Cardiovascular Outcomes After Arterial Switch TGA Boston

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D-looped transposition of the great arteries (D-TGA)accounts for 5% to 7% of congenital heart defects.1Intra-atrial baffle repair, as pioneered by Senning2and Mustard,3

radically altered the otherwise grim associated prognosis,

allowing most patients with D-TGA to thrive well into adult-

hood.4However, recognized long-term complications include

systemic right ventricular dysfunction, brady- and tachyar-

rhythmias, baffle obstructions and shunts, and sudden death.47

Clinical Perspective on p 339

In the mid 1970s, Jatene developed the arterial switchoperation (ASO),8 which soon after supplanted atrial

redirection as the preferred surgical option for D-TGA without

pulmonary stenosis. It involves translocating the pulmonary

artery and aorta above their sinuses and reimplanting the

ostia of the coronary arteries in the neoaorta. The so-called

Lecompte maneuver may be performed to place the main

pulmonary artery bifurcation anterior to the ascending aorta.9

The ASO allows the morphological left ventricle to remain

systemic and avoids multiple atrial incisions and suture

lines that predispose to arrhythmias. Midterm results appear

favorable, although identified complications include aortic

insufficiency, pulmonary stenosis, and obstructive coronary

disease.1014Follow-up data beyond a median of 5 to 10 years

remain scarce.1017We therefore sought to characterize longer-

term survival and cardiovascular outcomes in a single-center

early cohort of patients with D-TGA and ASO.

Materials and Methods

Study PopulationWe included all patients with D-TGA or a Taussig-Bing anomaly who

had an ASO at Boston Childrens Hospital between January 1983 and

December 1999 and a last known permanent contact address in the

New England area. Eligible candidates were identified by searching

BackgroundData regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries

are scarce.

Methods and ResultsA single-institution retrospective cohort study was conducted to assess cardiovascular outcomes

after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted

and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238

(59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In

perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.71.8% and 96.60.1%, respectively.

Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.52.5% remained

free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.91.9% at

25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-

15.90), P=0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P=0.0107). At

last follow-up, the left ventricular ejection fraction was 60.38.9%, 97.3% had class I symptoms, and 5.2% obstructive

coronary artery disease. Peak oxygen uptake was 35.17.6 mL/kg/min (86.115.1% predicted), with a chronotropic index


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332 Circulation January 22, 2013

institutional databases in the Departments of Cardiac Surgery andCardiology. Patients without follow-up data within 3 years were con-tacted by mail and telephone, and questioned about their health statussince last seen at our institution. For patients under 18 years of age,parents or guardians were contacted instead. When relevant, consentwas obtained via mailed medical record release forms to supplementfollow-up data with information from secondary sources (ie, fromother physician and hospital encounters). The study was conducted

in accordance with guidelines from the Committee on ClinicalInvestigation and approved by the institutional review board.

Primary anatomic subtypes were classified as D-TGA with an intactventricular septum, D-TGA with1 ventricular septal defects, or a Taussig-Bing anomaly.12The aortic arch was considered abnormal in the presenceof aortic coarctation, interruption, or hypoplasia. Atrioventricular valveswere classified as abnormally attached if portions of the mitral or tricus-pid valve were malattached, or if they were associated with positionalanomalies (ie, straddling or overriding). The coronary anatomy was cat-egorized as usual; circumflex originating from the right coronary artery(RCA); single RCA; single left coronary artery (LCA), inverted coronaryarteries, RCA originating from the left anterior descending (LAD) artery;intramural LCA; intramural LAD with circumflex from RCA; singleLCA with intramural RCA; intramural RCA, circumflex, and RCA sepa-rate from LAD; and other.18Any variant other than usual was considered a

coronary anomaly.

