Left main coronary artery compression in a young woman with Eisenmenger syndrome

Secondary pulmonary hypertension is a common finding in patients with congenital cardiac defects. Eisenmenger syndrome, a severe manifestation of pulmonary hypertension, affects quality of life, morbidity, and mortality. Patients usually present with cyanosis and reduced physical ability due to dyspnoea or sometimes chest pain on exertion. Nevertheless, coronary artery compression is an uncommon and probably underestimated cause for angina-like chest pain and ventricular dysfunction in patients with severe pulmonary artery hypertension. We report on an adolescent woman with unoperated ventricular septal defect and Eisenmenger syndrome who experienced myocardial infarction owing to a compression of the left main coronary artery (LMCA) by a distended pulmonary artery. A 16-year-old cyanotic woman was admitted to hospital because of severe chest pain during minimal exertion. On admission, she was in functional class III (New York Heart Association). She had a history of unoperated unrestrictive ventricular septal defect with a bidirectional shunt leading to Eisenmenger reaction. Clinical examination revealed a holosystolic heart murmur with fixed narrow splitting of the second heart sound. There were neither signs of acute heart failure nor arrhythmia. Electrocardiogram indicated regular sinus rhythm with delayed conduction, signs of biventricular hypertrophy as well as ST segment and T wave changes in the left precordial leads. Echocardiography showed a perimembranous malalignment-ventricular septal defect with a bidirectional shunt, biventricular hypertrophy and dilation with reduced biventricular systolic function, but no regional wall motion abnormalities (figure 1). Mild mitral regurgitation was assessed. Moreover, the pulmonary valve had a gradient of 28 mm Hg, and the …