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Abstract—Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete
transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation)
performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure
and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node
dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial
arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch
procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the
pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected
transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological
RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the
tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block
occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for
a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration
of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All
patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and
hemodynamic assessment of adult congenital heart disease is available. (Circulation. 2006;114:2699-2709.)
Key Words: transposition of great vessels 䡲 heart defects, congenital 䡲 pediatrics 䡲 cardiovascular diseases
(d-transposition) have survived because of prior radical repair portion of the right ventricle is to the right of the morpho-
and are seen increasingly in cardiology practice. Residua and logical left ventricle.
sequelae are common, and morbidity and mortality are L-loop: Refers to a leftward (levo⫽L) loop or bend of the
ongoing. In contrast, although some patients with congeni- embryonic cardiac tube resulting in the inflow portion of the
tally corrected transposition (C-TGA) have had surgery in morphological right ventricle being to the left of the morpho-
childhood, others may present for the first time in adulthood, logical left ventricle.
and the diagnosis is often overlooked. This review will focus Cardiac chambers: Morphological right and left ventri-
on the presentation, evaluation, and management of these 2 cles refer to the anatomic characteristics of the chambers
complex lesions. and not their positions (eg, the morphological right ven-
tricle is on the left in congenitally corrected transposition).
Glossary of Terms Congenitally corrected transposition: Atrioventricular dis-
cordance and ventriculoarterial discordance. The right atrium
enters the left ventricle, which gives rise to the pulmonary
Atrioventricular discordance: Inappropriate connections of artery, and the left atrium enters the right ventricle, which
the morphological right atrium to the morphological left gives rise to the aorta. Thus, the circulation continues in the
ventricle and morphological left atrium to right ventricle. appropriate direction but flows through the “wrong”
Ventriculoarterial discordance: The pulmonary artery ventricles.
arises from a morphological left ventricle, and the aorta arises
from a morphological right ventricle. Complete Transposition of the Great Arteries
Transposition of the great arteries: Refers to ventriculoar- In this anomaly, the aorta arises from the morphological right
terial discordance. The aorta arises from a morphological ventricle (RV), and the pulmonary artery arises from the
right ventricle, and the pulmonary artery arises from a morphological left ventricle (LV) (ie, there is ventriculoarte-
morphological left ventricle. rial discordance). Complete transposition of the great arteries
From the Division of Cardiovascular Diseases and Department of Internal Medicine, Mayo Clinic, Rochester, Minn.
Correspondence to Carole A. Warnes, MD, FRCP, Mayo Clinic, Gonda 5-368, 200 First St SW, Rochester, MN 55905. E-mail
warnes.carole@mayo.edu
© 2006 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org DOI: 10.1161/CIRCULATIONAHA.105.592352
2699
2700 Circulation December 12, 2006
the absence of severe ventricular dysfunction. function as a systemic pump, and therefore the pulmonary
artery must be banded to facilitate hypertrophy of the LV. If
Systemic Ventricular Dysfunction and successful, the atrial baffle may subsequently be taken down
Tricuspid Regurgitation and an arterial switch operation performed.28,29 This approach
Late RV dysfunction is a recognized outcome after the has been successful in some young patients but is more
Mustard or Senning procedures because the RV is still the problematic in adults.30 In addition to the technical challenges
systemic ventricle.10,17 Tricuspid regurgitation often coexists, of the extensive surgery, the LV appears less able to respond
and both lesions increase the propensity for atrial arrhyth- to the increased afterload, and ventricular failure occurs after
mias. Mild to moderate tricuspid regurgitation is very com- the pulmonary banding. In a series of 35 patients with
mon in adult survivors and tends to progressively worsen. pulmonary banding, 10 failed retraining, and this was more
Occasionally the tricuspid valve apparatus may be intrinsi- common in patients aged ⬎12 years.31 Whether a slower,
cally abnormal or may have been damaged at the time of prior more physiological banding process might be more effective
VSD repair or by endocarditis. In this circumstance, tricuspid in retraining the LV in adults remains to be seen.