Clinical Variables and Follow-UpData were retrospectively collected from a detailed review of medicalrecords, preoperative echocardiographic and cardiac catheterizationdata, operative notes, and follow-up reports, including physical exam-ination, interventions, functional status, long-term complications, andnoninvasive tests (ie, echocardiography, cardiac MRI, cardiopulmo-nary exercise testing, and nuclear imaging). Data quality checks con-sisted of identifying and tracking missing, incomplete, or inconsistentinformation. Invalid formats and codes were flagged and data queriesissued to clarify and resolve discrepancies on a per-patient basis.

Surgical reintervention was defined as any cardiovascular surgerysubsequent to the ASO, including aortic arch, caval thrombosis, andcoronary bypass surgery. An arrhythmic event was defined as docu-

mented atrial flutter/intra-atrial reentrant tachycardia, atrial fibrillation,bradycardia requiring pacemaker implantation, sustained ventriculartachycardia or fibrillation, resuscitated cardiac arrest, or sudden deathof presumed arrhythmic etiology. The combined adverse cardiovas-cular event outcome consisted of an arrhythmic event, acute coronarysyndrome, heart failure-related hospitalization, cerebrovascular acci-dent, or cardiovascular death. The diagnosis of coronary arterial ob-struction required documented stenosis 50% in at least 1 coronaryartery, an acute coronary syndrome, or objective evidence of myo-cardial ischemia by exercise or perfusion imaging studies. An acutecoronary syndrome was defined by electrocardiographic changes (ie,transient ST segment elevation 1 mm, ST segment depression 1mm, new T wave inversion 1 mm, pseudonormalization of previouslyinverted T waves, new Q-waves [1/3 the height of the R wave or >0.04seconds], new R wave > S wave in lead V

1 [posterior MI], new left

bundle-branch block), with an increase in cardiac enzymes (positivetroponin I or T or CK-MB more than twice upper limit of normal). 19

Echocardiographic assessment included color, continuous wave,and pulse wave Doppler data for all valves according to establishedcriteria.20Peak Doppler gradients were presented for neoaortic andpulmonary valves, with values 25 mm Hg and 36 mm Hg consid-ered to indicate at least moderate stenosis, respectively.21The levelof neoaortic and pulmonary valve obstruction was further classifiedas subvalvular, valvular, and supravalvular. Obstruction at the levelof left and right pulmonary arteries was also assessed. Estimates ofleft ventricular ejection fractions were based on all available echo-cardiographic data, as per standard practice. Aortic dimensions weremeasured at the level of the annulus, root, sinotubular junction,and ascending aorta when at their maximum in the cardiac cycle.For parameters duplicated by cardiac magnetic resonance imaging(ie, left ventricular volumes and ejection fraction and aortic dimen-sions) within 1 year of echocardiography, cardiac magnetic resonancedata were retained. In addition, right ventricular volumes and ejectionfractions were assessed by cardiac magnetic resonance.

For cardiopulmonary exercise testing, peak oxygen consumptionwas defined as the highest value of oxygen uptake attained in thefinal 20 seconds of exercise when the respiratory exchange ratio was>1.09. Heart rate reserve was defined as peak heart rate resting heartrate and chronotropic index as (peak heart rate resting heart rate)/(220 age resting heart rate).22Chronotropic incompetence wasdefined as a failure to achieve a chronotropic index >80%.

Mode of DeathMortality ascertainment was supplemented by linking to the NationalDeath Index (www.cdc.gov/nchs/ndi.htm) and original source mate-rial was reviewed for all fatalities. Death was considered perioperativeif it occurred within 30 days of surgery or before hospital dischargeand was subclassified as early (ie, within 30 days) or late (ie, after 30days but during the same hospitalization).23Death was classified as

secondary to heart failure if it complicated worsening heart failure,as defined by evidence of 1 of the following: orthopnea, nocturnaldyspnea, pulmonary edema, increasing peripheral edema, renal hypo-perfusion (ie, worsening renal function), or radiological signs of con-gestive heart failure.24Sudden death was defined as death occurringwithin 1 hour of acute symptoms.25Death was considered secondaryto myocardial infarction if it was preceded by a clinical presentation

consistent with an acute coronary syndrome or if the diagnosis wasconfirmed by autopsy. Remaining deaths were classified as other.

