Echocardiographic Trajectories Of Long Term Outcomes Of Congenitally Corrected Transposition Of The Great Arteries With Biventricular Physiology
Razleen Brar, M.D.1, Amol Pande, PhD2, Miza Salim Hammoud2, Hani Najm, M.D.2, Tara Karamlou, M.D.2, Rukmini Komarlu, M.D.1.
1Cleveland Clinic Children's, Cleveland, OH, USA, 2Cleveland Clinic, Cleveland, OH, USA.
Objective(s):Congenitally corrected transposition of great arteries (ccTGA) is a rare, heterogeneous entity with variable timing of presentation. Surgical options for biventricular circulation are anatomic repair (AR)/physiologic repair (PR). Medical management (MM) can be used in pts with no major associated defects. Despite new approaches for surgical management, optimum strategies remain unclear. We addressed this critical knowledge gap by analyzing echocardiographic trajectories of long term outcomes in these 3 groups.
Methods:Single center retrospective analysis with prospective cross sectional follow-up of pts with ccTGA with biventricular physiology from 1995-2022 was performed. Demographic, surgical, echocardiographic variables were analyzed from presentation to last follow-up using time-varying coefficient model/gradient boosting approach (968 echocardiograms reviewed: AR: 462; PR: 338; MM: 227).
Results:Of 248 pts with ccTGA, 169 met inclusion criteria. 53 (31%) had pacemaker. 44 (26%) had PA band (AR: 31; PR: 13). In PR group, 10 (23%) had TV repair; 23 (54%) had TV replacement. AR group had highest freedom from moderate or greater systemic ventricular dysfunction/systemic AV valve regurgitation (AVVR). Worsening TR correlated with worsened RV function in PR/MM groups. Pacemaker influenced systemic AVVR more profoundly in PR/MM groups.
Conclusions:AR group had best long term outcomes with regards to systemic ventricular and AV valve function. Early AR should be preferred surgical approach in pts with suitable anatomy. Fallibilities of systemic RV/systemic TV were manifest in PR/MM groups.
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