valve replacement may be warranted, but in most cases the Because pulmonary banding increases the LV pressure, a
regurgitation is secondary to annular dilatation from RV rightward “septal shift” may occur with improved coaptation
failure, and tricuspid valve replacement is not helpful. of the systemic AV valve and reduction in systemic AV valve
The treatment of systemic ventricular dysfunction is chal- regurgitation. Some authors have advocated pulmonary band-
lenging. Although it is tempting to extrapolate the use of ing as a therapy for the failing RV in its own right, but
angiotensin-converting enzyme inhibitors and -blockers whether this is an effective palliation has yet to be deter-
from trials of acquired heart disease to patients after an atrial mined.32 In many centers, these surgical approaches have
switch procedure, convincing data that they increase exercise been abandoned in adults in favor of cardiac transplantation.
time, ventricular function, or survival do not exist.18 None-
theless, angiotensin-converting enzyme inhibitors are often Atrial Baffle Obstruction and Leaks
used empirically. Caution should be exercised with the use of Scarring and narrowing of the atrial baffle are infrequent late
-blockers, which might exacerbate AV block and precipitate complications but should always be investigated. Obstruction
bradycardia. of the superior vena cava is more common than that of the
Many young patients have survived to their 20s and 30s, inferior vena cava and may produce “SVC syndrome.”
and an actuarial survival of 80% at 20 years has been reported Imaging of the superior limb before the implantation of an
from a single-center experience.19 Late attrition from RV endocardial pacing system will ensure that the lead will not
failure and arrhythmias is an ongoing issue, however, that cause obstruction and possible thrombosis. This imaging can
2702 Circulation December 12, 2006
Pulmonary Hypertension
Pulmonary hypertension is a serious complication of atrial
switch repairs for d-TGA and occurs in ⬇7% of those who
survive to adulthood.37,38 The exact cause is not completely
clear, but patients appear more likely to develop pulmonary Figure 3. The Rastelli operation. The VSD is patched to tunnel
blood from the LV into the aorta (1). The pulmonary valve is
vascular disease when undergoing operation at ⬎2 years of oversewn, and a valved conduit is inserted from the RV to the
age.39 Other risk factors include having had shunts at the pulmonary artery (2) to bypass the pulmonary stenosis. Repro-
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ventricular or great artery level before repair.40 Patients with duced from Hornung et al1 with permission from Elsevier.
mild elevation of pulmonary pressures at early postoperative
catheterization appear at increased risk for developing pul- arteries above the sinuses and detachment of the coronary
monary vascular disease.37 Pulmonary hypertension may also arteries along with a “button” from the aortic wall. The
be caused by pulmonary venous baffle obstruction, which great arteries are then switched into their new position,
should always be excluded. with the pulmonary artery brought forward anterior to the
aorta and the coronary buttons sutured into the “neoaorta.”
Pregnancy Adult survivors of this procedure are only now appearing
Pregnancy may be well tolerated after an atrial switch in adult congenital heart disease clinics. Complications in-
procedure,41 but careful prepregnancy counseling and evalu- clude distortion of the RV outflow tract and pulmonary
ation, including a frank discussion about the patient’s long- arteries and dilatation of the neoaortic root with aortic
term prognosis, are critical. Attention should be paid to the regurgitation. Coronary stenoses may also occur and cause
function of the systemic RV and degree of tricuspid regurgi- sudden death or myocardial infarction.44,45 Other coronary
tation because pregnancy poses a significant volume load that stenoses may require surgical or catheter intervention.46,47
may increase RV dimensions and is sometimes irreversible.42 Lifetime follow-up is clearly warranted.
