Long-term functional outcome of patients following the mustard procedure: the next decade of follow-up.
BACKGROUND
Nearly 10 years ago, we studied a population of patients with d-transposition of the great arteries (DTGA) who had undergone the Mustard procedure 8-26 years earlier. The present study was undertaken to determine how that cohort of patients is currently functioning.


METHODS
Of the 45 original patients, 44 were located. Six (13%) had either died (n = 4) or received a cardiac transplant (n = 2). In total, 35 of the remaining 38 patients (78%) chose to undergo testing. Systemic right ventricular ejection fraction (RVEF) was estimated using radionuclide angiocardiography. Exercise stress testing, echocardiography, Holter monitoring, and a quality of life questionnaire were also performed.


RESULTS
Those 6 that died or received cardiac transplantation did so between 16 and 25 years of age. The surviving participants ranged in age from 19 to 37 years. Peak oxygen consumption was significantly diminished at 27.7 +/- 6.9 mL/kg/min. Comparison of exercise duration from the original study demonstrated a significant decrease (11.2 +/- 2.2 minutes to 9.1 +/- 2.9 minutes) (P < 0.001). Comparison of RVEF data from the original study did not demonstrate a significant decrease (0.54 +/- 0.10 to 0.53 +/- 0.10) (P = 0.27). Quality of life data suggested that Mustard patients do not score as well in physical functioning, general health, and level of energy as normal adults, but are comparable with adult patients with other chronic diseases. Mustard patients are similar to normal adults and feel better than other adults with chronic illness in their interpretation of social functioning and bodily pain.


CONCLUSIONS
Cardiac mortality of 13% occurred during the second and third decade of life in this cohort of patients with DTGA palliated by the Mustard procedure. Despite continuing deterioration in exercise performance, right ventricular function, and cardiac rhythm, many surviving patients with DTGA continue to lead normal lives into the 4th decade after Mustard procedure.
Ebenroth
Eric S
ES
James Whitcomb Riley Hospital for Children-Pediatric Cardiology, Indianapolis, IN, USA. eebenrot@iupui.edu
Hurwitz
Roger A
RA
D000328:Adult; D015331:Cohort Studies; D004452:Echocardiography; D005080:Exercise Test; D017079:Exercise Tolerance; D005260:Female; D005500:Follow-Up Studies; D006339:Heart Rate; D006801:Humans; D008297:Male; D011237:Predictive Value of Tests; D011788:Quality of Life; D013318:Stroke Volume; D014188:Transposition of Great Vessels / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology* / Q000601:surgery*; D016278:Ventricular Function, Right