Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by endocarditis, myocardial infarction, or coronary aneurysms. This article reviews the clinical characteristics and surgical outcome of 10 patients with CAF in Rajae Heart Center. From 1990 to 2000, 10 patients (age, 6 months to 15 years; mean age, 8.5 years) were diagnosed with CAF by echocardiography and cardiac catheterization. Six were female and 4 were male. All patients with isolated CAF (9) were asymptomatic. One patient with associated anomaly (mitral valve prolapse with severe mitral regurgitation) had dyspnea on exertion and palpitation. Five fistulas originated from the right coronary artery-three from the left and two from the left circumflex. Drainage was to the right ventricle (7), right atrium (2), and pulmonary artery (1). The ratio of pulmonary to systemic flow ranged between 1 and 1.6. All patients had surgical ligation. In the symptomatic patient, in addition to ligation, mitral valve replacement was performed. There was no operative or late death. Follow-up evaluation (range, 1-6 years; mean, 4.2 years) showed no evidence of recurrent or residual CAF. Surgical management of CAF is a safe and effective treatment resulting in 100 survival and clousure rate. Â© Springer Science+Business Media, Inc. 2005

Malekahmadi, M.

Shahmohammadi, A.

eprints Iran University of Medical Sciences

Surgical Outcome of Coronary Artery Fistulas Repair in ChildrenM. Malekahmadi,1 A. Shahmohammadi21Pediatic Department, Hajar Hospital, Shahrekord University of Medical Sciences, Shahrekord, Iran2Pediatric Cardiology Department, Iran University of Medical Sciences, Tehran, IranAbstract. Coronary artery ﬁstula (CAF) is a rarecongenital anomaly that can be complicated byendocarditis, myocardial infarction, or coronaryaneurysms. This article reviews the clinical charac-teristics and surgical outcome of 10 patients withCAF in Rajae Heart Center. From 1990 to 2000, 10patients (age, 6 months to 15 years; mean age,8.5 years) were diagnosed with CAF by echocardi-ography and cardiac catheterization. Six were femaleand 4 were male. All patients with isolated CAF (9)were asymptomatic. One patient with associatedanomaly (mitral valve prolapse with severe mitralregurgitation) had dyspnea on exertion and palpita-tion. Five ﬁstulas originated from the right coronaryartery—three from the left and two from the leftcircumﬂex. Drainage was to the right ventricle (7),right atrium (2), and pulmonary artery (1). The ratioof pulmonary to systemic ﬂow ranged between 1 and1.6. All patients had surgical ligation. In the symp-tomatic patient, in addition to ligation, mitral valvereplacement was performed. There was no operativeor late death. Follow-up evaluation (range, 1–6 years;mean, 4.2 years) showed no evidence of recurrent orresidual CAF. Surgical management of CAF is a safeand eﬀective treatment resulting in 100% survival andclousure rate.Key words: Coronary artery ﬁstula — Surgical liga-tionSymptomatic coronary artery ﬁstulas (CAFs) havebeen associated with substantial morbidity andmortality at all ages [4, 6–8, 11, 12]. CAFs that havenot been detected or closed in childhood have beenreported to become symptomatic in adulthood be-cause of chronic volume load and ischemia [6]. Inci-dental detection of CAFs in asymptomatic patientshas been noted on coronary angiography, but theirclinical signiﬁcance is unclear [9]. Although someauthors recommend elective closure of CAFsregardless of symptoms [6], others advocate conser-vative management [3]. This study was performed todetermine the clinical characteristics and surgicaloutcome of children with CAFs.Patients and MethodsPatientsThe chart databases of the Department of Cardiology, ShaheedRajae Hospital, were reviewed for all patients with the diagnosis ofCAF noted on echocardiography or at catheterization from 1990 to2001. The study included patients who had isolated CAFs withoutany important other cardiac anomaly. Patients with CAFs orsinusoids associated with congenital cardiac malformation, such aspulmonary atresia with intact ventricular septum, or mitral stenosisand aortic atresia were excluded. Patients in whom CAFs weredetected following intracardiac surgery were also excluded. Tenpatients met the inclusion criteria. Patients’ charts were reviewedfor demographic, symptoms, clinical ﬁndings, indication forechocardiography, associated diagnosis, and radiologic and elec-trocardiograph (ECG) ﬁndings at presentation. At follow up, evi-dence of myocardial ischemia, congestive heart failure, andarrythmias was sought clinically and with electrocardiography.When performed, surgical or cardiac catheterization reports werereviewed. Surgical repair was approached via a median sternotomyand cardiopulmonary