BackgroundVentricular dyssynchrony and its relationship to clinical outcomes is not well characterized in patients following Fontan palliation.MethodsSingle-center retrospective analysis of cardiac magnetic resonance (CMR) imaging of patients with a Fontan circulation and age-matched healthy comparison cohort as controls. Feature tracking was performed on all slices of a ventricular short-axis cine stack. Circumferential and radial strain, strain rate, and displacement were measured; and multiple dyssynchrony metrics were calculated based on timing of these measurements (including standard deviation of time-to-peak, maximum opposing wall delay, and maximum base-to-apex delay). Primary endpoint was a composite measure including time to death or heart transplant listing (D/HTx); secondary outcomes were the presence of atrial or ventricular arrhythmias.ResultsA total of 503 cases (15y; IQR 10, 21) and 42 controls (16y; IQR 11, 20) were analyzed. Compared to controls, Fontan patients had increased dyssynchrony metrics, longer QRS duration, larger ventricular volumes, and worse systolic function. Dyssynchrony metrics were higher in patients with right ventricular (RV) or mixed morphology compared to those with LV morphology. At median follow-up of 4.3 years, 11% had D/HTx, 7% ventricular arrhythmia, and 38% atrial arrhythmia. Multiple risk factors for D/HTx were identified, including RV morphology, ventricular dilation, dysfunction, QRS prolongation, and dyssynchrony. Ventricular dilation and RV morphology were independently associated with D/HTx; ventricular dilation and global circumferential strain were independently associated with ventricular and atrial arrhythmias.ConclusionsMechanical dyssynchrony is highly prevalent in functional single ventricles palliated to the Fontan circulation and is more pronounced in hearts with RV or mixed ventricular morphology compared to those with LV morphology. Dyssynchrony is associated with death or need for heart transplantation and cardiac arrhythmias. These data add to the growing understanding regarding factors that can be used to risk-stratify patients with the Fontan circulation.
Introduction: Acute coronary syndrome in pediatric patients with graft-versus-host disease is rare.
Case Report: We present an 18-year-old male with graft-versus-host disease referred for chest pain, electrocardiogram abnormalities, and troponin leak. Additional risk factors for acute coronary syndrome included chronic steroid use, elevated triglyceride/low-density lipoprotein (LDL) levels and cannabis use. A catheterization revealed >90% occlusion of the left coronary artery and he underwent successful percutaneous intervention.
Conclusion: Given the overall rarity of acute coronary syndrome in pediatric patients, it may go unrecognized and underappreciated as a significant cause of morbidity in those with graft-versus-host disease.
Introduction:
The prevalence and significance of mechanical dyssynchrony is not well known in the Fontan population.
Methods:
Single-center, retrospective analysis of CMRs in Fontan patients compared to healthy controls. Feature tracking was performed on all slices of a short-axis cine stack and dyssynchrony index (DI) was defined as the standard deviation of time-to-peak circumferential strain for all segments. A composite outcome was defined as death, heart transplant listing, or new ventricular arrhythmias.
Results:
A total of 512 cases (17±9 y) and 42 controls (16±9 y) were included. Figure 1 depicts differences in EDV
i
, EF, DI, and QRS duration between the cohorts. DI correlated with EDV
i
(r=0.35; p<0.01), EF (r=-0.29; p<0.01), and QRS duration (r=0.29; p<0.01). At a median follow-up of 4.2 yrs, 10% had death or transplant listing and 4% had ventricular arrhythmias. RV dominance, EDV
i
, EF, DI, and QRS interval were associated with increased risk of the composite outcome (Table 1). Figure 2 demonstrates a significantly higher probability of the composite outcome in those with EDV
i
>120 ml/m
2
and DI>70 ms.
Conclusion:
Fontan patients have more mechanical dyssynchrony and longer QRS duration compared to controls. These abnormalities are more pronounced in RV or mixed ventricular morphology and are associated with a higher risk of ventricular arrhythmias, transplant listing, or death.
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