Two cases of anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) with review of previous published cases.

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1 FAIT clinique Cardiologie T u n i s i e n n e Two cases of anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) with review of previous published cases. Hakim Kaouthar, Boussaada Rafik, Hamdi imen,jabri Faten, msaad Hela, ouarda Fatma, Chaker Lilia Pediatric Cardiology Department, La Rabta Hospital, Tunisia Summary Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare but potentially fatal anomaly. We are presenting two cases of ARCAPA and reviewing the main previous published data on this lesion. The first patient presented at the age of 5 months with respiratory distress and severe chest infection. He was found to have heart murmur and cardiomegaly on chest X ray. Echocardiographic and angiographic data confirmed an ARCAPA associated to a large malalignment ventricular septal defect and distal pulmonary artery aneurysms. He underwend surgical closure of the ventricular septal defect and reimplantation of the ARCAPA on the aorta with good result. The second case is an 11 year male patient, complaining of dizziness and chest pain on exertion. Echocardiographic and angiographic data confirmed ARCAPA associated to a valvular pulmonary stenosis. He was operated on successfully. He got also direct reimplantation of the anomalous coronary artery on the aorta and a pulmonary valve commissurotomy. An anomalous origin of the right coronary artery is a rare condition but may lead to myocardial ischemia and sudden death. Diagnosis is mainly made by echocardiography and confirmed by conventional coronary arteriography. Nowadays, multislice computed tomography is a new noninvasive imaging technique with excellent spatial resolution which can detect the origin and course of an anomalous coronary vessel. Operative correction is the appropriate treatment for an anomalous coronary artery arising from the pulmonary trunk. Reimplantation of the abnormal coronary artery on the aorta should be considered whenever anatomical conditions allow its practice because it offers dual coronary circulation and therefore allows for normalization of coronary flow reserve and for greater protection against secondary coronary bypass graft changes because of age and atherosclerosis. Keywords Pulmonary artery Right coronary Correspondance Docteur HAKIM Kaouthar Service de Cardiologie pédiatrique de l'hôpital la Rabta 1007 Jebbari Tunis Telephone: Fax: kaoutharhakim@yahoo.fr Cardiologie tunisienne - Volume 09 n 02-2 eme trimestre

2 Hakim K. & al. IntroductIon anomalous origin of the right coronary artery originating from the pulmonary trunk (arcapa) is a rare but potentially fatal anomaly. it can be isolated or associated to various other congenital heart defects. different modalities are available to aid in establishing the diagnosis, including echocardiography, angiography, computed tomography coronary angiography and magnetic resonance angiography. We are presenting two cases of arcapa and reviewing the main previous published data on this lesion. case n 1 a 5 month old boy was admitted to pediatric intensive care unit for severe respiratory tract infection with congestive failure leading to mechanical ventilation. The parents report history of failure to thrive since the end of the neonatal period. The body weight was 4.2 Kg (- 3 sd) and he had a systolic murmur along left sternal border. The electrocardiogram was normal. Transthoracic echocardiogram revealed a large mal alignment ventricular septal defect with a left to right shunt with mild sub pulmonary artery gradient, enlarged left cardiac chambers and good biventricular function. The coronary arteries anatomy was not visualized on echocardiography. a Chest X ray showed cardiomegaly with cardio thoracic index of 0.6, congested lungs and multiple areas of atelectasis. a Multidetector computed tomography of the chest was performed in order to study the pulmonary parenchyma and eliminate any respiratory tract compression. it revealed multiple small pulmonary artery aneurysms (figure n 1). a cardiac catheterization was subsequently performed and confirmed the diagnosis of ventricular defect with increased pulmonary artery pressure and distal multiple pulmonary artery aneurysms. it also showed an anomalous right coronary artery arising from the pulmonary artery and filled via retrograde flow from the left coronary artery (figure n 2). Figure 1: Small pulmonary arteries aneurysms (arrows) on CT scan Figure 2: Pulmonary artery angiography showing the Right coronary artery (arrow) arising from the main pulmonary artery. The infant was operated on after the infection had resolved. He underwent direct reimplantation of anomalous right coronary artery into the aorta with closure of the large ventricular septal defect through a right atriotomy. The post operative period was uneventful. in particular, there was no clinical or electrical sign of ischemia. on the last routine follow up, 2 years after surgery, he is perfectly asymptomatic on acethyl salicylic acid therapy. He gained weight normally. The ecg is normal and the echographic study showed normal biventricular function with no residual shunt or residual pulmonary hypertension. The forward coronary flow from aorta was well seen. case n 2 a 11-year-old boy presented to the pediatric cardiology clinic complaining of dizziness and non specific chest pain on exertion for several months. Physical examination revealed a 4/6 grade systolic murmur over the pulmonary area. electrocardiogram was normal without evidence of myocardial ischemia or arrhythmia. an echocardiogram revealed normal segmental analysis, with normal biventricular contractility. a pulmonary stenosis with peak gradient of 58 mm Hg was noticed (figure n 3, 4). The left coronary artery was dilated (figure n 5). The origin of the right coronary artery could not be visualized on echocardiography. There was a diastolic flow into the main pulmonary artery, close to the pulmonary valve (figure n 6). angiographic images documented the right coronary artery arising from the pulmonary trunk, and selective left coronary arteriography showed retrograde filling of the coronary artery from collateral vessels (figure n 7). right ventricular angiography and pressure measurements confirmed pulmonary valvular stenosis with infundibular reaction (figure n 8).The patient underwent surgical reimplantation of the anomalous rca into the aorta (figure n 9 and figure n 10) with pulmonary valve Cardiologie Tunisienne 123

