Medicine and Health Sciences Commons^™

Is Biventricular Vascular Coupling A Better Indicator Of Ventriculo-Ventricular Interaction In Congenital Heart Disease?, Emily L. Yang, Shelby Kutty, Brian D. Soriano, Sathish Mallenahalli, Mark R. Ferguson, Mark B. Lewin, Sujatha Buddhe

Journal Articles: Pediatrics

BACKGROUND: Ventriculo-ventricular interactions are known to exist, though not well quantified. We hypothesised that the ventricular-vascular coupling ratio assessed by cardiovascular MRI would provide insight into this relationship. We also sought to compare MRI-derived ventricular-vascular coupling ratio to echocardiography and patient outcomes.

METHODS: Children with cardiac disease and biventricular physiology were included. Sanz's and Bullet methods were used to calculate ventricular-vascular coupling ratio by MRI and echocardiography, respectively. Subgroup analysis was performed for right and left heart diseases. Univariate and multivariate regressions were performed to determine associations with outcomes.

RESULTS: A total of 55 patients (age 14.3 ± 2.5 years) …

Intraventricular Septation In The Context Of Dilated Cardiomyopathy Associated With Ttn Mutation, Karl R. Khandalavala, Jean A. Ballweg, Stanley J. Radio, Jeffrey A. Robinson

Journal Articles: Pediatrics

A 6-month-old infant boy presented with symptomatic heart failure. Dilated cardiomyopathy was found in association with a mutation in TTN. Structural heart disease included novel septation of the left ventricle with a fenestrated membrane resulting from aberrant congenital mitral valve apparatus formation. (Level of Difficulty: Advanced.).

Williams Syndrome And Neonatal Cardiac Surgery For Congenital Single Ventricle, Taylor E. Katt, Robert L. Spicer, Anji T. Yetman, Ali N. Ibrahimiye, James M. Hammel, Jeffrey A. Robinson

Journal Articles: Pediatrics

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.).