Long-Term Impact of Tricuspid Valve Intervention on Survival, Tricuspid Valve, and Ventricular Function in Hypoplastic Left Heart Syndrome.

Abstract

Tricuspid regurgitation (TR) is a significant survival risk in hypoplastic left heart syndrome. This is a retrospective single-institution study of 485 patients with hypoplastic left heart syndrome who underwent Fontan from 1985 through 2017. Interstage death or failure and tricuspid valve replacement without attempted repair were excluded. The primary outcome was survival free of transplant or Fontan takedown. Secondary outcomes were conditional cumulative incidence of tricuspid valve failure (≥ moderate TR or surgical tricuspid valve intervention [TVI]) and ventricular failure (≥ moderate ventricular dysfunction or mechanical circulatory support). There were 83 patients who underwent TVI with 114 procedures. Surgical techniques included partial annuloplasty with posterior leaflet obliteration (68), commissuroplasty (23), cleft closure (11), ring annuloplasty (9), and others. Single TVI tended to have a more straightforward regurgitant mechanism and lesion. The survival free of transplant or Fontan takedown was 62% at 20 years. Single TVI showed comparable survival to non-TVI (non-TVI, 77%; single TVI, 79%; repeated TVI, 47%; P < .001). The conditional cumulative incidence of tricuspid valve and ventricular failure was 22.9% and 12% at 20 years, respectively. At 10 years, the repeated TVI showed a higher tricuspid valve failure rate vs non-TVI and single TVI (non-TVI, 6.3%; single TVI, 11.6%; repeated TVI, 39.3%). However, the ventricular failure rate was similar between single and repeated TVI at 10 years (non-TVI, 4.7%; single TVI, 15.0%; repeated TVI, 21.8%). Single successful TVI positively modified tricuspid valve function and survival after Fontan. Tricuspid valve repair should be considered, especially for simple TR.