3 anomalous origin of the RiGHt CoRonaRY artery FRom the pulmonary artery commissurotomy. The post operative course was smooth with no clinical or electrical sign of ischemia. The post operative echocardiographic study showed good biventricular size and function with good visualization of forward coronary flow from the aorta to both right and left coronary arteries. There was no residual pulmonary stenosis, and pulmonary regurgitation was mild. at 3- month follow-up, he is asymptomatic and has normal left ventricular function. Figure 5: The left coronary artery was dilated Figure 3: Pulmonary flow acceleration on the parasternal short axis view. PA: Pulmonary artery; Ao: Aorta a B Figure 6: RCA from the pulmonary artery (a) with diastolic coronary steal to the main pulmonary artery (B) Figure 4: Peak gradient of 58 mm Hg over the pulmonary valve. Figure 7: visualization of the RCA from collateral circulation after opacifying left coronary artery Cardiologie Tunisienne 124

4 Hakim K. & al. discussion Figure 8: Right ventricular angiography confirming pulmonary valvular stenosis with infundibular reaction. Figure 9: intraoperative view of the abnormal RCA (arrow) from the main pulmonary artery (PA) Figure 10 : intraoperative view of the RCA (arrow) after reimplantation on the aorta PA: pulmonary artery; Ao: aorta Congenital coronary artery anomalies are rare in the general population with an incidence of only 0.3 to 0.9% [1] increasing up to 36% in patients with congenital heart disease [2]. among these abnormalities, the anomalous coronary origin from the pulmonary artery is an extremely rare form. it was first described by Brooks in 1886 [3]. Four variations of this condition have been described: origin of the left coronary artery from the pulmonary artery (alcapa), origin of the right coronary artery from the pulmonary artery (arcapa), origin of an accessory coronary artery from the pulmonary artery, and origin of the entire coronary circulation from the pulmonary artery [4]. arcapa is extremely rare, with an estimated incidence in the general population of 0.002% [4]. Many patients are asymptomatic, and the true prevalence of arcapa is likely underestimated. in fact, Williams and al [5] published in 1990 a review of 70 previously reported cases of arcapa. They noticed that this anomaly was diagnosed on basis of a heart murmur, mostly continuous, in asymptomatic patients in 50% of cases. in the other patients, chest pain and congestive heart failure were the most frequent presentations, followed by palpitations, arrhythmias and myocardial infarction. These data were similar to those of Krishnamurthy [6]. only 25-30% cases of arcapa are associated with structural heart defects [5] like our two cases. The most common associated cardiac defects reported were tetralogy of Fallot and aorto-pulmonary window, followed by aortic stenosis, septal defects and aortic coarctation, etc.. [5, 6, 7, 8]. Two cases, similar to our first patient, with an arcapa associated to a large ventricular septal defect, were reported by Burakowskii in 1985 [9] and Maluf in 1997 [10]. another case similar to our second patient, having an arcapa with pulmonary valvular stenosis was published by donaldson et al [11]. Pathophysiology of arcapa is well established. it s a well tolerated condition at neonatal period, high neonatal pulmonary vascular resistances allowing forward perfusion of the abnormal pulmonary artery from the pulmonary artery. once the pulmonary vascular resistances fall, the fully oxygenated blood arriving in the anomalous coronary, via collateral vessels from the normally arising coronary, is stolen by the pulmonary trunk resulting in chronic myocardial ischemia [12, 13]. symptoms result from the chronic left to right shunt and from myocardial ischemia which can lead to ventricular dilatation and dysfunction. The severity of ventricular ischemia is determined by the shunt size, presence of collateral circulation, territory at risk, and myocardial oxygen demands [13]. in our first case, there was no collateral circulation. if the abnormal coronary was not noticed, a simple closure of the ventricular septal defect with acute drop in pulmonary artery pressure could Cardiologie Tunisienne 125

