DOI: 10.1016/j.athoracsur.2008.03.032 2008;86:170-176 Ann Thorac Surg

Khouqeer, Charles C. Canver, Avedis Kalloghlian and Zohair Al-Halees Bahaaldin Alsoufi, Ahmed Sallehuddin, Ziad Bulbul, Mansour Joufan, Fareed

Anomalous Left Coronary Artery Origin From the Pulmonary ArterySurgical Strategy to Establish a Dual-Coronary System for the Management of

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urgical Strategy to Establish a Dual-Coronaryystem for the Management of Anomalous Leftoronary Artery Origin From the Pulmonary Artery

ahaaldin Alsoufi, MD, Ahmed Sallehuddin, MD, Ziad Bulbul, MD,ansour Joufan, MD, Fareed Khouqeer, MD, Charles C. Canver, MD,vedis Kalloghlian, MD, and Zohair Al-Halees, MD

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Background. Optimal repair of anomalous origin of leftoronary artery from pulmonary artery (ALCAPA) reliesn the creation of a dual-coronary system. If the anoma-ous coronary arises at a long distance from the aorta, wese various coronary extension techniques to facilitate

ension-free implantation.Methods. Thirty patients underwent ALCAPA opera-

ions using direct coronary transfer (n � 11) or coronaryxtension techniques (n � 19). Surgical outcomes werenalyzed.Results. Median age and weight were 5.7 months

range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9g). Five patients had concomitant mitral annuloplasty.ean cardiopulmonary bypass and ischemic times were

08 � 38 and 57 � 25 minutes. Two patients requiredntraoperative revision of the implantation. There werehree hospital deaths (10%) and no late deaths. Follow-upchocardiograms demonstrated significant improvement

ostoperatively vs preoperatively in shortening fraction

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2008 by The Society of Thoracic Surgeonsublished by Elsevier Inc

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35% � 2% vs 16% � 2%, p < 0.00001), ejection fraction64% � 3% vs 32% � 4%, p < 0.00001), and mitralegurgitation (11% moderate vs 70% moderate or severe,

� 0.0002). Left ventricular end-diastolic dimension-score decreased from 9.1 � 0.9 to 1.2 � 0.5 (p < 0.00001).oth techniques were equally effective. Two patientsnderwent reoperation 1 and 12 years postoperatively

coronary artery bypass grafting, 1; mitral repair withoronary angioplasty, 1). Surviving patients remainsymptomatic (p < 0.00001).Conclusions. Dual-coronary system can be established

n patients with ALCAPA. Coronary extension implanta-ion techniques have acceptable operative mortality andxcellent cardiac recovery and late survival. Although theate of late coronary occlusion is low, continual ventric-lar or mitral dysfunction should trigger evaluation ofersistent coronary compromise.

(Ann Thorac Surg 2008;86:170–6)

© 2008 by The Society of Thoracic Surgeons

nomalous origin of the left coronary artery from thepulmonary artery (ALCAPA) is a rare congenital

eart disease that may result in infant death due to theevelopment of ischemic cardiomyopathy [1]. Severalurgical techniques have been implemented to repairhis lesion. Operative approaches that establish a dual-oronary supply to the heart have been associated withmproved outcomes compared with approaches that re-ult in one source for coronary circulation [1–13].

Different techniques have been described to achievedual-coronary system, including the creation of in-

rapulmonary tunnel and coronary transfer. At ournstitution we have adopted a surgical policy to estab-ish a dual-coronary supply since 1991. Because anntrapulmonary tunnel was associated with severalomplications, coronary transfer has been our surgical

ccepted for publication March 18, 2008.

resented at the Poster Session of the Forty-fourth Annual Meeting ofhe Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008.

ddress correspondence to Dr Alsoufi, King Faisal Heart Institute (MBC

trategy of choice [14]. When a significant gap existedetween the anomalous coronary artery and the aorta, wesed several previously described modified implantation

echniques to facilitate tension-free transfer of distantoronaries [14–16]. Our surgical strategy has evolvedith time to use those techniques in all ALCAPA pa-

ients. In the current series, we review our experienceith those modified implantation techniques and assessostoperative cardiac recovery and long-term clinicalutcomes.

atients and Methods

nclusion Criteriarom July 1991 to July 2007, 30 consecutive children withLCAPA underwent surgical correction at the King Faisalpecialist Hospital and Research Center in Riyadh, Saudirabia. The patients were identified using the surgicalatabase. Clinical, operative, and outcome data were ab-tracted from the medical records. Approval of this studyas obtained from the Research Ethics Board at our insti-

ution, and individual consent was waived.

