Adults with repaired systemic biventricular congenital heart disease with a systemic left ventricle and heart failure
Mohamed Ellabbad1, Zeyad Kholeif1, Naser M Ammash1
1From the Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.
Insights
Guideline-directed medical therapy (GDMT) for heart failure (HF) in adults with congenital heart disease (CHD) significantly reduces hospitalizations and mortality. However, many patients do not receive optimal GDMT, indicating a need for improved treatment strategies.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Heart Failure Management
Background:
- Conflicting results exist regarding the clinical benefits of guideline-directed medical therapy (GDMT) for heart failure (HF) in adults with congenital heart disease (CHD).
- Previous studies lacked standardized criteria for assessing HF therapy adequacy in heterogeneous populations.
- This study focused on adults with repaired systemic biventricular CHD, systemic left ventricle, and HF with reduced ejection fraction (HFrEF).
Purpose of the Study:
- To assess the effect of GDMT, using a standardized GDMT score, on clinical outcomes in adults with specific types of CHD and HFrEF.
- To investigate the relationship between baseline GDMT and GDMT uptitration with HF hospitalization and mortality.
Main Methods:
- Defined HFrEF as stage B/C HF and systemic left ventricular ejection fraction <50%.
- Assessed GDMT score at baseline and 1-year follow-up, calculating GDMT uptitration (∆GDMT score).
- Utilized Cox regression to analyze the association between GDMT (baseline and ∆GDMT) and outcomes (HF hospitalization, mortality).
Main Results:
- Higher baseline GDMT scores and increased GDMT uptitration were associated with a reduced risk of HF hospitalization.
- Both higher baseline GDMT and increased GDMT uptitration correlated with lower mortality rates.
- Only 33% of patients achieved GDMT uptitration (∆GDMT score >0), suggesting suboptimal therapy.
Conclusions:
- GDMT use at baseline and uptitration are linked to improved outcomes in adults with CHD and HFrEF.
- A dose-dependent relationship exists between GDMT use and the risk of adverse cardiovascular events.
- A significant proportion of patients (67%) did not receive GDMT uptitration, highlighting opportunities for therapeutic improvement.
Background:
Results of observational studies assessing clinical benefits of guideline-directed medical therapy (GDMT) for heart failure (HF) in adults with congenital heart disease are conflicting. This is because these studies were based on a heterogenous population and lacked standardized criteria for assessing adequacy of HF therapy across studies. The current study addressed these limitations by studying the effect of GDMT (using a standardized GDMT score) in adults with repaired systemic biventricular congenital heart disease, systemic left ventricle, and HF with reduced ejection fraction.
Methods:
HF with reduced ejection fraction was defined as stage B/C HF and systemic left ventricular EF < 50%. GDMT score was assessed at baseline encounter (baseline GDMT score) and 1-year follow-up. GDMT uptitration was calculated as defined as ∆GDMT (∆=delta or change in) score from baseline. Cox regression was used to assess the relationship between HF therapy (baseline and ∆GDMT score) and outcomes (HF hospitalization and mortality).
Results:
Of 778 patients, baseline and ∆GDMT scores were 2 (1, 3) and 0.56 (0.49, 0.62), respectively. Of 778, only 258 (33%) had GDMT uptitration (∆GDMT score >0). Higher use of GDMT at baseline (adjusted hazard ratio [HR] per 1 point increase in GDMT score 0.83, 95% confidence interval [CI] 0.74, 0.92, P < .001), and increased use of GDMT over time (adjusted HR for 1 point increase in ∆GDMT score HR 0.71, 95% CI 0.62, 0.82, P < .001) were associated with lower risk of HF hospitalization. Higher baseline GDMT score (adjusted HR 0.87, 95% CI 0.76, 0.98, P = .01), and ∆GDMT score (adjusted HR 0.84, 95% CI 0.72, 0.86, P = .002) were also associated with lower mortality.
Conclusions:
GDMT use at baseline, and GDMT uptitration were associated with improved outcomes and suggest a dose-dependent relationship between use of GDMT and risk of adverse outcomes. Furthermore, 67% of patients did not receive GDMT uptitration, suggesting suboptimal therapy and opportunities for improvement.
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