Congenital atresia of the ostium of the left coronary artery. Diagnostic difficulty and successful surgical revascularization in two patients

Abstract

Brief communicationCongenital anomalies of the coronary arteries, occurring in 1-2% of the population 1 , are classified as anomalies at the origin (abnormal exit from the pulmonary trunk of the left coronary artery), terminal (coronary fistulae) and distributive (one coronary artery only) 2 . Congenital atresia of the ostium of the left coronary artery is a rather rare occurrence that is not even mentioned in the above-cited literature. Classically, it consists of total ostial atresia of the left coronary artery, possibly in association with atresia of the trunk of this vessel. Clinically, during the first months of life such patients may present with cardiac insufficiency (infantile form) or a delayed (including adults) picture of coronary insufficiency (adult form). We report below two patients of different ages and clinical presentations, having congenital atresia of the ostium of the left coronary artery of difficult diagnosis, who underwent successful surgical myocardial revascularization. Case reportsCase 1 -A nine-month-old boy from Divinolândia, SP, with a clinical history of tiredness on sucking, accompanied by discrete diaphoresis from the first month of life on. At six months of age, after a case of pneumonia, a sudden worsening of his condition occurred accompanied by cardiac insufficiency. A noninvasive investigation diagnosed dilated myocardiopathy. The patient showed significant improvement following medication, receiving a discharge from the hospital with a prescription of digoxin, furosemide and captopril. At nine months of age, he was referred to clinical examination at the Heart Hospital of Ribeirão Preto and he was found to be in excellent general condition (weight: 10 kg). On physical examination, he had a normal pulse and upon cardiac auscultation a ++/6 systolic murmur on the 2 nd -3 rd left intercostal space with normal heart sounds. Thoracic antero-posterior radiography showed significant cardiomegaly ( fig.1 upper side). The electrocardiogram showed unequivocal signs of anterolateral infarction ( fig. 2 A). In view of this consistent clinical evidence, an initial diagnosis of anomalous origin of the left coronary artery from the pulmonary atretik was made. Due to lack of evidence of an anomalous coronary artery on a previously performed echocardiogram, a diagnostic hemodynamic study was indicated. Depressed left ventricular function was observed during this procedure. The right coronary artery was dilated, and collateral circulation nourished the left coronary, which was atretic in its ostium. No shunting to the pulmonary artery was detected ( fig. 3). Once the diagnosis of congenital atresia of the ostium of the left coronary artery had been established, the infant immediately underwent surgical treatment by revascularization of the left coronary artery with a graft from the internal thoracic artery. The postoperative period was uneventfull, and 11 months following surgery the patient remains asymptomatic, in good general condition and under the use of digoxin. Thoracic radiography sh...

Congenital mitral regurgitation due to papillary muscle infarction: a case report and approach to evaluation

“…Two methods are described in children-coronary artery bypass graft using internal mammary artery and/ or saphenous vein graft and reconstruction of the left main coronary artery with aortic wall or autologous pericardial patch [1,5]. Long-term prognosis and the quality of life of the patients with this rare condition are unclear, especially in cases with mitral regurgitation, even if revascularization and mitral valve repair are successful.…”

Angor in a child: Left main coronary artery atresia

“…In infants, failure to thrive, acute myocardial infarction and congestive heart failure are the most frequent findings, whereas older children often present syncope and dyspnea (but we can also find some patients with angina and arrhythmias). Adults presentation was mainly angina, as well as dyspnea and myocardial infarction in some cases [1].We have found 3 asymptomatic patients in the literature [1][2][3] in the other hand, 4 patients of Musiani et al review [1] had suddenly died, and in two cases, it was the first symptom.Differential diagnosis includes: abnormal origin of the left coronary artery from pulmonary trunk, single coronary artery, and total obstruction of the left coronary artery [1].Revascularization therapy provides adequate flow to the left-sided arteries, and most authors recommend revascularization therapy in asymptomatic patients, due to the risk of 0167-5273/$ -see front matter D …”

“…This finding is of particular importance, as it usually generates critical myocardial ischemia, even myocardial infarction and sudden death, and given that surgical revascularization shows good results [1][2][3][4].A 3 month-old girl was referred to our unit presenting a cardiac murmur and failure to thrive. The electrocardiogram was within normal limits, and the echocardiography showed dilated left cavities, and a dysplastic mitral valve, with flaps prolapse, and severe regurgitation.…”

“…This finding is of particular importance, as it usually generates critical myocardial ischemia, even myocardial infarction and sudden death, and given that surgical revascularization shows good results [1][2][3][4].…”

Congenital atresia of the left main coronary artery: successful surgical treatment (myocardial revascularisation and mitral valve repair) in a 1-year-old boy

“…The variety of clinical symptoms of this condition depend on the degree of the collateral circulation between the right and left coronary arteries [1]. We report on a 1-year-old boy with congenital atresia of the LMCA and mitral insufficiency (MI), who successfully underwent two separate surgical procedures at the same time.…”