Unguarded Tricuspid Orifice with Congenitally Corrected Transposition of the Great Arteries and Aortic Atresia- A Highly Unusual Case

Unguarded tricuspid orifice is a very rare anomaly. It is characterised by the absence of one or more of the tricuspid valve leaflets resulting in severe tricuspid regurgitation and right heart failure. It is rarely an isolated anomaly but more often associated with pulmonary atresia and intact ventricular septum. When the ventricles are inverted however, the result of outflow tract obliteration is not pulmonary atresia, but aortic atresia. This anomaly has been described in the literature in only 2 cases so far. We present a case of a neonate with unguarded tricuspid orifice with absence of all tricuspid leaflets, congenitally corrected transposition of the great arteries and aortic atresia. The severe tricuspid regurgitation and right ventricular enlargement would have required a Norwood-like procedure combined with a right ventricular plication. Due to the complexity of this lesion no surgical therapy was attempted in consent with the parents.

Reduction in Right Atrial Pressures Is Associated With Hemodynamic Improvements After Transcatheter Edge-to-Edge Repair of the Tricuspid Valve

Background: Investigational transcatheter edge-to-edge repair (TEER) for severe tricuspid regurgitation (TR) has shown promise as an alternative to surgery, but factors influencing outcomes, optimal patient selection, and procedural timing remain incompletely defined. Given the limitations of determining TR severity by conventional echocardiography, our objectives were to determine whether invasive right atrial (RA) pressures performed during the procedure are related to patient outcomes. Methods: This study was a retrospective review of patients who underwent off-label tricuspid TEER using MitraClip (Abbott Vascular, Menlo Park, CA) for significant TR at a single institution. Intraprocedural mean RA pressure, RA peak V-wave, RA pressure nadir, and systolic increase in RA pressure (XV height) were recorded. Results: Thirty-eight patients underwent tricuspid TEER; 33 underwent concomitant mitral TEER for mitral regurgitation. The study cohort was 39% female with a mean age of 78.6±14.3 years. Median follow-up was 339 days (interquartile range, 100–601). Any reduction in mean RA pressure, RA peak V-wave, RA nadir, and XV height occurred in 74%, 82%, 45%, and 87% of patients, respectively. At 1 year, event-free survival was 47%. Postprocedure XV height correlated with TR severity as determined by echocardiography ( P <0.0001). The highest quartile of postprocedure XV height (>8 mm Hg) had worse event-free survival compared with those who had concluding XV height ≤8 mm Hg ( P =0.02). Attainment of a concluding XV height less than or equal to median value was associated with a lower creatinine the next day (1.27±0.47 versus 1.64±0.47 mg/dL, P= 0.04). Conclusions: Intraprocedural XV height correlates with TR severity after tricuspid TEER, and lower concluding pressures are associated with improved outcomes. Analysis of RA pressures may serve as a complementary tool for the evaluation of disease severity and procedural guidance.

Abstract 16851: Simultaneous Mitral Valve Replacement, Aortic Valve Replacement, Tricuspid Valve Repair, Left Atrial Appendage Ligation, Maze Procedure And Cabg In A 57-year Old Female With History Of Tavr And Severe Mitral Annular Calcification

Background: Open heart surgery is preferred for patients with multiple valvular issues with conflicting physiology. Case: A 57-year-old female with a past medical history of Type I Diabetes Mellitus complicated by kidney & pancreatic transplant in 1999 s/p failed kidney transplant in 2016 subsequently back on dialysis, aortic stenosis leading to Transcatheter Aortic Valve Replacement (TAVR) in 2016 who presented to the hospital for profound cardiogenic shock and was found to have severe tricuspid regurgitation, severe mitral stenosis secondary to mitral annular calcification with severe concentric hypertrophy of left ventricle. Decision Making: Her conflicting right and left heart physiology due to Tricuspid Regurgitation and Mitral Stenosis was very difficult to manage medically. She was not deemed a candidate for a heart transplant due to a history of a failed kidney transplant. She underwent surgery which included Mitral Valve Replacement, explant TAVR and repeat aortic valve replacement, Tricuspid repair, Left atrial appendage ligation and maze procedure, and CABG x1 with saphenous vein graft to PDA. She stayed on V-V extracorporeal membrane oxygenation (ECMO) post-operatively and was discharged on post-operative Day # 14. Conclusion: Severe tricuspid Regurgitation & coexisting mitral stenosis pose a dilemma for medical management and only feasible option is surgery which could be extremely challenging.

Neonatal Rupture of the Tricuspid Valve and Maternal Lupus: Is There a Correlation?

Valve dysfunction is not widely recognized as a feature in newborns born to mothers with positive anti-Ro/SSA antibodies, and only scarce reports have suggested an association between rupture of the atrioventricular valve tensor apparatus and these maternal antibodies. We report the follow-up from fetal life to the time of postnatal surgery of a patient with severe tricuspid regurgitation due to a flail of the anterior tricuspid valve leaflet who was born to an anti-Ro/SSA antibodies positive mother.

Renal and hepatic function of patients with severe tricuspid regurgitation undergoing inferior caval valve implantation

Due to progressive abdominal-venous congestion severe tricuspid regurgitation (TR) is a common cause of cardiorenal and cardiohepatic syndrome. We initiated the TRICAVAL study to compare interventional valve implantation into the inferior vena cava (CAVI) versus optimal medical therapy (OMT) in severe TR. In the present subanalysis, we aimed to evaluate the effects of CAVI on clinical signs of congestion, renal and hepatic function. TRICAVAL was an investigator-initiated, randomized trial. Twenty-eight patients with severe TR were randomized to OMT or CAVI using an Edwards Sapien XT valve. Probands who completed the 3-month follow-up (CAVI [n = 8], OMT [n = 10]) were evaluated by medical history, clinical examination, and laboratory testing at baseline, 3 and 12 months. After 3 months, the CAVI group exhibited a significant reduction of body weight (from 80.7 [69.0–87.7] kg to 75.5 [63.8–84.6] kg, p < 0.05) and abdominal circumference (from 101.5 ± 13.8 cm to 96.3 ± 15.4 cm, p ≤ 0.01) and a trend to lower doses of diuretics compared to OMT. Renal and hepatic function parameters did not change significantly. Within a short-term follow-up, CAVI led to an improvement of clinical signs of venous congestion and a non-significant reduction of diuretic doses compared to OMT.