Statistical AnalysisContinuous variables are summarized by meanstandard deviation ormedian and interquartile range (IQR; 25th, 75thpercentile) dependingon normality of distribution. Categorical variables are represented byfrequencies and percentages. Baseline comparisons between patientswith D-TGA and intact ventricular septum, ventricular septal defect,and Taussig-Bing anomaly were performed by 1-way analysis ofvariance (ANOVA), Kruskal-Wallis tests, or Fisher-Freeman-Halton

tests, where appropriate.Factors associated with perioperative mortality were explored in

univariate and multivariate logistic regression analyses from which

odds ratios (OR) and 95% confidence intervals (CI) were generated.For time-to-event analyses, time 0 was defined as time of ASO. Patient-time was accrued until the outcome of interest (ie, all-cause mortality,surgical or catheter-based reintervention, arrhythmic event, and adversecardiovascular event), with censoring at the time of last follow-up.No patient underwent cardiac transplantation. Event-free survival was

plotted using the KaplanMeier method, with comparisons by log-rankstatistics. After verification of proportionality assumptions, univariateand stepwise multivariate Cox proportional hazards models were usedto assess factors associated with the outcome of interest. Variablessignificant at the 0.2 level in univariate analyses were considered inthe respective stepwise (P entry=0.05; P=removal 0.10) multivariatelogistic and Cox regression models. Correlations between normallydistributed continuous variables were assessed using Pearsonscorrelation coefficient. Two-tailed probability values


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Khairy et al Arterial Switch Operation for TGA 333

136 (34%) patients, the most common variant being a circum-

flex artery originating from the RCA (n=72). Baseline charac-

teristics are summarized in Table 1.

Perioperative DeathsFigure 1 provides an overview of vital status. Perioperative deaths

occurred in 26 (6.5%) patients during a 17-year time-span. The

perioperative mortality rate decreased significantly over time,

from 15.1% (8 of 53) during the first 4 years (19831986) to

3.9% (4 of 103) during the final 4 years (19961999), P=0.0226.

The only multivariate predictors of perioperative mortality were

a Taussig-Bing anomaly (OR, 8.4; 95% CI, 2.0-35.6; P=0.0057)

and postoperative hemorrhage requiring surgical re-exploration

(OR, 10.3; 95% CI, 1.9-56.6; P=0.0073).

Later DeathsDuring a median follow-up of 18.7 (IQR 14.7, 22.5) years,

later deaths occurred in 6 of 374 (1.6%) perioperative survi-

vors, yielding a survival rate of 99.20.5% at 10 years and

96.71.8% at 25 years. Three deaths in perioperative survivors

were classified as sudden. A 15-year-old patient with supra-

systemic pulmonary pressures and a circumflex artery origi-

nating from the RCA died suddenly at rest. A 24-year-old with

a single RCA, postoperative complete heart block, severe left

ventricular dysfunction, a biventricular implantable defibril-

lator, and recurrent episodes of ventricular tachycardia with

appropriate shocks, was found in asystole. The third sudden

death occurred in a 28-day-old infant 5 days after hospital dis-

charge after ASO at 4 days of life, which was complicated by

pulmonary hypertension.

Two deaths were attributable to myocardial infarction. The

first occurred in a 6-week-old infant with inverted coronary

arteries and a postoperative course complicated by severe left

ventricular dysfunction and moderate right ventricular dys-

kinesis. Autopsy revealed a recent biventricular myocardial

infarction with occlusion of the ostial LCA and a


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recent myocardial infarction resulting from a near-complete

ostial occlusion of the single RCA. The only noncardiovascu-

lar late death occurred in a 17-year-old who was well until a

fatal motor vehicle accident.

