scholarly journals Szemléletváltozás a Down-szindrómás szívbetegek szívsebészeti kezelésében, 1974–2016

2016 ◽  
Vol 157 (40) ◽  
pp. 1601-1603
Author(s):  
István Hartyánszky ◽  
Gábor Bogáts
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Life Expectancy ◽  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Cardiac Surgeons ◽  
The Impact

Introduction: Congenital heart defects are frequently present in patients with Down syndrome. Aim: The authors analyzed the impact of changing approach in surgical management of congenital heart defect on the life expectancy of patients with Down syndrome. Method: Between 1974 and 1997 the data of 359 children with Down syndrome were collected. Among them 255 patients had no surgery and the mortality in this group was 25.9%, whereas the mortality in the group of 104 patients who underwent palliative surgery was 8.6%. Results: Surgical management of congenital heart defects provides the same life expectancy for these patients as compared to Down patients without cardiac defects. Primary reconstruction is the preferable surgical procedure in infancy that provides good results. Nowadays the number of the operated grown-up congenital heart disease patients with Down syndrome is increasing. During the last three years 82 grown-up congenital heart disease patients, including 4 patients with Down syndrome (aged between 24 and 60 years) were reconstructed successfully. Conclusions: Due to the successful surgery in infancy the population of grown-up congenital heart disease patients with Down syndrome is increasing. The cardiac surgeons are ready to do everything for the optimal life expectancy of these patients. However, management of special problems (indication and necessity of reoperation, optimal age) in patients with Down syndrome poses a great challenge for cardiologists and cardiac surgeons. Orv. Hetil., 2016, 157(40), 1601–1603.

Abstract MP67: Pregnancy Outcomes in Women With Congenital Heart Defects in Colorado: Results From the Colorado CHD Surveillance System in Adolescents and Adults (COCHD)

Circulation ◽  
2018 ◽  
Vol 137 (suppl_1) ◽  
Author(s):  
Tessa Crume ◽  
Lindsey Duca ◽  
Christopher Rausch ◽  
Amber Khanna
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Surveillance System ◽  
Congenital Heart ◽  
Heart Defects ◽  
Cyanotic Congenital Heart Disease ◽  
Cardiac Events ◽  
Adolescents And Adults

Introduction: The normal hemodynamic changes of pregnancy can trigger cardiac dysfunction in women with congenital heart defects (CHD), leading to an increased risk of cardiac events. Further, utero-placental flow is impaired in women with CHD which can result in higher risk of adverse offspring outcomes. Hypothesis: Severity of maternal CHD is associated with adverse maternal cardiovascular events and offspring events by severity of CHD in 1074 pregnancies that resulted in a live birth in Colorado between 2011-13. Methods: Using data from the Colorado CHD Surveillance System in Adolescents and Adults, 926 pregnant women with CHD were identified that resulted in 1074 live births between 2011-2013. Maternal CHD type was dichotomized as severe (n=149) and moderate (total n=882). The risk of adverse maternal cardiac and offspring events were calculated out of the total number of pregnancies in each severity category. Results: Primary maternal cardiac events including cardiovascular mortality, arrhythmia, heart failure, thromboembolic events (pulmonary embolism, valve thrombosis or deep venous thrombosis), vascular events (stroke, myocardial infarction or dissection) and endocarditis occurred in 15% and 7% of women with severe and moderate CHD, respectively. Premature birth (delivery <37 weeks) and/or low birth weight (<2,500 grams) occurred in >18% of the women with severe CHD and 17% of women with moderate CHD. The risk of offspring congenital anomalies among women with severe CHD was extremely high for circulatory/respiratory, musculoskeletal/integumentary, cyanotic congenital heart disease and Down syndrome. The risk of infant congenital anomalies was lower for women with moderate CHD, with the most common being circulatory/respiratory, urogenital, Down syndrome and cyanotic congenital heart disease. Conclusions: Population-level surveillance system of CHD in Colorado provides novel assessment of the substantial risk of maternal and neonatal cardiac events associated with pregnancy in women with CHD.

scholarly journals Personalized Genetic Diagnosis of Congenital Heart Defects in Newborns

2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Malformations ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Genetic Diagnosis ◽  
Single Nucleotide Variants ◽  
Personalized Approach

Congenital heart disease is a group of pathologies characterized by structural malformations of the heart or great vessels. These alterations occur during the embryonic period and are the most frequently observed severe congenital malformations, the main cause of neonatal mortality due to malformation, and the second most frequent congenital malformations overall after malformations of the central nervous system. The severity of different types of congenital heart disease varies depending on the combination of associated anatomical defects. The causes of these malformations are usually considered multifactorial, but genetic variants play a key role. Currently, use of high-throughput genetic technologies allows identification of pathogenic aneuploidies, deletions/duplications of large segments, as well as rare single nucleotide variants. The high incidence of congenital heart disease as well as the associated complications makes it necessary to establish a diagnosis as early as possible to adopt the most appropriate measures in a personalized approach. In this review, we provide an exhaustive update of the genetic bases of the most frequent congenital heart diseases as well as other syndromes associated with congenital heart defects, and how genetic data can be translated to clinical practice in a personalized approach.

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 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.

Cause of death in adults with congenital heart disease — An analysis of the German National Register for Congenital Heart Defects

2016 ◽  
Vol 211 ◽  
pp. 31-36 ◽  
Author(s):  
Claudia C. Engelings ◽  
Paul C. Helm ◽  
Hashim Abdul-Khaliq ◽  
Boulos Asfour ◽  
Ulrike M.M. Bauer ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Cause Of Death ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
National Register

scholarly journals Surgical Options for Pulmonary Valve Pathology in the Current Era

2021 ◽  
Author(s):  
Sameh M. Said
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Carcinoid Syndrome ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Clinical Profile ◽  
Surgical Options ◽  
Valve Pathology

Pulmonary valve pathology occurs mostly in the settings of congenital heart disease whether primary or as the result of repair of a variety of congenital heart defects. Acquired pulmonary valve disorders, albeit rare, can occur in the settings of endocarditis, tumors, carcinoid syndrome, or rheumatic fever. Surgical options include repair and replacement of pulmonary valve. Several options for replacement are available, which can be tailored based on the patient’s clinical profile and the primary valve pathology. In this chapter, we present the surgical options that are currently available for pulmonary valve disorders and the current outcomes.

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