scholarly journals Chromosomal Anomalies Influence Parental Treatment Decisions in Relation to Prenatally Diagnosed Congenital Heart Disease

2009 ◽  
Vol 30 (8) ◽  
pp. 1105-1111 ◽  
Author(s):  
Sinai C. Zyblewski ◽  
Elizabeth G. Hill ◽  
Girish Shirali ◽  
Andrew Atz ◽  
Geoffrey Forbus ◽  
...  
Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Pirouz Shamszad ◽  
Shaine A Morris ◽  
Deipanjan Nandi ◽  
Andrew T Costarino ◽  
Bradley S Marino ◽  
...  

Introduction: The management of neonates with congenital heart disease (CHD) may be complicated by necrotizing enterocolitis (NEC), however, there is limited multicenter data describing the incidence and outcomes of NEC in the CHD population. Objective: We aimed to assess the incidence and risk factors for the development of NEC in neonates with major CHD and the impact on survival. Methods: A retrospective cohort study of neonates with CHD was performed for all index hospitalizations of neonates (<28 days) with major CHD between 2004 and 2014 using the Pediatric Health Information System database. The diagnosis of NEC was determined by the presence of ICD-9 code 777.5x. The incidence of NEC was determined as were risk factors for the development of NEC. Mortality was the primary outcome measure. Results: Of 38770 neonates with major CHD, 1448 (3.6%) were diagnosed with NEC. The rate of NEC varied between 0-8% by hospital and was not associated with hospital volume (p=0.4). Among neonates with a single, major CHD diagnosis, the rate of NEC was 6% in hypoplastic left heart syndrome (HLHS), 6% in truncus arteriosus (TA) , 4% in tetralogy of Fallot (TOF), 3% in aortic arch obstruction (AO), and 2% in transposition of the great arteries (TGA); these diagnoses accounted for 47% of all NEC. Prematurity and chromosomal anomalies were independently associated with the diagnosis of NEC (p≤0.01 for both). Unadjusted mortality among neonates with NEC was 24% compared to 12% in neonates without NEC (OR 2.4, 95%CI 2.1-2.7). When evaluating changes in adjusted mortality associated with NEC by CHD diagnosis, TOF mortality increased from 8% to 16% (p<.01), TGA increased from 5% to 21% (p<0.01), AO increased from 6% to 20% (p<0.01), HLHS increased from 22% to 28% (p=.07), and TA decreased from 13% to 12% (p=0.7). Median LOS was higher in neonates with NEC than without NEC (54d [IQR 31-93] vs. 18d [IQR 9-34], p<0.01) as was median hospital charge ($600k [IQR 310k-1.1m] vs. $220k [IQR 100k-430k], p<0.01). Conclusions: The incidence of NEC among neonates with major CHD is highest in HLHS and TA. NEC is associated with significantly higher hospital mortality, LOS, and charges. Determining modifiable factors associated with NEC may allow for interventions to reduce morbidity in this population.


1991 ◽  
Vol 1 (1) ◽  
pp. 8-11 ◽  
Author(s):  
L. D. Allan ◽  
G. K. Sharland ◽  
S. K. Chita ◽  
S. Lockhart ◽  
D. J. Maxwell

Children ◽  
2019 ◽  
Vol 6 (9) ◽  
pp. 98
Author(s):  
Kylat

Tracheal rings (TR) are rare, congenital cartilaginous defect of the upper airway and are usually due to complete or near complete circumferential cartilaginous tracheal rings, with variable degrees of tracheal stenosis (TS) and shortening. Chromosomal anomalies like trisomy 21 are characteristically associated with a wide range of upper airway anomalies including TS and congenital heart disease (CHD). However, the overall prevalence of severe forms of TS is rare and reported in 1.2% of all CHD patients. Herein, we present a rare association of severe TS due to complete tracheal rings in a trisomy 21 patient with CHD and the challenges in the management.


2021 ◽  
Vol 8 ◽  
Author(s):  
Jef Van den Eynde ◽  
Cedric Manlhiot ◽  
Alexander Van De Bruaene ◽  
Gerhard-Paul Diller ◽  
Alejandro F. Frangi ◽  
...  

Built on the foundation of the randomized controlled trial (RCT), Evidence Based Medicine (EBM) is at its best when optimizing outcomes for homogeneous cohorts of patients like those participating in an RCT. Its weakness is a failure to resolve a clinical quandary: patients appear for care individually, each may differ in important ways from an RCT cohort, and the physician will wonder each time if following EBM will provide best guidance for this unique patient. In an effort to overcome this weakness, and promote higher quality care through a more personalized approach, a new framework has been proposed: Medicine-Based Evidence (MBE). In this approach, big data and deep learning techniques are embraced to interrogate treatment responses among patients in real-world clinical practice. Such statistical models are then integrated with mechanistic disease models to construct a “digital twin,” which serves as the real-time digital counterpart of a patient. MBE is thereby capable of dynamically modeling the effects of various treatment decisions in the context of an individual's specific characteristics. In this article, we discuss how MBE could benefit patients with congenital heart disease, a field where RCTs are difficult to conduct and often fail to provide definitive solutions because of a small number of subjects, their clinical complexity, and heterogeneity. We will also highlight the challenges that must be addressed before MBE can be embraced in clinical practice and its full potential can be realized.


2010 ◽  
Vol 10 (1-3) ◽  
pp. 131-139 ◽  
Author(s):  
Smitha Rame Gowda ◽  
Harshavardhan M. Gawde ◽  
Abbas Hyderi ◽  
Mysore R. Savitha ◽  
Balasundaram Krishnamurthy ◽  
...  

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