Congenital Heart Disease in Infants With Downʼs Syndrome

Wells, Gretchen; Barker, Sara; Finley, Sara; Colvin, Edward V.; Finley, Wayne H.

doi:10.1097/00007611-199407000-00010

Cited by 60 publications

(37 citation statements)

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“…Al-Aama et al (2012) reported a greater prevalence of 86.6%, possibly due to the study being conducted from a genetic referral center. International studies ranged from 40 to 55% (Evans, 1950;Wells et al, 1994;de Rubens Figueroa et al, 2003;Vida et al, 2005;Al-Aama et al, 2012;Roizen et al, 2014). Similar to CHD in the general population, VSD was the most common lesion in DS, which is also consistent with most international studies (Abbag F, 1998;Al-Jarallah, 2009 andAl-Aama et al, 2012).…”

Section: Discussionsupporting

confidence: 63%

“…The association of CHD with DS was first described by Evans in 1950(Carlgren, 1959. Since then a number of studies have consistently shown an association between the two conditions (Evans, 1950;Granzotti et al, 1995;Wells et al, 1994;de Rubens Figueroa et al, 2003;Vida et al, 2005;Roizen et al, 2014). As CHD is the greatest cause of death in infants and young children with DS, describing the prevalence and types of defects found in DS is relevant in facilitating early intervention and appropriate management (Evans, 1950).…”

Section: Discussionmentioning

confidence: 99%

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The epidemiology of congenital heart diseases in Saudi Arabia: A systematic review

Amirah¹,

Nada²,

Anna³

et al. 2015

J. Public Health Epidemiol.

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Several studies have been conducted on congenital heart disease in different regions of Saudi Arabia. However, no recent systematic review has examined the growing scientific evidence with respect to the epidemiology of CHD in the Kingdom. The aim of this review is to provide a comprehensive summary of CHD incidence, prevalence, burden and impact on the Saudi population. A literature search was conducted through PubMed and Google Scholar using relevant keywords to identify studies performed in Saudi Arabia regarding CHD from 1993 to December 2013. Articles written in English that described or investigated the epidemiology, etiology, distribution, impact and burden of CHD in the Saudi Arabian population were included. Twenty one articles met these criteria. Cross-sectional studies found the prevalence of CHD ranging between 2.1 and 10.7 per 1,000 persons. The most prevalent type of CHD was the ventricular septal defect ranging from 29.5 to 39.5% of all diagnosed CHDs, followed by atrial septal defect (8.9 to 18.1%) and pulmonary stenosis (6 to 12.4%). Overall, the incidence of severe CHD was approximately 5.4 per 1,000 live births per year. Occurrence of CHD in Saudi Arabia was significantly associated with Down's syndrome, consanguinity and maternal diabetes. Studies on the burden of these anomalies on children, families and society are scarce. This systematic review found that prevalence of CHD is comparable to that in other developing countries. Several modifiable risk factors have been identified emphasizing the importance of public health programs that are aimed at tackling such potentially preventable risk determinants.

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Section: Discussionsupporting

confidence: 63%

Section: Discussionmentioning

confidence: 99%

The epidemiology of congenital heart diseases in Saudi Arabia: A systematic review

Amirah¹,

Nada²,

Anna³

et al. 2015

J. Public Health Epidemiol.

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“…Although there is a wide variation among individuals in the clinical manifestations, particularly in neonates, diagnosis of DS is based on its characteristic features and associated systemic and functional malformations. Congenital heart disease (CHD), which is the most frequent systemic malformation, is present in 16-62% of individuals with DS (3)(4)(5). Although DS remains one of the most common causes of mental retardation, the quality of life and mortality of an individual with DS depends on the seriousness of the CHD (6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Evaluation of congenital heart diseases and thyroid abnormalities in children with Down syndrome

