Key result
Long-term follow-up of patients with DTGA post-Mustard procedure showed a significant decrease in exercise duration (11.2 to 9.1 minutes, P<0.001) and a 13% rate of death or cardiac transplant.
Why the study?
What are the long-term functional outcomes, survival, and quality of life in patients with d-transposition of the great arteries who underwent the Mustard procedure?
Cohort (n=45)
What are the long-term functional outcomes, survival, and quality of life in patients with d-transposition of the great arteries who underwent the Mustard procedure?
Despite a 13% mortality/transplant rate and declining exercise performance over time, many DTGA patients palliated by the Mustard procedure maintain stable right ventricular function and lead normal lives into their fourth decade.
Answers are grounded in this paper and cite their sources.
Declining exercise capacity warrants closer surveillance in Mustard patients; extends outcome data but leaves optimal intervention thresholds open.
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. CONCLUSION: 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.
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Ebenroth et al. (2007) conducted a cohort in d-transposition of the great arteries (DTGA) (n=45). Mustard procedure vs. Original study baseline was evaluated on Exercise duration (minutes) (p=<0.001). Long-term follow-up of patients with DTGA post-Mustard procedure showed a significant decrease in exercise duration (11.2 to 9.1 minutes, P<0.001) and a 13% rate of death or cardiac transplant.
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