Figure 3: Right coronary artery angiograms showing right dominant coronary artery circulation, with a posterior descending artery supplying the inferior aspect of the interventricular septum. A: left anterior oblique view. B: right anterior oblique view.

Mentions: A 20-year-old man presented to the emergency department with two hours of persistent substernal chest pain, which started after heavy alcohol consumption. He had experienced dyspnea on exertion for the past two years. He was a seven pack-year smoker, but he had no other significant clinical history. Initial physical examination was normal, and ECG showed ST-segment elevation with Q waves in the anterior precordial leads (Fig. 1). Cardiac enzymes were normal, and echocardiography showed a dilated left ventricle (LV) with apical dyskinesia and moderate LV dysfunction (EF=33%). Emergent coronary angiography showed no atherosclerotic lesion, but did reveal hypoplasia of the mid-to-distal left anterior descending (LAD) artery without compensatory collateral vessels supplying the apex (Fig. 2). A myocardial bridge was noted at the mid-LAD. The other coronary arteries were unremarkable and of normal size. A posterior branch of the right coronary artery (RCA) supplied the inferior aspect of the interventricular septum (Fig. 3). Intracoronary infusion of nitroglycerin did not change the diameter or the morphology of any of the coronary arteries (Fig. 4).

Sim DS, Jeong MH, Choi S, Yoon NS, Yoon HJ, Moon JY, Hong YJ, Kim KH, Park HW, Kim JH, Ahn Y, Cho JG, Park JC, Kang JC - Korean Circ J (2009)

Bottom Line: Sixty-four-slice cardiac CT and cardiac MR imaging demonstrated thinning of the apical wall with calcification and delayed enhancement, supporting the diagnosis of long-standing MI.The patient was discharged symptom-free on medication for ischemic heart failure two weeks after admission.Although HCAD is very uncommon, it should be considered in children and young adults who suffer MI or sudden cardiac death.

Affiliation: The Heart Center of Chonnam National University Hospital, Cardiovascular Research Institute of Chonnam National University, Gwangju, Korea.

Abstract: Hypoplastic coronary artery disease (HCAD) is a rare condition that may lead to myocardial infarction (MI) and sudden death. We discovered HCAD in a young man who developed chest pain after heavy drinking and who was found to have suffered an MI. His ECG showed ST-segment elevation with Q waves in the anterior leads, and echocardiography revealed apical dyskinesia with moderate left ventricular (LV) dysfunction. Coronary angiography showed hypoplasia of the left anterior descending (LAD) artery. (99m)Tc-tetrofosmin-gated myocardial perfusion scintigraphy showed a large, fixed perfusion defect in the anteroseptal and apical segments. Sixty-four-slice cardiac CT and cardiac MR imaging demonstrated thinning of the apical wall with calcification and delayed enhancement, supporting the diagnosis of long-standing MI. The patient was discharged symptom-free on medication for ischemic heart failure two weeks after admission. Although HCAD is very uncommon, it should be considered in children and young adults who suffer MI or sudden cardiac death.