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PubMed1992

Social adaptation of young adults with congenital heart disease.

The social status of 71 young adults (30 females, 41 males, aged 22.9 +/- 1.9 yr) with congenital heart disease was compared with that of 211 (106 females, 105 males, mean age 23.2 +/- 1.4 yr) randomly selected controls. The clinical examinations included clinical study with ultrasonic visualization, electrocardiography, X-ray and measurement of exercise capacity. In the study group 87% had completed school at the secondary level and another 21% at high school level, while the corresponding figures in the control group were 98% and 51%. About half of both groups continued to vocational training and a fifth to a higher level. Those without any vocational education made up 32% of the study group (95% confidence interval 21-43%) and 11% of the controls (95% confidence interval 7-15%). State benefits were the main source of income for 13% of the study group as compared to 2% among the controls. The employment status of the actual labour force showed no significant difference between the groups. Among patients with congenital heart disease there were significantly more of those who had developed a dependent life style, living with their parents without a marital or quasi-marital relationship. A cyanotic type of severe heart defect is one of the factors predisposing to poor success at school and a dependent lifestyle. Congenital heart disease, even when treated, is a significant factor which influences adult life. The results call for more practical support to assist psychosocial maturation, especially in patients with residual defects.

Adult↗
PubMed1986

Emotional and social effects of congenital heart disease in Singapore.

A cohort of patients with congenital heart disease (n = 29) who had undergone corrective cardiac surgery was studied and compared with normal controls. The mean age of cardiac patients was 10.3 years with a range of 6 years to 16 years. Their emotional maladjustment as measured on a behavioural checklist was significantly higher (P less than 0.01) than controls, and their social adjustment was also similarly less satisfactory (P less than 0.05). Regression analysis indicated that emotional maladjustment in the child was related to maternal maladjustment (P less than 0.01), maternal guilt (P less than 0.01) and maternal anxiety (P less than 0.05). Social maladjustment in the child was significantly related to maternal guilt (P less than 0.05) and pampering (P less than 0.02). It was considered that the child with heart disease was perceived and 'labelled' by the mother as abnormal even after surgery and was therefore the object and focus of maternal guilt and anxiety. Hence, the child's emotional and social maladjustment is probably the result of maternal factors rather than the physical handicaps of the illness.

Adolescent↗
PubMed2004

[Risks and benefits of cardiac surgery in Down's syndrome with congenital heart disease].

UNLABELLED: Down's syndrome is the most frequent chromosomic anomaly. Fifty percent are associated with a congenital heart disease. Life expectancy and quality of life are increasing since 15 years with improving surgical techniques. PATIENTS AND METHODS: This study presents 73 patients with Down's syndrome who underwent a surgical repair of congenital heart disease between 1992 and 2002. Among them, 37 (50.6%) had a complete atrio-ventricular septal defect (AVSD) and 36 an other anomaly. RESULTS: Five children died in the post operative period: all had an AVSD. Two late deaths occurred due to extra cardiac causes during the follow up. Two children who underwent a repair of AVSD had to be reoperated. The majority of the children are doing well, without any cardiac symptom. Thirty-eight percent of patients with AVSD repair have a middle mitral or tricuspid regurgitation and three had a massive mitral or tricuspid regurgitation. One child presents neurological sequelae related to surgery. CONCLUSION: Congenital heart diseases in Down's syndrome can be repaired with a limited risk of death. Final results are good for many children with significant improvement of the quality of life and life expectancy.

Cardiac Surgical Procedures↗
PubMed1994

Psychosocial functioning of young adults after surgical correction for congenital heart disease in childhood: a follow-up study.

To investigate the long-term psychosocial outcome of congenital heart disease, the emotional, intellectual and social functioning of 288 (young) adult patients was assessed with standardized assessment procedures 9-23 years (mean follow-up interval: 16 years) after surgical correction for congenital heart disease in childhood, and compared with that of reference groups. With respect to emotional functioning, the patients reported significantly fewer feelings of hostility, fewer neurotic complaints and a better self-esteem than reference subjects. Overall, the results concerning social functioning showed favourable outcomes on daily activities (school, employment) and leisure-time activities for (young) adults with congenital heart disease. No significant differences were found between scores of different cardiac diagnostic groups on hostility, neuroticism, self-esteem and leisure-time activities. The possibility whether the 'denial'-mechanism might have contributed to the positive outcomes is discussed.

Activities of Daily Living↗
PubMed2001

Consanguinity and congenital heart disease in Saudi Arabia.

