Heart on the Heart
A 44-year-old woman presented with exertional dyspnea and atypical chest pain. On physical examination, severe kyphotic deformity was noted. The ECG demonstrated right-axis deviation and right ventricular hypertrophy. Echocardiography demonstrated normal left ventricular systolic function, no segmental wall-motion abnormality, and mild pulmonary hypertension. The pulmonary function test showed severe restrictive disease. An exercise treadmill test was attempted but terminated at stage 1 because of severe dyspnea. Coronary angiography was performed. A left anterior oblique-caudal view of left coronary angiography with a JL4 catheter showed no stenosis on the epicardial coronary arteries. The left anterior descending artery and left circumflex artery made a “heart” on the heart because of the counterclockwise rotation and the anterior displacement of the cardiac apex (Figure). Results of right coronary angiography were normal. The presumptive diagnosis was severe restrictive disease due to chest wall deformity.
The online-only Data Supplement, which contains two supplemental figures, can be found at http://circ.ahajournals.org/cgi/content/full/112/15/e262/DC1.