Borges, Vera T.M.

Bregagnollo, Edson A.

Carvalho, Fabio C.

Hueb, João Carlos

Magalhães, Claudia G.

Matsubara, Beatriz B.

Roscani, Meliza Goi

Salmazo, Pericles S.

Zanati, Silmeia Garcia

English

Cadernos Espinosanos (E-Journal)

CASE REPORTCongenital aneurysmal circumflex coronary arteryfistula in a pregnant womanMeliza Goi Roscani, Silmeia Garcia Zanati, Pericles S. Salmazo, Fabio C. Carvalho, Claudia G. Magalha˜es,Vera T.M. Borges, Edson A. Bregagnollo, Beatriz B. Matsubara, Joa˜o Carlos HuebUniversidade Estado de Sa˜o Paulo (UNESP), Botucatu Medical School, Department of Internal Medicine, Botucatu/SP, Brazil.Email: meliza10@hotmail.comTel.: 55 14 3882-2969INTRODUCTIONCoronary artery fistula (CAF) is an abnormal commu-nication between an epicardial coronary artery and a singleor multiple cardiac chambers, large vessels or other vascularstructures (1). Although it is a rare condition, it is the mostfrequent congenital coronary anomaly (2), and it accountsfor 0.2-0.4% of congenital cardiac anomalies. Approximately50% of pediatric coronary vasculature anomalies arecoronary artery fistulae. Small fistulae remain clinicallysilent and are recognized during routine echocardiographyor at autopsy. Larger fistulae enlarge progressively overtime, and complications, such as congestive heart failure,arrhythmias, infectious endocarditis, aneurysm formation,rupture, and death, are more likely to arise in older patients.The most common feature is a right coronary artery fistulaconnecting to the right ventricle. Reports on the clinicalrepercussions of CAFs in pregnancy are uncommon (3,4).We describe a pregnant woman with a congenital aneur-ysmal circumflex coronary artery fistula to the rightventricle and discuss the appropriate management for thissituation.CASE DESCRIPTIONA 23-year-old woman was admitted at the generalhospital for a prenatal examination in her 10th week ofpregnancy. She was asymptomatic, and a physical examina-tion found that her blood pressure was 110/80 mmHg, herheart rate was 80 bpm, and her respiration rate was 18 ipm.Heart auscultation revealed a grade 2 continuous murmuralong the left sternal border. There were no signs ofcongestive heart failure. Electrocardiography showed nor-mal sinus rhythm and no specific changes in the ST segmentor T wave. Transthoracic echocardiography showed aslightly enlarged left ventricle and a markedly dilated leftcoronary artery. Additionally, there was an abnormal flowto the right ventricle, suggesting the diagnosis of a coronaryfistula to a right cardiac chamber (Figure 1). Cardiaccatheterization performed in the second trimester ofpregnancy showed normal pulmonary artery pressure andno significant increase in oxygen saturation in the right sideof the heart. Coronary angiography revealed a markedlydilated tortuous circumflex coronary artery connected to theright ventricle, indicating a coronary artery fistula (Figure 2).Aspirin (100 mg/day) and endocarditis prophylaxis wereinitiated. A cesarean section was performed at the 37th weekof pregnancy without complications, and the health of thenewborn was satisfactory. Six months after delivery,surgical treatment was recommended to correct the fistula.However, the patient refused treatment and was lost tofollow-up. Two years later, she returned in the thirdtrimester of pregnancy. The delivery occurred withoutcomplications, and the patient did not agree to treatment.She remains asymptomatic under clinical management.DISCUSSIONWe discussed an asymptomatic young pregnant womanwith the rare condition of an aneurysmal circumflexcoronary artery connecting to the right ventricle.Congenital CAFs are defined as an abnormal precapillarycommunication between one or several normally originat-ing coronary arteries and any cardiac vein, chamber or largethoracic vessel that bypasses the myocardial capillary bed(1).Fistulae arise from the right coronary artery (60%) or leftanterior descending artery (30%) and terminate in the rightside of the heart (90%). The most frequent termination sites,in descending order, are the right ventricle, right atrium,coronary sinus, and pulmonary vasculature. The circumflexcoronary artery is rarely affected (2).CAFs are isolated in 55% to 80% of cases and associatedwith other congenital heart diseases in 20% to 45% (1). Themost frequently associated anomalies are tetralogy of Fallot,atrial septal defects, patent ductus arteriosus, ventricularseptal defects, and pulmonary atresia with an intactventricular septum.The natural history of a coronary fistula is unknown.There is a consensus that this anomaly does not typicallyproduce symptoms before the age of 30 years (5), and insome cases, only a continuous murmur is audible, with noother signs. However, if the CAF is sufficiently large tocause a considerable left-to-right shunt, symptoms ofangina, syncope, dizziness and fatigue may be present andtypically occur after the fourth decade of life (6).Electrocardiography (ECG) is normal in approximately50% of cases and shows left or right hypertrophy in theCopyright  2012 CLINICS – This is an Open Access article distributed underthe terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided theoriginal work is properly cited.No potential conflict of interest was reported.CLINICS 2012;67(12):1523-1525 DOI:10.6061/clinics/2012(12)301523other 50%. Atrial fibrillation and an ischemic pattern arerarer manifestations (5).Chest x-rays are typically normal, but cardiomegaly maybe present if there is a large left-to-right shunt (5).Typically, two-dimensional and color Doppler echocar-diography are not useful for establish a definitive diagnosisor determining the course of the coronary fistula; however,these methods