Congenital heart disease in the adult

2010 ◽  
pp. 2842-2872
Author(s):  
S. A. Thorne
Keyword(s):  
Congenital Heart Disease ◽  
United Kingdom ◽  
Heart Disease ◽  
Congenital Heart ◽  
Specialist Care ◽  
Term Survival ◽  
Long Term Survival ◽  
The United Kingdom ◽  
Growing Population

Adults with congenital heart disease are a growing population, and now outnumber children with congenital heart disease in the United Kingdom. Many patients with repaired hearts can now, with specialist care, expect to live a normal or near normal lifespan. Other survivors have complex, surgically altered hearts and circulations that reflect the surgical and interventional practices of the preceding two decades. Their long-term outlook is unknown and they remain at lifelong risk of complications that may require further intervention. The organization of services to provide specialist care is key to their long-term survival....

Congenital heart disease in the adult

2020 ◽  
pp. 3559-3595
Author(s):  
S.A. Thorne
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Specialist Care ◽  
Term Survival ◽  
The United Kingdom ◽  
Developing Heart ◽  
Growing Population

Adults with congenital heart disease are a growing population, and now outnumber children with congenital heart disease in the United Kingdom. Many patients with repaired hearts can now, with specialist care, expect to live a normal or near normal lifespan. Other survivors have complex, surgically altered hearts and circulations that reflect the surgical and interventional practices of the preceding two decades. Their long-term outlook is unknown and they remain at lifelong risk of complications that may require further intervention. The organization of services to provide specialist care is key to their long-term survival. As the population of adults with congenital heart disease ages, so the number developing heart failure increases. Services caring for patients need to develop a robust end-of-life pathway that focuses on symptoms and quality of life, and runs in parallel with other therapies.

Eisenmenger syndrome and long-term survival in patients with Down syndrome and congenital heart disease

Heart ◽  
2016 ◽  
Vol 102 (19) ◽  
pp. 1552-1557 ◽  
Author(s):  
Marc-André Körten ◽  
Paul C Helm ◽  
Hashim Abdul-Khaliq ◽  
Helmut Baumgartner ◽  
Deniz Kececioglu ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Eisenmenger Syndrome ◽  
Term Survival ◽  
Long Term Survival

Extracorporeal membrane oxygenation and cyanotic congenital heart disease

1992 ◽  
Vol 2 (4) ◽  
pp. 359-360 ◽  
Author(s):  
Gale A. Pearson ◽  
Richard K. Firmin ◽  
Ranjit Leanage
Keyword(s):  
Congenital Heart Disease ◽  
United Kingdom ◽  
Heart Disease ◽  
Extracorporeal Membrane Oxygenation ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Membrane Oxygenation ◽  
The United Kingdom

AbstractWorldwide figures suggest that two percent of appropriate referrals for neonatal extracorporeal membrane oxygenation turn out to have previously covert congenital heart disease. This is despite the fact that expert cardiological evaluation is routine prior to cannulation. The experience in the United Kingdom includes such a case which is reported here. The implications for the role of pediatric cardiologists in such a service are considered.

Prostaglandins in congenital heart disease—potential for confusion

1995 ◽  
Vol 5 (2) ◽  
pp. 202-203 ◽  
Author(s):  
R. John Madar ◽  
Tim J.D. Donaldson ◽  
Stewart Hunter
Keyword(s):  
Congenital Heart Disease ◽  
United Kingdom ◽  
Heart Disease ◽  
Congenital Heart ◽  
Prostaglandin E ◽  
The United Kingdom ◽  
Arterial Duct

the use of prostaglandians in maintaining the patency of the arterial duct in congenital heart disease is well established. Intravenous1-3 and ora12–4 administration has been used, although for acute use intravenous and possibly intraosseous5 routes are favored. Both prostaglandin E1 (alprostadil—ProstinVR: Upjohn)1,4 and prostaglandin E2 (dinoprostone—Prostin E2: Upjohn)2,3 are used for this purpose, although only prostaglandin E1 is licensed for this indication in the United Kingdom. Prostaglandin E1 costs approximately 8 times more than prostaglandin E2 (£56.96 versus £7.43 per vial).

