Somatic Development after Total Cavopulmonary Connection: Factors Influencing Catch-up Growth

Background: Patients after Fontan palliation might suffer from impaired somatic growth. Various factors may be implicated as underlying causes. However, studies have suggested that the key to avoid minor corporal development may lie in the timing of volume-unloading procedures. We aimed to retrospectively analyze the somatic growth of patients after total cavopulmonary connection (TCPC) procedure as well as risk factors that might adversely affect postoperative catch-up growth.

Method: A total of 620 patients underwent TCPC in our institution between 1994 and 2021. Weight and height at the time of TCPC and at the serial postoperative periods were collected in all patients. Patients who underwent TCPC below 5 years old were selected for a better analysis. Weight and height at the time of TCPC and different times afterward were selected. Weight, height, and BMI for age Z-score (WAZ, HAZ, and BMIZ) were calculated using WHO anthro software. Factor analysis was performed for WAZ, HAZ, BMIZ, and delta WAZ.

Results: A total of 310 patients were included in the study. Median age at TCPC was 2.0 (1.7–2.5) years. Most frequent diagnosis was HLHS (n = 205), followed by tricuspid atresia (n = 50), and double inlet left ventricle (n = 39). Associated anomalies included heterotaxy syndrome (n = 21) and anomalous systemic venous anomalies (n = 31). Weight Z-score (WAZ: −0.39 ± 1.1 to −0.19 ± 1.10, p < 0.001) and BMI Z score (0.01 ± 1.26 to 0.30 ± 1.41, p < 0.001) were significantly increased after TCPC. TCPC older than 2 years (p < 0.01), BCPS older than 5 months (p = 0.012), heterotaxy (p = 0.003), and anomalous systemic venous return (p = 0.002) were identifies as risk factors for low WAZ pre-TCPC, TCPC older than 2 years (p < 0.01), DORV (p = 0.008), and anomalous systemic venous return (p = 0.031) were identified as risk factors for low WAZ post-TCPC. Risk factor analysis using delta WAZ (post WAZ – pre WAZ) revealed that age at TCPC older than 2 years (p = 0.013) was at risk for impaired catch-up growth.

Conclusion: Patients demonstrated a significant catch-up growth in weight-for-age Z-score after TCPC. Several variables were associated with postoperatively reduced somatic growth. Among those TCPC after 2 years of age showed significant impact on both pre- and postoperative somatic development. Heterotaxy and anomalous systemic venous connection impacted on WAZ, HAZ, and BMZ, respectively.

Publication History

Article published online:
28 January 2023