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atient Characteristicsuring the study period, 30 children (12 boys, 18 girls)ith ALCAPA underwent surgical treatment. The median

ge at the time of operation was 5.7 months (range, 46 dayso 5.45 years), and 16 were aged younger than 6 months.he median weight was 5.35 kg (range, 3.3 to 15.9 kg).Upon clinical presentation, 12 patients required inten-

ive care unit admission for inotropic support and man-gement of severe congestive heart failure, and 4 werentubated preoperatively. Overall, 23 patients had historyf congestive heart failure, whereas 7 were asymptomaticnd were diagnosed based on the presence of a hearturmur.

reoperative Echocardiography Datahe preoperative mean shortening fraction was 0.16 �.02 and the mean ejection fraction was 0.32 � 0.04, andpatients (27%) had a preoperative ejection fraction of

ig 1. Direct coronary transfer for treatment of anomalous left coronas transected. The coronary artery is excised with a wide cuff of pulmary button is widely mobilized to allow a tension-free anastomosis.

o serve as the site of the anastomosis of the anomalous coronary arte

ig 2. Coronary implantation with autologous flap extensions from aary artery is transected. The anomalous left coronary artery is exciseary artery wall. This tongue is used to form the anterior half of theflap to form the posterior half of the tunnel. The aortic flap is first s

ongue of pulmonary artery wall is then sutured to complete the tunnel as

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ess than 0.20. Mean preoperative left ventricular end-iastolic diameter was 4.0 � 0.14 cm, with a mean Z score.1 � 0.9. The preoperative degree of mitral valve regur-itation (MR) was none in 1 patient, mild in 6, mild tooderate in 2, moderate in 15, moderate to severe in 4,

nd severe in 2. Left ventricular wall motion abnormali-ies were present in 24 patients.

perative Techniquell procedures were performed though a midline ster-otomy. Cardiopulmonary bypass (CPB) was establishedith standard aortic and bicaval venous cannulation. The

eft ventricle was decompressed by venting through theight superior pulmonary vein. Mild hypothermia (32° to4°) was used. Once CPB was initiated, the right and leftulmonary arteries were snared to prevent myocardial

schemia secondary to steal of coronary blood flow intohe pulmonary artery.

tery from the pulmonary artery. (Left panel) The pulmonary arteryarterial wall to form a coronary button. (Middle panel) The coro-

t panel) An incision is made at the posteromedial wall of the aortathe aorta.

osteriorly and pulmonary artery anteriorly. (Left panel) The pulmo-a button together with a tongue of tissue from the anterior pulmo-ed tunnel. (Middle panel) The aortic incision is fashioned to created to the lower margin of the coronary button. (Right panel) The

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orta pd asplannuture

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The aorta was cross-clamped, and the heart was ar-ested with antegrade blood cardioplegia infused into thescending aorta and into the main pulmonary artery.dditional doses of cardioplegia were infused directly

nto the orifice of the left coronary artery if needed.Several techniques have been used in our series. Ear-

ier in our experience, when the anomalous left coronaryrtery originated from the posterior pulmonary sinus, theoronary ostia was excised with a wide cuff of pulmonaryrterial wall to form a coronary button. The coronaryutton was widely mobilized to allow a tension-freenastomosis. Similarly, an incision was made at theosteromedial wall of the aorta for the anastomosis. Theesultant defect in the pulmonary artery was patchedith autologous pericardium (Fig 1).When the anomalous coronary artery originated from

he nonfacing pulmonary sinus or from the posteriorulmonary sinus close to the commissure with the non-

acing sinus, we used a modified technique that has beenescribed previously by Sese and colleagues [14] thatombines aortic and pulmonary flaps. As we gained morexperience with this technique, it became our operativetrategy of choice regardless of the origin of the anoma-ous coronary artery. In this technique, the pulmonaryrtery was transected. A tunnel was created using nativeissue from both the aorta and the pulmonary arterialalls. The anomalous left coronary artery was excised asbutton together with a tongue of tissue from the

nterior pulmonary artery wall. This tongue was used toorm the anterior half of the planned tunnel. The aorticncision was fashioned to create a flap to form theosterior half of the tunnel. The aortic flap was firstutured to the lower margin of the coronary button. Theongue of pulmonary artery wall was then sutured toomplete the tunnel and also to close the aortotomy. The

