Congenital heart anomalies and non-white ethnicity are among the factors associated with poor survival rates in people with Down syndrome

2018 ◽  
Vol 22 (1) ◽  
pp. 17-17
Author(s):  
Pauline Heslop
2021 ◽  
Vol 12 (4) ◽  
pp. 473-479
Author(s):  
Orlando José Tamariz-Cruz ◽  
Luis Antonio García-Benítez ◽  
Hector Díliz-Nava ◽  
Felipa Acosta-Garduño ◽  
Marcela Barrera-Fuentes ◽  
...  

Background: Early extubation is performed either in the operating room or in the cardiovascular intensive care unit during the first 24 postoperative hours; however, altitude might possibly affect the process. The aim of this study is the evaluation of early extubation feasibility of patients undergoing congenital heart surgery in a center located at 2,691 m (8,828 ft.) above sea level. Material and Methods: Patients undergoing congenital heart surgery, from August 2012 through December 2018, were considered for early extubation. The following variables were recorded: weight, serum lactate, presence or not of Down syndrome, optimal oxygenation and acid–base status according to individual physiological condition (biventricular or univentricular), age, bypass time, and ventricular function. Standardized anesthetic management with dexmedetomidine–fentanyl–rocuronium and sevoflurane was used. If extubation in the operating room was considered, 0.08 mL/kg of 0.5% ropivacaine was injected into the parasternal intercostal spaces bilaterally before closing the sternum. Results: Four hundred seventy-eight patients were operated and 81% were early extubated. Mean pre- and postoperative SaO2 was 92% and 98%; postoperative SaO2 for Glenn and Fontan procedures patients was 82% and 91%, respectively. Seventy-three percent of patients who underwent Glenn procedure, 89% of those who underwent Fontan procedure (all nonfenestrated), and 85% with Down syndrome were extubated in the operating room. Reintubation rate in early extubated patients was 3.6%. Conclusion: Early extubation is feasible, with low reintubation rates, at 2,691 m (8,828 ft.) above sea level, even in patients with single ventricle physiology.


2021 ◽  
pp. 1-6
Author(s):  
Daniel Rinnström ◽  
Mikael Dellborg ◽  
Ulf Thilén ◽  
Peder Sörensson ◽  
Niels-Erik Nielsen ◽  
...  

Abstract Background: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients. Methods and results: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]). Conclusions: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  

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.


2017 ◽  
Vol 183 ◽  
pp. 67-73.e1 ◽  
Author(s):  
Anna Lonyai Harbison ◽  
Jodie K. Votava-Smith ◽  
Sylvia del Castillo ◽  
S. Ram Kumar ◽  
Vince Lee ◽  
...  

2014 ◽  
Vol 7 (3) ◽  
pp. 445-452 ◽  
Author(s):  
Jacqueline M. Evans ◽  
Madan Dharmar ◽  
Erin Meierhenry ◽  
James P. Marcin ◽  
Gary W. Raff

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos

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.


Author(s):  
Rutika, B. Maske

The purpose of this review is to provide the latest information on Down syndrome. The author conducted a literature search of available sources describing the issue of down syndrome with special focus on syndrome and made a comparison and evaluation of relevant findings.The results of this review indicate that Down syndrome (DS) is one of the commonest disorders with huge medical and social cost. DS is associated with number of phenotypes including congenital heart defects, leukemia, Alzeihmer’s disease, Hirschsprung disease etc. DS individuals are affected by these phenotypes to a variable extent thus understanding the cause of this variation is a key challenge. In the present review article, we emphasize an overview of DS, DS-associated phenotypes diagnosis and management of the disease.


Sign in / Sign up

Export Citation Format

Share Document