Pregnancy poses a definite risk of deterioration of functional
class, even if the patient is reportedly asymptomatic before Rastelli Procedure
pregnancy.42 In 1 series, 2 of 16 pregnant women required This operation is used when d-TGA coexists with a large
heart failure therapy during or soon after pregnancy.42 The subaortic VSD and pulmonary stenosis.48 A patch is placed to
recurrence risk of congenital heart disease in the offspring, in direct blood from the LV through the VSD to the aorta. The
the absence of a family history, is probably ⬍5%, although pulmonary valve is oversewn, and continuity is established
no large series have been reported.41 between the RV and the pulmonary artery by means of a
valved conduit (Figure 3). This operation has the advantage
Arterial Switch Procedure that the LV functions as the systemic ventricle, but conduit
This operation restores the normal anatomic arrangement of degeneration and stenosis are inevitable, necessitating reop-
the circulation and, as such, is a more attractive physiological eration with all of its attendant risks. Atrial and ventricular
long-term option. From its first description by Jatene in arrhythmias are frequent, and sudden death may occur,
1976,43 it has steadily become the procedure of choice when particularly when significant conduit obstruction exists. RV
the anatomy is appropriate and is usually performed in the and LV failure also may occur. Regular clinical and echocar-
first month of life. It involves transection of the great diographic surveillance is necessary, and the development of
Warnes Transposition of the Great Arteries 2703
detection of arrhythmias, occult coronary disease, and serial commonly subvalvular. It may result from an aneurysm of the
functional capacity. Periodic Holter monitoring allows detec- interventricular septum or may be associated with fibrous
tion of sinus node disease and atrial arrhythmias, although it tissue tags or a discrete ring of tissue in the subvalvular area.
may be of little help in identifying those at risk of sudden Associated valvar pulmonary stenosis also occurs. Patients
death.14 Most patients need endocarditis prophylaxis unless who have both a VSD and pulmonary stenosis may be
they have had an atrial switch procedure and have no residual hemodynamically well balanced or have varying degrees of
valve dysfunction or outflow tract disturbance. cyanosis.
Figure 6. Chest radiographs of a patient with C-TGA and levocardia (A) and dextrocardia (B). A, The vascular pedicle on the left, where
the usual rounded convexities of the descending aorta and pulmonary artery are seen in a normal heart, is unusually straight. In addi-
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tion, the ventricular border has a “humped” appearance and is more vertical than usual. B, The apex of the heart points to the right,
but the gastric bubble is on the left. This should always raise the suspicion of C-TGA because dextrocardia occurs in ⬇20% of cases.
The gross cardiomegaly is the result of profound heart failure secondary to severe AV valve regurgitation.
also occurs but is different from the typical right-sided Diagnostic Evaluation
Ebstein’s anomaly, the only real similarity being that the Chest Radiograph
valve is displaced inferiorly closer to the cardiac apex.51 No With mesocardia or levocardia, the diagnosis may be sus-
large “saillike” anterior leaflet exists, adherence of the septal pected from the chest radiograph. The vascular pedicle
and posterior leaflets is limited, and the atrialized portion of appears abnormally straight because the normal arterial rela-
the RV inflow is also relatively small. In addition, this tionships are lost. The ascending aorta is not visible on the
systemic tricuspid valve can rarely be surgically repaired with right side, and the convexities from the descending aortic
success. These delicate valves are inherently vulnerable to knob and pulmonary artery are absent on the left side. The
developing regurgitation, which is an additional burden for ventricular border on the left may also appear more vertical
the systemic morphological RV. Sometimes the tricuspid than usual (Figure 6A). C-TGA is one of the most common
valve may straddle the ventricular septum across a VSD, anomalies associated with dextrocardia and should be sus-
making a biventricular repair much more difficult and some- pected when there is abdominal situs solitus (gastric bubble
times impossible. Such patients may need single ventricle on the left) and dextrocardia (Figure 6B).
repair or palliation with a Fontan operation or bidirectional
Electrocardiogram
cavopulmonary anastomosis.
The ECG may be misinterpreted as inferior myocardial
Conduction System infarction. Because of the ventricular inversion, the right and
The AV node and His bundle have an unusual position and left bundles are also inverted, causing septal activation to
course, and many patients have dual AV nodes.52,53 The occur from right to left. This produces Q waves in the right
second anomalous AV node and bundle are usually anterior, precordial leads and absent Q waves in the left precordial
and the long penetrating bundle is vulnerable to fibrosis with leads (Figure 7). Varying degrees of AV block are also
advancing age. This makes the conduction system somewhat common.