bypass (CPB).EchocardiographyEchocardiograms were performed with available cardiac scanners,with transducer frequency and focus appropriate for patient size.The origin, course, and exit site of the ﬁstula were recorded for eachpatient. Coronary artery diameter was determined and considerednormal if measurements were within 2 SD of normal controls.Cardiac CatheterizationCardiac catheterization data and angiography were reviewed whenavailable. The origin, course, and exit site of the ﬁstula, right andCorrespondence to: M. Malekahmadi, email: mr_malekahmadi@yahoo.comPediatr Cardiol 26:328–330, 2005DOI: 10.1007/s00246-004-0656-yleft ventricular end diastolic pressures, pulmonary artery (PA)pressure, pulmonary-to-systemic ﬂow ratio, and assessment of leftventricular (LV) function were recorded.ResultsTen patients had an incidental ﬁnding of a small CAFdetected by echo and angiography. The salientdemographic, clinical, echocardiographic, and out-come data are summerized in Table 1. The mean ageat diagnosis was 8.5 years (range, 6 months to15 years). The primary indication for echocardiog-raphy at presentation was murmur in 9 patients.Dyspnea on exertion and palpitation in addition tomurmur were noted in 1 patient. This patient, inaddition to CAF, had mitral valve prolapse (MVP)and severe mitral regurgitation (MR). A systolicmurmur was audible in 6 patients and was assessed asinnocent in all. A continuous murmur, which wasinterpreted as a small patent ductus arteriosus, wasaudible in 4 patients. No patient had symptomssuggestive of angina or congestive heart failure.Associated cardiac abnormality was present in 1 pa-tient (Table 1). Cardiomegaly was present radio-graphically in 4 patients. At presentation, 2 patientshad electorcardiographic criteria for biventricularhypertrophy—1 patient for left ventricular hypertro-phy and 1 patient for right ventricular hypertrophy.No patient had abnormal Q wave or ST segment or Twave changes suggestive of ischemia on EC.The origin of the CAF was clearly deﬁned bycolor Doppler and angiography in 10 patients. Theorigin of the ﬁstula was from the right coronary ar-tery system in 5 patients, from the left coronary ar-tery in 3 patients, and from the left circumﬂex in 2patient. Coronary artery dimentions, were normal inall patients. The ﬁstula drained into the right ventriclein 7 patients, right atrium in 2 patients, and pulmo-nary artery in 1 patient. Cardiac catheterization wasperformed in all patients. The pulmonary-to-systemicﬂow ratio ranged from 1 to 1.6. All patients hadsurgical ligation of CAF by cardiopulmonary bypassand midsternotomy approach. All patients had fol-low-up from 1 to 6 years (mean, 4.2 years). None hadevidence of recurrent or residual ﬁstula.DiscussionCAF is a rare anomaly and occurs in 0.2–0.4% ofcases are congenital heart disease. There are newtechniques for closing the CAF, such as transcatheterembolization techniques using coils, a detachableballoon, polyvinil foam, and a double-umberella de-vice. The transcatheter approach is a fairly compli-cated intervention and requires an experiencedoperator and interventional specialist with expertisein both coronary arteriography and embolizationtechniques. Embolization often requires complicatedcatheter manipulation as well as selection of variouscatheters and wires. In the literature, there are onlycase reports and reports of small series [1, 5]. Al-though there was some experience with patent ductusarteriosus closure by coil and Amplatzer in our cen-ter, there was not any experience with CAF clousureusing these devices, therefore, surgical treatment wasperformed in all of our patients.Of the 10 patients who had undergone surgery, 6were female and 4 were male. In a study from Thai-land, a female preponderance had also been observed[14].In this study, the most common indication forECG, as in the Sherwood et al [10] study, was heartmurmur. Most patients in our study, as in that ofTable 1. Clinical, and paraclinical ﬁndings and outcome data of 10 patients with CAFPatientNo. GenderAge(years)Reason forechoCardiomegallyon CXR ECGAssociatedcardiac anomalyFistulaorigin Exit site Qp/QsFollow-up(years)1 F 9 CMM 0 NL 0 RCA RV 1.2 3.92 F 3.5 CMM + RVH 0 LCX RA 1.3 1.03 F 13 SMM 0 NL 0 RCA RV 1.4 4.24 F 5 SMM + BVH 0 LCA RA 1.3 2.45 F 0.5 SMM + BVH 0 LCX RV 1.6 7.56 M 12 SMM 0 NL Q RCA RV 1.4 5.07 M 6 CMM 0 NL 0 LCA RV 1.0 5.58 M 14 SMM 0 NL 0 RCA RV 1.5 4.09 M 7 SMM + NL 0 RCA RV 1.3 6.010 F 15 CMM, DOE 0 LVH MR, MVP LCA PA 1.1 5.5BVH, biventricular hypertrophy; CMM, continuos murmur; DOE, dyspnea on exertion;.F, female; LAD, left axis deviation; LCA, leftcoronary artery; LCX, left circumﬂex; LVH, left ventricular hypertrophy; M, male; MR, mitral regurgitation; MVP, mitral valve