Abstract No. 182 Evaluation of adjunctive collimated C-arm computed tomography in adrenal venous sampling and its effect on successful selective catheterization

Abstract

Pulmonary arteriovenous malformations (PAVMs) are low-resistance, high-flow vascular structures that create a shunt in the pulmonary circulation. This shunting affects regional and systemic hemodynamics in Hereditary Hemorrhagic Telangiectasia (HHT). We hypothesized that this could lead to differential cardiac presentations in this setting. Thus, we investigated the cardiac structural and functional differences between HHT patients with PAVMs (HHT-P) compared to other presentations of HHT (HHT-O). Materials and Methods: We conducted a retrospective, crosssectional, single-center study of HHT patients who underwent transthoracic echocardiograms (TTE) between 2005 and 2020. HHT diagnosis was determined by Curacao score of !3 or positive genetic testing. PAVM diagnosis was made by angiography. Results: 176 HHT-O and 125 HHT-P patients were included. HHT-O patients had severely dilated right ventricles (RVIDD) and reduced tricuspid annular plane systolic excursion (TAPSE), a parameter of right ventricular (RV) function. In the left heart, HHT-O patients had larger left atriums (LASD) and increased mitral E/e' ratios, signifying elevated left sided filling pressures. HHT-O patients also had larger left ventricular diameters (LVEDD), lengths (LVEDL), and septal and posterior wall thicknesses (IVSd and LVPW). Cardiac output (CO) and stroke volumes were not significantly different (Table ). Conclusions: In our study, HHT patients without PAVMs had differences in right and left ventricular structure and function suggestive of a potential trend toward adverse remodeling as indicated by large right ventricles and thicker walls. PAVMs may therefore have a cardioprotective effect in HHT. Longitudinal studies should investigate these changes in cardiac structure/function to understand potential cardiovascular effects of PAVM embolization in this setting.