BACKGROUND Myocardial damage in myocarditis is mediated, in part, by immunological mechanisms. High-dose intravenous gamma-globulin (IVIG) is an immunomodulatory agent that is beneficial in myocarditis secondary to Kawasaki disease, as well as in murine myocarditis. Since 1990, the routine management of presumed acute myocarditis at Children's Hospital, Boston, and Children's Hospital, Los Angeles, has included administration of high-dose IVIG. METHODS AND RESULTS We treated 21 consecutive children presenting with presumed acute myocarditis with IVIG, 2 g/kg, over 24 hours, in addition to anticongestive therapies. A comparison group comprised 25 recent historical control patients meeting identical eligibility criteria but not receiving IVIG therapy. Left ventricular function was assessed during five time intervals: 0 to 7 days, 1 to 3 weeks, 3 weeks to 3 months, 3 to 6 months, and 6 to 12 months. At presentation, the IVIG and non-IVIG groups had comparable left ventricular enlargement and poor fractional shortening. Compared with the non-IVIG group, those treated with IVIG had a smaller mean adjusted left ventricular end-diastolic dimension and higher fractional shortening in the periods from 3 to 6 months (P = .008 and P = .033, respectively) and 6 to 12 months (P = .072 and P = .029, respectively). When adjusting for age, biopsy status, intravenous inotropic agents, and angiotensin-converting enzyme inhibitors, patients treated with IVIG were more likely to achieve normal left ventricular function during the first year after presentation (P = .03). By 1 year after presentation, the probability of survival tended to be higher among IVIG-treated patients (.84 versus .60, P = .069). We observed no adverse effects of IVIG administration. CONCLUSIONS These data suggest that use of high-dose IVIG for treatment of acute myocarditis is associated with improved recovery of left ventricular function and with a tendency to better survival during the first year after presentation.

Introduction: Fifteen-year survival after Fontan operation approaches 95%, but morbidities are increasingly prevalent. It is unknown how well cardiologists can predict which pts are at risk for major adverse events (MAE), but awareness is critical to enable appropriate counseling. We asked cardiologists the “surprise” question: would you be surprised if your patient has a MAE in the next year? Methods: We performed a prospective, multi-center study across New England. The attending cardiologist was asked the yes/no “surprise” question. Inclusion criteria included lateral tunnel or extracardiac Fontan and age >10 yrs; individuals were excluded for current transplant evaluation, history of Fontan conversion, or pregnancy. Baseline clinical data were collected. After 12 months, cardiologists were surveyed to assess for MAE (see Table) and multivariate analysis (MVA) was performed for those deemed to be at risk and those with MAE. Results: From 2018-19, 146 pts and 40 cardiologists were enrolled at 9 sites. 99/146 pts (68%) were predicted to be a “Good Fontan.” After 1 yr, 17 (12%) patients experienced a MAE (Table). The simple kappa coefficient of ability to predict MAE was 0.17 (95% CI 0.02-0.32), suggesting ability to predict MAE was only 17% better than random chance. In MVA of those at risk (47/146), diuretic/beta blocker use (p=<0.0001) and systolic dysfunction (p=0.0046) were associated with identified risk. MVA of pts who experienced MAE (n=17) found prior unplanned cardiac admission (p=0.0062), diuretic/beta blocker use (p=0.028) and significant AV valve regurgitation (p=0.049) to be associated with MAE. Conclusions: Cardiologists are only marginally able to identify which Fontan pts are at risk for MAE in the next year. There was overlap between factors associated with prediction of “at risk” group and MAE, namely use of diuretic/blocker and the linked systolic dysfunction/AV valve regurgitation, which merits close attention in follow-up and further study.

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