Coronary Artery Aneurysm and Coronary Fistula in Tetrology of Fallot: A Case Presentation of A Young Adult UndergoingTotal Correction

ABSTRACT

Congenital presentation of a coronary artery fistula is a rarely observed pathologic finding; especially an associ-ation with tetralogy of Fallot is reported in only three cases previously. This is a presentation of a patient with tet-ralogy of Fallot who had an aneurysmatic fistula communication between the circumflex artery and left ventricle.Preoperative computerized tomography angiographic examination showed a communication between the cir-cumflex artery and left ventricle. 27-year-old female patient underwent an operation for total correction. Intraope-ratively, the fistula could not be seen and it had been left unligated. The patient had been discharged from hospi-tal without problems on the tenth postoperative day. Aneurysmatic coronary artery fistula is not found to cause adeleterious effect on the hemodynamics or myocardial performance of the heart after the total correction surgery Key Words: Tetralogy of Fallot; Coronary artery; aneurysm; coronary fistula.

ÖZET

Fallot Tetrolojisinde Koroner Arter Anevrizmas› ve Koroner Fistul: Tam Düzeltme Yap›lan Genç Eriflkin BirVaka Sunumu

Konjenital koroner arter fistülü oldukça nadir gözlenen bir patolojik bulgudur. Özellikle Fallot tetralojisi ile birlikte-li¤i ise daha önce sadece üç vaka olarak rapor edilmifltir. Bu yaz›m›zda sircumflex arter ile sol ventrikül aras›ndafistül bulunan fallot tetrolojili bir hastan›n sunumu yap›lmaktad›r. Ameliyat öncesi bilgisayarl› tomografik anjiogra-fide sol ventrikül ile sircumflex arter aras›nda anevrizmatik fistül iliflkisi gözlemlendi. 27 yafl›ndaki bayan hasta tamdüzeltme ameliyat›na al›nd›. Operasyonda fistül bulunamad› ve ba¤lanmadan b›rak›ld›. Problemi olmayan hasta,ameliyat sonras› onuncu günde taburcu edildi. Anevrizmatik koroner arter fistülün tam düzeltme ameliyat› sonra-s›nda miyokardiyal performansda ve hemodinamide bozulma yaratan bir etkisi gözlemlenmedi.Anahtar Kelimeler: Fallot tetralojisi; koroner arter; anevrizma; koroner fistül.

INTRODUCTION

Congenital presentation of a coronary arteriovenous fistula (CAVF) is a rare abnormal finding and it was reportedto present in approximately 1 in every 50.000 in patients with congenital heart disease (1,2). The association of co-ronary fistula and tetrology of fallot is reported only in three cases in the literature (3). However, aneurysm in thecoronary artery fistula has not been previously reported. The early recognition of coronary arteriovenous fistulasis imperative, as management and treatment could prevent serious complications. In this case report, in the twocoronary arteries high degree of fusiformal aneurysmatic dilatation and coronary fistula between circumflex arteryand left ventricle was detected in the angiographic study preoperatively in a patient with tetrology of Fallot. Thepresentation of this case and the surgical intervention and subsequent postoperative course is presented to dis-cuss this unusual case and necessary precautions that is required during management in preoperative, operativeand postoperative periods.

Ali Fedakar, MD1, Ahmet fiaflmazel MD1, Onursal Bugra, MD1, Kamil Boyac›o¤lu MD1, Ayfle Baysal MD2, Ayfle ‹nci MD3, Mehmet Balkanay MD1

1 Kartal Kofluyolu Yüksek ‹htisas E¤itim ve Araflt›rma Hastanesi, Department of Cardiovascular Surgery, Istanbul, Turkey2 Kartal Kofluyolu Yüksek ‹htisas E¤itim ve Araflt›rma Hastanesi, Anesthesiology and Reanimation Clinic, Istanbul, Turkey 3 Kartal Kofluyolu Yüksek ‹htisas E¤itim ve Araflt›rma Hastanesi, Department of Pediatric Cardiology, Istanbul, Turkey

Address for ReprintsAli Fedakar, MDDepartment of Cardiovascular Surgery, Kartal Kofluyolu Yüksek ‹htisas E¤itim ve Araflt›rma Hastanesi, 34846 Kartal, IstanbulTelephone: +90 216 459 40 41 / 1007 Fax: +90 216 459 63 21 e-mail: alfdkr67@hotmail.com

Kofluyolu Kalp Dergisi 2010;13(3):23-25

24 Kofluyolu Kalp Dergisi Ali Fedakar, MD

CASE REPORT

A 27 year old woman presented to our clinic with diffi-culty in breathing, blue discoloration in lips and hands,palpitation. The patient was told to have congenital he-art disease but had not received any treatment previo-usly. In the family history, there is no kinship or any sib-ling with congenital heart disease. She has a healthybrother and a sister who are both alive.In the physical exam, the blood pressure was 100/60mmHg, heart rate was 95/minute, respiratory rate was28/minute, arterial oxygen saturation was 79%. Thecyanosis in lips was more prominent than the peripheryand there was pertinent respiratory insufficiency andclubbing in hand nails. The auscultation showed a 5/6systolic murmur which is best heard at the mesocardi-ac location that is also heard in all other locations aswell. Bilateral crackles are heard in lower lobes of lungfields. The palpation of the liver was 2 cm below the

