Right ventricular and tricuspid valve function during staged reconstruction for hypoplastic left heart syndrome: A longitudinal echocardiographic study

Background: Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy. Methods: From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1). Echocardiographic evaluation of tricuspid regurgitation (TR) and RV function was assessed at multiple time points. Clinical outcomes including tricuspid valvuloplasty, transplantation, and death were reviewed. Results: The most prevalent HLHS subtype was aortic atresia/mitral atresia. The presence of significant TR and/or RV dysfunction was 14.78% and 9.36%, respectively, at the time of initial HS1. There were 185 survivors following HS1 (91.13%, n = 185/203), while 147 patients underwent comprehensive stage II or bidirectional Glenn shunt (72.41%, n = 147/203). Tricuspid valvuloplasty was undertaken in nine patients (4.86%, n = 9/185). Ultimately, 100 patients underwent the Fontan procedure. The odds of development of significant TR and/or RV dysfunction were not statistically different throughout the stages of palliation (TR: odds ratio [OR] = 0.14-0.25, P = .5260; RV dysfunction: OR = 0.02-0.13, P = .3992). However, the risk of death and/or transplant was 2.5- to 3.8-fold when either were present alone or in combination (TR: OR = 2.58, P = .0356; RV dysfunction: OR = 3.84, P = .0262). Transplant-free survival at 15 years was 44.8%. Conclusion: Following hybrid palliation for HLHS, the majority of survivors have normal RV and TV functions. Tricuspid valvuloplasty was required in few patients. Once significant TR and/or RV dysfunction ensues, there is a two- to three-fold risk of death and/or transplant.

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Tricuspid valve repair improves early right ventricular and tricuspid valve remodeling in patients with hypoplastic left heart syndrome

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2012.10.040 ◽

2013 ◽

Vol 145 (2) ◽

pp. 446-450 ◽

Cited By ~ 16

Author(s):

Shinya Ugaki ◽

Nee Scze Khoo ◽

David B. Ross ◽

Ivan M. Rebeyka ◽

Ian Adatia

Keyword(s):

Right Ventricular ◽

Hypoplastic Left Heart Syndrome ◽

Tricuspid Valve ◽

Tricuspid Valve Repair ◽

Left Heart ◽

Valve Repair ◽

Hypoplastic Left Heart ◽

Valve Remodeling ◽

Left Heart Syndrome

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Tricuspid valve repair in children with hypoplastic left heart syndrome: impact of timing and mechanism on outcome

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezaa004 ◽

2020 ◽

Vol 57 (6) ◽

pp. 1083-1090

Author(s):

Masamichi Ono ◽

Benedikt Mayr ◽

Melchior Burri ◽

Nicole Piber ◽

Christoph Röhlig ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Tricuspid Valve ◽

Stage Ii ◽

Tricuspid Valve Repair ◽

Left Heart ◽

Anterior Leaflet ◽

Hypoplastic Left Heart ◽

Staged Reconstruction ◽

Left Heart Syndrome ◽

Leaflet Prolapse

Abstract OBJECTIVES Our aim was to evaluate the results of tricuspid valve repair (TVr) in patients with hypoplastic left heart syndrome during staged reconstruction, focussing on the timing of the repair and the mechanisms of tricuspid regurgitation (TR). METHODS Records of 44 children with hypoplastic left heart syndrome who underwent a total of 62 tricuspid valve (TV) procedures during staged reconstruction were retrospectively analysed. RESULTS TVr was performed before stage II in 4 (9%) patients, at stage II in 23 (52%) patients, between stages II and III in 3 (7%) patients and at stage III in 14 (32%) patients. The median age at the first TV procedure was 5 months. At surgery, TR emanated commonly from the anteroseptal commissure in 21 (48%) patients. Anterior leaflet prolapse was observed most frequently (n = 23; 52%), followed by septal leaflet restriction (n = 22; 50%), dilated annulus (n = 21; 48%) and cleft anterior leaflet (n = 9; 21%). Surgical techniques included commissuroplasty in 27 (61.4%) patients, leaflet adaptation in 20 (44%) patients, partial annuloplasty in 11 (25%) patients, chordal reconstruction in 10 (23%) patients and cleft closure in 10 (23%) patients. Among all 44 patients, 27 (61%) patients had preoperative grade III TR and 17 (39%) patients had grade IV; postoperatively, there were no patients with grade IV, 25 patients with grade III (57%), 10 patients with grade II (23%) and 6 patients with grade I (14%). Fifteen patients required redo TV surgeries. Reoperation-free survival was 52% at 5 years. Lower weight at initial TVr predicted mortality [hazard ratio (HR) 0.7, P = 0.044] and reoperation (HR 0.8, P = 0.015). TVr before stage II was a risk for both reoperation (HR 5.5, P = 0.042) and TV replacement (HR 36.9, P = 0.013). Among morphological factors, septal leaflet restriction was a risk for reoperation (HR 4.7, P = 0.017) and anterior (HR 4.7, P = 0.037) and posterior (HR 7.3, P = 0.015) leaflet chordal anomaly for TV replacement. CONCLUSIONS Anterior leaflet prolapse and septal leaflet restriction are the main mechanisms of TR in hypoplastic left heart syndrome. Early-onset TR before stage II predicts worse outcome. Refinements to repair techniques in early infancy, especially for septal leaflet restrictions and chordal anomalies, are mandatory to improve outcomes.

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