Hypoplastic Left Heart Syndrome in US Military Family Members: Trends in Intervention, Survival, and Prevalence

Scott, John; Niebuhr, David W.

doi:10.1111/j.1747-0803.2007.00067.x

Cited by 5 publications

(3 citation statements)

References 24 publications

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“…In a US population-based study, Boneva et al 1 reported that mortality from congenital heart disease declined by 40% from 1979 through 1997 but first-year mortality for HLHS declined by only 7.5%. Scott and Niebuhr, 24 in their study of survival in HLHS cases in US military families, reported a marked increase in survival comparing cases from the 1980s and the 1990s, but no change was observed thereafter. In a single-center study of HLHS, Mahle et al 5 reported progressive improvement in hospital survival after each stage of palliation (ie, Norwood, bidirectional Glenn, and Fontan procedures).…”

Section: Fixler Et Al Five-year Mortality Of Functional Single Ventriclementioning

confidence: 98%

Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003

et al. 2010

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Section: Fixler Et Al Five-year Mortality Of Functional Single Ventriclementioning

confidence: 98%

Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003

et al. 2010

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“…A trend of increasing use of aggressive treatment has been suggested for hypoplastic left heart syndrome; from the 1980s to mid 2000s, its occurrence remained stable but deaths declined. This was thought to be due to more families choosing surgical intervention over comfort care (Scott and Niebuhr, 2007;Gordon et al, 2008). The surgical mortality rate has also declined (Connor and Thiagarajan, 2007).…”

Section: Discussionmentioning

confidence: 96%

Time trends in the prevalence of birth defects in Texas 1999-2007: Real or artifactual?

Langlois

Marengo

Canfield

2011

Birth Defects Research Part A: Clinical and Molecular Teratolog

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In our opinion, the weight of evidence in our study suggests that the observed increase over time in total birth defects and in many specific birth defects is artifactual. This likely reflects increased awareness, referral, detection, or documentation in health care facilities visited by TBDR staff, resulting in more complete ascertainment by the registry, rather than a true change over time in the occurrence of most birth defects.

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“…A recent survey of European surgeons reported that parental intention to treat was 95% in the UK, with the remaining 5% opting for comfort care ( 22 ). According to a US study of military personnel, the proportion of parents opting for comfort care is minimal (3%) since the 2000's and so these cases probably make up a very small proportion of our cohort ( 23 ).…”

Section: Discussionmentioning

confidence: 98%

The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales

et al. 2021

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Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012.Methods: Cases of HLHS notified to four congenital anomaly registers in England and Wales during 1998–2012, matched to Office for National Statistics mortality information, were included. Kaplan-Meier survival estimates to age 15 were reported. Cox regression models were fitted to examine risk factors for mortality.Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality.Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.

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Hypoplastic Left Heart Syndrome in US Military Family Members: Trends in Intervention, Survival, and Prevalence

Cited by 5 publications

References 24 publications

Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003

Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003

Time trends in the prevalence of birth defects in Texas 1999-2007: Real or artifactual?

The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales

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