Surgical and Catheter-Based InterventionsSurgical and catheter-based interventions after ASO are

listed in Table 2. The most common interventions were

for pulmonary artery stenosis at the site of anastomosis or

involving the main branches. Overall, 48 (12.8%) patients

had 1 cardiac surgical reintervention, with corresponding

freedom from surgical reintervention rates of 89.81.6% at 10years and 85.72.0% at 25 years of follow-up. Sixty patients

(16.0%) had catheter-based procedures (excluding diagnostic

studies), with an intervention-free survival rate of 86.81.8%

at 10 years and 82.12.3% at 25 years. Freedom from surgical

or catheter-based intervention was 81.82.0% at 10 years and

75.52.5% at 25 years. Figure 2 depicts intervention-free

survival according to whether patients had an intact ventricular

septum, ventricular septal defect, or a Taussig-Bing variant.

Factors associated with surgical or catheter interventions are

listed in Table 3. The only multivariate predictors were an

aortic arch anomaly (hazard ratio, 3.25; 95% CI, 1.69-6.27;

P=0.0004) and lower weight at the time of ASO (hazard ratio,

0.61 per kg; 95% CI, 0.41-0.91; P=0.0144).

Cardiovascular OutcomesOver the course of follow-up, 21 (5.6%) postoperative sur-

vivors had the following cardiac events: atrial flutter/intra-

atrial reentrant tachycardia (n=4), atrial fibrillation (n=2),

bradyarrhythmia requiring pacemaker (n=3), myocardial

infarction (n=4), heart failure-related hospitalization (n=1),

and cerebrovascular accident (n=6). Arrhythmia-free sur-

vival was 98.40.7% at 10 years and 96.60.1% at 25 years

(Figure 3A). Freedom from an adverse cardiovascular event

was 96.80.9% at 10 years and 92.91.9% at 25 years

(Figure 3B). Associated factors are listed in Table 4. The only

multivariate predictors were a single RCA (hazard ratio, 4.58;

95% CI, 1.32-15.90; P=0.0166) and a low cardiac output syn-

drome in the immediate postoperative period (hazard ratio,

6.93; 95% CI, 1.57-30.62; P=0.0107).

Table 5 summarizes clinical data at the last follow-up. The

mean left ventricular ejection fraction was 60.38.9% and

97.3% of patients had New York Heart Association (NYHA)

class I symptoms. Nineteen (5.2%) patients had documented

coronary disease, and 12 (3.3%) had systemic hypertension.

At least moderate neoaortic and pulmonary regurgitation were

documented in 3.4% and 6.6% of patients, respectively. Some

degree of right ventricular outflow tract or pulmonary artery

stenosis was present in 62.1% of patients, with a mean gradientof 2517 mm Hg by echocardiography, and at least moderate

stenosis in 10.3%. Neoaortic stenosis was present in 11.9% of

patients, with an average gradient of 197 mm Hg and at least

moderate stenosis in 3.2%. The mean aortic root size was 30.88.3

mm, with 6.5% of patients having an aortic root dimension 40

mm. Only 1 patient had an aortic root >50 mm (ie, 51 mm).

A total of 161 patients had cardiopulmonary exercise test-

ing. The peak oxygen uptake was 35.17.6 mL/kg/min, corre-

sponding to 86.115.1% of predicted values. The percentage

of maximum predicted heart rate averaged 90.77.0% but was

Figure 1. Overview of vitalstatus.

Table 2. Surgical and Catheter-Based Interventions in

Perioperative Survivors After the Arter ial Switch Operation(n=374)

n (%)

Surgical Reintervention

Coronary intervention 2 (0.5)

Aortic reconstruction at anastomosis 2 (0.5)

Pulmonary artery reconstruction/plasty at anastomosis 25 (6.7)

Aortic valve plasty 4 (1.1)

Aortic valve replacement 2 (0.5)

Subaortic stenosis resection 5 (1.3)

Aortic arch/coarctation surgery 5 (1.3)

Pulmonary valve plasty 4 (1.1) Subpulmonary stenosis/right ventricular outflow tract resection 1 (0.3)

Left pulmonary artery plasty 6 (1.6)

