Congenital Heart Disease and Down Syndrome

2016 ◽  
pp. 1301-1310 ◽  
Author(s):  
Kenneth J. Dooley
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart

scholarly journals Down syndrome and congenital heart disease: perioperative planning and management

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Defects ◽  
Careful Consideration ◽  
Children With Down Syndrome ◽  
Perioperative Planning

AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

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

2014 ◽  
Vol 7 (3) ◽  
pp. 445-452 ◽  
Author(s):  
Jacqueline M. Evans ◽  
Madan Dharmar ◽  
Erin Meierhenry ◽  
James P. Marcin ◽  
Gary W. Raff
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Hospital Death

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

Targeted Next-Generation Sequencing of 406 Genes Identified Genetic Defects Underlying Congenital Heart Disease in Down Syndrome Patients

2018 ◽  
Vol 39 (8) ◽  
pp. 1676-1680 ◽  
Author(s):  
Khalid M. Alharbi ◽  
Abdelhadi H. Al-Mazroea ◽  
Atiyeh M. Abdallah ◽  
Yousef Almohammadi ◽  
S. Justin Carlus ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Next Generation Sequencing ◽  
Congenital Heart ◽  
Genetic Defects ◽  
Next Generation ◽  
Targeted Next Generation Sequencing ◽  
Generation Sequencing

scholarly journals Congenital Heart Disease among Down Syndrome Children at Dr. Hasan Sadikin General Hospital from 2008 to 2013

2018 ◽  
Vol 5 (1) ◽  
pp. 6-11
Author(s):  
Thyviyaa Rajamany ◽  
Rahmat Budi Kuswiyanto ◽  
Leonardo Lubis
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
General Hospital ◽  
Congenital Heart

Screening for Congenital Heart Disease in Infants with Down Syndrome: Is Universal Echocardiography Necessary?

2016 ◽  
Vol 37 (7) ◽  
pp. 1222-1227 ◽  
Author(s):  
Soujanya Bogarapu ◽  
Nelangi M. Pinto ◽  
Susan P. Etheridge ◽  
Xiaoming Sheng ◽  
Kirk N. Liesemer ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart

scholarly journals Penetrance of Congenital Heart Disease in a Mouse Model of Down Syndrome Depends on a Trisomic Potentiator of a Disomic Modifier

Genetics ◽  
2016 ◽  
Vol 203 (2) ◽  
pp. 763-770 ◽  
Author(s):  
Huiqing Li ◽  
Sarah Edie ◽  
Donna Klinedinst ◽  
Jun Seop Jeong ◽  
Seth Blackshaw ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Mouse Model ◽  
Congenital Heart

scholarly journals Does Congenital Heart Disease Affect Neurodevelopmental Outcomes in Children with Down Syndrome?

2016 ◽  
Vol 11 (1) ◽  
pp. 26-33 ◽  
Author(s):  
Tarek Alsaied ◽  
Bradley S. Marino ◽  
Anna J. Esbensen ◽  
Julia S. Anixt ◽  
Jeffery N. Epstein ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Neurodevelopmental Outcomes ◽  
Children With Down Syndrome

scholarly journals ADULTS WITH DOWN SYNDROME AND CONGENITAL HEART DISEASE: HOSPITALIZATIONS AND MORTALITY IN THE UNITED STATES

2012 ◽  
Vol 59 (13) ◽  
pp. E788
Author(s):  
Fernando E. Baraona ◽  
Michelle Gurvitz ◽  
Michael Landzberg ◽  
Alexander Opotowsky
Keyword(s):  
United States ◽  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
The United States

Altered autonomic cardiac regulation in individuals with Down syndrome

2005 ◽  
Vol 289 (6) ◽  
pp. H2387-H2391 ◽  
Author(s):  
Ferdinando Iellamo ◽  
Alberto Galante ◽  
Jacopo M. Legramante ◽  
Maria Enrichetta Lippi ◽  
Claudia Condoluci ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Sinus Node ◽  
Control Subjects ◽  
Arterial Blood ◽  
Cardiac Regulation ◽  
Autonomic Cardiac Regulation ◽  
And Control

We tested the hypothesis that individuals with Down syndrome, but without congenital heart disease, exhibit altered autonomic cardiac regulation. Ten subjects with Down syndrome (DS) and ten gender-and age-matched healthy control subjects were studied at rest and during active orthostatism, which induces reciprocal changes in sympathetic and parasympathetic traffic to the heart. Autoregressive power spectral analysis was used to investigate R-R interval variability. Baroreflex modulation of sinus node was assessed by the spontaneous baroreflex sequences method. No significant differences between DS and control subjects were observed in arterial blood pressure at rest or in response to standing. Also, R-R interval did not differ at rest. R-R interval decreased significantly less during standing in DS vs. control subjects. Low-frequency (LFNU) and high-frequency (HFNU) (both expressed in normalized units) components of R-R interval variability did not differ between DS and control subjects at rest. During standing, significant increase in LFNU and decrease in HFNU were observed in control subjects but not in DS subjects. Baroreflex sensitivity (BRS) did not differ between DS and control subjects at rest and underwent significant decrease on going from supine to upright in both groups. However, BRS was greater in DS vs. control subjects during standing. These data indicate that subjects with DS exhibit reduced HR response to orthostatic stress associated with blunted sympathetic activation and vagal withdrawal and with a lesser reduction in BRS in response to active orthostatism. These findings suggest overall impairment in autonomic cardiac regulation in DS and may help to explain the chronotropic incompetence typically reported during exercise in subjects with DS without congenital heart disease.

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