5 anomalous origin of the RiGHt CoRonaRY artery FRom the pulmonary artery cause a fatal myocardial infarction. ecg in arcapa may be normal or it may show left ventricular hypertrophy or deep Q waves in inferior leads [5, 14]. Transthoracic echocardiography is an essential tool for the diagnosis of arcapa. in pediatric patients, the echocardiographic diagnosis of this anomaly has improved. However, in the adult group the location of right coronary artery ostium is always difficult to detect by transthoracic two-dimensional (2d) imaging because of a low spatial resolution with poor penetration through the chest wall [6, 14]. Cardiac computed tomography coronary angiogram and cardiovascular magnetic resonance provide excellent visualization of coronary artery anomalies. They complete coronary angiography by providing detailed anatomic informations of origin, course, and relationship of the anomalous coronary artery [6, 14]. Because adverse outcomes including increased risk for myocardial infarction and sudden cardiac death have been described in arcapa patients regardless previous symptoms of myocardial ischemia, surgical correction is recommended whenever this anomaly is diagnosed [5, 15]. The aim of surgical correction is to eliminate the left-to-right shunt and establish dual coronary circulation to prevent the potential risk of myocardial ischemia from coronary steal. The location of the ostium of the right coronary artery in the pulmonary artery will influence the technique used for surgical repair [16, 17]. Transfer of the anomalous vessel to the aorta is the treatment of choice since it provides establishment of bicoronary circulation that allows for normalization of coronary flow reserve and for greater protection against secondary coronary bypass graft changes because of age and atherosclerosis [16, 18, 19, 20, 21]. When anatomical considerations make this attitude impractical, ligation of the abnormal pulmonary origin of the coronary artery, arterial bypass grafting or confection of aorto-pulmonary window should be considered as alternative therapeutic options [17]. surgical correction of arcapa is associated to low operative mortality ranging from 2 to 3% [5-14]. Because reports describing arcapa follow-up are rare, there is only limited knowledge about the long term changes of coronary vasculature after surgical correction. nevertheless, surgical and pathology reports [3] describe the anomalous right coronary as being thinwalled, dilated, and vein-like structured; therefore, it is thought that the right coronary artery does not tend to normalize in diameter after surgical correction of arcapa. conclusion an anomalous origin of the right coronary artery is a rare condition which might lead to myocardial ischemia and sudden death. diagnosis is mainly made by echocardiography and confirmed by conventional coronary angiography. nowadays, multislice computed tomography is a new noninvasive imaging technique with excellent spatial resolution which can detect the origin and course of an anomalous coronary vessel. surgical correction is the appropriate treatment for an anomalous coronary artery arising from the pulmonary trunk. references 1. Topaz o, demarchena ej, Perin e, et al. anomalous coronary arteries: angiographic findings in 80 patients. int J Cardiol 1992;34: Carvalho Js, silva CM, rigby Ml, shinebourne ea. angiographic diagnosis of anomalous coronary artery in tetralogy of Fallot. Br Heart J 1993;70: Brooks HsJ. Two cases of abnormal coronary artery of the heart arising from the pulmonary artery: with some remarks upon the effect of this anomaly in producing cirsoid dilation of the vessels. J anat Physiol 1885;20: Yamanaka o and Hobbs r. Coronary artery anomalies in patients undergoing coronary arteriography. Cath Cardiovasc diagnosis 1990;21: Williams i a, gersony W, Hellenbrand W. anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of the literature. am Heart J 2006;152: Krishnamurthy J, Chung T, Vick g. W, et al. P. anomalous right coronary artery from the pulmonary artery: noninvasive diagnosis and serial evaluation. J Cardiovasc Magnetic resonance 2007;9: Bossert T, Walther T, doll n, et al. anomalous origin of the right Coronary artery From the Pulmonary artery Combined With aortic Valve stenosis. ann Thorac surg 2005;79: Warraich H, Matyal r, shahul s, et al. anomalous right Coronary artery arising From the Pulmonary artery. ann Thorac surg 2012;93:75 9. Burakowskii Vi. anomalous derivation of the right coronary artery from the pulmonary artery with an interventricular septal defect. grud Knir 1981; Maluf Ma, smith M, abellan dm, et al. anomalous origin of right coronary artery from the pulmonary artery in association with a ventricular septal defect. Tex heart inst J Cardiologie Tunisienne 126

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