Abstract:ObjectiveGiven the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome.MethodsWe performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000–2018. The primary outcome was mortality, ascertained from the Office fo… Show more
“…Hypoplastic aortic arch was defined as an aortic arch diameter less than the number of kilogram body weight in millimetres plus 1 millimetre, in at least one segment from the first and to the last aortic arch artery. UVH ascertainment among patients with DORV was done by combining baseline diagnostic data with the actual procedures done 14…”
Section: Methodsmentioning
confidence: 99%
“…UVH ascertainment among patients with DORV was done by combining baseline diagnostic data with the actual procedures done. 14 …”
ObjectivesFew 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.DesignRetrospective.SettingTertiary centre for congenital cardiology and congenital heart surgery.ParticipantsAll 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020.ResultsOne 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 surgeryConclusionsSurgery 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.
“…Hypoplastic aortic arch was defined as an aortic arch diameter less than the number of kilogram body weight in millimetres plus 1 millimetre, in at least one segment from the first and to the last aortic arch artery. UVH ascertainment among patients with DORV was done by combining baseline diagnostic data with the actual procedures done 14…”
Section: Methodsmentioning
confidence: 99%
“…UVH ascertainment among patients with DORV was done by combining baseline diagnostic data with the actual procedures done. 14 …”
ObjectivesFew 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.DesignRetrospective.SettingTertiary centre for congenital cardiology and congenital heart surgery.ParticipantsAll 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020.ResultsOne 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 surgeryConclusionsSurgery 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.
“…The lower survival rate could be due to the inclusion of patients with HLHS and those who received comfort care. However, this is not surprising, as univentricular heart patients have low survival in other studies 19–21. Nonetheless, tricuspid atresia shows a comparatively better survival rate among children with univentricular hearts, with 50% surviving until age 15.…”
ObjectiveLimited population-based studies are available on the survival of congenital heart disease (CHD) from lower- and middle-income countries. Therefore, we evaluated the survival from birth until 15 years and associated factors for mortality.MethodsThis population-based cohort study included all children with CHD registered in the Pediatric Cardiology Clinical Information System born between 2006 and 2020 in Johor, Malaysia. The mortality rate was calculated, and Cox proportional hazard regression analysis was used to determine factors associated with mortality. The Kaplan-Meier analysis was used to estimate the survival rates at 1, 5, 10 and 15 years.ResultsThere were 5728 patients with CHD studied, with 1543 (27%) lesions resolved spontaneously, 322 (5.6%) were treated with comfort care, 1189 (21%) required no intervention, and 2674 (47%) needed surgery or intervention. The overall mortality rate was 15%, with a median age of death of 3.7 months (IQR 0.9–9.8 months). Preoperative/intervention death was observed in 300 (11%), and 68 (3.2%) children died within 30 days of surgery or intervention. The overall estimated survival at 1, 5, 10 and 15 years was 88%, 85%, 84% and 83%, respectively. The independent factors associated with mortality were male gender, associated syndrome or extra-cardiac defect, pulmonary hypertension, antenatal diagnosis and severe lesions.ConclusionsEight out of 10 patients with CHDs survived up to 15 years of age. However, 10% of CHDs who require intervention die before the procedure. Thus, improving congenital cardiac surgery and enhancing the overall healthcare system are crucial to improve survival.
“… 5 Figure 1 Flowchart of f-SV study population inclusion and exclusion steps and classifications. Identification of patients with and without HLHS, and classification into diagnosis types were done using an algorithm described previously 2 , 6 and further in the Appendix E1 . NHS , National Health System; f-SV , functionally single ventricle; HLHS , hypoplastic left heart syndrome.…”
Section: Methodsmentioning
confidence: 99%
“… 1 Advances in surgical techniques, critical care, and follow-up improved the outcomes, which still remain less favorable than for other complex CHD. 2 , 3 , 4 , 5 , 6 …”
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