Right pulmonary artery plasty 6 (1.6)

Ventricular septal defect closure 1 (0.3)

Atrial septal defect closure 3 (0.8)

Mitral valvuloplasty 3 (0.8)

Permanent pacemaker 3 (0.8)

Implantable cardioverter-defibrillator 3 (0.8)

Total 48 (12.8)

Catheter-based intervention

Aortic plasty/stenting at anastomosis 5 (1.3)

Pulmonary artery plasty/stenting at anastomosis 33 (8.8)

Aortic valve plasty 1 (0.3)

Aortic coarctation dilation/stenting 4 (1.1)

Pulmonary valve plasty 13 (3.5)

Right pulmonary artery dilation/stenting 20 (5.3)

Left pulmonary artery dilation/stenting 28 (7.5)

Superior vena cava dilation/stenting 2 (0.5)

Closure of aortopulmonary collaterals 6 (1.6)

Right ventricle to pulmonary artery homograft dilation 3 (0.8)

Intracoronary thrombolysis 1 (0.3)

Cavotr icuspid isthmus ablation for typical atrial flutter 2 (0.5)

Total 60 (16.0)

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completeness of data collection, the catchment area was lim-

ited to patients living in New England. Moreover, data were

supplemented by directly contacting patients and by acquiring

records from secondary sources. To minimize misclassifica-

tion errors, outcomes were uniformly defined, and detailed

standardized case-report forms were subject to data quality

checks.

In this early surgical experience, the perioperative mortal-

ity rate declined over time. Since the original description of

the ASO in 1976,8modifications such as the Lecompte pro-

cedure, coronary artery relocation, and pulmonary artery

reconstruction have likely contributed to improved periop-

erative outcomes.9,2730The association between the Taussig-

Bing anomaly and poorer outcomes observed in our study

has been previously described,31

and postoperative complica-tions, including hemorrhage, have been linked to increased

Supravalvar 104 (37.8)

Left pulmonary artery 61 (22.2)

Right pulmonary artery 66 (24.0)

Neoaortic stenosis Present, n (%) 37 (11.9)

Gradient in patients with neoaortic stenosis, mm Hg 197

At least moderate aortic stenosis, n (%) 10 (3.2)

Level of stenosis (nonexclusive), n (%)

Subaortic 4 (1.3)

Aortic valve 6 (1.9)

Supravalvar (below anastomosis) 15 (4.8)

Aortic anastomosis 17 (5.5)

Aortic dimensions, mm

Annulus 21.46.2

Root 30.88.3

Aortic root 40 mm, n (%) 18 (6.5)

Sino-tubular junction 23.713.8

Ascending aorta 26.38.7

Right ventricular ejection fraction, (%) 597

Right ventricular end-diastolic volume, mm3 15669

Right ventricular end-systolic volume, mm3 7030

Functional capacity

New York Heart Association functional class, n (%)

Class I 290 (97.3)

Class II 8 (2.7)

Class III or IV 0 (0)

Peak heart rate, bpm 18018

Peak percent heart rate predicted, % 90.77.0

Heart rate reserve, bpm 10121

Chronotropic index, % 83.910.9

Respiratory exchange ratio (RER) 1.160.09

Peak oxygen uptake, mL/kg/min 35.17.6

Percent maximum predicted peak oxygen uptake, % 86.115.1

All percentages presented in the table are based on the denominator of

patients with available data for the variable of interest.

