PMID- 35839154 OWN - NLM STAT- MEDLINE DCOM- 20220823 LR - 20240216 IS - 1522-1539 (Electronic) IS - 0363-6135 (Print) IS - 0363-6135 (Linking) VI - 323 IP - 3 DP - 2022 Sep 1 TI - Statistical shape modeling reveals the link between right ventricular shape, hemodynamic force, and myocardial function in patients with repaired tetralogy of Fallot. PG - H449-H460 LID - 10.1152/ajpheart.00228.2022 [doi] AB - Patients with repaired tetralogy of Fallot (rTOF) can develop chronic pulmonary insufficiency (PI) with right ventricular (RV) dilation, progressive RV dysfunction, and decreased exercise capacity. Pulmonary valve replacement (PVR) can help reduce the amount of PI and RV dilation; however, optimal timing remains controversial; a better understanding of rTOF pathophysiology is of fundamental importance to inform clinical management of patients with rTOF and optimal timing of PVR. In this study, we hypothesize a tight interplay between RV shape, intracardiac biomechanics, and ventricular function in patients with rTOF. To explore this hypothesis and derive quantitative measures, we combined statistical shape modeling with physics-based analysis of in vivo 4D flow data in 36 patients with rTOF. Our study demonstrated for the first time a correlation between regional RV shape variations, hemodynamic forces (HDF), and clinical dysfunction in patients with rTOF. The main findings of this work include 1) general increase in RV size, due to both volume overload and physiological growth, correlated with decrease in strain magnitude in the respective directions, and with increased QRS; 2) regional PI-induced remodeling accounted for approximately 10% of the shape variability of the population, and was associated with increased diastolic HDF along the diaphragm-to-right ventricular outflow tract (RVOT) direction, resulting in a net RV deformation along the same direction and decreased tricuspid annular plane systolic excursion (TAPSE); and 3) three shape modes independently correlated with systolic HDF and exercise capacity. Identification of patients based on the shape variations described in this study could help identify those at risk for irreversible dysfunction and guide optimal timing of PVR.NEW & NOTEWORTHY We combine statistical shape modeling with physics-based analysis of 4D flow data to elucidate the interplay between RV shape, hemodynamic forces, and clinical dysfunction in repaired tetralogy of Fallot. We are the first to show that ventricular remodeling is related to hemodynamic force magnitude and direction, global and regional functional parameters, and exercise intolerance. Identification of patients based on the shape variations described in this study could help identify those at risk for irreversible dysfunction. FAU - Kollar, Sarah AU - Kollar S AUID- ORCID: 0000-0002-3036-7407 AD - Division of Cardiology, Children's National Medical Center, Washington, District of Columbia. FAU - Balaras, Elias AU - Balaras E AD - Department of Mechanical and Aerospace Engineering, George Washington University, Washington, District of Columbia. FAU - Olivieri, Laura J AU - Olivieri LJ AD - Division of Cardiology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania. FAU - Loke, Yue-Hin AU - Loke YH AD - Division of Cardiology, Children's National Medical Center, Washington, District of Columbia. FAU - Capuano, Francesco AU - Capuano F AUID- ORCID: 0000-0003-0274-5260 AD - Department of Fluid Mechanics, Universitat Politecnica de Catalunya-BarcelonaTech, Barcelona, Spain. LA - eng GR - R01 HL143468/HL/NHLBI NIH HHS/United States GR - R21 HL156045/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20220715 PL - United States TA - Am J Physiol Heart Circ Physiol JT - American journal of physiology. Heart and circulatory physiology JID - 100901228 SB - IM MH - Heart Ventricles MH - Hemodynamics MH - Humans MH - *Pulmonary Valve Insufficiency/diagnostic imaging/etiology/surgery MH - *Tetralogy of Fallot/surgery MH - *Ventricular Dysfunction, Right/complications/etiology MH - Ventricular Function, Right/physiology PMC - PMC9394773 OTO - NOTNLM OT - hemodynamic forces OT - statistical shape modeling OT - tetralogy of Fallot OT - ventricular remodeling COIS- No conflicts of interest, financial or otherwise, are declared by the authors. EDAT- 2022/07/16 06:00 MHDA- 2022/08/24 06:00 PMCR- 2023/09/01 CRDT- 2022/07/15 13:23 PHST- 2022/07/16 06:00 [pubmed] PHST- 2022/08/24 06:00 [medline] PHST- 2022/07/15 13:23 [entrez] PHST- 2023/09/01 00:00 [pmc-release] AID - H-00228-2022 [pii] AID - 10.1152/ajpheart.00228.2022 [doi] PST - ppublish SO - Am J Physiol Heart Circ Physiol. 2022 Sep 1;323(3):H449-H460. doi: 10.1152/ajpheart.00228.2022. Epub 2022 Jul 15.