Challenges of Diagnosing Congenital Heart Defects — Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Abstract

Background. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) also known as Bland — White — Garland syndrome is a rare congenital heart defect that affects 1 in every 300 000 newborns, thus comprising 0.22% of all congenital heart defects and 0.4–0.7% of critical congenital heart defects. In case of a more favorable disease course, symptoms typically appear between the 1st and 2nd months after birth. The ECG may show typical signs of ischemia, myocardial infarction, and left ventricular hypertrophy. The EchoCG is more informative as it enables the visualization of coronary artery orifices. Surgical correction is the only treatment method for this heart defect.Case report.Patient G.S.V., one month of age, was admitted to the neonatal pathology unit. Based on the physical examination the patient’s condition was severe. The skin was pale with cyanosis of the nasolabial triangle. The respiration rate was accelerated (50–52 breaths per minute) with the indrawing of the intercostal spaces. The displacement of the apex beat 1 cm to the left of the left midclavicular line was revealed by palpation. The displacement of the left border of the relative cardiac dullness to the anterior axillary line was revealed by percussion. Upon auscultation, the first heart sound at the heart apex was decreased, and there was a blowing systolic murmur radiating to the left anterior axillary line. Upon examination the child was diagnosed with severe acute acquired non-rheumatic diffuse viral bacterial carditis. The lack of improvement in the child’s condition following the myocarditis treatment and the examination results were suggestive of the anomalous coronary artery. The child was transferred by emergency to the Penza Federal Center of Cardiovascular Surgery where the diagnosis was confirmed.Conclusion. Despite being rare, this congenital heart defect may be diagnosed in clinical pediatric practice. A thorough record of complaints and medical history is an important step in its early diagnosis, and the presence of signs of heart failure requires additional examination. In case of suspected myocardial lesions, presence of high troponin levels and other markers of myocardial injury in the blood, ECG signs of myocardial ischemia and lack of improvement despite the treatment conducted, pathologic changes in the coronary arteries must be ruled out.