costal margin and mild pretibial edema is palpated inthe lower extremities. The investigations included; an electrocardiogramwhich revealed a right ventricular hypertrophy, rightaxial deviation, atrial fibrillation without any ischemicfinding. A chest X-ray showed; cardiomegaly, conges-tion in basal lung fields bilaterally and biatrial dilatationof the heart. Echocardiographic evaluation revealed; 1)Fallot tetralogy and atrial septal defect (ASD), 2) leftventricular hypertrophy, 3)biatrial dilatation, giant leftatrium (right atrium diameter 90 mm). Coronary arteri-ography by computerized tomographic (CT) study sho-wed; 1) All coronary arteries visualized as having fusi-form aneurysmatic presentation and run in an unevencourse that especially the diameter of the circumflexartery increases to 2.2 cm at the proximal region and insome regions during its course this diameter is evenhigher than the diameter of the aorta that has been in adextraposition. 2) The first obtuse marginal (OM1)branch of the circumflex artery is fistulized into the leftventricle. 3) Left pulmonary artery diameter was 15.3mm, the right pulmonary artery diameter was 10.2 mmand truncus pulmonalis diameter was 24 mm. 4) Therewas serious stenosis at the pulmonary infundibulumand the diameter was 11 mm. The patient was scheduled for total correction and wasadmitted to surgery electively. The operation was per-formed under general anesthesia and cardiopulmonarybypass was performed under mild hypothermia (28 ºC).Also anterograde blood cardioplegia was used formyocardial protection. During surgical procedure, rightatriotomy was performed. An organized thrombus for-mation is observed at the proximal end of the vena ca-va superior and a thrombectomy was performed. After

Figure 1: Presentation of aneurysmatical coronary arteries.

Figure 2: CT showing aneurysmatical coronary arteries and coronary-ventricular fistula.

Figure 3: CT showing aneurysmatical coronary arteries.

the administration of cardioplegia, the blood was re-moved from the heart and with appropriate positioningthe area to perform right ventriculotomy and pulmo-nary arteriotomy is visualized between the right coro-nary artery (RCA) and left anterior descending coronaryartery (LAD) which were both appeared as aneurysma-tic arteries. ASD was repaired primarily. Ventricularseptal defect (VSD) was closed with dacron patch.Right ventricle outflow tract reconstruction was perfor-med with pericardial patch after precautions was takento protect the posterior cusp of the pulmonary valve.An unsuccessful search was performed to find the firstobtuse marginal (OM1) branch of the circumflex arterythat was fistulized into the left ventricle. The coronaryarteries that were aneurysmatic were left in their placeswithout any further intervention. Patient received inot-ropic support of dobutamine infusion for a total of 48hours including the weaning period and was extubatedon the postoperative second day. She was dischargedto home without any complications on the tenth posto-perative day.

DISCUSSION

In tetralogy of Fallot the abnormalities related to the co-ronary arteries is commonly observed (4). The associ-ation of coronary fistula and tetrology of fallot is repor-ted in only three cases in the literature (3).The patholo-gic findings related to these abnormalities may vary andthe clinical presentation and subsequent treatment mo-dalities change accordingly depending on the clinicalsymptoms. Some anurysmatic coronary arteries mayrequire medical and surgical treatments whereas othersremain asymptomatic (5,6). In the diagnosis of coronary artery aneurysms, conven-tional angiography and 64-sectionmultidetector com-puterized tomography (MDCT) was used extensively asa significant diagnostic tool in several studies (7,8,9). Inour case MDCT angiography study was used to evalua-te the coronary arteries and revealed aneurysmatic di-latation in two coronary arteries and a fistula betweencircumflex artery and left ventricle. Arteriovenous fistulas were reported more commonlybetween the coronary arteries and the pulmonary arte-ries. Dabizzi et al. (10) observed fistula between coro-nary artery and pulmonary artery in 13 patients in a se-ries of 119 patients after preoperative selective coro-nary angiographic evaluation. Of these 13 cases only 1patient had fistula between right coronary artery anteri-or branch and right atrium. Badak et al. (11) have repor-ted a fistula between right coronary artery and right at-rium. Saxena et al. (12) have reported a fistula betweencircumflex artery and coronary sinus. Ito et al. (13) haveshown a coronary artery pulmonary artery fistula origi-nating from three major coronary branches associatedwith exertional chest pain and tachycardia-dependentleft bundle branch blockThe aneurysm in two coronary arteries especially RCAand LAD and coronary fistula presentation in circumflexartery was significant pathologic findings to hesitate toproceed with total correction procedure in an adult pa-tient with tetralogy of Fallot. We have evaluated the pat-

hologic findings with dual slice CT coronary angiog-raphy and were able to make a plan to perform rightventriculotomy without any intervention to the aneury-smatic coronary arteries. Although this surgical planwas successfully performed, the coronary fistula wasunable to be detected for further evaluation and forpossible repair. Postoperatively no problems related tocardiac performance occurred and this suggests thatcoronary fistula is a benign presentation that does notrequire intervention.

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Coronary Artery Aneurysm and Coronary Fistula in Tetrology ... 25Kofluyolu Kalp Dergisi