Table 5. Continued

Characteristic

Table 5. Clinical Data on Latest Follow-Up

Characteristic

Physical examination

Height, cm 156.027.4

Weight, kg 57.725.1

Heart rate, bpm 7521

Systolic blood pressure, mm Hg 11514

Diastolic blood pressure, mm Hg 649

Oxygen saturation, % 98.61.3

Recognized comorbidities

Coronary artery disease, n (%) 19 (5.2)

Hypertension, n (%) 12 (3.3)

Dyslipidemia, n (%) 2 (0.5)

Diabetes mellitus, n (%) 1 (0.3)

Thyroid disease, n (%) 2 (0.5)

Cancer, n (%) 1 (0.3)

Cardiac imaging

Left ventricular ejection fraction, % 60.38.9

Left ventricular dysfunction, n (%)

None 307 (95.6)

Mild 14 (4.4)

Moderate or severe 0 (0)

Left ventricular end-diastolic volume, mm3 13667

Left ventricular end-systolic volume, mm3 5535

Mitral regurgitation, n (%)

None 188 (63.7)

Mild 105 (35.6)

Moderate 2 (0.7)

Severe 0 (0)

Tricuspid valve regurgitation, n (%)

None 104 (35.3)

Mild 184 (62.4)

Moderate 7 (2.4)

Severe 0 (0)

Neoaortic regurgitation, n (%)

None 163 (49.8)

Mild 153 (46.8)

Moderate 11 (3.4)

Severe 0 (0)

Pulmonary regurgitation, n (%) None 91 (39.7)

Mild 123 (53.7)

Moderate 10 (4.4)

Severe 5 (2.2)

Pulmonary stenosis

Present, n (%) 171 (62.2)

Gradient in patients with pulmonary stenosis, mm Hg 2517

At least moderate pulmonary stenosis, n (%) 28 (10.3)

Level of stenosis (nonexclusive), n (%)

Subpulmonary 17 (6.2)

Pulmonary valve 17 (6.2)

(Continued)



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perioperative mortality.12Additional reported risk factors for

early death include, but are not limited to, aortic arch repair

prior to ASO, right ventricular hypoplasia, lower birth weight,

and longer intraoperative support.10

In perioperative survivors, the observed 96.7% actuarial

survival rate at 25 years further extends results from previous

longitudinal studies.12,16,26,27With the exception of 1 accidental

death, all late fatalities were cardiovascular and attributable to

myocardial infarction or sudden death of presumed arrhyth-

mic etiology. Three of the 6 cardiovascular deaths occurred

in infants, an observation consistent with studies suggesting a

bimodal distribution of coronary events, with most transpiring

during the first year of life.16,32Optimal screening remains a

contentious issue, especially considering the fact that patients

with ASO and denervated coronary arteries may not experi-

ence typical symptoms of angina. Some authors have, there-

fore, recommended serial coronary angiographic studies to

identify potential lesions of concern.33 Although coronary

artery disease was identified in 5% of patients in the current

study, acute coronary syndromes were far less common andoccurred exclusively in infants aged


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AcknowledgmentsWe thank Hugues Leduc from the Montreal Heart InstituteCoordinating Center (MHICC) for his expert statistical assistance.

Sources of FundingThe study was supported, in part, by the Dunlevie Foundation anda Canada Research Chair in Adult Congenital Heart Disease andElectrophysiology.

DisclosuresNone.

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CLINICAL PERSPECTIVEAs the initial cohort of patients with arterial switch procedures for transposition of the great arteries reach their adult years, it

is essential to characterize sequelae and longer-term outcomes to inform and guide screening and surveillance. We, therefore,

conducted a retrospective cohort study on 400 patients with arterial switch surgery between 1983 and 1999. Excellent overall

(96.7%) and arrhythmia-free (96.6%) survival was observed at 25 years of follow-up. Late fatalities were predominantlyattributable to myocardial infarction or sudden death of presumed arrhythmic cause. The few acute coronary events occurred

exclusively in infants aged


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W. Newburger, Michael J. Landzberg and John E. Mayer, JrPaul Khairy, Mathieu Clair, Susan M. Fernandes, Elizabeth D. Blume, Andrew J. Powell, Jane

Great ArteriesCardiovascular Outcomes After the Arterial Switch Operation for D-Transposition of the

Print ISSN: 0009-7322. Online ISSN: 1524-4539Copyright 2012 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/CIRCULATIONAHA.112.135046

2013;127:331-339; originally published online December 12, 2012;Circulation.

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