Key result
Tricuspid valve repair in children with hypoplastic left heart syndrome yielded a 5-year reoperation-free survival of 52%, with repair before stage II increasing reoperation risk (HR 5.5, P=0.042).
Why the study?
The results of tricuspid valve repair in patients with hypoplastic left heart syndrome during staged reconstruction required evaluation, focusing on timing and mechanisms of regurgitation.
What are the outcomes and risk factors for mortality and reoperation following tricuspid valve repair in children with hypoplastic left heart syndrome?
Cohort (n=44)
What are the outcomes and risk factors for mortality and reoperation following tricuspid valve repair in children with hypoplastic left heart syndrome?
Hazard Ratio: 5.5
p-value: p=0.042
In children with hypoplastic left heart syndrome, early-onset tricuspid regurgitation requiring repair before stage II reconstruction predicts worse outcomes, including higher rates of reoperation and valve replacement.
Repair before stage II may warrant caution due to higher reoperation risk; leaves open optimal timing and prospective validation in HLHS.
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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Ono et al. (2020) conducted a cohort in Hypoplastic left heart syndrome (n=44). Tricuspid valve repair before stage II vs. Tricuspid valve repair at later stages was evaluated on Reoperation (HR 5.5, p=0.042). Tricuspid valve repair in children with hypoplastic left heart syndrome yielded a 5-year reoperation-free survival of 52%, with repair before stage II increasing reoperation risk (HR 5.5, P=0.042).
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