ig 3. Coronary implantation with autologous flap extension from puwo transverse parallel incisions, one proximal and the other distal tistance on both sides of the coronary orifice. (Middle panel) The isollous artery at its center is folded with the orifice of the coronary arteension tube of tissue that lengthens the coronary artery. (Right paneleromedial wall of the aorta to serve as the site of the anastomosis of

esultant defect in the pulmonary artery was patched o

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ith autologous pericardium, and the transected pulmo-ary artery was reanastomosed end-to-end (Fig 2).In addition, a few patients underwent a modified

echnique that has been described previously by Turleynd colleagues [15] and was popularized by Ilbawi andolleagues [16] in which the pulmonary artery wasransected. Two transverse parallel incisions, one proxi-

al and the other distal to the anomalous coronary arteryrifice, were made and extended an equal distance onoth sides of the coronary orifice. The commissure be-

ween the posterior and the nonfacing pulmonary si-uses was often mobilized if found close to the anoma-

ous coronary. The isolated segment of the pulmonaryrtery containing the origin of the anomalous artery at itsenter was folded with the orifice of the coronary arterys its fulcrum and its side edges sutured to each other toorm an extension tube of tissue that lengthens theoronary artery. With the use of a 5-mm aortic punch, anncision was made at the posteromedial wall of the aortao serve as the site of the anastomosis of the lengthenedoronary artery to the aorta. The resultant defect in theulmonary artery was patched with autologous pericar-ium, and the transected pulmonary artery was reanas-

omosed end-to-end (Fig 3).In all techniques after completion of the coronary

mplantation, antegrade warm cardioplegia was infusednto the aortic root, air was removed from the left side ofhe heart, and the aortic cross-clamp was released. Theeconstruction of the pulmonary artery was performed asescribed on an empty beating heart.Mitral valve repair was performed in 5 patients with

ither severe regurgitation or mitral dysfunction thateemed to be more organic than functional. Our repairechnique involved annuloplasty along the posterior leaf-et using a polypropylene suture, reinforced with a strip

ary artery sinuses. (Left panel) The pulmonary artery is transected.anomalous coronary artery orifice, are made and extended an equalsegment of the pulmonary artery containing the origin of the anom-its fulcrum, and its side edges sutured to each other to form an ex-

h the use of a 5-mm aortic punch, an incision is made at the pos-ngthened coronary artery to the aorta.

lmono theatedry as) Wit

f autologous pericardium, in two layers of running

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attress fashion. In addition, papillotomy was per-ormed in 1 patient, and division of secondary chordaerom the posterior mitral leaflet was needed in 2 patientso improve the mobility of the retracted leaflet.

Weaning from CPB was done with the use of inotropicupport. The sternum was left open in 12 patients, withelayed chest closure performed in the intensive carenit after the swelling had subsided.Mean CPB and cardiac ischemic times were 108 � 38

nd 57 � 25 minutes, respectively, for the whole group.hese times were somewhat longer when coronary

engthening implantation techniques were used com-ared with simple direct coronary transfer technique:ean CPB duration was 99 minutes for direct transfer vs

22 minutes for coronary lengthening procedures (p �.12), and mean cardiac ischemic duration was 50 min-tes for direct transfer vs 65 minutes for coronary length-ning procedures (p � 0.09).

ollow-Upate outcomes were determined from recent office visitst King Faisal Specialist Hospital and Research Center orrom direct correspondence with the patient’s family. The

ean follow-up duration was 4.2 � 4.5 years (range, 2ays to 14.6 years).

tatistical Analysisll the data were analyzed with SAS 9 software (SAS

nstitute Inc, Cary, NC). Data are presented as frequency,edian with range, or mean � standard deviation, as

ppropriate, with the number of nonmissing values in-icated. Characteristics and outcomes of patients in thereoperative period vs those at the last follow-up wereompared using paired t tests and �2 or the Fisher exactest, as appropriate. Unrelated two-group comparisonsere done with unpaired, two-tailed t tests for continu-us variables and the �2 or Fisher exact test for categoricata.

esults

perative Mortalityhree patients (10%) died postoperatively of multisystemrgan failure (n � 1), ventricular fibrillation (n � 1), andardiogenic shock after a persistent low cardiac outputtate (n � 1). One patient in our series received postop-rative support with extracorporeal membrane oxygen-tion (ECMO) and eventually died of multisystem organailure and inability to wean off ECMO assistance. Oneatient had sudden cardiac arrest due to ventricularbrillation 12 days postoperatively and did not receiveCMO; whereas the other patient’s operation was in991, before wide use of ECMO for postoperative cardiacupport at our institution.