First-cousin marriage may be a significant risk factor for specific types of congenital heart disease in a consanguineous population. Inbreeding studies suggest an autosomal recessive component in the cause of some congenital heart defects. We studied a large sample of patients with structural congenital heart defects (CHD) identified through the Congenital Heart Disease Registry at King Faisal Specialist Hospital in Riyadh, Saudi Arabia. After exclusions of chromosome abnormalities and non-participation, data were collected on 891 consecutive patients who were registered between January and August, 1998. Data on first-cousin consanguinity and type of CHD diagnosis were collected. A z test of proportions was used to determine the association between consanguinity and subtypes of CHD. Data indicate that the proportion of first cousins in the CHD sample is higher than the proportion in the general population, supporting a hypothesis of autosomal recessive gene involvement in congenital heart disease. When subgroups of CHD were analyzed, first-cousin consanguinity was significantly associated with ventricular septal defect (VSD), atrial septal defect (ASD), atrioventricular septal defect (AVSD), pulmonary stenosis (PS), and pulmonary atresia (PA). There was no relationship between consanguinity and tetralogy of Fallot (TOF), tricuspid atresia (TA), aortic stenosis (AS), co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA). Thus, in a population with a high degree of inbreeding, consanguinity may exacerbate underlying genetic risk factors, particularly in the offspring of first cousins. There may be a recessive component in the causation of some cardiac defects.

Adolescent↗
PubMed1999

Interventional procedures for congenital heart disease.

Interventional techniques available for use in treating congenital heart disease include balloon dilation of valves and vessels, stent placement and coil embolization of collaterals, patent ducts and other arterial fistulae. In addition, a variety of devices for closure of atrial and ventricular septal defects and patent ducts currently are under investigation. Radiofrequency ablation of arrhythmias also is applicable to the pediatric population.

Angioplasty, Balloon, Coronary↗
PubMed1992

Transesophageal echocardiography in infants and children with congenital heart disease.

TEE applications for children and infants with congenital heart disease are now clinically established and TEE is being used in most major pediatric cardiovascular centers. This technology has evolved to the point that it is capable of providing diagnostic quality imaging, even for small infants. With expanding technology and increasing experience, new and more sophisticated applications will be found for pediatric TEE.

Child↗
PubMed2000

Exercise prescription in adults with congenital heart disease: a long way to go.

OBJECTIVE: To determine if appropriate advice had been given to adults with congenital heart disease regarding safe and effective exercise, and to assess pre-existing misconceptions of the potential benefits and dangers of exercise. DESIGN: An anonymous self assessment questionnaire. SETTING: A tertiary referral clinic. PATIENTS: 99 adults (57 men, 42 women) with congenital heart disease, mean age 25.6 years. MAIN OUTCOME MEASURES: The extent and nature of exercise advice given over previous years; a measure of current activity level compared with the American Heart Association recommendations; and an assessment of exercise limiting symptoms and a description of barriers to further exercise. RESULTS: 44% of the cohort assumed all exercise was safe despite their cardiac disease. A health care professional had only raised the issue of specific exercise advice in 28 cases. Of those given instruction it was more common to receive prohibitive advice (30%) than to be encouraged to take more exercise (19%). Despite this 61% were involved in some form of at least light exercise. The most prevalent barriers to exercise were current symptoms (32.3%), lack of interest in exercise (24.2%), and health fears (16.1%). CONCLUSIONS: The education of adults with congenital heart disease regarding exercise and its potential benefits and limitations is suboptimal even in a specialist clinic.

Adolescent↗
PubMed2006

Serum vascular endothelial growth factor in cyanotic congenital heart disease functionally contributes to endothelial cell kinetics in vitro.

BACKGROUND: Remarkable amounts of neovascularization develop in patients with cyanotic congenital heart disease who have low pulmonary blood flow and systemic cyanosis, but the factors functionally responsible for angiogenesis in cyanotic congenital heart disease have not been determined. METHODS AND RESULTS: To investigate the functional angiogenic molecules in serum from these patients, serum angiogenic activity was studied in 21 patients (systemic oxygen saturation: 82+/-1.9%) and in 17 healthy controls. Patient serum was more active in stimulating the tube formation of human umbilical vein endothelial cells (HUVECs) into capillary-like structures than control serum (150% vs 104% of internal control; p<0.001). This increased serum angiogenic activity normalized after total cardiac repair (p<0.001). The migration activity of HUVECs was also accelerated in patient serum (p=0.007). To identify the molecules in patient serum affecting tube formation of HUVECs, we examined the effects of an inhibitor or a neutralizing antibody against various angiogenic molecules on in vitro angiogenesis. Both the soluble vascular endothelial growth factor (VEGF) receptor 1 and the VEGF receptor 2 tyrosine kinase inhibitor SU5416 reduced the basal serum angiogenic activity of patients and this was reversed by a supplement of recombinant human VEGF. CONCLUSION: Our results indicate that serum VEGF functionally contributes to vascular endothelial cell kinetics in patients with cyanotic congenital heart disease.

