The Impact of Congenital Heart Disease on Cognitive and Behavioral Functioning

Author(s):  
Jo Wray

Congenital heart disease (CHD) has been defined as “. . . a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance” (Mitchell, Korones, and Berendes 1971). Congenital heart disease is the most common single group of congenital abnormalities, accounting for about 30% of the total. The incidence is reported as varying between 0.3% and 1% of all live births. Ten to 15% of children with congenital heart defects have more than one cardiac abnormality; up to one-third also have one or more associated noncardiac congenital abnormalities (Wernovsky 2006). Although some forms of CHD are minor and do not require any medical or surgical intervention, others are very complex and may necessitate a series of staged surgical procedures and/or require life-long medications. Significant improvements in medical and surgical techniques have resulted in increasing numbers of children and adults living with CHD, and it is currently anticipated that 80%–85% of children born with CHD today will survive into adulthood (British Cardiac Society 2002). However, although survival rates have improved dramatically over the last 40 years or so, morbidity remains a concern. Congenital heart defects can be broadly subdivided into two groups, based on changes in the circulation. Acyanotic defects may be due to either a left-to-right shunt or to an obstructive lesion; there is no mixing of desaturated blood in the systemic arterial circulation. With cyanotic defects, there may be either increased or diminished pulmonary flow, and desaturated blood enters the systemic arterial circulation, regardless of whether cyanosis is clinically evident. Unsaturated venous blood bypassing the lungs can result in secondary polycythemia, which is a compensatory mechanism to carry more oxygen to the tissues. This causes increased viscosity, which in turn results in sluggish blood circulation and impeded blood flow, particularly in the capillaries. Poor peripheral blood flow and clubbing of the fingers and toes can result, breathlessness and fatigue often result in a reduced exercise tolerance, and growth may be affected.

2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  

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.


2016 ◽  
Vol 211 ◽  
pp. 31-36 ◽  
Author(s):  
Claudia C. Engelings ◽  
Paul C. Helm ◽  
Hashim Abdul-Khaliq ◽  
Boulos Asfour ◽  
Ulrike M.M. Bauer ◽  
...  

2021 ◽  
Author(s):  
Sameh M. Said

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.


1986 ◽  
Vol 67 (4) ◽  
pp. 265-268
Author(s):  
I. F. Matyushin ◽  
I. K. Okhotin ◽  
V. Ya. Ovsyanikov

We have undertaken a study of thymus gland structure in children with congenital heart defects aged 3 to 10 years. We analyzed thymus biopsy specimens obtained from the marginal zone of the gland during surgical correction of cardiac malformations after thoracic cavity dissection. The biopsy size was 1.5 cm3.


2016 ◽  
Vol 73 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Vesna Miranovic

Bacground/Aim. Congenital heart defects (CHDs) are structural or functional abnormalities of the heart present at birth even if they are detected much later. Their importance lies in the fact that, depending on the severity, they change the quality of life, and may be life threating. In addition, we should not ignore the high costs of treating people with congenital heart disease. The aim of this study was to analyze the incidence of congenital heart disease in relation to the severity in the world based on the available literature. Methods. All the available literature on the incidence of CHD cases regarding the severity of CHD published from 1955 to 2012 was analyzed. The researcher was able to read the titles and abstracts of 128 papers on the subject. Due to methodological inconsistency, 117 of the papers were rejected. Based on the criteria of reliability, availability and comparability, our analysis included 11 studies testing CHD incidence regarding the severity of the defect conducted all over the world. The Yates' ?2-test was used to compare the observed incidences. Results. The frequency of severe congenital heart defects, ranged from 0.414 to 2.3/1,000 live births, the incidence of moderate congenital heart defects from 0.43 to 2.6/1,000 live births while in the group of minor congenital heart defects the incidence ranged from 0.99 to 10.3/1000 live births. There were no statistically significant differences in the incidence of mild, moderate and severe CHDs. Conclusion. The results obtained studying of the available data suggest that no statistically significant difference in the incidence of mild, moderate and severe congenital heart defects. A universal methodological approach to the incidence of CHD is essential.


PEDIATRICS ◽  
1956 ◽  
Vol 18 (3) ◽  
pp. 491-500 ◽  
Author(s):  
John D. Keith

HEART failure is associated with an inability of the heart to empty itself adequately, with the result that there is a high venous filling pressure and a decrease in the effective work done by the heart muscle. There are several factors that, if sufficiently severe, will produce congestive heart failure in either infancy or childhood. These include valvular obstruction or insufficiency; mechanical obstruction of the heart as a whole, as in pericardial disease; the physical effects of large intracardiac shunts which increase the load on one or both ventricles; the presence of raised pressure in the pulmonary or systemic circulation; inflammatory reactions in the heart muscle or oxygen lack; and, finally, certain metabolic disturbances, such as hyperthyroidism or hypothyroidism. One or more of these factors may be operating in the same child, as in rheumatic fever where myocarditis is associated with valvular insufficiency, or in congenital heart disease with pulmonary stenosis and patent foramen ovale, where the right ventricle has a high pressure to maintain and is at the same time being offered cyanotic blood from the coronaries. PATIENT MATERIAL In analyzing 1,580 cases of congenital heart disease at the Hospital for Sick Children, Toronto, 20 per cent were found to have had failure at some time. In 90 per cent of these failure occurred in the first year of life. A list of the various causes of heart failure in the pediatric age group in order of frequency follows. [see table in source pdf] In certain types of heart defects failure develops in characteristic age groups. For example, during the first week of life the most common cause of heart failure is aortic atresia. From 1 week to 1 month, coarctation of the aorta leads. From 1 to 2 months, transposition of the great vessels predominates. From 2 to 3 months, endocardial fibroelastosis is the chief cause of heart failure, with transportation of the great vessels second to it. The actual incidence of type of heart defect in relation to age at onset of heart failure is as follows.


Sign in / Sign up

Export Citation Format

Share Document