Multiple demographic, operative, and era variablesere entered into both univariate and multivariable

egression analyses to identify factors associated with

perative mortality. None of the assessed variables was e

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ignificantly associated with hospital death, likely due tohe small sample size (Table 1).

ime-Related Survival and Freedom Fromorbid Eventso late deaths occurred in our series, and none of theatients required orthotopic heart transplantation. Twoatients required late cardiac reoperations. One patientontinued to have persistent left ventricular dilatation,itral regurgitation (MR), and ventricular dyskinesia on

erial postoperative echocardiograms, with persistentymptoms of congestive heart failure. Cardiac catheter-zation 8 months postoperatively revealed an occludedeft coronary artery with collateral filling from the rightoronary artery. He underwent reoperation with coro-ary artery bypass grafting using the internal mammaryrtery at the age of 1 year, in addition to mitral annulo-lasty. He is currently 3.5 years old, asymptomatic, withn ejection fraction of 0.76, trace MR, and normal leftentricular dimensions with no dyskinesia. The otheratient is a girl who underwent ALCAPA repair at thege of 3 years in 1993. Her ejection fraction improvedrom 0.32 preoperatively to 0.65 on follow-up, with im-rovement of her moderate MR. However, she waseevaluated 12 years postoperatively for fatigue and wasound to have worsening MR and left ventricular dyski-esia. Cardiac catheterization showed stenosis at therigin of the left main coronary artery. She underwenteoperation with patch angioplasty of the left coronaryrtery and mitral valve repair, with significant clinicalnd echocardiographic recovery.

linical Status and Echocardiography Assessmentt the most recent follow-up, all survivors had normal

linical exercise ability and were in New York Heartssociation function class I except for 1 patient who wasdmitted to another hospital for pneumonia a fewonths postoperatively and had a respiratory arrest

esulting in ischemic brain injury.Follow-up echocardiography in survivors demon-

trated a mean shortening fraction of 0.35 � 0.2 postop-

able 1. Logistic Regression Analysis of Variables Affectingospital Survival in the Surgical Treatment of Anomalouseft Coronary Origin From the Pulmonary Artery

ariable

p Value Analysis

Univariate Multivariable

mplantation technique 0.90 0.88ge 0.47 0.96eight 0.43 0.87

PB duration 0.47 0.69schemic duration 0.38 0.78re-op ejection fraction 0.64 0.91re-op mitral regurgitation 0.93 0.74re-op LVED dimension Z score 0.16 0.66

PB � cardiopulmonary bypass; LVED � left ventricular end-diastolic.

ratively compared with 0.16 � 0.02 preoperatively (p �

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.00001) and an ejection fraction of 0.64 � 0.3 postopera-ively compared with 0.32 � 0.04 preoperatively (p �.00001). Mitral regurgitation was absent to mild in 24urvivors and was moderate in 3 (11%) compared with0% of patients who had moderate or severe MR preop-ratively (p � 0.0002). The left ventricular end-diastolicimension Z-score decreased from 9.1 � 0.9 preopera-

ively to 1.2 � 0.5 at the latest follow-up (p � 0.00001).No difference was noted in the echocardiographic

ecovery between patients who underwent direct coro-ary transfer vs those who underwent coronary length-ning implantation techniques. In most patients, systolicunction, left ventricular dilatation, and MR had recov-red by 6 months postoperatively.

omment

urgical management of ALCAPA has evolved over theears into the currently generally accepted standardshat include urgent surgical repair soon after diagno-is, the establishment of a dual-coronary artery system,igilant myocardial protection with administration ofultiple doses of blood cardioplegia into the aorta and

electively into the coronary arteries while preventingteal into the pulmonary arteries, creation of a widenastomosis between the anomalous artery and theorta, liberal use of delayed sternal closure, and ECMOupport when indicated [1–13].