Adolescent↗
PubMed1993

Employment and insurance for young adults with congenital heart disease.

OBJECTIVE: To determine the life and health insurability and employability of young adults with congenital heart disease. DESIGN: Questionnaire study. SETTING: Cardiac department of a tertiary referral hospital for children. PATIENTS: Young adults 18-30 years old with a variety of congenital heart defects, both simple and complex, including postoperative patients. MAIN OUTCOME MEASURES: Availability of insurance at normal or high rates, with or without special conditions or exclusions. Prospects for employment. RESULTS: Questionnaires were sent to eight large life insurance companies, five health insurance companies and, 15 employers, and 26 replies were received (93%). The consensus for life insurability was that young adults with mitral valve prolapse without regurgitation, postoperative ductus arteriosus, and aortic coarctation were insurable at standard rates. Those with any of the other heart defects listed were either insurable at high rates, or in the case of many lesions, not insurable at all. The consensus for health insurance was that insurance was available, but with complete exclusion of benefit for the cardiac disorder. Employment prospects were good for those with simple defects, but poorer for those with complex lesions. CONCLUSIONS: Prospects for insurance and employment for young adults with complex congenital heart lesions are poor. Inconsistencies found in insurance and job policies may be due to lack of appropriate guidelines for the outcome of young adults with corrected and uncorrected congenital heart disease.

Adolescent↗
PubMed2004

Parental decision-making in congenital heart disease.

OBJECTIVE: To explore whether prenatal diagnosis of congenital heart disease is associated with lower levels of parental distress and greater satisfaction with decisions about cardiothoracic surgery when compared to postnatal diagnosis. METHODOLOGY: A combined quantitative-qualitative design was used. Participants included the parents of 31 neonates (30 mothers and 22 fathers) admitted to the cardiac intensive care unit between 1 November 2001 and 1 May 2002 for repair of congenital cardiac malformations. Participants completed self-report measures of anxiety, optimism, and life events pre-operatively, and semi-structured qualitative interviews assessing satisfaction with decision-making within 1 week of the operation. RESULTS: At the time of surgery, mothers of neonates receiving the diagnosis prenatally did not differ from mothers of neonates receiving the diagnosis postnatally on measures of anxiety, optimism, and life events. Fathers of neonates receiving the diagnosis prenatally, however, reported more optimism, lower state and trait anxiety, and fewer negative life events than fathers of neonates receiving the diagnosis postnatally. When we analyzed the interviews, we found that, regardless of the timing of the diagnosis, parents felt as though they made a genuine choice for their baby to have surgery. CONCLUSIONS: In this pilot study, fathers who learned prenatally that their child had a congenital cardiac malformation were less distressed than those who discovered this fact only postnatally. From the parental perspective, nonetheless, distress and urgency do not impair their ability to make decisions about neonatal cardiac surgery.

Adult↗
PubMed1982

Hypertrophic osteoarthropathy in cyanotic congenital heart disease: its prevalence and relationship to bypass of the lung.

The frequency of hypertrophic osteoarthropathy in cyanotic congenital heart disease has previously been considered to be very low. Only a few isolated reports have described such an association. We studied 32 consecutive patients older than 6 years with various types of cyanotic congenital heart disease and examined each case for hypertrophic osteoarthropathy. We also assessed the role of the altered cardiopulmonary hemodynamics in the development of hypertrophic osteoarthropathy by means of cardiac catheterization. Our results showed that 31% of the patients had hypertrophic osteoarthropathy. When the hemodynamic parameters of the group of patients with hypertrophic osteoarthropathy were compared with those of the remaining patients, we found significant differences in the systemic blood flow (P less than or equal to 0.05), right-to-left shunt (P less than or equal to 0.05), and arterial oxygen unsaturation (P less than or equal to 0.005). Thus, we found a much higher prevalence of hypertrophic osteoarthropathy in patients with cyanotic congenital heart disease than has previously been recognized. The development of hypertrophic osteoarthropathy is related to the degree of bypass of the lung. This is consistent with the concept that hypertrophic osteoarthropathy results from mediators in the systemic venous circulation that escape inactivation in the pulmonary capillary bed.