tenuous, with a progressive incidence of complete AV block Two-Dimensional Echocardiography
occurring at ⬇2% per year. Tricuspid valve or VSD surgery Echocardiography can be difficult for those without experi-
may also precipitate heart block. ence of congenital heart disease, particularly because dextro-
Warnes Transposition of the Great Arteries 2705
view may show the abnormal arterial relationships, with the therefore the morphological RV is perfused by a single right
aorta usually anterior and to the left of the pulmonary artery. coronary artery. In such a situation, there may be limitations
Cardiac Catheterization of myocardial perfusion and a mismatch between oxygen
Cardiac catheterization permits evaluation of systemic AV supply and demand. In a study of 20 patients in which
valve regurgitation and systemic ventricular function.54 A exercise sestamibi imaging was used, all 20 had perfusion
hemodynamic assessment of associated anomalies can be defects at rest, and in 17 these defects worsened with
performed, including measurement of left and right heart exercise.60 In a study in which positron emission tomography
pressures and pulmonary resistance. In older patients, coro- imaging was used, myocardial blood flow in the systemic
nary angiography is necessary before any operative ventricle was normal at rest, but the hyperemia resulting from
intervention. adenosine-induced vasodilatation was significantly less than
in the controls, suggesting that coronary flow reserve is
MRI/Radionuclide Imaging greatly attenuated.61
MRI may help to define ventricular function and volumes,55
A retrospective multi-institutional study clearly demon-
although it requires some expertise in imaging congenital
strated an increasing incidence of systemic ventricular dys-
heart disease. Because some patients have cardiac pacemak-
function and clinical congestive heart failure with advancing
ers, it is not always a feasible imaging modality. Radionu-
age.62 Even in patients with C-TGA and no significant
clide angiography may also be used.56
associated lesions, more than one third had congestive heart
Natural History/Long-Term Sequelae failure by the fifth decade. In patients with significant
The minority of patients may be relatively normal from a associated defects and prior open heart surgery, two thirds of
functional standpoint, and survival to the seventh and eighth patients had congestive heart failure by the age of 45 years.62
decades has been reported when no associated anomalies In addition to the morphological RV’s inherent vulnerabil-
exist.57–59 Failure of the systemic ventricle is much more ity to failure, it has a complex relationship with systemic AV
common earlier in life, usually with concomitant tricuspid valve regurgitation, and controversy exists as to which is the
regurgitation. “chicken” and which is the “egg.” In general, however, it
appears that primary RV failure, while uncommon, is a
Systemic Ventricular Failure and AV frequent sequel to systemic AV valve regurgitation.63 Thus,
Valve Regurgitation when systemic ventricular function deteriorates, AV valve
There has been much debate about the cause of systemic regurgitation should be considered the explanation until proof
ventricular failure. The coronary anatomy is concordant, and of a different explanation can be provided, and surgery should
2706 Circulation December 12, 2006
be considered early, before irreversible ventricular function with a median age at diagnosis of 1 month. Asymptomatic
ensues. patients had isolated C-TGA, a small VSD, or mild pulmo-
Late referral is common. In the Mayo Clinic series of 44 nary stenosis, or they were hemodynamically well balanced
patients aged 20 to 79 years with no prior cardiac surgery, 26 with a VSD and pulmonary stenosis. In contrast, patients with
patients (59%) were referred with significant (⬎3/4) AV heart failure had a large VSD, a VSD with mild pulmonary
valve regurgitation.49 Of 30 patients who needed systemic stenosis, or a regurgitant systemic AV valve. Those with
AV valve replacement, 16 were referred late with clinical cyanosis had a VSD and either pulmonary stenosis or
ventricular dysfunction ⬎6 months. The mean preoperative pulmonary atresia. In a median follow-up of 9.3 years, the 5-,
ejection fraction was 39%, which would be considered 10-, and 20-year survival rates were 92%, 91%, and 75%,
unacceptably late in patients with normal ventricular anatomy respectively. There were 20 deaths (16.5%) at a median age
and mitral regurgitation. Although there was no early mor- of 13.2 years and 5 cardiac transplants (4%). Surgery was
tality, 4 patients ultimately needed cardiac transplantation for performed in 86 patients with an operative mortality of 2.5%.
