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.”
References:
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