We discuss the results of the modified implantationechniques that we have adopted for the surgical treat-

ent of ALCAPA at our institution.

mplantation Techniqueeveral techniques have been used by different surgeons

o reestablish a dual-coronary system for the treatment ofLCAPA [1–16]. Direct anomalous coronary transfer and

mplantation into the aorta provides the simplest man-gement option. It is the most frequently adopted surgi-al technique and has gained popularity through in-reased experience with coronary artery transferechniques adapted from the arterial switch operationFig 1). The reported mortality rate for direct reimplan-ation is 0% to 16% [1–13]. Nonetheless, direct coronaryransfer has major limitations. When the anomalousoronary arises from the posterior pulmonary sinus closeo the commissure between the posterior and the non-acing sinus, or less frequently when it arises from theonfacing sinus, there is a long distance between theoronary artery orifice and aorta. In these cases, directoronary transfer can result in tension applied to theortocoronary anastomosis resulting in increased inci-ence of stenosis and obstruction with the risk of persis-

ent ventricular ischemia and development of postoper-tive cardiac arrest.Coronary artery bypass grafting using a saphenous

ein, the internal mammary artery, or the subclavianrtery is associated with major shortcomings such asinking, stenosis, and late occlusion and is usually notecommended in infants and small children [1].

Creation of an intrapulmonary artery tunnel with aor- t

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opulmonary window (Takeuchi operation) has been rec-mmended as the treatment of choice in infants by someurgeons [7, 13]. This procedure has been associated withumerous complications such as the development ofupravalvular pulmonary stenosis, baffle leaks creating aoronary–pulmonary artery fistula, and aortic valve in-ufficiency [7, 13] Overall reported operative mortalityas been 0% to 23%, with the reported late reoperation oratheter intervention rate as high as 30% [7, 13].

Several techniques have been described in the litera-ure to avoid any tension on the implanted anomalousoronary artery [14–16]. Sese and colleagues [14] recom-ended the use of a flap of pulmonary artery wall with a

orresponding flap from the aorta to lengthen the im-lanted coronary artery (Fig 2). We adopted this modifi-ation selectively early in our practice in cases of distantoronaries; and later on, our strategy evolved to use thisechnique in all children with ALCAPA.

This technique allows adequate lengthening of themplanted vessel to bridge the distance between thenomalous coronary and the aorta and to avoid tensionn the aortocoronary anastomosis and prevent the devel-pment of obstruction that may be amplified in the

mmediate postoperative period by the dilated left ven-ricular and the tense pulmonary artery. Extensive dis-ection of the anomalous coronary artery is not necessaryn this technique, which minimizes bleeding complica-ions and the risk of distortion. This procedure preserveshe growth potential of the implanted artery because viableative tissues are used. Although this tunnel had to beevised twice intraoperatively in our experience due tonaccurate orientation of the autologous flaps, this was theesult of a learning curve and was associated with excellentecovery in both patients after proper implantation. Theisk of distortion should not be any different than that inirect coronary transfer when performed by experiencedurgeons. We have not noted any evidence of distortiono the aortic valve on follow-up echocardiograms.

Nonetheless, we have noted two late postoperativeailures, one at about 8 months and one at about 12 years,s described in the Results section. As noted in manyrior publications, most of the patients show significantesolution of their ventricular dilatation, systolic func-ion, and MR between 4 and 8 months postoperatively [1,, 10]. Failure to do so should trigger evaluation ofersistent coronary ischemia, as was the case in our firsteoperation. In addition, late development of MR orentricular dysfunction, as was the case in our secondeoperation, should also encourage further investigationo rule out coronary compromise. No late failures oc-urred in the patients in our series who received directoronary transfer, and late stenosis after coronary length-ning techniques does not seem to be higher than thateported in other series mainly using direct coronaryransfer in the treatment of ALCAPA [1–13] (Fig 4).

Another coronary lengthening technique has beenescribed by Turley and colleagues [15] and was popu-

arized by Ilbawi and colleagues [16]. It involves the usef a noncircumferential segment of the pulmonary artery

o lengthen the coronary artery to provide a wide tunnel

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f redundant, autologous tissue to bridge the gap be-ween the anomalous coronary and the aorta (Fig 3).imilar to the first technique, it allows future growth bysing viable native tissue. This technique was used in

ew patients in our center with good early and lateesults. Detachment of the commissure between theosterior and the noncoronary sinuses was often neces-ary. We have not experienced any late complicationsith this technique, although our follow-up in thoseatients does not exceed 5 years.