poor ventricular function, and the only marker for poor Biventricular repair was performed in 47 patients and varied
survival was a poor preoperative ejection fraction.49 according to the underlying cardiac anatomy. Risk factors for
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Because complete heart block is common, it should be mortality included age at biventricular repair, moderate or
noted that implantation of an endocardial pacemaker may severe systemic AV valve regurgitation, and poor RV func-
also precipitate deterioration in systemic ventricular function tion. In this study, as in others, most patients undergoing
and worsening of AV valve regurgitation. This procedure surgical repair had worsening systemic AV valve regurgita-
presumably alters the position of the ventricular septum, tion to a moderate or severe degree. Implicated factors
inducing “septal shift” and failure of tricuspid valve coapta- include the “insult” of cardiopulmonary bypass leading to
tion64 (Figure 9). annular dilatation,66 annular distortion caused by VSD clo-
sure,67 changes in the position of the ventricular septum, and
Surgical Repair
annular dilatation after relief of pulmonary stenosis.68,69 In
Systemic AV valve replacement is relatively common in the
adult age group. Surgery can be accomplished with accept- addition, poor tolerance of the systemic AV valve to systemic
able perioperative mortality in experienced centers. The pressure after VSD closure is possible because afterload is
outcomes of 40 patients aged 5 months to 70 years who increased, and postoperative complete AV block may com-
underwent systemic AV valve replacement at the Mayo pound the adverse hemodynamics.59,69 –71 The observation of
Clinic between 1964 and 1993 were reported by van Son.59 progressive systemic AV valve regurgitation after operative
Of these 40 patients, 36 had severe regurgitation, and 29 intervention has prompted some authors to recommend sys-
(72.5%) had associated cardiac anomalies. The preoperative temic AV valve replacement at the time of intracardiac repair
ejection fraction ranged from 20% to 60% (mean, 48%). The if the systemic AV valve regurgitation is more than mild at
in-hospital mortality in this high-risk group was 10%, but the time of surgery.66
with follow-up to 26 years (mean, 4.7), another 8 patients Hraska et al72 recently reported the outcomes of 123
died. The survival was 78% at 5 years and 61% at 10 years. patients with C-TGA who underwent a variety of surgical
The cause of death in all 12 patients in this series was procedures. The 10-year survival was only 67%, and systemic
systemic ventricular failure, an outcome which emphasizes ventricular dysfunction occurred in 44% of patients. Ventric-
the need for early operation before the development of ular dysfunction was again closely linked to systemic AV
ventricular dysfunction. Functional status improved in the 28 valve regurgitation, particularly when Ebstein’s anomaly of
survivors, with 27 patients in New York Heart Association the systemic AV valve was present. The authors also specu-
class I or II. Survivorship correlated with a preoperative lated that the change in RV geometry precipitated more
ejection fraction ⬎44%. tricuspid regurgitation, which in turn led to more annular
Rutledge et al65 reported a pediatric series of 121 patients dilatation and then more tricuspid regurgitation. They too
seen at Texas Children’s Hospital between 1952 and 1999 were disappointed with the results of classic biventricular
Warnes Transposition of the Great Arteries 2707
Pregnancy
Successful pregnancy can be achieved in most women with
Figure 10. “Double-switch” operation for C-TGA using the Mus-
C-TGA, but careful prepregnancy evaluation is mandatory.77
tard atrial baffle technique and arterial switch procedure. The A comprehensive cardiovascular examination should include
VSD has been closed with a patch. Venous blood from the an assessment of cardiac rhythm, ventricular function, AV
superior and inferior vena cava (SVC, IVC) is directed to the RV valve regurgitation, associated lesions, and postoperative
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of subtle deterioration in exercise capacity in patients who 19. Wilson NJ, Clarkson PM, Barratt-Boyes BG, Calder AL, Whitlock RM,
may believe that they are asymptomatic and permits exercise Easthope RN, Neutze JM. Long-term outcome after the Mustard repair
for simple transposition of the great arteries: 28-year follow-up. J Am Coll
guidelines to be prescribed. Symptoms or signs of arrhythmia Cardiol. 1998;32:758 –765.
warrant detailed investigation and a review of underlying 20. Roos-Hesselink JW. Decline in ventricular function and clinical condition
hemodynamic problems. Most patients require endocarditis after Mustard repair for transposition of the great arteries (a prospective
study of 22–29 years). Eur Heart J. 2004;25:1264 –1270.
prophylaxis unless they have no valvular dysfunction, out- 21. Salehian O, Schwerzmann M, Merchant N, Webb GD, Siu SC, Therrien
flow obstruction, or VSD. J. Assessment of systemic right ventricular function in patients with
transposition of the great arteries using the myocardial performance
Disclosures index: comparison with cardiac magnetic resonance imaging. Circulation.
