Patients with hypoplastic left heart syndrome (HLHS) and tricuspid valve regurgitation receiving tricuspid valve (TV) repair have similar transplant-free survival rates as those with HLHS without clinically significant TV regurgitation (TVR), according to a study in Pediatric Cardiology.
The systematic review and meta-analysis evaluated the effect of TV repair in modifying the prognosis of patients with HLHS, the risk for TVR recurrence, and the need for reintervention.
A literature search was conducted in the PubMed, Web of Science, and Scopus databases in January 2023. Eligible studies included patients affected by HLHS with TVR receiving TV repair; reported survival, and/or TVR recurrence, and/or risk for reoperation for TVR displayed as Kaplan-Meier curves; and were published in English after 1970. A random-effect meta-analysis was performed on the study outcomes.
A total of 9 articles with 203 patients who received surgical repair of TVR were included. Participants’ median/mean age at TV repair ranged from 0.02 to 1.9 years, and the median/mean follow-up ranged from 0.4 to 7.9 years. The most common surgical technique for TV repair was commissuroplasty (139/191 patients, 72.8%), followed by annuloplasty (113/191, 59.2%).
Pooled analysis showed that in-hospital mortality after TV repair was 9% (95% CI, 1%-21%; I2= 76.9%; P <.001). The pooled risk for early (in-hospital) TV reoperation was 1% (95% CI, 0%-5%; I2= 32.9%; P =.15).
Based on 5 studies, the transplant-free survival rates at 1, 2, 5, and 10 years of follow-up were 75.5% (95% CI, 67.6%-84.3%), 69.4% (95% CI, 60.9%-79%), 63.6% (95% CI, 54.6%-73.9%), and 61.9% (95% CI, 52.7%-72.6%), respectively. The pooled transplant-free survival rate of patients who had TV repair was not different vs the 323 patients with HLHS without TVR (control group; P =.59).
In pooled analysis of 4 studies with 91 patients, freedom from TVR recurrence at 1, 2, 5, and 10 years of follow-up was 65.9% (95% CI, 56.7%-76.7%), 63.2% (95% CI, 53.8%-74.3%), 57% (95% CI, 46.7%-69.7%), and 48.7% (95% CI, 37.3%-63.7%), respectively.
Pooled analysis from 5 studies with 115 patients showed that rates of freedom from TV reoperation at 1, 2, 5, and 10 years of follow-up were 77% (95% CI, 69.4%-85.4%), 71.4% (95% CI, 63.1%-80.7%), 63.6% (95% CI, 54.5%-74.3%), and 63.6% (95% CI, 54.5%-74.3%), respectively.
Age at surgery was an effect modifier (hazard ratio [HR], 0.05; 95% CI, 0.01-0.25; P <.001), and younger patients had an increased risk for TV reoperation. The rate of patients who needed TV repair during a Norwood operation had a modifier effect on the freedom from TV reoperation (HR, 1.02; 95% CI, 1.01-1.02; P <.001).
Limitations of the study include the use of observational studies in the meta-analysis, and comparing outcomes of cases vs controls from different populations may result in a selection bias. Also, the relatively short mean follow-up times did not allow reliable inferences on the long-term fate of TV and patient survival after TV repair.
“At a medium-term follow-up, TV repair can effectively modify the prognosis of patients with HLHS and loss of systemic TV competence, re-establishing a comparable transplant-free survival to HLHS peers without TVR,” wrote the study authors. “However, the durability of surgery seems to be time-dependent and a significant quota of patients will experience TVR recurrence, requiring more than 1 surgical procedure on the TV.”
Ponzoni M, Azzolina D, Vedovelli L, Gregori D, Vida VL, Padalino MA. Tricuspid valve repair can restore the prognosis of patients with hypoplastic left heart syndrome and tricuspid valve regurgitation: a meta‑analysis. Pediatr Cardiol. Published online August 9, 2023. doi: 10.1007/s00246-023-03256-0