Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life?

Masuda, Mitsuharu; Kado, Hideaki; Tanoue, Yoshihisa; Fukae, Kouji; Onzuka, Tatsushi; Shiokawa, Yuichiro; Shirota, Toshihiko; Yasui, Hirofumi

doi:10.1016/j.ejcts.2004.11.027

Cited by 40 publications

(28 citation statements)

References 22 publications

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“…1,2,[11][12][13]19,22,23,30 In a study using the 2000 version of the KID to examine the role of sex as a risk factor for in-hospital mortality after pediatric cardiac surgery, DS was 1 of 4 comorbidities used as at CONS CALIFORNIA DIG LIB on January 21, 2016 http://circoutcomes.ahajournals.org/ Downloaded from independent, nonprocedural variables. The findings of this study are consistent with our findings and demonstrate lower odds of in-hospital mortality for patients with DS.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

“…[14][15][16] The vast majority of infants born with an AVSD also have DS [11][12][13][17][18][19][20][21] ; however, DS is not a known risk factor for morbidity and mortality after biventricular AVSD repair. 12,22 In fact, infants with DS tend to have better outcomes after surgical repair of an AVSD compared with infants without DS.…”

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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“…Current best practice suggests that prompt surgical correction of cardiac defects is imperative to prevent the development of irreversible pulmonary vascular disease [12]. The mainstay of management of lower airways disease is the use of prophylactic antibiotics and regular inhaled corticosteroids, alongside oxygen and physiotherapy [4].…”

Section: Discussionmentioning

confidence: 99%

“…At the current time, it is clear that all those with large left-to-right shunts have the potential to develop pulmonary vascular disease, but some do not demonstrate any symptoms due to the raised pulmonary vascular resistance, perhaps associated with Upper Airway Obstruction (UAO) [3,9]. This is ameliorated but not abolished by the screening programme and the advent of earlier definitive cardiac surgery at 3-6 months of age [12]. However, even with screening, a number will develop PAH and require investigationpart of the assessment of this should be a vigorous search for other causes (especially respiratory).…”

Section: Introductionmentioning

confidence: 97%

Management of pulmonary hypertension in Down syndrome

et al. 2011

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“…Today surgery is offered to all Down syndrome patients with CHD although this was not always the case. Surgical results are good, and there is believed to be no extra risk from the concomitant presence of Down syndrome [22,23]. Surgical repair is aimed in the first few months of life and certainly before 6 months old as irreversible pulmonary vascular disease is more likely to develop quickly in patients with Down syndrome and AVSD.…”

Section: Atrioventricular Septal Defect (Avsd)mentioning

confidence: 99%

Congenital Heart Disease in Down Syndrome

Morrison¹,

McMahon²

2018

Advances in Research on Down Syndrome

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Down syndrome remains the most common chromosomal abnormality in live-born infants in the world today. The association between Down syndrome and congenital heart disease (CHD) is well known, and it is widely recognized that CHD contributes significantly to the morbidity of children with Down syndrome. The reported incidence of CHD in Down syndrome patients is between 40 and 60%. The most commonly described defect is complete atrioventricular septal defect (AVSD), which comprises 30-40% of all cardiac defects. Complex genetic factors are involved. Routine cardiac screening of all newborn babies with Down syndrome is recommended. Expert groups suggest that the cardiac status of all children with Down syndrome should be established by 6 weeks of age to permit appropriate and timely treatment avoiding the establishment of irreversible pulmonary vascular disease that would make corrective surgery impossible.

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Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life?

Abstract: Down syndrome does not affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life.

Cited by 40 publications

References 22 publications

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

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

Management of pulmonary hypertension in Down syndrome

Congenital Heart Disease in Down Syndrome

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