Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study.

OBJECTIVES: The purpose of this study was to determine long-term outcome in adults with congenitally corrected transposition of the great arteries (CCTGA), with particular emphasis on systemic ventricular dysfunction and congestive heart failure (CHF). BACKGROUND: Patients with CCTGA have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and CHF being relatively common in older adults. METHODS: Retrospective analysis of records of 182 patients from 19 institutions were reviewed to determine current status and possible risk factors for systemic ventricular dysfunction and CHF. Factors considered included age, gender, associated cardiac defects, operative history, heart block, arrhythmias and tricuspid (i.e., systemic atrioventricular) regurgitation (TR). RESULTS: Both CHF and systemic ventricular dysfunction were common in groups with or without associated cardiac lesions. By age 45, 67% of patients with associated lesions had CHF, and 25% of patients without associated lesions had this complication. The rates of systemic ventricular dysfunction and CHF were higher with increasing age, the presence of significant associated cardiac lesions, history of arrhythmia, pacemaker implantation, prior surgery of any type, and particularly with tricuspid valvuloplasty or replacement. Aortic regurgitation (a previously unreported problem) was also relatively common in this patient population. CONCLUSIONS: Patients with CCTGA are increasingly subject to CHF with advancing age; this complication is extremely common by the fourth and fifth decades. Tricuspid (systemic atrioventricular) valvular regurgitation is strongly associated with RV (anatomical right ventricle connected to aorta in CCTGA patients; systemic ventricle in CCTGA) dysfunction and CHF; whether it is causative or a secondary complication remains speculative.

Alpha-adrenergic blockade in vasotonic angina: lack of efficacy of specific alpha 1-receptor blockade with prazosin.

To clarify the possible role of the alpha 1-adrenergic receptor in angina due to coronary artery spasm, a double-blind, randomized, placebo-controlled trial of the specific alpha 1-antagonist, prazosin, was performed. Six patients with vasotonic angina were studied, with efficacy measured by continuous electrocardiographic recording and the tabulation of chest pain and nitroglycerin usage. Despite plasma prazosin levels adequate to produce a six-fold shift in the response to phenylephrine, there was no significant difference in the number of ischemic episodes while taking prazosin (9.8 +/- 6.3 episodes/24 h) compared with placebo (10.5 +/- 6.9). There was also no difference in the length of ischemic episodes, which averaged 231 +/- 35 seconds with placebo and 231 +/- 33 with prazosin. Chest pain and nitroglycerin usage were not altered by prazosin. These data suggest that coronary artery spasm is not primarily caused by an effect on or an abnormality of the coronary vascular alpha 1-receptor.