ortality and Risk Factors for Deathublished mortality after dual-coronary repair of ALCAPA

s 0% to 16% [1–12]. Hospital mortality after ALCAPA repairas 10% in our series. Previous reports have identified

everal risk factors for operative mortality such asounger age, operations done before ECMO availability,ecreased preoperative left ventricular function, andeverity of preoperative MR [1, 3, 9, 10]. Although alleaths in our series were in infants, age was not identi-ed as a risk factor. Similarly, the degree of ventricularysfunction or MR was not a significant factor in our risknalysis. More important, neither the implantation tech-ique nor the site of origin of the anomalous left coronaryrtery was associated with hospital death. Nonetheless,ur small cohort size and the multiple variables involved

imit the power of the study to identify clinically signifi-ant risk factors.

ate of the Mitral Valvehe management of the regurgitant mitral valve at the

ime of ALCAPA repair varies between institutions andemains controversial. Many surgeons suggest that themprovement in coronary perfusion, ventricular dilation,nd papillary muscle dysfunction associated with suc-essful ALCAPA surgery will result in improvement in

R, and therefore, mitral valve repair is not recom-

ig 4. Freedom from reoperation after anomalous left coronaryrtery from the pulmonary artery implantation stratified by implan-ation technique for direct coronary transfer (dashed line) and coro-ary extension (solid line) techniques.

ended [3, 8, 9–12]. On the other hand, other surgeons

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ecommend surgical repair of severe MR at the time ofhe ALCAPA procedure owing to the high incidence ofersistent MR after coronary reimplantation [2, 7]. More-ver, several reports have shown that the improvement

n the degree of MR is generally slower than that ofentricular dysfunction [1, 9, 10]. In our experience, theLCAPA operation was associated with significant im-rovement of MR. Nonetheless, because our philosophy

owards mitral valve repair has evolved with time, sev-ral patients in our series with significant preoperativeR underwent concomitant mitral valve repair at time of

heir ALCAPA operation, with considerable improve-ent. Several others with significant MR who did not

ndergo mitral repair also showed considerable im-rovement of regurgitation. Therefore, our series is notuitable to address the issue of mitral valve repair re-uirement. Our current approach is to perform concom-

tant mitral valve repair in patients with severe MR or inhom preoperative echocardiographic assessment of

alve dysfunction reveals a likely organic rather thanunctional pathology.

Most important, 2 patients who continued to showvidence of persistent or recurrent MR were found toave obstruction of the implanted coronary, and there-

ore continual MR and ventricular dysfunction shouldrompt further evaluation of coronary ischemia.

onclusiondual-coronary system in the surgical management of

LCAPA can be established in all patients regardless ofhe site of origin of the anomalous coronary artery.oronary extension implantation techniques allow ten-

ion-free anastomosis with minimal risk of distortion andlow rate of late occlusion. Regardless of the implanta-

ion technique, the ALCAPA operation is associated withcceptable operative mortality, excellent cardiac recov-ry, and late survival. Although the rate of late coronarycclusion is low, continual ventricular dysfunction oritral regurgitation should prompt evaluation of persis-

ent coronary compromise.

he illustrations in this article were created by one of theuthors but were adapted from similar work by Rachid Idrisshat can be seen in reference 1.

eferences

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9. Azakie A, Russell JL, McCrindle BW, et al. Anatomic repairof anomalous left coronary artery from the pulmonary arteryby aortic reimplantation: early survival, patterns of ventric-ular recovery and late outcome. Ann Thorac Surg 2003;75:1535–41.

0. Schwartz ML, Jonas RA, Colan SD. Anomalous origin of the

left coronary artery from the pulmonary artery: recovery of

udicious lifelong follow-up.

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left ventricular function after dual coronary repair. J Am CollCardiol 1997;30:547–53.

1. Caspi J, Pettitt TW, Sperrazza C, Mulder T, Stopa A. Reim-plantation of anomalous left coronary artery from the pul-monary artery without mitral valve repair. Ann Thorac Surg2007;84:619–23.

2. Lange R, Vogt M, Hörer J, et al. Long-term results of repairof anomalous origin of the left coronary artery from thepulmonary artery. Ann Thorac Surg 2007;83:1463–71.

3. Takeuchi S, Imamura H, Katsumoto K, et al. New surgicalmethod for repair of anomalous left coronary artery frompulmonary artery. J Thorac Cardiovasc Surg 1979;78:7–11.

