Author + information
- Warren G. Guntheroth, MD⁎ ()
- ↵⁎Department of Pediatrics (Cardiology), University of Washington School of Medcine, Seattle, WA 98195-6320
The report by Tsirka et al. (1) provided a very useful update of the outcomes for infants and children who develop dilated cardiomyopathy (DCM). Their use of a category combining death and cardiac transplant (“heart death”) as end points was sensible and helpful. However, some of the diagnostic categories were less than fully explained and somewhat arbitrary. If patients with muscular dystrophy (n = 4) and inherited metabolic disorders (n = 5), both uniformly fatal diseases, were not included, their total population of DCM would be only 82 instead of 91, and the population of survivors with recovery of normal contractility would amount to 40% of that population. This raises a serious question about proceeding to transplantation in the first year of the disease, as four centers have advised on the basis of their perception that DCM had such a poor prognosis. In this published cohort, some recovered as late as six years after onset; would 40% of the transplanted children have recovered with normal contractility had they not been transplanted?
Although the title of their study asserts that transplantation for DCM will provide “improved outcomes,” that can only apply to the first few years after cardiac transplantation, which lasts on average 10 to 13 years. In contrast, 40% of all patients with DCM recovered systolic function without transplantation and were still normal eight years later. The difference between transplants and complete recovery does not take into account the morbidity of rejections and anti-immune treatments for the transplanted patients. If 40% of the transplanted children would have recovered, the overall outcome would certainly not be improved.
Finally, the reliability of separating myocarditis from idiopathic DCM is raised by their finding of no differences between the two groups for either “heart death” or for recovery of function. This important finding throws doubt on the criteria used to diagnose myocarditis.
In sum, this important study by Tsirka et al. (1) provided several important advances in our knowledge, even though the title made promises that were unwarranted.
- American College of Cardiology Foundation