2004;110:3229 –3233.
None. 22. Vogel M, Derrick G, White PA, Cullen S, Aichner H, Deanfield J,
Redington AN. Systemic ventricular function in patients with transpo-
References sition of the great arteries after atrial repair: a tissue Doppler and con-
1. Hornung TS, Derrick GP, Deanfield JE, Redington AN. Transposition ductance catheter study. J Am Coll Cardiol. 2004;43:100 –106.
complexes in the adult: a changing perspective. Cardiol Clin. 2002;20: 23. Hurwitz RA, Caldwell RL, Girod DA, Mahony L, Brown J, King H.
405– 420. Ventricular function in transposition of the great arteries: evaluation by
2. Levin DL, Paul MH, Muster AJ, Newfeld EA, Waldman JD. radionuclide angiocardiography. Am Heart J. 1985;110:600 – 605.
d-Transposition of the great vessels in the neonate: a clinical diagnosis. 24. Redington AN, Rigby ML, Oldershaw P, Gibson DG, Shinebourne EA.
Arch Intern Med. 1977;137:1421–1425. Right ventricular function 10 years after the Mustard operation for trans-
3. Rashkind WJ, Miller WW. Creation of an atrial septal defect without position of the great arteries: analysis of size, shape, and wall motion. Br
thoracotomy: a palliative approach to complete transposition of the great Heart J. 1989;62:455– 461.
arteries. JAMA. 1966;196:991–992. 25. Rees S, Somerville J, Warnes C, Underwood R, Firmin D, Klipstein R,
4. Senning A. Surgical correction of transposition of the great arteries. Longmore D. Comparison of magnetic resonance imaging with echocar-
Surgery. 1959;45:966 –980. diography and radionuclide angiography in assessing cardiac function and
5. Mustard WT. Successful two-stage correction of transposition of the great anatomy following Mustard’s operation for transposition of the great
vessels. Surgery. 1964;55:469 – 472. arteries. Am J Cardiol. 1988;61:1316 –1322.
6. Blalock A, Hanlon CR. Surgical treatment of complete transposition of 26. Paul MH, Wessel HU. Exercise studies in patients with transposition of
aorta and pulmonary artery. Surg Gynecol Obstet. 1950;90:1–15. the great arteries after atrial repair operations (Mustard/Senning): a
7. Duster MC, Bink-Boelkens MT, Wampler D, Gillette PC, McNamara review. Pediatr Cardiol. 1999;20:49 –55; discussion 56.
DG, Cooley DA. Long-term follow-up of dysrhythmias following the 27. Derrick GP, Narang I, White PA, Kelleher A, Bush A, Penny DJ,
Mustard procedure. Am Heart J. 1985;109:1323–1326. Redington AN. Failure of stroke volume augmentation during exercise
8. Hayes CJ, Gersony WM. Arrhythmias after the Mustard operation for and dobutamine stress is unrelated to load-independent indexes of right
transposition of the great arteries: a long-term study. J Am Coll Cardiol. ventricular performance after the Mustard operation. Circulation. 2000;
102(suppl 3):III-154 –III-159.
1986;7:133–137.
28. Cochrane AD, Karl TR, Mee RB. Staged conversion to arterial switch for
Downloaded from http://ahajournals.org by on May 1, 2019
39. Clarkson PM, Neutze JM, Wardill JC, Barratt-Boyes BG. The pulmonary replacement in corrected transposition. J Thorac Cardiovasc Surg. 1995;
vascular bed in patients with complete transposition of the great arteries. 109:642– 652; discussion 652– 653.
Circulation. 1976;53:539 –543. 60. Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Celermajer DS,
40. Newfeld EA, Paul MM, Muster AJ, Idriss FS. Pulmonary vascular disease Hawker RE. Myocardial perfusion defects and associated systemic ven-
in complete transposition of the great arteries: a study of 200 patients. tricular dysfunction in congenitally corrected transposition of the great
Am J Cardiol. 1974;34:75– 82. arteries. Heart. 1998;80:322–326.
