Page 27 - Journal of Structural Heart Disease Volume 3, Issue 5

153
Case Report
metabolic acidosis (pH = 6.7 and lactate = 17 mmol/L). Echocardiography showed akinetic left ventricle and re- duced contraction of the right ventricle. Due to severe left-sided cardiogenic shock, extracorporeal heart lung assist with catheters through the left femoral artery and vein was initiated. Coronary angiography revealed se- vere stenosis of the left main stem and anomalous ori- gin of the left coronary artery rising from the opposite sinus and running between the pulmonary artery and aorta. The right coronary artery had a normal course. The lesion in the left main coronary artery was treated with a 3.0/18-mm drug eluting stent (Figure 1), and TIMI 3 ow was achieved. Although left ventricle function was slightly improved, left ventricle ejection fraction was still severely reduced.
In the intensive care unit, the patient was treated with milrinone, norepinephrine, and epinephrine in the following hours, but he died due to cardiogenic shock with multiple organ failure and irreversible is- chemic damage to his legs.
Autopsy showed an anomalous left coronary artery originating from the opposite sinus of Valsalva close to the semilunar valve, and a failed attempt to probe the lumen due to increased resistance revealed a slit- like ori ce narrowing the coronary ostium. The left coronary artery had a short intramural course inside the aorta vessel wall and continued in an inter-arte- rial course between the aorta and pulmonary artery (Figure 2). There was ischemic damage to both ventri- cles, likely associated with the coronary abnormality. The heart was of normal size and weight, and the right coronary vessel showed a normal course. There was no evidence of arteriosclerosis, thrombosis, or stenosis in the coronary arteries in the non-stented areas.
Examination of the patient’s pediatric medical records showed that echocardiography (Figure 3) was performed at 4 years of age on the suspicion of cor- onary abnormalities described in the patient’s adop- tion information papers, which did not clarify which diagnostic tools were initially used to investigate the suspected coronary abnormalities. The echocardiog- raphy was performed by a pediatric cardiologist, and its results were described as normal with no coronary abnormalities. The indication for the echocardiogra- phy was unknown, and the patient had no history of chest pain or symptoms suggesting episodic coro- nary ischemia in early or late childhood.
Discussion
Anomalous coronary origin is a rare condition that can result in sudden cardiac death, most frequently when the coronary artery courses between the aor- ta and pulmonary artery. In active young adults with anomalous coronary origin, sudden cardiac death can be caused by exercise-induced ischemia [3]. The exact pathophysiological mechanism is not fully un- derstood but could be related to the compression, torsion, or kinking of the coronary artery when it courses between the great vessels, particularly when blood pressure and heart rate increase [2]. Coronary artery spasms have also been mentioned as having an important role in the pathogenesis of myocardial infarction [4].
Attention de cit hyperactivity disorder is a com- mon behavioral disorder in children and adolescents. The treatment consists of both stimulant drugs such as methyphenidate and amphetamine and non-stim- ulant drugs such as atomoxetine. Recently, however, the safety of these medications has come into ques- tion [5]. Atomoxetine elevates diastolic and systolic blood pressure and increases heart rate but does not signi cantly increase incidents of myocardial infarc- tion and sudden death in normal people [6].
In the present case, both angiography and autopsy revealed that the left coronary artery originated from the opposite sinus of Valsalva with an intramural and inter-arterial course between the aorta and pul- monary artery. Because there were no macroscopic traces of compression of the artery or thrombosis in our case, it was not possible to determine the exact cause of the sudden obstruction of the artery, but the anomalous origin of the left coronary artery with a short intramural and subsequent inter-arterial course combined with coronary spasm may have played a signi cant role [4]. Thus, the patient’s abnormal cor- onary artery anatomy, atomoxetine treatment, and coronary artery spasm provoked by cold water could together have caused the heart attack.
We re-evaluated the echocardiogram obtained when the patient was 4 years old. In the parasternal short-axis view, the left main coronary artery became visible close to the aorta at 3 o’clock after its course between the pulmonary artery and aorta and then split into left anterior descendent and left circum ex
Hansen, K. N. et al.
Anomalous Coronary Artery Origin and Heart Attack