Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990-2015

Gunnar Erikssen; Knut Liestøl; Jamil Aboulhosn; Gunnar Wik; Henrik Holmstrøm; Gaute Døhlen; Ola Gjesdal; Sigurd Birkeland; Tom Nilsen Hoel; Kjell Johan Saatvedt; Egil Seem; Erik Thaulow; Mette E Estensen; Harald Lauritz Lindberg

doi:10.1136/bmjopen-2022-069531

Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990-2015

BMJ Open. 2023 Jul 25;13(7):e069531. doi: 10.1136/bmjopen-2022-069531.

Authors

Affiliations

¹ Department of Cardiology, Oslo universitetssykehus Rikshospitalet, Oslo, Norway gunnar.erikssen@yahoo.com.
² Department of Informatics, University of Oslo, Oslo, Norway.
³ Ahmanson Adult Congenital Heart Disease Center, UCLA, Los Angeles, California, USA.
⁴ Department of Pediatrics, Sørlandet Hospital, Kristiansand, Norway.
⁵ Department of Women's and Children's, Oslo University Hospital, Oslo, Norway.
⁶ Department of Women's and Children's, Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
⁷ Department of Cardiology, Oslo University Hospital rikshospitalet, Oslo, Norway.
⁸ Department of Cardiothoracic Surgery, Oslo University Hospital, Oslo, Norway.
⁹ Department of Coardiothoracic Surgery, Oslo universitetssykehus Rikshospitalet, Oslo, Norway.
¹⁰ Department of Pediatric Cardiology, Oslo universitetssykehus Rikshospitalet, Oslo, Norway.
¹¹ Department of Cardiology, Oslo University Hospital, Oslo, Norway.
¹² Department of Cardiothoracic Surgery, Oslo universitetssykehus Rikshospitalet, Oslo, Norway.

Abstract

Objectives: Few data exist on mortality among patients with univentricular heart (UVH) before surgery. Our aim was to explore the results of intention to perform surgery by estimating preoperative vs postoperative survival in different UVH subgroups.

Design: Retrospective.

Setting: Tertiary centre for congenital cardiology and congenital heart surgery.

Participants: All 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020.

Results: One quarter (151/595; 25.4%) were not operated. Among these, only two survived, and 125/149 (83.9%) died within 1 month. Reasons for not operating were that surgery was not feasible in 31.1%, preoperative complications in 25.2%, general health issues in 23.2% and parental decision in 20.5%. In total, 327/595 (55.0%) died; 283/327 (86.5%) already died during the first 2 years of life. Preoperative survival varied widely among the UVH subgroups, ranging from 40/65 (61.5%) among patients with unbalanced atrioventricular septal defect to 39/42 (92.9%) among patients with double inlet left ventricle. Postoperative survival followed a similar pattern. Postoperative survival among patients with hypoplastic left heart syndrome (HLHS) improved significantly (5-year survival, 42.5% vs 75.3% among patients born in 1990-2002 vs 2003-2015; p<0.0001), but not among non-HLHS patients (65.7% vs 72.6%; p=0.22)-among whom several subgroups had a poor prognosis similar to HLHS. A total of 291/595 patients (48.9%) had Fontan surgery CONCLUSIONS: Surgery was refrained in one quarter of the patients, among whom almost all died shortly after birth. Long-term prognosis was largely determined during the first 2 years. There was a strong concordance between preoperative and postoperative survival. HLHS survival was improved, but non-HLHS survival did not change significantly. This study demonstrates the complications and outcomes encountering newborns with UVH at all major stages of preoperative and operative treatment.

Keywords: cardiac epidemiology; cardiac surgery; congenital heart disease; epidemiology; paediatric surgery.

MeSH terms

Adult
Child
Heart Septal Defects* / complications
Humans
Hypoplastic Left Heart Syndrome* / complications
Hypoplastic Left Heart Syndrome* / surgery
Infant, Newborn
Retrospective Studies
Treatment Outcome
Univentricular Heart* / complications