scholarly journals Epidemiology and Evaluation of Congenital Cyanotic Heart Disease in Children- A Review

Author(s):  
Ahmed Abdelsamie Fadl ◽  
Yaslam Mabruk S. Alsaiari ◽  
Khadijah Nasr Aldeen M. Dhafer ◽  
Marwan Saleh S. Alharbi ◽  
Rakan Hamdan M. Alelyani ◽  
...  

Structure abnormalities of the heart or intrathoracic great vessels that arise during fetal development are known as congenital heart disease (CHD). It is the most frequent type of birth defect. Despite great success in surgical and medicinal care of CHD. many operations are palliative rather than curative. and some survivors still have significant residual hemodynamic and electrical conduction abnormalities. as well as long-term cardiovascular problems. The field of congenital heart defect treatment has come a long way in its infancy. With heart problems that were an automatic death sentence 60 years ago, to contemporary surgical survival rates of more than 96 percent for all defects evaluated together. No other branch of research or medicine has done so much in such a short time. In this paper, we overview epidemiology, evaluation and management of congenital cyanotic heart diseases in children.

2019 ◽  
Vol 13 (4) ◽  
pp. 189-199
Author(s):  
Luciano Daliento ◽  
Elettra Pomiato ◽  
Giovanni Vescovo ◽  
Massimo Padalino ◽  
Giovannella Russo

More than 60 years have passed since Lillehei “The father of open surgery” started the era of correction of congenital heart diseases1. At the end of the sixties, only the minority of the patients with complex congenital heart diseases survived the first year after birth2; nowadays on the contrary, the grown-up congenital heart population exceeds the pediatric one3–5. The reasons of such a change are different: fetal echocardiography has made prenatal diagnosis of heart anomalies possible, thus causing the raise of interruptions of pregnancy. On the other hand, the high diagnostical accuracy and the brilliant surgery outcome along with an improved medical management allow higher survival rate6–8. Early during seventies Perloff foretold: “…it is a simple matter of time before a population of adult with congenital heart disease would emerge […]; we are obliged to look beyond the present and define our ultimate goal: the quality of life of long-term survival “9.


Circulation ◽  
1999 ◽  
Vol 100 (suppl_2) ◽  
Author(s):  
Jacqueline M. Lamour ◽  
Linda J. Addonizio ◽  
Mark E. Galantowicz ◽  
Jan M. Quaegebeur ◽  
Donna M. Mancini ◽  
...  

Background —Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. Methods and Results —We reviewed the pretransplantation and posttransplantation courses of 24 patients >18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50%), and d -transposition of the great vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79% at 1 year and 60% at 5 years . No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD ( P =0.83). Conclusions —Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients.


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.


2020 ◽  
Vol 40 (2) ◽  
pp. 72-77
Author(s):  
Nita Sharma ◽  
Pratima Sharma ◽  
Tulashi Adhikari Mishra

Introduction: Congenital Heart Disease (CHD) is a problem with structure and function of the heart that is present at birth.  Children with CHD require special care, treatment and follow up for a number of common conditions which may be quite straining to the care givers. The objective of the study was to find out the burden of care among mothers having children with CHD. Methods: This descriptive cross-sectional study was carried out in a cardiac centre of Nepal. A total of 95 mothers having children with CHD attending outpatient department of our institute were selected as the sample for the study using non-probability purposive sampling technique. A semi structured interview questionnaire consisting of the Modified Caregiver Strain Index was used to assess the burden of care among mothers having children with CHD. Frequency and percent were used to describe the variables and chi- square test at 0.05 significance level was used to analyse associations. Results: Most (77.9%) of the mothers were regularly strained to find that their children’s health condition was deteriorating due to CHD. Nearly half (44.2%) of the mothers always had financial constrain while giving care to the child, nearly half (40%) of the mothers had done emotional adjustments to take care of their children with CHD, another two-fifths (28.4%) of the mothers sometimes had disturbed sleep and almost half (46.3%) of the mothers were always upset due to some behaviour of their child with CHD. Half (50.5%) of the mothers had high level of burden of care. Statistically significant association were found between age of the mother and level of burden of care (p value = 0.05). Similarly, the type of family (p value = 0.005), age of the children (p value = 0.000) and type of CHD (p value = 0.002) were significantly associated with the level of burden of care among the mothers. Conclusion: The study concluded that mothers tend to feel less burden of care as the child grows older, mothers having children with cyanotic heart disease tend to experience more burden of care. Mothers of less than thirty years of age and living in a joint family also experience more burden of care.  


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.


2021 ◽  
Vol 126 (3) ◽  
pp. 365-379
Author(s):  
Gianluca Pontone ◽  
Ernesto Di Cesare ◽  
Silvia Castelletti ◽  
Francesco De Cobelli ◽  
Manuel De Lazzari ◽  
...  

AbstractCardiac magnetic resonance (CMR) has emerged as new mainstream technique for the evaluation of patients with cardiac diseases, providing unique information to support clinical decision-making. This document has been developed by a joined group of experts of the Italian Society of Cardiology and Italian society of Radiology and aims to produce an updated consensus statement about the current state of technology and clinical applications of CMR. The writing committee consisted of members and experts of both societies who worked jointly to develop a more integrated approach in the field of cardiac radiology. Part 1 of the document will cover ischemic heart disease, congenital heart disease, cardio-oncology, cardiac masses and heart transplant.


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