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

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.

Figure 1. Flowchart showing the number of patients and their outcome during different phases of the study. ¹Both patients pulmonary hypertension. ²Two patients BCPC as final operation; four two ventricle correction. ³Five patients with aorto-pulmonary shunt. ⁴Thirteen patients heart transplantation (HTX). ⁵BCPC (bidirectional cavopulmonar connection) as first operation in 48 patients. ⁶Fontan as first operation in 6 patients.

Figure 2. Cumulative 15 years HTX-free survival since birth among subgroups of patients with univentricular heart. DILV, double inlet left ventricle; DORV, double outlet right ventricle; HLHS, hypoplastic left heart syndrome; HTX, heart transplantation; MISC, miscellaneous; PA-IVS, pulmonary atresia with intact ventricular septum; TA, tricuspid atresia; uAVSD, unbalanced atrioventricular septal defect.

Figure 3. Distribution of time from birth do death within 30 days among patients who were not operated.

Figure 4. Five years HLHS versus non-HLHS postoperative survival among patients born in 1990–2002 versus 2003–2015. HLHS, hypoplastic left heart syndrome.

Figure 5. Thirty days postoperative survival among patients with hypoplastic left heart syndrome operated with aorto-pulmonary shunt (AP-shunt) versus Sano shunt. AP, aorto-pulmonary; HLHS, hypoplastic left heart syndrome;