prolapse;NL, normal; PA, pulmonary artery; RA, right atrium; RCA, right coronary artery; RV, right ventricle; RVH, right ventricular hypertrophy;SMM, systolic murmur.Malekahmadi and Shahmohammadi: CAF Surgical Correction 329Sherwood et al [10] and Wang et al [15], wereasymptomatic and most included the isolated form ofCAF, as in the study of Wang et al [15] and that ofSunder et al [13]. Symptomatic forms were seen morein patients older than 20 years [1, 10, 15].Electrocardiography revealed that the mostcommon changes were ventricular hypertrophy, aspreviously reported [14]. The most common ﬁndingin chest radiography was cardiomegaly, as reportedby Thongtang [14].In this study, the origin of the CAF was the rightcoronary artery in 50% of patients and the left cor-onary artery in 50% of patients, consistent with otherstudies [2, 14, 16], and the exit site in decreasing orderof frequency was the right ventricle, right atrium, andpulmonary artery, as stated in other reports [2, 13,15]. However, in some studies the common exit sitewas the pulmonary artery [10, 14]. The range of Qp/Qs in this report was 1–1.6, and that in Thohgtang etal.’s [14] report was 1.2–1.6.All patients in this study were repaired surgicallyusing CPB. There was no mortality, and no residualshunt was found at the time of discharge from thehospital.No abnormal clinical ﬁndings, were encounter inoutpatients during follow-up, which ranged from 1 to6 years (mean, 4.2 years). This was consistent withother reports [14, 15].ConclusionEarly and proper surgical management of CAF issafe and eﬀective, resulting in 100% survival andclosure rates.References1. Balanescu S, Sangiorgi G, Castelvecchio S, Medda M, IngleseL (2001) Coronary atery ﬁstulas: clinical conequences andmethods of closure. A literature review. Ital Heart J 2:669–6762. Barbosa MM, Katina T, Oliveria HG, Neuenschwander FE,Oliveria EC (1999) Doppler echocardiographic features ofcoronary artery ﬁstula: report of 8 cases. J Am Soc Echocar-diogr 12:149–1543. Hobbs RF, Millit HD, Raghavan PV, Moodie DS, SheldonWC (1982) Coronary artery ﬁstulae: a ten-year review. Cleve-land Clin Q 49:191–1974. Kovalchin JP, Grifka RG, McQuinn TC, Pignatelli RH, GevaT (1995) Right coronary artery to right atrium ﬁstula mim-icking a right atrial mass. Am Heart J 130:907–9095. Latson LA, Forbes TJ, Cheatham JP (1992) Trancatheter coilembolization of a ﬁstula from the posterior descending coro-nary artery to the right ventricle in a two-year-old child. AmHeart J 124:1624–16266. Liberthson PR, Sagar K, Berkoben JP, Weintraub RM, LevineFH (1979) Congenital coronary arteriovenous ﬁstula. Reportof 13 patients, review of literature and delineation of man-agement. Circulation 59:849–8547. Ludomirsky A, O’Laughlin MP, Reul GJ, Mullins CE (1990)Congenital aneurysm of the right coronary artery with ﬁstulousconnection to the right atrial atrium. Am Heart J 119:672–6758. Rein AJJT, Yatsiv I, Simcha A (1988) An unusual presentationof right coronary atery ﬁstula. Br Heart J 59:598–6009. Sapin P, Frantz E, Jain A, Nichols TC, Dehmer GJ (1990)Coronary artery ﬁstula: an abnormality aﬀecting all agegroups. Medicine 69:101–11310. Sherwood MC, Rockenmacher S, Colan SD, Geva T (1999)Prognostic signiﬁcance of clinically silent coronary artery ﬁs-tulas. Am J Cardiol 83:407–41111. Stansel HC Jr, Fenn JE (1994) Coronary arteriovenous ﬁstulabetween the left coronary artery and persistent left superiorvena cava complicated by bacterial endocarditis. Ann Surg160:292–29612. Starc TJ, Bowman FO, Hordof AJ (1986) Congestive heartfailure in a newborn secondary to coronary artery–left ven-tricular ﬁstula. AM J Cardiol 58:366–36713. Sunder KR, Balakrishnan KG, Tharakan JA, et al. (1997)Coronary artery ﬁstula in children and adults: a review of 25cases with long-term observations. Int J Cardiol 58:47–5314. Thongtang V, Panchavinnin P, Chaithiraphan S, Sabasakul Y(1996) Congenital coronary artery ﬁstula: a report of 24 pa-tients. J Med Assoc Thai 79:630–63415. Wang S, Wu Q, Hu S, et al. (2001) Surgical treatment of 52patients with congenital coronary artery ﬁstulas. Chin Med J(Engl) 114:752–75516. Wong KT, Menahem S (2000) Coronary arterial ﬁstulas inchildhood. Cardiol Young 10:15–20330 Pediatric Cardiology Vol. 26, No. 4, 2005

Surgical outcome of coronary artery fistulas repair in children

http://eprints.iums.ac.ir/12138/1/Pediatric%20Cardiology%20Volume%2026%20issue%204%202005%20%5Bdoi%2010.1007_s00246-004-0656-y%5D%20M.%20Malekahmadi%3B%20A.%20Shahmohammadi%20--%20Surgical%20Outcome%20of%20Coronary%20Artery%20Fistulas%20Repair%20in%20Children.pdf

Surgical outcome of coronary artery fistulas repair in children

Abstract

Similar works

Full text

Available Versions

eprints Iran University of Medical Sciences