Anomalous Origin of Coronary Arteries

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Veras FHAP, et al. Anomalous Origin of Coronary Arteries. Rev Bras Cardiol Invas 2007; 15(3).murmur in the mitral area, as a result of secondarymitral insufficiency to the increase of the cardiac areadue to its dilatation or due to dysfunction of the papillarymuscle 10,11 . Mortality during the first year of life isestimated at 90%, even with clinical treatment 10,11 .The adult or mature form can occur in childrenand necessarily in adults in around 10 – 15% of cases.It is characterized by an abundance of intercoronarycollateral circulation, of sufficient magnitude to allowa survival rate until adult age, in some cases, it hasbeen told, until 72 years of age.Sometimes, there are no appreciable clinical manifestationsand the diagnosis of OACEP is suspecteddue to the presence of murmurs: continuous, as aresult of the passage of blood through the coronarypulmonaryfistula, or systolic, because of dysfunctionof the mitral valve 10 . Some patients evolve towardscardiac insufficiency; others towards angina 10,11 .In the OACEA, some symptoms such as syncope,chest pain and palpitations may be present 12,28 . Butmost bearers are asymptomatic and the only expressionof this coronary anomaly in young athletes might besudden death 12,28 . The studies suggest that these coronaryanomalies can be lethal only during or immediatelyafter extenuating physical exercise, typically in youngsubjects 8 . (Table 1).COMPLEMENTARY EXAMSElectrocardiogram (ECG) and ergometric test (ET)One study demonstrated that the presence of the“Q” wave with a depth over 3 mm, duration higherthan 30 ms and a “QR” pattern in at least one of thedeviations – DI, aVL, V5-V7, was present in 100% ofexams of patients with OACEP 29 . The absence of the“Q” wave in the lower wall is also a prevalent finding(82% of cases) 13,15,29 . The axel deviation towards theleft is less frequent (64% of cases) 13 . Atypical electrocardiographicalterations can mask the illness whenassociated to lesions with important pulmonary hipoflux.In the OACEA, the ECG and the TE are almostalways normal 8,9,12,28 . In spite of the OACEA being thecause of myocardial ischemia, this is incidental, with manifestationsunder extenuating clinical conditions 8,9,12,28 .Thorax X-RayIn general, it shows alterations in the cases associatedto cardiac insufficiency. In the OACEP, young agedchildren can present an increase of the cardiac area 10 .Lung circulation is normal, however, when cardiacinsufficiency is present, a picture of venous pulmonarycongestion can be observed 10 .EchocardiogramThe bidimensional echocardiogram with coloredDoppler is useful in the morphofunctional assessmentof the heart. For the diagnosis of anomalous origin ofthe coronary arteries, the studies show a variablesensibility, with better results in children 11,13,15,26 .The transesophageal echocardiogram (TEE) is anexam with a much higher sensibility for the detectionof the anomalous origin of coronary arteries 1,26 . TheTEE, specially with the use of a multi-plane probe/catheter, is capable of drawing, with precision, theproximal course and the flux pattern in the anomalouscoronary arteries 1,26 .Computer Tomography of Coronary Arteries (CTCA)The studies show a quick evolution of this noninvasivemethod in the assessment of coronary arteries.Initially, the studies with the Electron Beam ComputedTomography (EBCT), demonstrated excellent accuracyin the assessment of the origin and proximal course ofcoronary anomalies 19,25 .With the advent of Computer Tomography withMultiple Detectors (16 and 64 acquisitions), a betterassessment of the middle and distal segments waspossible, for the detection of obstructive coronary arteryillness 16. It uses ionizing radiation and requires theuse of medication to diminish heart frequency 25. Themethod requires the administration of a contrast meansin the veins, which is potentially allergic and nephrotoxic.The data in the literature show it to be a safe andefficient method with excellent spatial resolution 16,24,25,30 .Comparative studies with coronary angiography haveshown a higher efficacy, suggesting that ComputerTomography with Multiple Detectors is the ideal examfor the detection of coronary anomalies 16,19 .TABELA 1Incidência de morte súbita relacionada a anomalias de artérias coronárias (adaptado de Angelini et al. 8 ).Grupo (idade) Número de mortes Mortes relacionadas a anomalias coronarianas,%Indivíduos fazendo exercício (8-66 a) 550 11População geral (< 40 a) 162 0,6Atletas competitivos(idade média de 17 a) 134 23Corredores (30-46 a) 120 1,6Indivíduos fazendo exercício, Maryland - EUA 62 04
Veras FHAP, et al. Anomalous Origin of Coronary Arteries. Rev Bras Cardiol Invas 2007; 15(3).Figure 2 illustrates a CTCA where an OACEA withan inter-arterial course is evidenced.Nuclear Magnetic Resonance (NMR)of the coronary arteriesCoronary angiography through MR is a noninvasiveexam, with high accuracy for the anatomicalidentification of the origin and proximal course of thecoronary arteries. The free choice of the image plane,with a trydimensional view, is an important advantageover the limited angle possibilities of conventionalcoronary angiography. It requires a non-nephrotoxiccontrast and does not use ionizing radiation 16 .It has the disadvantage of the long time of theexam (45-50 minutes), the need of periods of apnea,and the limitation of the analysis of the distal coronarysegments 19,31-34 .Bunce et al. 34 developed a technique for the detectionof coronary anomalies were there is no needof apnea and with a shorter time of exam (average time26 minutes).It is an exam that possibilitates cardiac morphofunctionalassessment, being of great usefulness in congenitalcardiopathies, since it allows a perfect spatialassessment between the coronary arteries, the greatvessels and all other cardiac structures 19,31-34 .Figure 3 illustrates a NMR diagnosing an athresiaof the TCE.CineangiocoronarographyIt is traditionally considered the ideal exam for thediagnosis of coronary anomalies. It is an invasive exam,which uses nephro-toxic contrast and ionizing radiation.The evolution of the RNM and of the TCAC, bothwith trydimensional analysis, have shown failures inthe CA for the diagnosis of coronary anomalies 19,31-33 .The CA might be incapable of differentiating, duringthe course through an anomalous coronary, the highrisk intra-arterial anatomy, from the septal anatomyretro-aortal or of the anterior wall, as well as the identificationof the coronary sinus and the morphology ofthe coronary ostium in the wall of the aorta 19,31-33 .The CA is still the best method for the assessmentof the distal coronary bed and associated arteroscleroticlesions 19,35,36 .TREATMENTOnce diagnosed, the OACEP must be surgicallycorrected immediately after the diagnosis, to avoidcomplications and sequels, characteristic of the naturalhistory of this illness 10,11,13,37-40 .The chosen surgical technique is the reimplantationwith translocation of the left coronary artery of thepulmonary trunk to the aorta, which is possible inmost cases 11,13,37-40 .In the case in which this technique is impossible toapply, “tunnelization” or Takeuchi Technique is recommended,which consists of creating a tunnel inside thepulmonary artery, to connect the left coronary arterywith the aorta 11,13,37-40 . Other surgical options are simplyto string or thread, to tie the left coronary artery with theaorta 11,13,37-40 . Surgical mortality for all the combinedFigura 2 - Origem anômala da artéria coronária esquerda com curso interarterial visualizada por TCAC. A: corte axial para avaliação da origemdas artérias coronárias. B: reconstrução tridimensional. As setas indicam o trajeto da artéria descendente anterior entre a aorta ascendentee o tronco da artéria pulmonar. A cabeça da seta indica a artéria coronária esquerda. Ao: aorta; AP: artéria pulmonar; AD: átrio direito; AE:átrio esquerdo(adaptado de Ropers et al. 24 ).5
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