Publications by authors named "D D Ivy"
Background: Right ventricular-arterial coupling (RVAC) describes the relationship between right ventricular contractility and pulmonary vascular afterload. Noninvasive surrogates for RVAC using echocardiographic estimates of right ventricular function, such as tricuspid annular plane systolic excursion (TAPSE), have been shown to correlate with invasively measured RVAC and predict clinical outcomes in pediatric pulmonary arterial hypertension. However, given the limitations of TAPSE at accurately estimating right ventricular function in children, we hypothesized that a multivariable estimate of RVAC using right ventricular free-wall longitudinal strain (RVFW-LS) may perform better than those utilizing TAPSE at predicting clinical outcomes.
View Article and Find Full Text PDFObjective: To characterize clinical, hemodynamic, imaging, and pathologic findings in children with pulmonary arterial hypertension (PAH) and variants in SRY-box transcription factor 17 (SOX17), a novel risk gene linked to heritable and congenital heart disease-associated PAH.
Study Design: We assembled a multi-institutional cohort of children with PAH and SOX17 variants enrolled in the Pediatric Pulmonary Hypertension Network (PPHNet) and other registries. Subjects were identified through exome and PAH gene panel sequencing.
Article Synopsis
- Pulmonary veno-occlusive disease (PVOD) is a rare pulmonary vascular condition that can be mistaken for pulmonary arterial hypertension (PAH), and they are believed to have different genetic causes.
- This report details a case of a woman diagnosed with PAH at 42, who unfortunately passed away despite treatment, and her son, who was diagnosed with PAH at 16 and required a heart-lung transplant due to worsening conditions.
- The autopsy of the mother showed evidence consistent with PAH, while the son's lung pathology confirmed PVOD, and neither had identifiable genetic mutations linked to these diseases.
The purpose of this study was to investigate neo-aortic curvature and diameter variation using the principal component analysis in patients who underwent a Norwood procedure for hypoplastic left heart syndrome. We further assessed whether neo-aortic curvature and diameter features are associated with clinical outcomes, single right ventricle function and flow hemodynamic patterns derived by 4D-Flow MRI. 55 patients with Fontan circulation who underwent a Norwood procedure in infancy underwent cardiac MRI as part of surveillance of their Fontan circulation.
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