those in the LPA as determined with MR images in 19 patients. The flow rate in the RPA was significantly lower than that in the LPA. B, Box plots show comparison of peak flow velocity (Vmax) in the RPA with that in the LPA as measured with phase-shift velocity maps. The peak flow velocity in the RPA was significantly higher than that in the LPA.

Figure 7. A, Box plots show comparison of minimum vessel diameter in the RPA with minimum vessel diameter in the LPA during systole in 19 patients. The mean diameter in the RPA was smaller than that in the LPA, but this difference was not significant (P 5 .18). B, Box plots show comparison of maximum vessel diameter in the RPA with maximum vessel diameter in the LPA during diastole in 19 patients. The mean diameter during diastole in the RPA was smaller than that in the LPA, but this difference was not significant (P 5 .59).

correlation (r 5 0.75) with those measured at cardiac catheterization (Fig 5a), but the tendency toward overestimation of pressure gradients with TTE was greater than that with MR imaging. In three patients in whom complete catheterization and MR imaging data were obtained, we could not use Doppler TTE to quantify a gradient in the PA owing to an inadequate acoustic window. Therefore, complete data for comparison among the three modalities were available in 10 patients (Fig 4). Volume 214 • Number 2

DISCUSSION Visualization In agreement with the results in other reports (24–27), our data demonstrated the superiority of MR imaging over Doppler TTE for assessment of the anatomy in patients after complete correction of transposition of the great arteries, in particular for assessment of a stenosis in the PA and its primary branches. This is of particular importance because this area often is poorly demonstrated or is not demon-

Peak velocity.—Simple depiction of anatomic structures during a single phase of the cardiac cycle often is not sufficient for evaluation of the complex dynamics involved. In addition to demonstration of a stenosis, therefore, the hemodynamics of the stenosis must be evaluated by means of flow measurements. As in other studies (18,27), a good correlation between MR imaging data and cardiac catheterization data was obtained in patients with a mild or moderate stenosis (Fig 5c). Use of the modified Bernoulli equation provides an estimate of the instantaneous gradient, which differs from that measured at cardiac catheterization. This difference increases with the severity of stenosis (28–30). In the present study, however, estimation with the Bernoulli equation was shown to be adequate for clinical practice. The criteria for further invasive diagnostic procedures and/or repeated surgery at our institution are a pressure gradient of more than 50 mm Hg, even in the absence of clinically detectable symptoms, or a gradient of more than 40 mm Hg in the presence of symptoms and/or changes in ventricular function or morphology. No further invasive diagnostic procedure is necessary in patients with a gradient of less than 40 mm Hg. In all patients, we were able to distinguish among categories by using MR imaging. Volume flow rate in phantoms.—Our phantom study results demonstrated the accuracy of the flow-sensitive gradientecho MR sequence with phase-shift velocity mapping for flow quantification. The influence of breathing on the accuracy of flow measurements has previously been described (31) and was not evaluated in the present study. The influence of pulsatile or continuous flow was investigated, and there was no significant difference between the two. In vivo volume flow rate.—Mild or moderate dynamic obstruction of the LPA and RPA is caused by bulging during systole of the neoaorta into the pulmonary arterial bifurcation. This bulging is due to the special nonphysiologic anatomic situation of the pulmonary arterial branches surrounding the AA after performance of the Lecompte maneuver (Fig 1) (15,16). This situation causes changes in the hemodynamics in the RPA and LPA, whereas the larger vessels (the PA and AA) are less affected and show only a slight increase


in mean peak velocity. In the more affected RPA, we found a significantly elevated peak velocity caused by dynamic or temporary obstruction, whereas the mean volume flow rate was significantly less in the RPA than in the LPA (Fig 6). Volume flow measurements were obtained not only in the LPA and RPA but also in the PA and AA to serve as internal standards for the in vivo flow measurements. No significant differences between these arteries were found, which indicated the internal consistency of the in vivo measurements. There was only one patient who had high-grade incompetence of the pulmonary valve: The ratio of pulmonary flow to systemic flow in this patient was substantially greater than 1.0 (Table); this high ratio was due to turbulence, which prevented adequate measurements of flow rates. In all other patients, the results showed a good agreement, with a pulmonary flow–to–systemic flow ratio of 1.0 6 1.0.

