Case report : ALCAPA syndrome in a 14 year old Iraqi girl presented with acute myocardial infarction

Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) syndrome is a rare congenital coronary artery anomaly. There are two types of ALCAPA syndrome: the infant and the adult types, each type presents with different clinical findings and outcomes. In infantile type, infants usually suffer myocardial infarction and congestive heart failure, and about 90% of them die within the first year of life if untreated surgically. In rare instances, ALCAPA syndrome presents in adolescents and adults; it is considered as an important cause of sudden cardiac death. The ideal treatment of ALCAPA syndrome is surgical repair performed by restoration of a dualcoronary-artery system. [13] We reported a case of a 14 year old girl presented with an acute myocardial infarction and her coronary angiography revealed ALCAPA syndrome. Four months after ALCAPA diagnosis, she was successfully under went surgical reconstruction without any major complications. نايرشلل ذاش ءوشن( اباكلا ةمزلاتم يوئرلا نايرشلا نم يجاتلا ةاتف في )يوئرلا دالحا ةيبلقلا ةلضعلا ءاشتحا تناع اماع رشع ةعبرا تاذ اباكلا( يوئرلا نايرشلا نم رسيلاا يجاتلا نايرشلل ذاشلا ءوشنلا عونلا ،ضرملا اذى نم ناعون دجوي .ةيجاتلا نييارشلل ردان يدلاو هوشت ةلاح سى ) لاع ول امهنم لكو ،غولبلا وا ةقىارملا نس يف رهظي يذلا رابكلا عون وى يناثلا عونلاو ةركبم نس يف رهظي يذلا يلوفطلا عونلا وى لولاا ةيريرسلا وتام فطلاا ونأب دجن يلوفطلا عونلا يف .وتافعاضمو ابيرقتو ،بلقلا زجعو ةيبلقلا ةلضعلا ءاشتحا نم نوناعي عضرلا لا 09 ةنسلا للاخ نوتومي مهنم % لا نم ةرتفلا هذى يف يئاجفلا توملل امهم "اببس نوكت دقو ،غولبلاو ةقىارملا نس يف اباكلا ةمزلاتم دىاشتام "اردان .ةايحلا نم ىلولاا ،"ايخيرات .رمع نع اهصيخشت مت اباكلا ةمزلاتم ينوزلحلا سارفملاك ةثيدحلا قرطلا لخدا صيخشتلا لاجم يف روطتلا نأف ،كلذ عم ،ةيدايتعلاا ةرطسقلا قيرط بولطملا ةيحارجلا ةيلمعلا عون رايتخا نم حارجلا نكمي يذلا هوشتلا اذى نع "ادج ةقيقد تامولعم يطعي امم ةلاحلا هذى صيخشت يف نينرلاو جلاعل ة فل ةلاح انقثو نحن .ةلاحلا يتلا ةيبلقلا ةرطسقلا ءارجا للاخ نم اباكلا ةمزلاتم تتبثو ةيبلقلا ةلضعلل داح ءاشتحا تناع اىرمع نم رشع ةعبارلا يف ةات طبار ةريغص نييارش قيرط نع نايرشلا اذى ءلاتماو يوئرلا نايرشلا نم رسيلاا يجاتلا نايرشلل ذاش ءوشن تنيب يتلاو انزكرم يف اهل تيرجا نايرشلا عم ة تلا دعب .بلقلا فاغش يف يعيبط ريغ لئاس دوجو مدع عم ،يليلكلاا مامصلا يف فيفح عاجرتسا عم ةديج تناك رسيلاا نيطبلا ةءافك ،نميلاا يجا نودبو ةيعيبط رسيلاا نيطبلا ةءافك نأب اندجو نيتنس رورم دعب .ةمهم تافعاضم نودب ةيحارج ةيلمع اهل تيرجا صيخشتلا نم رهشا ةعبرا يضم يا رعا .ةيبلق ضا


INTRODUCTION
he incidence of ALCAPA syndrome is very rare (1 of 300,000 live births) and represents about 0.4 percent of congenital cardiac anomalies.It was reported for the first time in 1885 by Brooks [1] and described clinically in conjunction with autopsy findings for the first time by Bland, et al. in 1933 [3] , therefore, it is also known as Bland-White-Garland-syndrome.Usually, ALCAPA syndrome is an isolated cardiac anomaly which does not manefist prenatally due to favorable fetal physiology that is characterized by equivalent pressures in the aorta and pulmonary artery resulted from a nonrestrictive patent ductus arteriosus (PDA), and the relatively equivalent O 2 saturations resulted from parallel circulations, consequently the perfusion of myocardium will be normal, so that there is no stimulus for the formation of collateral vessels between the right and left coronary artery systems.Shortly after birth, the parallel circulation will be abolished and becomes in one series, the pulmonary artery pressure and pulmonary resistance decrease, and also the O 2 saturation of pulmonary blood flow.Consequently, there will be a decrease in antegrade flow and O 2 saturation of the T anomalous left coronary artery, causing ischemia in myocardium.This results in initiation of collateral circulation between the right and left coronary systems, and reversion of flow in the left coronary artery flow and enters the pulmonary trunk because of the low pulmonary arterial pressure, resulting in what is called coronary steal phenomena that causes a fixed ischemia or myocardial infarction.85% of all ALCAPA cases present within the first 2 months of life.The clinical presentation and course of ALCAPA include severe left-sided heart failure with significant mitral valve regurgitation due to ischemia of papillary muscle, permanent fibrosis, or left ventricular dilatation.The presentation at late stage is very rare and usually with significant cardiac dysfunction. [14-16]A case report of a 14 year old girl with ALCAPA syndrome will be discussed.

