Introduction: Timothy syndrome (TS) is a rare genetic disorder of the L-type cardiac channel Cav1.2 encoded by CACNA1C. The seminal report in 2004 characterized TS as a multisystem disorder, consis...
Abstract Patients with repaired tetralogy of Fallot (rTOF) are at risk of long-term left ventricular (LV) dysfunction associated with poor outcomes. In this study, we examined the association of LV end-diastolic (ED) shape with components of systolic wall motion (SWM) that contribute to global systolic dysfunction in an rTOF patient cohort. Features of LV shape associated with conicity and septal wall curvature correlated with components of SWM. The effect of ED shape perturbations on SWM were examined in a finite element analysis of systolic ventricular mechanics. Variations in the combination of ED shape and myocardial contractility were able to match predicted measures of SWM. From these results, we hypothesize that greater LV conicity and a flatter septal wall are markers of reduced global myocardial contractility and should be examined further for clinical prognostic utility to improve patient outcomes.
Introduction: Intermittent pre-excitation (I-PX) in patients with Wolff Parkinson White syndrome (WPW) is thought to indicate low risk of a life-threatening event (LTE), which is reflected by recen...
Congenital heart disease is the most common birth defect, with an incidence of 75 in every 1000 births. As a result of improved interventions, 90% of people with congenital heart disease now survive to adulthood. They must undergo regular imaging to assess their biventricular (left and right ventricular) function. Analysis of the images is problematic due to the large variety of shapes and complex geometry. In this paper we extend a biventricular modeling method to improve the analysis of MR images from congenital heart disease patients. We used a subdivision surface method to create three customizable exemplars, representing common manifestations of anatomy, and incorporated these as priors into an interactive biventricular customization procedure. The CHD-specific priors were tested on 60 cases representing a variety of congenital heart diseases for which the gold standard manual contours were available. The introduction of multiple priors showed a significant decrease in analysis time while maintaining good correlation between the two methods (R 2 >.82).
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