Daniel Peck 1, Konstantin Averin 2, Philip Khoury 1, Grant Veldhuis 1, Tarek Alsaied 1 3, Adam M Lubert 1, Russel Hirsch 1, Wendy M Whiteside 4, Gruschen Veldtman 5, Bryan H Goldstein 1 3
J Am Heart Assoc. 2023 Jan 3;12(1):e026508. doi: 10.1161/JAHA.122.026508.
PMID: 36565206
Take Home Points:
- Rapid volume expansion during cardiac catheterization may unmask diastolic dysfunction.
- Single‐ventricle diastolic dysfunction, even subclinical in nature, may portend a worse clinical prognosis.
- However, the duration of Fontan physiology exposure coupled with ventricular diastolic dysfunction, rather than just diastolic dysfunction alone is associated with adverse clinical outcomes.
Commentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch:
Diastolic dysfunction has been recognized as a major contributor to heart failure in adults with and without impaired systolic function. Its effect is well studied in adults with biventricular physiology, but in a single ventricle post Fontan palliation, it is not. This study investigated the medium-term effect of occult diastolic dysfunction (ODD) on Fontan circulation and whether it contributes to the long-term risks of morbidity and mortality.
This is a retrospective study, examining 89 patients (47% female; 44% dominant right ventricle; median age, 14.0 years; median duration of Fontan: 10.6 years), with 31% showing evidence of ODD. ODD was unmasked by demonstrating a final ventricular end diastolic pressure (edp) of > 15mmHg after rapid volume expansion (RVE), in patients with normal edp during cardiac catheterization. Table 1 summarizes the baseline demography of the study cohort.
Over a median duration of 2.9 years, ODD was found to be more prevalent in patients with longer durations of Fontan circulation and higher baseline edp (Table 2). Composite adverse clinical outcome was met by 29 (35%) patients and was more frequently encountered in the ODD‐positive group (52% versus 26%; P=0.03) (Table 3). Death, the need for a ventricular assist device, or transplant was seen in 9 (11%) patients and occurred at a similar rate between ODD groups.
The presence of ODD was associated with an increased hazard for the composite adverse clinical outcome (hazard ratio, 2.68 [95% CI, 1.28–5.63]; P=0.02) (Figure 2). Fontan duration, baseline single‐ventricle EDP, and atriopulmonary Fontan type were also associated with an elevated risk for adverse clinical outcomes on univariate analysis. On multivariable analysis, after adjustment for the duration of Fontan circulation, no factors remained significantly associated with the primary outcome (Table 4). The cohort was subsequently stratified into subgroups based on the Fontan duration (<10 years or ≥10 years). In patients with Fontan duration < 10 years, no difference in survival was observed between those with and without ODD (Figure 3). However, patients with a Fontan duration of > 10 years were associated with poorer survival (Figure 4). A greater hazard of the composite adverse clinical outcomes was also seen in patients with Fontan duration > 10 years and ODD (HR, 2.57 [95% CI, 1.03–6.57]; P=0.04.
This study suggests that the presence of ODD may indicate a higher risk of clinical deterioration in Fontan patients, especially as they age. Long Fontan duration might be the primary hazard for adverse outcomes, reflecting the duration of Fontan circulatory physiology exposure. Although its cause has yet to be demonstrated, this article underscores the importance of screening for ventricular diastolic dysfunction as it may help in prognosis and guide management strategies. ODD’s presence might predict a progression to overt diastolic dysfunction or may simply be an indicator of future challenges without a direct cause-effect relationship.
