Abstract 14882: Outcomes of Heart Failure in Hypoplastic Left Heart Syndrome and the Impact of Recovery

Abstract

Introduction: Survival for children with hypoplastic left heart syndrome (HLHS) and right ventricular (RV) dysfunction (dysfxn) remain poor. We describe outcomes of HLHS patients with RV dysfxn and the long-term impact of interval normalization. Methods: The SickKids Heart Failure (HF) Database is a retrospective cohort of HF patients from 2001 to 2017. We included patients with HLHS and at least mild RV dysfxn on echocardiogram lasting >30 days, or death or transplant within 30 days of onset of dysfxn. We defined normalization as normal RV function (fxn) lasting > 30 days after dysfxn onset. The primary endpoint was death or transplant. Descriptive statistics and time to event analysis were used. Results: We identified 99 patients with a median (IQR) age at onset of dysfxn of 2.6 (1 - 5.3) months. Of these, 51 (52%) had normalization of RV fxn for a median (IQR) duration of 3.5 (0.8 - 9.1) years and 28 (55%) had multiple periods of normal fxn. Of those who normalized, 4 (8%) normalized within a few months post Glenn procedure and 6 (13%) had normal fxn at last follow up. Patients with and without normalization had similar frequency of severe dysfxn (41 vs 52%, p=0.6). Normalization of RV fxn correlated with improved transplant free survival (78 % vs. 14 %, p<0.001, Figure 1C) independent of surgical stage (p=0.03, figure 1D). Table 1 shows risk factors independently associated with transplant-free survival, including angiotensin converting enzyme inhibition. Conclusions: HLHS patients with RV dysfxn have poor transplant-free survival, especially those at interstage, but outcomes improve in those with persistent normalization of fxn. Factors predicting normalization need further study