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Outcomes following prenatal diagnosis of isolated persistent left superior vena cava
Isabelle Durand1, Tristan Hazelzet1, André Gillibert2
1Department of Paediatrics, Rouen University Hospital, 37, boulevard Gambetta, 76031 Rouen cedex, France.
Insights
Infants with isolated persistent left superior vena cava (PLSVC) show good short-term outcomes. However, PLSVC often co-occurs with other malformations, impacting long-term prognosis, necessitating thorough prenatal screening.
Area of Science:
- Perinatology
- Pediatric Cardiology
- Medical Genetics
Background:
- Prenatal diagnosis of persistent left superior vena cava (PLSVC) is increasing.
- Limited data exists on the outcomes of infants with prenatally diagnosed isolated PLSVC.
Purpose of the Study:
- To compare the postnatal outcomes of infants with isolated PLSVC diagnosed prenatally against those with associated malformations.
Main Methods:
- Prospective registry of PLSVC cases identified via fetal echocardiography.
- Unfavorable outcome defined as pregnancy termination, in utero/postnatal death, or severe genetic syndrome.
Main Results:
- 256 infants included: 113 isolated PLSVC, 143 with associated malformations.
- Five-year survival with good outcome was 100% for isolated PLSVC, and ranged from 78.9% to 91.0% for those with associated anomalies.
- All genetic findings were associated with non-isolated PLSVC.
Conclusions:
- Infants with isolated PLSVC generally have favorable short-term postnatal outcomes.
- Associated malformations significantly affect outcomes in PLSVC cases.
- Comprehensive prenatal evaluation for associated anomalies is crucial for PLSVC cases.
Background:
Prenatal diagnosis of persistent left superior vena cava is increasing, but little is known about outcomes of infants with prenatally diagnosed isolated persistent left superior vena cava.
Objective:
To assess the outcomes of infants with isolated persistent left superior vena cava diagnosed prenatally compared with infants with associated malformations.
Methods:
All cases of persistent left superior vena cava confirmed by specialized fetal echocardiography in pregnant women were included from a single-centre prospective registry. Unfavourable outcome was defined as termination of pregnancy, in utero death, postnatal death or severe genetic syndrome missed prenatally.
Results:
A total of 256 infants were included: 113 cases (44.1%) with isolated persistent left superior vena cava and 143 cases (55.9%) with associated malformations; respectively, 111 (98.2%) and 101 (70.6%) had a live birth. The median postnatal clinical follow-up was 3.6 years. Five-year postnatal survival with good outcome was estimated at: 100% (95% confidence interval 90.7% to 100%) in infants with isolated persistent left superior vena cava; 91.0% (74.0% to 98.1%) in infants with associated cardiac anomalies; 87.5% (51.8% to 97.3%) in infants with associated extracardiac anomalies; 81.0% (52.6 to 94.6%) in infants with both cardiac and extracardiac anomalies; and 78.9% (36.7% to 95.9%) in infants with non-structural anomalies. All genetic findings and syndromes were detected in fetuses or infants with non-isolated persistent left superior vena cava.
Conclusion:
Infants with isolated persistent left superior vena cava have good short-term outcomes postnatally, but persistent left superior vena cava is frequently associated with other malformations that have an effect on outcomes, which should be thoroughly searched for prenatally.
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