Abstract 4369193: Burden of Heart Failure in 4,573 Children and Adults with Fontan Physiology: Epidemiology and Outcomes from a Decade-Long Seven-State US-Based Surveillance Network

The prevalence of heart failure (HF) among U.S. patients (pts) with Fontan physiology is poorly defined, with estimates generally derived from single centers. We leveraged a 7-state administrative dataset—the largest single ventricle (SV) cohort to date—to estimate HF prevalence across the lifespan and its associated comorbidities. This is a study from the CDC-funded STAR1 registry of CHD pts ages 0-45. All ICD diagnostic/procedural codes from 2010-2019 were collected. SV pts ≥5 yrs (presumed post-Fontan) were identified via ICD-9/10 codes associated with SV physiology and Fontan palliation. HF of any type was identified via ICD9/10 query. To depict HF burden over the lifespan (Figure 1), HF prevalence was calculated per 5-year age range via the following: total number of pts with HF coded in an encounter for that age range divided by the total number of pts at risk in that age range. Of the 4,753 Fontan pts identified, 2,836 (60%) were 5-17 years old (yo), 1,358 (28%) were 18-30 yo and 559 (12%) were ≥31 yo at first encounter. Diagnoses included hypoplastic left heart in 1,752 (37%), tricuspid atresia in 1,945 (41%), and SV not otherwise specified in 1,056 (22%), of which 36%, 41% and 46% were adults, respectively; 168 SV patients were >45 yo by end of the surveillance period. Overall, 1,401 (29%) had a diagnosis of HF at any time. HF prevalence increased over the lifespan, ranging from 8.3% in patients 5-9 yo to 58.3% of pts ≥45 yo ( ). HF patients had higher rates of cardiac and non-cardiac comorbidities than their non-HF peers ( ). Traditional modifiable cardiovascular risk factors, including hypertension, hyperlipidemia, smoking, and diabetes, were significantly higher among pts with HF across all ages. Heart transplant occurred in 22% of all HF pts (occurring in 30% of 5-17 yo, 16% of ≥ 31 yo, p < 0.05) vs. 0.2% in non-HF pts. 231 (16%) of HF pts died (mortality rate 184 deaths/10,000 person-years) vs. 81 deaths among 3,352 (2%) pts without HF (mortality rate 28 deaths/10,000 person-years, p < 0.05). In the largest Fontan cohort studied to date, ~1/3 were diagnosed with HF at any time, with steady increase to >50% after age 45. The HF cohort had a significantly higher burden of comorbid disease, including potentially targetable risk factors. This emphasizes the need for close surveillance and early diagnosis in this complex patient population.

Duke Scholars

Author Richard Andrew Krasuski Medicine, Cardiology