“Quality of Life” Analysis in the Long-Term Follow-up after “Fontan's” Palliation for Congenital Heart Defects—A Single Center Experience

Objectives: Complex CHD like hypoplastic left (HLHS) or right syndrome (HRHS) is life-threatening and surgical treatment during infancy is unavoidable. Palliative surgery is often the only way to achieve Fontan's circulation and consists of up to three surgeries during childhood: the Norwood1 procedure (NOR-1), Glenn anastomosis, and TCPC. Next to increase of surveillance up to adulthood, long-lasting assurance of QoL is essential to live as unrestricted as possible. Despite there is knowledge about reduced QoL compared with healthy children, detailed knowledge is lacking. Thus, the aim of this study was to investigate the individual sensitiveness of particular QoL areas like physical, psychological, or cardiac health. Furthermore, effect of different ages, gender or performance of NOR-1 on QoL development of UVH patients is interesting.

Methods: n = 62 patients with complex CHD were screened for QoL analysis. Surgeries during infancy of patients were one staged (TCPC), two staged (Glenn's anastomosis and TCPC), or three staged (NOR-1, Glenn's anastomosis, and TCPC). Outcome after TCPC was analyzed in detail (mortality and morbidity) and QoL. Data were collected and analyzed adequately using validated questionnaire “pediatric quality of life inventory,” version 4.0 (PedsQL), including cardiac module as parent-proxy report. Total health score of patients was compared with mean score of healthy children from literature.

Result: Overall survival 133 months (11.0 years) after TCPC was 93.5%. A mean follow-up of 76.6 ± 21.4 months after TCPC, patients exhibited mean total QoL. There was no difference in QoL between younger (5–7 years) and older patients (8–12 years). Age at TCPC and gender had no effect on later impaired QoL. In contrast to that, patients with first-stage surgery (NOR-1 or shunt) exhibited worse QoL than patients without first stage. Thereby, type of first stage palliation did not play any role. Furthermore, patients with first stage palliation prior TCPC showed impaired QoL earlier (82.5 months, p = 0.033), in contrast to patient without first stage surgery. Patients with one- or two-stage palliation suffer from impaired QoL later, approximately 105.3 months after TCPC.

Conclusion: TCPC affects physical and psychological QoL, as well as cardiac health independently from age, gender, and first-stage palliation. QoL is worse in later life of TCPC treated patients with NOR-1 as part of surgical procedure in early childhood, than in patients which got two-staged procedure without NOR-1 or shunt.

Publication History

Article published online:
21 February 2021