CASE PRESENTATION
A 14-year-old girl presented with dyspnea and chest pain for two days that was misdiagnosed and treated as a case of musculoskeletal chest pain.She experienced worsening left-sided chest pain and subsequently referred to our cardiac center.She had no coronary risk factors and no family history of premature coronary artery disease or congenital heart disease.Physical examination was unremarkable apart from sinus tachycardia.

DISCUSSION
ALCAPA syndrome is very rarely presented late at adolescence and adulthood because of few patients can survive childhood without surgical repair.
So, echocardiography may be used as the first examination for diagnosis of ALCAPA syndrome. [20]In most cases of infantile type who were surgically corrected, have normalization of left ventricular systolic function and mitral valve insufficiency. [11,12]he long-survival at 20 years was estimated as 94.8%. [12]The prognosis of ALCAPA syndrome is usually determined by the degree of irreversible left ventricular systolic dysfunction and presence of myocardial scar tissue.Our patient had completely normal left ventricular systolic function two years after surgical repair, which is indicated the absence of irreversible LV systolic dysfunction at the time of diagnosis.

Conclusion:
The diagnosis of ALCAPA syndrome in adolescence and adulthood needs high index of suspicion in patients with left ventricular dysfunction or evidence of ischemic heart disease.
ECG showed sinus tachycardia with ST segment depression at anterolateral and inferior leads-Non ST elevation myocardial infarction (Figure-1).Chest X-ray showed no cardiomegaly.Cardiac troponin was elevated Echocardiography showed preserved left ventricular function (LV ejection fraction=65%) with anterior hypokinesia and mild mitral regurgitation, no pericardial effusion.A subsequent cardiac catheterization in Basrah Cardiac Center confirmed the diagnosis of ALCAPA with retrograde filling in left coronary artery through collaterals arising from an enlarged right coronary artery, significant left-to-right shunting left main coronary artery into main pulmonary artery trunk (Figure-2).The patient underwent surgical treatment in India by ligation of left coronary artery ostium with left internal mammary artery graft to obtuse marginal artery and right internal mammary artery to left anterior descending artery.She had no major complications or ischemic symptoms two years after surgical operation.A follow up echocardiography showed normal size cardiac chambers and normal left ventricular systolic function and absence of valvular abnormalities.

Fig 1 .
Fig 1. ECG at time of presentation: Sinus Tachycardia with ST depression atanterolateral and inferior leads.

Fig 2 .
Fig 2. Right Coronary angiogram in LAO view: A retrograde flow seen via collaterals arising from dilated right coronary artery(RCA) to a large left descending artery (LAD) and left main artery (LMA) arised from pulmonary artery trunk.A significant left to right shunt filling the pulmonary artery via LAD and LMA.

REFERENCES 1 .
Brooks HSJ, "Two cases of an abnormal coronary artery of the heart, arising from the pulmonary artery, with some remarks upon the effect of this anomaly in producing cirsoid dilatation of the vessels," Journal of Anatomy and Physiology 1885; 20: 26-29.2. Menahem S, Venables AW. "Anomalous left coronary artery from the pulmonary artery: TP, Di Carli MF, Sullivan NM, Leonen MF, Morrow WR., "Myocardial flow reserve in long-term survivors of repair of anomalous left coronary artery from pulmonary artery," Journal of the American College of Cardiology 1998; (31) 2: 437-443. 5. T. Kristensen KF.Kofoed S. Helqvist, M. Helvind, L. Søndergaard, "Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report, "Journal of Cardiothoracic Surgery 2008; vol.3, no. 1, article 33. 6. Facciorusso P, Lanna C, Vigna, et al. "Anomalous origin of the left coronary artery from the pulmonary artery in an elderly patient, football player in youth, "Journal of Cardiovascular Medicine 2008; 9(10): 1066-1069.7. Separham, Aliakbarzadeh P. "Anomalous left coronary artery from the pulmonary artery presenting with aborted sudden death in an octogenarian: a case report," Journal of Medical Case Reports 2012; vol.6, article 12. 8. Das M, Mahindrakar P, Das D, Behera SKS.Chowdhury R, Bandyopadhyay B. "Anomalous left coronary artery from pulmonary artery with mitral stenosis," Annals of Thoraic Surgery 2011; 92(2): 735-737.9. Bajona P, Maselli D, Dore R, Minzioni G. "Anomalous origin of the left main artery from the pulmonary artery: adult presentation with systemic collateral supply and giant right coronary artery aneurysm, "Y.Imai, T. Shin'oka M. Aoki and Iwata Y. "Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery: the Tokyo experience,AJ.In: Mandell GL, Bennett JE, Dolin R. Goldman: Cecil Medicine.23rd ed.Philadelphia, Pa: Saunders Elsevier; 2007:chap 68.