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Objective Speckle tracking echocardiography (STE) may be a useful modality for assessing ventricular performance in patients with single ventricle physiology. However, STE’s ability to accurately assess ventricular performance in this population is unknown. The objective of this study was to perform a preliminary comparison of STE measures of myocardial deformation to reference standard measures of function derived from pressure–volume loop (PVL) analysis. Design This was a secondary analysis of a prospective study investigating PVLs in patients with Fontan physiology. PVLs were recorded using microconductance catheters. PVL indices included end-systolic elastance (Ees), arterial elastance (Ea), ventriculo-arterial coupling (Ea/Ees), and the isovolumic relaxation time constant (tau). Patients were included if they had an echocardiogram within 1 month of their catheterization. STE was performed retrospectively using vendor independent software. Results Seventeen patients had echocardiograms available for analysis, 12 were right ventricular (RV) dominant. The median age was 8 years (IQR 5–17 years). Circumferential strain (r=−.72, P≤.01) and strain rate (r=−.61, P=.04) correlated with Ea/Ees in those with RV-dominant morphology. Longitudinal strain rate correlated with Ees in those with LV-dominant morphology (r=−.98, P≤.01). Longitudinal EDSR correlated with tau in those with LV-dominant morphology (r=−.90, P=.04). Conclusions In this limited sample, circumferential measures of deformation correlated with PVL measures better in patients with RV morphology, while longitudinal measures correlated better with PVL measures in patients with LV morphology. Further validation and investigation into the clinical usefulness of these measures are warranted.

We present the case of a 10-month-old female with a right coronary sinus of Valsalva aneurysm with rupture into the right atrial appendage who presented with a murmur. Surgical repair was performed shortly after diagnosis with pericardial patch closure from within the aorta and closure of the right atrial rupture site. To our knowledge, this is the youngest child with sinus of Valsalva aneurysm with rupture to be identified in the literature.

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