Hypoplastic left heart syndrome: Prognosis and management options

Fruitman, Deborah

doi:10.1093/pch/5.4.219

Cited by 40 publications

(24 citation statements)

References 34 publications

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“…Surgical and medical interventions are also not recorded on the NorCAS, but likely impact survival. In particular, for cases of hypoplastic left heart, survival may be improved with palliative surgery, though many parents opt for comfort care . Moreover, younger age at surgical intervention appears to improve survival in children with CHD .…”

Section: Discussionmentioning

confidence: 99%

Survival, by Birth Weight and Gestational Age, in Individuals With Congenital Heart Disease: A Population‐Based Study

Best

Tennant

Rankin

2017

JAHA

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BackgroundCongenital heart disease (CHD) survival estimates are important to understand prognosis and evaluate health and social care needs. Few studies have reported CHD survival estimates according to maternal and fetal characteristics. This study aimed to identify predictors of CHD survival and report conditional survival estimates.Methods and ResultsCases of CHD (n=5070) born during 1985–2003 and notified to the Northern Congenital Abnormality Survey (NorCAS) were matched to national mortality information in 2008. Royston–Parmar regression was performed to identify predictors of survival. Five‐year survival estimates conditional on gestational age at delivery, birth weight, and year of birth were produced for isolated CHD (ie, CHD without extracardiac anomalies). Year of birth, gestational age, birth weight, and extracardiac anomalies were independently associated with mortality (all P≤0.001). Five‐year survival for children born at term (37–41 weeks) in 2003 with average birth weight (within 1 SD of the mean) was 96.3% (95% CI, 95.6–97.0). Survival was most optimistic for high‐birth‐weight children (>1 SD from the mean) born post‐term (≥42 weeks; 97.9%; 95% CI, 96.8–99.1%) and least optimistic for very preterm (<32 weeks) low‐birth‐weight (<1 SD from mean) children (78.8%; 95% CI, 72.8–99.1).ConclusionsFive‐year CHD survival is highly influenced by gestational age and birth weight. For prenatal counseling, conditional survival estimates provide best‐ and worst‐case scenarios, depending on final gestational age and birth weight. For postnatal diagnoses, they can provide parents with more‐accurate predictions based on their baby's birth weight and gestational age.

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Section: Discussionmentioning

confidence: 99%

Survival, by Birth Weight and Gestational Age, in Individuals With Congenital Heart Disease: A Population‐Based Study

Best

Tennant

Rankin

2017

JAHA

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“…2,7,8 Medical advances have dramatically decreased mortality, although it continues to remain at~40% 5 years after palliative surgeries. 9 Continued research is required to better understand the unique course and psychosocial complexity of these medically fragile children.…”

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confidence: 99%

Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses

et al. 2017

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“…The antenatal diagnosis allows time for parents to accept the diagnosis and explore the options available to them (eg, termination of pregnancy, compassionate care postnatally, palliative staged reconstructive surgery or, in some countries, cardiac transplantation) 26 – 28…”

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confidence: 99%

Antenatal perspective of hypoplastic left heart syndrome: 5 years on

Rasiah

Ewer

Miller

et al. 2008

Archives of Disease in Childhood - Fetal and Neonatal Edition

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The number of parents choosing termination after an antenatal diagnosis of HLHS has almost halved since 5 years ago. Despite the significant increase in surgical survival following stage 1 Norwood in this period, in the intention-to-treat cohort the survival was 46.9%. These data again highlight the poorer outcome for babies with congenital malformations diagnosed in utero in comparison with those identified postnatally.

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Hypoplastic left heart syndrome: Prognosis and management options

Cited by 40 publications

References 34 publications

Survival, by Birth Weight and Gestational Age, in Individuals With Congenital Heart Disease: A Population‐Based Study

Survival, by Birth Weight and Gestational Age, in Individuals With Congenital Heart Disease: A Population‐Based Study

Children with hypoplastic left heart syndrome have lower quality of life than healthy controls and children with other illnesses

Antenatal perspective of hypoplastic left heart syndrome: 5 years on

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