Tricuspid valve surgery in patients with a systemic right ventricle Roderick Scherptong, Hubert Vliegen, Michiel Winter, Barbara Mulder, Ernst van der Wall, Dave Koolbergen, Mark Hazekamp Eduard Holman, Leiden University Medical Center and Amsterdam Medical Center Netherlands
Background: Right ventricle that sustains systemic circulation Associated conditions: 1. Mustard or Senning correction for transposition of the great arteries (d-tga) 2. Congenitally corrected transposition of the great arteries (l-tga)
Tricuspid valve insufficiency in systemic right ventricle Tricuspid insufficiency is common Severe insufficiency is associated with RV failure and decreased survival Mechanisms of insufficiency: Annular dilatation Leaflet tethering Structural anomalies of the valve (Ebstein/ post VSD closure)
Surgical options 1. Repair/ replacement of the tricuspid valve 2. Restoration of the left ventricle into systemic position (high risk, bad results in adults) 3. Pulmonary artery banding (shifts septum towards the RV) 4. Cardiac transplantation
Tricuspid valve repair or replacement Several papers report on the association of tricuspid regurgitation and progressive RV failure Literature about the benefits of tricuspid valve surgery is very scarce and it is unclear whether repair of the tricuspid valve is beneficial
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle Patients: Right systemic ventricle More than moderate tricuspid regurgitation Functional impairment Tricuspid valve repair or replacement (1998-2009) Evaluation: Post-operative complications Survival: Maximum follow-up, death or recurrent TR TR, RV function: Echocardiography Functional class: NYHA
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle ALL patients had previous surgery TGA: Senning/ Mustard/ VSD closure cctga: VSD closure, relief of pulmonary valve stenosis Pacemaker in 6 Mean age at tricuspid valve surgery: 35,7 ± 12,8 years
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle Preoperative data RV function mildly to moderately severe decreased in all Tricuspid insufficiency: mean grade 3,2 ± 0,8 Mechanism of tricuspid insufficiency: Annular dilatation Leaflet tethering Ebsteinoid valve Structural valve damage (after VSD closure)
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle 19 patients 11 with congenitally corrected TGA 8 with TGA and Senning or Mustard operation Tricuspid valve repair: 11 Tricuspid valve replacement: 8 Associated procedures very common: Baffle repair (Senning/Mustard) Pulmonary artery banding Pacemaker, ICD, surgical AF ablation Mitral valve repair, CABG, residual VSD closure
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle Mortality N=3 ( 16 % ) 109, 180 and 659 days after surgery Reoperation in 1 ( 5 % ) Tricuspid valve replacement after failure of repair Postoperative implantation of pacemaker/ ICD in 4 ( 21 % ) Important postoperative complications 10 ( 52 % ) Low cardiac output, IABP Renal failure, temporary dialysis arrhythmias
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle mean follow-up: 4,6 ± 3,3 years (0,3 12 years) Follow-up complete in all
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle Tricuspid insufficiency improved from preoperative 3,2 ± 0,8 to postoperative 0,9 ± 1,0 ( p= 0,001) Mild to moderate residual tricuspid insufficiency was common after tricuspid repair Main cause: leaflet tethering due to dysfunctional and dilated RV
Leiden/ Amsterdam experience with tricuspid valve surgery in patients with a systemic right ventricle RV function remained unchanged overall No difference between repair and replacement Deterioriation to bad RV function was observed in 3 patients after 8, 10 and 11 years Clinical condition improved overall from preoperative NYHA class 2,7 ± 0,6 to 2,1 ± 0,8 after surgery ( p= 0,007)
Discussion Tricuspid regurgitation is a common long-term complication in patients with a right systemic ventricle Repair or replacement is technically/ practically the most feasible surgical option High incidence of post-operative complications common Complications mostly treated successful
Discussion Survival is acceptable TVR outperforms TVP ( theoretically TVP superior ) High recurrence rate of moderate TR after TVP ( tethering )
Conclusion In patients with a right systemic ventricle and moderate to severe tricuspid regurgitation who undergo tricuspid surgery: High incidence of complications; no influence on survival Survival is acceptable Results of TVP seem unfavorable as compared to TVR Right ventricular function may stabilize Functional class improves
recommendations Tricuspid valve repair/ replacement should be considered in a relatively early stage, when RV function is still preserved Tricuspid valve repair should probably not be used when RV function is poor (leaflet tethering will cause recurrent TI) Concomitant pulmonary artery banding may be beneficial to RV geometry and function
Limitations Semi-quantitative assessment of RV function; Subjective estimation of functional class; Small number of patients: Prospective analysis, (inter)national Powered to stratify on important baseline characteristics (l-tga/d-tga; TVP/TVR; RV function and volume; timing; associated defects; age)