4. Sese A, Imoto Y. New technique in the transfer of ananomalously originated left coronary artery to the aorta. AnnThorac Surg 1992;53:527–9.

5. Turley R, Szarnicki R, Flachsbart K, Richter R, Popper R,Tarnoff H. Aortic implantation is possible in all cases ofanomalous origin of the left coronary artery from the pul-monary artery. Ann Thorac Surg 1995;60:84–9.

6. Barth MJ, Allen BS, Gulecyuz M, Chiemmongkoltip P,Cuneo B, Ilbawi MN. Experience with an alternativetechnique for the management of anomalous left coronaryartery from the pulmonary artery. Ann Thorac Surg

2003;76:1429 –34.

NVITED COMMENTARY

erhaps because anomalous origin of the left coronaryrtery from the pulmonary artery (ALCAPA) is so rare annomaly, presents so dramatically, and is so effectivelyreatable, it appears regularly in our surgical literature.lsoufi and coworkers [1] have added to this knowledgeith a cogent analysis of their experience with 30 patientsith this disorder with encouraging results.There remain a few dilemmas with this lesion. The first

ilemma is the mortality, which remains about 10% inecent reports. Most of the mortality occurs in patientsresenting with shock or significant end-organ injury.nfortunately, there is no “signature” of the lesion either

n fetal life or in the neonatal period. The mortality mayecrease coincidently with the increasing use of echocar-iography to investigate “transitional” murmurs in therst month of life, as long as cardiologists check theoronary anatomy. An astute pediatrician might detect aurmur of mitral regurgitation before symptoms de-

elop. The challenge is to increase the awareness, at thefront line,” of the possibility of this abnormality.The second dilemma is the quality of the coronary recon-

truction. As mentioned by the authors, reimplantation tech-iques (most of them showcased in numerous reports in Thennals of Thoracic Surgery during the past 15 years) have highuccess rate in the hands of experienced surgeons whooutinely perform the arterial switch operation. On thether hand, long-term follow-up of patients who under-ent the latter operation show a significant incidence of

oronary ostial or proximal coronary artery stenosis. Oneight expect the incidence to be similar in repairedLCAPA. A recent report demonstrates that myocardialow reserve, evaluated with adenosine challenge and

3NH4 dynamic positron emission tomographic imaging, ismpaired after ALCAPA repair. Thus, these patients need

The final dilemma is the non-zero incidence of persis-ent mitral regurgitation (MR) after ALCAPA repair. Inome reports in which the mitral valve is not repaired athe time of ALCAPA repair, the incidence of persistentignificant MR is 25% to 35%. In the present report, theuthors repaired the mitral valve at initial operation in allpatients with severe MR, and on late follow-up only 1 of5 patients had even moderate MR. (Two other patientsith significant MR also had important coronary stenosisr occlusion and ventricular dysfunction and underwenteoperation). These results would suggest that severeitral regurgitation should be addressed at the time ofLCAPA repair. There is certainly not uniform agree-ent on this approach. Therefore, the challenge is to

ome up with better predictors of the need for initialitral valve repair other than the initial presence of

evere MR. Careful analysis of the mitral valve usinghree-dimensional echocardiography and future tech-iques to differentiate ischemic papillary muscle dys-

unction from permanent injury may help guide theecision.

illiam M. DeCampli, MD, PhD

ediatric Cardiothoracic Surgeryhe Congenital Heart Instituternold Palmer Hospital for Children and Womenrlando, FL 32806-2036

-mail: wdecampli@orhs.org

eference

. Alsoufi B, Sallehuddin A, Bulbul Z, et al. Surgical strategy toestablish a dual-coronary system for the management ofanomalous left coronary artery origin from the pulmonary

artery. Ann Thorac Surg 2008;86:170–6.

0003-4975/08/$34.00doi:10.1016/j.athoracsur.2008.03.076

Zohair Al-Halees on August 23, 2009

DOI: 10.1016/j.athoracsur.2008.03.032 2008;86:170-176 Ann Thorac Surg

Khouqeer, Charles C. Canver, Avedis Kalloghlian and Zohair Al-Halees Bahaaldin Alsoufi, Ahmed Sallehuddin, Ziad Bulbul, Mansour Joufan, Fareed

Anomalous Left Coronary Artery Origin From the Pulmonary ArterySurgical Strategy to Establish a Dual-Coronary System for the Management of

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