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https://www.selleckchem.com/products/guggulsterone.html Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare lesion with wide anatomic variability. A rare variant exists, marked by right-sided dilatation, an Ebsteinoid tricuspid valve (TV), and severe tricuspid regurgitation. Neonatal cone valvuloplasty allows for a biventricular circulation and avoids complications of prosthetic valves; however, this technique inevitably requires reintervention to exchange the RV-PA conduit as the patient grows. We present a successful complete repair cone tricuspid valvuloplasty in a 9-day-old (2.8 kg) neonate with trisomy 21, PA/IVS, and an Ebsteinoid TV. Valve-sparing root replacement (VSRR) is more challenging with eccentric aortic insufficiency (AI) due to cusp and root asymmetry, which may impact valve durability and survival. This study analyzed the effect of jet eccentricity on long-term outcomes in tricuspid (TAV) and bicuspid (BAV) valves. From 2005-2019, 111 patients (65 TAV, 46 BAV) with >2+ AI underwent VSRR at an academic center. Pre- and post-operative echocardiograms were analyzed. Of these, 32 patients presented with concentric jets (29 TAV, 3 BAV) and 71 with eccentric jets (28 TAV, 43 BAV). Median (IQR) follow-up was 49 (12-93) months. Kaplan-Meier analysis and cumulative risk were used to compare long-term survival and valve-related reintervention. The mean age was 44 ± 12 years. Compared to TAV, more BAV patients presented with eccentric jets (93.5% vs 43.1%, p<0.001). All BAV patients received cusp repair, compared to 52.3% of TAV patients (p<0.001). At 3-, 5-, and 10-years, the cumulative risk of AVR for TAV (4.7%, 6.4%, and 6.4%) versus BAV (5.8%, 7.8%, and 7.8%) patients (p=0.87) and concentric (0%, 0%, and 0%) versus eccentric (6.4%, 9.4%, and 9.4%) jets (p=0.98) were similar. Overall survival at 10-years was 71% for TAV and 97% for BAV (p=0.19) and 86% for concentric and 79% for eccentric jets (p=0.17). In patients presenting for VSRR with

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