Maternal Phenylketonuria: Experiences From the United Kingdom

PEDIATRICS ◽  
2003 ◽  
Vol 112 (Supplement_4) ◽  
pp. 1553-1556
Author(s):  
Philip J. Lee ◽  
Maggie Lilburn ◽  
Jenny Baudin
Keyword(s):  
Congenital Heart Disease ◽  
United Kingdom ◽  
Heart Disease ◽  
Birth Weight ◽  
Head Circumference ◽  
Congenital Heart ◽  
Developmental Quotient ◽  
The United Kingdom ◽  
Postnatal Environment ◽  
The Mean

Charles Dent was one of the first physicians to recognize the teratogenic effects of maternal phenylalanine (Phe) on the fetus in 1956. This article describes the clinical experiences of women with phenylketonuria (PKU) within the unit that was established by Dent in the United Kingdom. Between 1977 and 2002, 79 infants were born to women with PKU. Of the 79, 18 (23%) were conceived while the women were on a normal diet with high blood Phe levels. The mean birth weight was 2.89 kg, and head circumference was 32.8 cm. At 1 year, the mean developmental quotient was 105.5 and at 4 years was 82.3. Four of these infants had congenital heart disease (2 of whom died as a result). In the remaining 61 infants, Phe-restricted diet started before conception. None of them had congenital heart disease. The mean birth weight was 3.23 kg, and head circumference was 34.0 cm. At 1 year, mean developmental quotient was 108.0 and at 4 years was 90.9. They continue to be followed up with additional neuropsychometric assessments at 8 and 14 years of age. This cohort is a proportion of infants who were born to mothers with PKU in the United Kingdom. Between 1978 and 1997, 255 live births were reported. Of these, 56% were conceived on unrestricted diet with subsequently poor outcome. This relatively high rate of conception off PKU diet is likely to reflect the scarcity of medical services for adults with metabolic disorders. We conclude that many features of the maternal PKU syndrome can be prevented but still occur because of the lack of appropriate resources to care for at-risk women. The precise targets for blood Phe and other nutrients during pregnancy are not entirely clear, neither are the reasons that some offspring are spared the harmful effects of Phe. The impact of the postnatal environment in which these infants find themselves requires additional assessment, too.

Surgery for Congenital Heart Disease: Improvements in Outcomes

2018 ◽  
Vol 35 (06) ◽  
pp. 557-560 ◽  
Author(s):  
Richard Jonas ◽  
Gerard Martin
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease ◽  
Term Outcome ◽  
Term Survival ◽  
Younger Age ◽  
Care Outcomes ◽  
Moderate Complexity

AbstractCardiac surgery for congenital heart disease has changed dramatically since the first surgery in 1938. During the early era, children underwent surgery at older ages often with palliative procedures before their corrective operation. Not surprisingly, in the early era, there was considerably higher early and late mortality, including the additive risks of having more than one procedure and a long period of living with an unphysiological palliated circulation. Over time with advances in noninvasive diagnosis, surgical approach, cardiopulmonary bypass techniques, and team-based care, outcomes have improved. Children now undergo corrective surgery at a younger age and have fewer palliative procedures. Short-term outcome as measured by the commonly used metric “procedural early mortality” (i.e., death before hospital discharge or less than 30 days following a surgical procedure) is now as low as 1 or 2% for many low-to-moderate complexity procedures. Late outcomes have also improved with long-term survival of hospital survivors for simple lesions being close to population controls. Late outcomes for more complex defects still show diminishing survival relative to a control population. Examination of the causes of death provides insights into areas in which clinical improvements may further improve the outlook for children with complex congenital heart disease.

Sign in / Sign up

Export Citation Format

Share Document