Extrinsic compression of the left main coronary artery (LMCA) secondary to pulmonary artery dilatation is a rare syndrome. Most cases of pulmonary artery hypertension but no atherosclerotic risk factors rarely undergo coronary angiography, and hence, diagnoses are seldom made and proper management is often delayed in these patients. We describe a patient that presented with pulmonary hypertension, clinical angina, and extrinsic compression of the LMCA by the pulmonary artery, who was treated successfully by percutaneous coronary intervention. Follow-up coronary angiography showed patent stent in the LMCA in the proximity of the dilated main pulmonary artery. This case reminds us that coronary angiography and percutaneous coronary intervention should be considered in pulmonary hypertension patients presenting with angina or left ventricular dysfunction.
Angina Pectoris/etiology ; Angioplasty, Balloon, Coronary ; Coronary Angiography ; Coronary Stenosis/radiography/therapy ; Coronary Vessels/radiography/*ultrasonography ; Dilatation, Pathologic ; Female ; Humans ; Hypertension, Pulmonary/etiology/radiography ; Middle Aged ; Pulmonary Artery/radiography/*ultrasonography ; Stents ; Tomography, X-Ray Computed ; Ventricular Dysfunction, Left

Cardiac resynchronization therapy (CRT) has been proven its value in adult patients with congestive heart failure of low ejection fraction and wide QRS duration. Contrast to adult patients, CRT has been rarely applied for young patients. We report on a 9-yr-old boy with progressive left ventricular (LV) dilatation and dysfunction following chronic VVI pacemaker therapy for congenital complete atrioventricular block associated with maternal anti-SSA/Ro and SSB/La antibody. His LV dysfunction was improved after epicardially established CRT.
Antibodies, Antinuclear/metabolism ; Atrioventricular Block/congenital/therapy ; Cardiac Pacing, Artificial/*adverse effects ; *Cardiac Resynchronization Therapy ; Child ; Chronic Disease ; Electrocardiography ; Heart Ventricles ; Humans ; Male ; Natriuretic Peptide, Brain/blood ; Sjogren's Syndrome/immunology ; Ventricular Dysfunction, Left/etiology/radiography/*therapy