Mıhçı¹,

Akçurin²,

Eren³

et al. 2010

Anadolu Kardiyol Derg

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ÖZETAmaç: Down sendromunda (DS), konjenital kalp hastalığı (KKH) ve tiroid hastalığı (TH) birlikteliği bildirilmiştir. Bu çalışmanın amacı, başvuru sırasında DS'lu çocuklardaki konjenital kalp hastalığının tipi ve sıklığı ile tiroid bozukluğu arasındaki ilişkiyi belirlemektir. Yöntemler: Bu retrospektif çalışma, Ağustos 1993 ile Aralık 2005 arasındaki DS'lu 187 çocuğu içermekteydi. DS'lu tüm hastalara karyotip analizi, tiroid fonksiyon testi ve ekokardiyografik çalışmalar yapıldı. Gereken hastalara kalp kateterizasyonu uygulanarak hemodinamik çalışmalar yapıldı. Tiroidi uyarıcı hormon (TSH) düzeyi yüksek olanlara tirotropin salgılatıcı hormon uyarı testi yapıldı. İstatistiksel analizlerde Ki-kare, bağımsız örneklem "t" veya Mann-Whitney U testleri kullanıldı. Bulgular: DS'lu 136 (%72.73) hasta konjenital kalp hastalığına sahipti. KKH'lığı olan ve olmayan DS'lu hastalardan oluşan iki grup arasında, başvuru zamanlarındaki yaşlara göre istatistiksel anlamlılık (p<0.001) vardı. DS'lu konjenital hipotiroidisi olan 12 (%11.88) hasta vardı, bunların 11'inin KKH'lıklıydı. Konjenital, subklinik hipotiroidi ile ötiroidler arasında TSH düzeyi ve total tiroksin (tT4) düzeyi arasında istatistiksel (TSH için p< 0.001 ve tT4 için p< 0.001) anlamlı fark vardı. Ancak TSH ve total tT4 düzeyi ile KKH'nın bulunma durumunun istatistiksel anlamlığı yoktu. Sonuçlar: Çalışmamız, DS'lu tüm hastalar başvurularında dikkatli bir şekilde fizik ve ekokardiyografik muayene yapılması gerekli olduğu göstermektedir. Buna ek olarak, DS'lu çocuklarda konjenital ve subklinik hipotiroidinin akılda tutulması ve takibi de gereklidir. (Anadolu Kardiyol Derg 2010; 10: 440-5) Anahtar kelimeler: Down sendromu, konjenital kalp hastalığı, konjenital hipotiroidi, subklinik hipotiroidi ABSTRACT Objective: Congenital heart disease (CHD) associated with thyroid disease has been reported in Down syndrome (DS). The purpose of this work was to assess abnormalities of the thyroid in relation to the frequency and type of CHD on admission among children with DS. Methods: This retrospective study included 187 children with DS between August 1993-December 2005. Karyotype analysis, thyroid function tests and echocardiographic studies were performed in all children with DS. If necessary, hemodynamic study by catheterization was carried out. Thyrotropin releasing hormone (TRH) stimulation test was performed in patients having elevated thyroid stimulating hormone (TSH) level. Statistical analyses were performed using Chi-square, "t" test for independent samples or Mann-Whitney U test. Results: It was found that 136 (72.73%) patients with DS had CHD. The age difference at the time of admission was statistically significant for these two groups (p<0.001) in children with /without CHD. There were 12 (11.88%) patients with congenital hypothyroidism and DS, of whom 11 had CHD. There were statistically significant differences in the levels of TSH and total thyroxine (tT4) between congenital and subclinical hypothyroid and euthyroid groups (p<0.001 for TSH and p< 0.001 for tT4). ...

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“…5,6 Forty to fifty percent of children with DS have some form of congenital heart disease (CHD). 1,[7][8][9] Although the average life expectancy for children born with DS has increased from 12 years in the 1940s to 60 years in current times, infant mortality for patients with DS still remains 5× to 8× higher than that of the general population. 3 The relatively greater infant mortality rate in the DS population has been largely attributed to their having a higher incidence of CHD 2,9,10 ; however, there are limited data suggesting that children with DS may actually have superior outcomes after CHD repairs compared with their eugenic counterparts.…”

mentioning

confidence: 99%

Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease

Evans

Dharmar

Meierhenry

et al. 2014

Circ: Cardiovascular Quality and Outcomes

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Background— The prevalence of Down syndrome (DS)–affected births has increased during the past 30 years; moreover, children with DS have a higher incidence of congenital heart disease compared with their peers. Whether children with DS have better or worse outcomes after repair of congenital heart disease is unclear. We sought to identify differences in in-hospital mortality after cardiac surgery in pediatric patients with and without DS using a large national database. Methods and Results— Children aged <18 years who underwent surgical intervention for congenital heart disease were identified using the Kids’ Inpatient Database (2000, 2003, 2006, and 2009). Patients were stratified using the Risk Adjustment for Congenital Heart Surgery algorithm. A total of 4231 (8.2%) of the 51 309 patients studied had a diagnosis of DS. In-hospital death for patients with DS was significantly lower than that for patients without DS overall (1.9% versus 4.3%; P <0.05) as well as within risk categories 2 (1.0% versus 1.8%; P <0.05) and 3 (2.3% versus 5.1%; P <0.05). Multivariable logistic regression showed a lower odds of death among children with DS (odds ratio=0.60; 95% confidence interval, 0.47–0.76; P <0.05) after adjusting for Risk Adjustment for Congenital Heart Surgery risk category, premature birth, major noncardiac structural anomaly, and age. Conclusions— In this large national study, children with DS who underwent repair of congenital heart disease were more likely to survive to discharge than children without DS. Future work is needed to better understand the factors underlying these differences.

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Congenital Heart Disease in Infants With Downʼs Syndrome

Cited by 60 publications

References 0 publications

The epidemiology of congenital heart diseases in Saudi Arabia: A systematic review

The epidemiology of congenital heart diseases in Saudi Arabia: A systematic review

Evaluation of congenital heart diseases and thyroid abnormalities in children with Down syndrome

Association Between Down Syndrome and In-Hospital Death Among Children Undergoing Surgery for Congenital Heart Disease

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