Cardiovascular abnormalities in Down's syndrome: spectrum, management and survival over 22 years

Irving, Claire; Chaudhari, Milind

doi:10.1136/adc.2010.210534

Cited by 137 publications

(122 citation statements)

References 19 publications

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“…Down's syndrome is strongly associated with the development of pulmonary hypertension, predominantly associated with upper airway obstruction and CHD. The prevalence of CHD in neonates with Down's syndrome is around 42-58%, with AVSD and VSD representing the most common defects [9,[69][70][71]. A number of factors in individuals with Down's syndrome may contribute to the high rates of PAH reported in this population.…”

Section: Review: Pah Associated With Chdmentioning

confidence: 99%

Pulmonary arterial hypertension associated with congenital heart disease

2012

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Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD.The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges.This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

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Section: Review: Pah Associated With Chdmentioning

confidence: 99%

Pulmonary arterial hypertension associated with congenital heart disease

2012

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“…own syndrome (DS) affects 1 to 2 per 1000 live births [1][2][3] and is the most commonly occurring chromosomal abnormality in live-born infants. 4 Despite increases in antenatal detection, the prevalence of babies born with DS has risen by 25% during the past 30 years and parallels the increase in advanced maternal age pregnancies.…”

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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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“…Las malformaciones más prevalentes asociadas a esta condición son las del sistema cardiovascular, con una incidencia que varía entre 42 a 48% para las malformaciones cardíacas 34,35 , siendo el hallazgo más frecuente el defecto aurículoventricular.…”

Section: Patología Cardíacaunclassified