are useful for demonstrating the dilation ofthe affected coronary, the related chamber enlargement andthe drainage site. Transesophageal echocardiography (TEE)is better than transthoracic echocardiography for identifyingthe precise origin and drainage site (1,5).Cardiac catheterization is considered the gold standardfor the diagnosis of CAF and should be performed todetermine the hemodynamic significance of the lesion andprovide a detailed angiographic assessment of the abnormalanatomy (1,5,7).It is recommended that endocarditis prophylaxis andaspirin be provided to prevent thrombosis for all patients(8). Asymptomatic patients with small fistulae should bemonitored clinically for signs of growth and increasing flow.Typically, lesions enlarge progressively and warrant opera-tive repair, either by transcatheter or surgical techniques.Both techniques are associated with an excellent prognosis,and the choice of the procedure depends on the morphologyof the fistula.Patients with large and tortuous fistulae, multiple open-ings, or significant aneurysmal dilatation may not beoptimal candidates for transcatheter closure, and a poten-tially curative surgical treatment is preferred (9,10). Olderpatients with large fistulae that are not tortuous oraneurysmal may benefit from transcatheter closure.Studies on CAFs have not emphasized the hazards of thiscondition when it is associated with pregnancy, althoughthis association may cause overt heart failure, which mayoccur due to volume overload (3). Fistula dissection andrupture are described as rare (11).The optimal time for intervention in pregnant women isnot clear. An asymptomatic patient should be treated usinga multidisciplinary approach during the prenatal period. Ifsymptoms of myocardial ischemia are present, surgicalintervention should be performed during pregnancy.In the present case, although the patient was asympto-matic, we recommended surgery after her cesarean sectionbased on studies emphasizing that the incidence ofsymptoms and complications in aneurysmal arteriesincreases with age, including coronary thrombosis causingmyocardial infarction and angina and symptomatic cardi-omyopathy. Therefore, corrective treatment should beperformed prior to these events.In conclusion, we describe a rare case of the discovery ofan aneurysmal circumflex artery in pregnancy and advocatethat the approach to management and therapeutic strategyFigure 1 - A transthoracic echocardiogram showing the markedly dilated left coronary artery (left) and an abnormal flow to the rightventricle, suggesting the diagnosis of a coronary fistula to a right cardiac chamber (right).Figure 2 - Coronary angiography revealing a markedly dilatedtortuous circumflex coronary artery connected to the rightventricle, indicating a coronary artery fistula.Fistula in pregnancyRoscani MG et al.CLINICS 2012;67(12):1523-15251524be based on symptoms, pathological changes and possiblecomplications.AUTHOR CONTRIBUTIONSRoscani MG wrote the manuscript. Zanati SG helped with the literaturereview. Carvalho FC and Bregagnollo EA performed the hemodynamicstudy and assisted in obtaining the figures. Matsubara BB helped with theliterature review and reviewed the manuscript. Hueb JC performed theechocardiogram and assisted in obtaining the figures. Salmazo PS helpedwith the literature and assisted in obtaining the figures.REFERENCES1. Chirantan V, Mangukia MBBS. Coronary artery fistula. Ann Thorac Surg.2012;93(6):2084-92.2. Levin DC, Fellows KE, Abrams HL. Hemodynamically significantprimary anomalies of the coronary arteries. Angiographic aspects.Circulation. 1978;58(1):25-34.3. Tay SM, Ong BC, Tan SA. Cesarean section in a mother with uncorrectedcongenital coronary to pulmonary artery fistula. Canadian journal ofanaesthesia = Journal canadien d’anesthesie. 1999;46(4):368-71, http://dx.doi.org/10.1007/BF03013230.4. Hipona FA. Congenital coronary arterial fistula to a persistent leftsuperior vena cava. The American journal of roentgenology, radiumtherapy, and nuclear medicine. 1966;97(2):355-8.5. Qureshi SA. Coronary arterial fistulas. Orphanet J Rare Dis. 2006;1:51,http://dx.doi.org/10.1186/1750-1172-1-51.6. Canga Y, Ozcan KS, Emre A, Kul S, Guvenc TS, Durmus G, et al.Coronary artery fistula: review of 54 cases from single center experience.Cardiology J. 2012;19(3):278-86, http://dx.doi.org/10.5603/CJ.2012.0050.7. VavuranakisM, BushCA, Boudoulas H. Coronary artery fistulas in adults;incidence, angiographic characteristics,natural history. Cathet CardiovascDiagn. 1995;35(2):116-20, http://dx.doi.org/10.1002/ccd.1810350207.8. Kasravi B, Reid CL, Allen BJ. Coronary artery fistula presenting asbacterial endocarditis. J Am Soc Echocardiogr. 2004;17(12):1315-6.9. Chamberlain MH, Henry R, Brann S, Angelini GD. Surgical managementof a gigantic circumflex coronary artery aneurysm with fistulousconnection to the coronary sinus. Eur J Cardiothorac Surg. 2001;20(6):1255-7, http://dx.doi.org/10.1016/S1010-7940(01)00998-8.10. Mastroroberto P, Olivito S. Giant congenital fistula of the circumflex coronaryartery with drainage into right atrium. Heart Lung Circ. In press 2012.11. Rajs J, Brodin LA, Hertzfeld I, Larsen FF. Death related to coronary arteryfistula after rupture of an aneurysm to the coronary sinus. Am J Forensic MedPathol. 2001;22(1):58-61, http://dx.doi.org/10.1097/00000433-200103000-00011.CLINICS 2012;67(12):1523-1525 Fistula in pregnancyRoscani MG et al.1525

Congenital aneurysmal circumflex coronary artery fistula in a pregnant woman

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Congenital aneurysmal circumflex coronary artery fistula in a pregnant woman

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