Diameter Measurements Changes in vessel diameter during the cardiac cycle were examined. By using cine gradient-echo MR imaging, we found a reduction in vessel diameter and vessel area during systole in the RPA at the site of greatest contact with the AA (Fig 3a), whereas in the LPA some patients had a decrease in diameter and others had an increase, as is normal for the vessel (Fig 3b). In all patients, expansion of the AA during systole was the reason for this transitory stenosis. In most of the patients, the great arteries were not positioned one behind the other. The neo-PA was usually positioned somewhat to the side of the neoaorta (32). In most patients who have undergone the Lecompte maneuver, therefore, the course of the LPA from the PA is at a steep angle and is less close to the AA (33), as compared with the course of the RPA, which, in most cases, is at a flat angle close to the AA. This caused the RPA to be more affected by the expanding AA during systole, resulting in a transitory stenosis of the RPA. As a result, an increase in the mean peak velocity and a decrease in the volume flow rate in the RPA were observed. The complex dynamics of the AA, LPA, and RPA in this particular anatomic situation could not be assessed at cardiac catheterization. Cardiac catheterization usually cannot simultaneously demonstrate the AA and PA. Simultaneous depiction of the right and left ventricular systems is one of the advantages of cine MR imaging. Cine 474 • Radiology • February 2000

gradient-echo MR imaging in the present study demonstrated that the proximity of the right ventricular outflow tract, PA, RPA, and LPA to the high-pressure left ventricular system may be one reason for the development of stenosis in patients such as those in our study. Depending on the particular anatomic situation present before surgery, the PA, RPA, or LPA could be affected. Paul and Wernovsky (34) reported that the LPA is more often stenosed, but our results, like those of other investigators (15,25), do not support this claim. The only occlusion observed in the present study was in the LPA, 0.5 cm distal to the PA bifurcation (Fig 2).

Clinical Importance The mechanism for obstruction of pulmonary blood flow is multifactorial. Mild to moderate obstruction of the LPA and RPA immediately distal to the bifurcation may be caused by systolic bulging of the AA into the bifurcation, which, in addition to kinking of the RPA and/or LPA due to cranial displacement of the branches, may lead to a permanent obstruction. Other mechanisms of permanent supravalvular obstruction of the pulmonary outflow tract include inadequate growth of the pulmonary arteries during somatic growth (35,36), circumferential narrowing at the suture line, or a combination of anatomic mechanisms, resulting in multiple obstructions at various levels. The different lesions respond to various treatments, including balloon angioplasty, which can successfully help relieve discrete branch stenosis or circumferential narrowing in the PA. It is clinically important to determine prospectively which patients may be candidates for an attempt at nonsurgical treatment of an obstruction. MR imaging in concert with phase-shift velocity mapping may be the noninvasive method of choice to help select patients for balloon angioplasty and for follow-up after the intervention. Under normal circumstances, slightly more than 50% of the cardiac output flows through the RPA (37–40). Therefore, a marked change in the distribution of blood flow may be of clinical importance and could influence the long-term outcome in patients. Thus, progressive reduction in blood flow in the RPA, as well as in the LPA, will necessitate a second surgical or interventional procedure.

Limitations of the Present Study The small sample size of the study group and the wide range of patient ages

and sizes may have been among the major limitations of this study. Another limitation was the fact that cardiac catheterization data were available for only 14 of 20 patients. Although the arterial switch operation has been in use for more than 2 decades, there still is not a large population being followed up and who are, therefore, possible candidates for MR imaging. Despite the small sample size and heterogeneity of the group in our study, however, MR imaging demonstrated temporary obstructions in pulmonary arterial branches that caused important changes in the pulmonary blood distribution after the arterial switch operation in almost all patients; such changes can go undetected with other imaging modalities, including cardiac catheterization. A temporary or systolic obstruction may affect the long-term outcome in these patients and should be studied further, because such an obstruction may progress and finally lead to right ventricular overload, which must be treated with repeated surgery. Although such stenoses are not permanent, they are, nonetheless, hemodynamically significant because they occur during systole. The accuracy of flow measurements can be affected by breathing. This effect was not evaluated in our study, but it has been examined by other investigators. In one study (31), MR imaging resulted in overestimation of actual flow during systole and underestimation during diastole. The errors in estimation decrease with measurements obtained during increasing numbers of phases of the cardiac cycle. Peak flow rate was more affected than was volume flow rate. Breathing should not have substantially altered our findings, because the origin of the pulmonary arteries is not much affected by thoracic movement during respiration. Furthermore, the RPA and LPA are affected by this respiratory motion in the same way. Therefore, the significant difference in blood distribution of the mean volume flow rates between the RPA and the LPA should not have been altered. In addition, we acquired measurements during at least 12 phases per cardiac cycle; in the aforementioned experimental study (31), measurements were obtained during one to six phases. In general, there is a tendency toward overestimation of pressure gradients with phase-shift velocity mapping (18,19), similar to the results with Doppler TTE, especially in the setting of severe stenosis. This may not be clinically relevant for decision making with regard to intervention. Finally, noninvasive MR imaging Gutberlet et al

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