41. Clarkson PM, Wilson NJ, Neutze JM, North RA, Calder AL, 61. Hauser M, Bengel FM, Hager A, Kuehn A, Nekolla SG, Kaemmerer H,
Barratt-Boyes BG. Outcome of pregnancy after the Mustard operation for Schwaiger M, Hess J. Impaired myocardial blood flow and coronary flow
transposition of the great arteries with intact ventricular septum. J Am reserve of the anatomical right systemic ventricle in patients with con-
Coll Cardiol. 1994;24:190 –193. genitally corrected transposition of the great arteries. Heart. 2003;89:
42. Guedes A, Mercier LA, Leduc L, Berube L, Marcotte F, Dore A. Impact 1231–1235.
of pregnancy on the systemic right ventricle after a Mustard operation for 62. Graham TP Jr, Bernard YD, Mellen BG, Celermajer D, Baumgartner H,
transposition of the great arteries. J Am Coll Cardiol. 2004;44:433– 437. Cetta F, Connolly HM, Davidson WR, Dellborg M, Foster E, Gersony
43. Jatene AD, Fontes VF, Paulista PP, Souza LC, Neger F, Galantier M, WM, Gessner IH, Hurwitz RA, Kaemmerer H, Kugler JD, Murphy DJ,
Sousa JE. Anatomic correction of transposition of the great vessels. Noonan JA, Morris C, Perloff JK, Sanders SP, Sutherland JL. Long-term
J Thorac Cardiovasc Surg. 1976;72:364 –370. outcome in congenitally corrected transposition of the great arteries: a
44. Bonnet D, Bonhoeffer P, Piechaud JF, Aggoun Y, Sidi D, Planche C, multi-institutional study. J Am Coll Cardiol. 2000;36:255–261.
Kachaner J. Long-term fate of the coronary arteries after the arterial 63. Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM. Pro-
switch operation in newborns with transposition of the great arteries. gressive tricuspid valve disease in patients with congenitally corrected
Heart. 1996;76:274 –279. transposition of the great arteries. Circulation. 1998;98:997–1005.
45. Legendre A, Losay J, Touchot-Kone A, Serraf A, Belli E, Piot JD, 64. Warnes CA. The adult with congenital heart disease: born to be bad? J Am
Lambert V, Capderou A, Planche C. Coronary events after arterial switch Coll Cardiol. 2005;46:1– 8.
operation for transposition of the great arteries. Circulation. 2003; 65. Rutledge JM, Nihill MR, Fraser CD, Smith OE, McMahon CJ, Bezold LI.
108(suppl 1):II-186 –II-190. Outcome of 121 patients with congenitally corrected transposition of the
46. Hausdorf G, Kampmann C, Schneider M. Coronary angioplasty for great arteries. Pediatr Cardiol. 2002;23:137–145.
coronary stenosis after the arterial switch procedure. Am J Cardiol. 66. Lundstrom U, Bull C, Wyse RK, Somerville J. The natural and
1995;76:621– 623. “unnatural” history of congenitally corrected transposition. Am J Cardiol.
47. Prifti E, Bonacchi M, Luisi SV, Vanini V. Coronary revascularization 1990;65:1222–1229.
after arterial switch operation. Eur J Cardiothorac Surg. 2002;21: 67. Metcalfe J, Somerville J. Surgical repair of lesions associated with cor-
111–113. rected transposition: late results. Br Heart J. 1983;50:476 – 482.
48. Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transpo- 68. McGrath LB, Kirklin JW, Blackstone EH, Pacifico AD, Kirklin JK,
sition of the great arteries with ventricular septal defect and subpulmo- Bargeron LM Jr. Death and other events after cardiac repair in discordant
nary stenosis. J Thorac Cardiovasc Surg. 1969;58:545–552. atrioventricular connection. J Thorac Cardiovasc Surg. 1985;90:
49. Beauchesne LM, Warnes CA, Connolly HM, Ammash NM, Tajik AJ, 711–728.
Danielson GK. Outcome of the unoperated adult who presents with 69. Sano T, Riesenfeld T, Karl TR, Wilkinson JL. Intermediate-term outcome
congenitally corrected transposition of the great arteries J Am Coll after intracardiac repair of associated cardiac defects in patients with
Cardiol. 2002;40:285–290. atrioventricular and ventriculoarterial discordance. Circulation. 1995;
Downloaded from http://ahajournals.org by on May 1, 2019