Assessing prognostic outcomes in cardiac sarcoidosis with advanced heart failure: How do current guidelines fare?

Cardiac sarcoidosis (CS) affects between 5 % to 25 % of systemic sarcoid cases. CS patients may present with heart failure (HF), with ultimate progression to advanced heart failure (AHF) associated with heightened mortality.

American guidelines emphasise using the 'I NEED HELP' criteria to identify AHF patients. The European Society of Cardiology (ESC) have alternative AHF diagnostic criteria. Both have demonstrated prognostic value, but their utility in prognosticating CS is unknown. This study aimed to address this.

109 patients, with baseline left ventricular ejection fraction (LVEF) <50 %, referred to the Royal Brompton Hospital between 2006 and 2019, were analysed. 48 patients had ≥1 'I NEED HELP' criteria consistent with AHF. Comparisons were made between the AHF and non-AHF CS patients. Sub-analysis was performed between the AHF patients that did or did not meet ESC-AHF criteria. Primary combined outcome measure was all-cause-mortality, urgent orthotopic cardiac transplant or urgent left ventricular assist device insertion. Secondary outcome measure was sustained ventricular tachycardia.

The AHF cohort had significantly lower LVEF and higher brain natriuretic peptide values. More AHF CS patients reached combined primary outcome measure (AHF 16/48 [33 %]), than the non-AHF group (8/61 [13 %]), p < 0.019. The AHF group had shorter time to both primary and secondary events on Kaplan-Meier analysis (logrank p < 0.014 and p < 0.040 respectively). Sub-analysis revealed the ESC-AHF group had the poorest prognoses according to both outcome measures.

In this study, both the AHA and ESC-AHF criteria had prognostic value. The ESC-AHF criteria best identifies CS patients with poorest prognoses.