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

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.

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

Revista Paulista de Pediatria ◽

10.1590/0103-0582201432218913 ◽

2014 ◽

Vol 32 (2) ◽

pp. 159-163 ◽

Cited By ~ 17

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.

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

Pediatric Cardiology ◽

10.1007/s00246-018-1951-3 ◽

2018 ◽

Vol 39 (8) ◽

pp. 1676-1680 ◽

Cited By ~ 7

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

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

Althea Medical Journal ◽

10.15850/amj.v5n1.1328 ◽

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

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

Pediatric Cardiology ◽

10.1007/s00246-016-1419-2 ◽

2016 ◽

Vol 37 (7) ◽

pp. 1222-1227 ◽

Cited By ~ 8

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

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

Genetics ◽

10.1534/genetics.116.188045 ◽

2016 ◽

Vol 203 (2) ◽

pp. 763-770 ◽

Cited By ~ 20

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

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Congenital Heart Disease and Down Syndrome

Health Care for People with Intellectual and Developmental Disabilities across the Lifespan ◽

10.1007/978-3-319-18096-0_105 ◽

2016 ◽

pp. 1301-1310 ◽

Cited By ~ 2

Author(s):

Kenneth J. Dooley

Keyword(s):

Congenital Heart Disease ◽

Down Syndrome ◽

Heart Disease ◽

Congenital Heart

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P1802Management and outcomes of infective endocarditis in adults with congenital heart disease

European Heart Journal ◽

10.1093/eurheartj/ehz748.0554 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

R Ly ◽

D Lebeaux ◽

F Pontnau ◽

F Compain ◽

B Gaye ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Infective Endocarditis ◽

Congenital Heart ◽

Hospital Death ◽

Oral Streptococci ◽

Mortality And Morbidity ◽

Risk Of Death ◽

Duke Criteria ◽

Increased Risk

Abstract Background Causes, epidemiology and microbiology of infective endocarditis (IE) have evolved in recent decades. Although novel tools for the diagnosis and therapeutic strategies have emerged, mortality and morbidity remain high. These trends may particularly concern the growing population of adults with congenital heart disease (CHD) who are at increased risk for IE. Purpose We aimed to characterize IE in CHD patients and describe management and outcome in this setting. We also sought to determine the risk factors associated with in-hospital death in CHD patients. Methods From January 2000 to June 2018, 666 consecutive episodes of IE in adults were recorded in our center. Among them, 143 concerned CHD, including 5 implantable cardiac electronic devices-lead infections, all managed by an IE team including CHD specialists. Cases were classified according to modified Duke criteria. Results CHD patients were significantly younger (37 years IQR [26–52]), with a more common history of cardiac reoperations (numbers of sternotomies≥2 in 35.7%) and infective endocarditis (19.7%, p<0.01) compared to non-CHD patients. There were more infections of valve-containing prosthetics (44% vs. 30%, p<0.04), and the right heart side (41.5%, p<0.01) in CHD patients. Forty-nine percent of them had a simple CHD, 12.7% a moderate, and 36.4% a complex. A predisposing event could be identified in only 34% of cases. Oral streptococci/Streptococci bovis and Staphylococcus aureus were the most frequently microorganisms isolated (32.4% and 20.4%, respectively). Surgery was performed in 90 episodes (62%), and was selected in emergency (<24h) in 61% (figure 1). In-hospital mortality was 12.7% and was directly related to IE in 10/18 cases. CHD patients had a significant lower risk of death compared to non-CHD patients (OR=0.47, p=0.026, p<0.01), even after adjustment for age, and the infected heart side. On multivariate analysis the complexity of CHD (if simple CHD: OR=0.07 IQR [0.01 to 0.44], p<0.01) and the white blood cell count (OR=1.18 IQR [1.04 to 1.33], p=0.01) were the strongest predictive factors of in-hospital death in the CHD group. Conclusions Mortality associated with IE in CHD patients is lower than in acquired heart disease. The multidisciplinary approach by IE team and CHD specialists may have improved management and outcome in this setting. However, risk for death remains high in complex lesions. Larger prospective studies on IE in adults with CHD are needed to develop guidelines in these complex patients.

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