Adolescent↗
PubMed2006

Cardiopulmonary bypass for adults with congenital heart disease: pitfalls for perfusionists.

The fixed incidence of congenital heart defects and improved survival have resulted in increasing numbers of adults with congenital heart disease (CHD) who have undergone complex repairs and/or palliations. Eventually, there will be more adults with CHD than children. They will require cardiac surgical interventions associated with progression of their CHD or for age-related disease, such as coronary revascularization. During bypass, anatomical shunts may exist within or without the heart. Left-to-right shunts can result in dramatically lower systemic blood flow than pump flow due to 'steal', while pulmonary edema ensues due to excessive pulmonary flow. Right-to-left shunts carry risks of massive air embolism and double or triple venous cannulation may be necessary. Cannulation of composite reconstructed aortas may be difficult, risking dissection or aortic obstruction, and double arterial cannulation may be indicated. Aberrant coronary arterial and venous anatomy may .preclude adequate myocardial preservation with common techniques and can be complicated by aortic insufficiency. Valves and conduits may exhibit failure. Conventional monitoring, such as central venous oximetry, may be misleading. Monitoring, such as serial lactate measurement, near-infrared spectroscopy and transcranial Doppler blood velocity, offer advantages for such patients. The perfusionist needs to be aware of such conditions as much congenital aberrancy may present unexpectedly during cardiac surgery.

Adult↗
PubMedDate not supplied

The recurrence risk in congenital heart disease.

We have studied 1148 'isolated" cases of congenital heart disease, taken from a general series of 1255 cases. The recurrence risk ranged from 0.9% for relatives of male cases of VSD and for female cases of aortic stenosis to 10.5% for relatives of female cases of atrioventricularis communis; mean value 2.5%. Heritability ranged from 50% to 95%, mean 65%. Full concordance was more frequent in 1st-degree relatives of Fallot's tetralogy patients, while discordance was common in more distant relatives. We propose a hypothesis of several overlapping polygenic systems for the causation of CHD.

Alleles↗
PubMedDate not supplied

Embolization of collateral vessels using mechanically detachable coils in young children with congenital heart disease.

Our objective was to evaluate the usefulness of embolizing collateral vessels using mechanically detachable coils (MDCs) in children aged 3 years or younger with congenital heart disease. The subjects were 8 children with congenital heart disease featuring collateral vessels (age 18 days-3 years): 3 with a single ventricle, 2 with the tetralogy of Fallot, 2 with pulmonary atresia, and 1 with a ventricular septal defect. The embolized vessels were the major aortopulmonary collateral artery (MAPCA) in 5 patients, the persistent left superior vena cava in 2, and the coronary arteriovenous fistula in 1. A 4 or a 5 F catheter was used as the guiding device, and embolization was performed using MDCs and other conventional coils introduced through the microcatheter. One patient had growth of new MAPCAs after embolization, and these MAPCAs were also embolized with MDCs. Thus, a total of 9 embolization procedures were performed in 8 patients. Complete occlusion of the collateral vessels was achieved in 8 of 9 procedures (89%). Seven of 8 patients (88%) had uneventful courses after embolization, and MDC procedures appeared to play important roles in avoiding coil migration and achievement of safe coil embolization. One patient who underwent MAPCA embolization showed no improvement in heart function and died 2 months and 19 days later. Embolization of collateral vessels using MDCs in young children with congenital heart disease can be an effective procedure and a valuable adjunct to surgical management.

Child, Preschool↗
PubMed2003

Pulmonary agenesis with congenital heart disease.

The combined anomalies of pulmonary agenesis and congenital heart disease are exceedingly rare and a highly lethal association. We describe four cardiovascular operations in three patients with this association. Open-heart surgery was performed in two patients, and palliative heart surgery was performed in two patients. One patient died 100 days after the operation because of severe tracheal stenosis and renal failure, but the other two patients are doing well. Surgical access through a thoracotomy, instead of a median approach, was adequate in all patients. Careful evaluation and a precise understanding of the anatomy are necessary for the surgical management of these patients.

Abnormalities, Multiple↗
PubMed1997

A post-receptor defect of adenylyl cyclase in severely failing myocardium from children with congenital heart disease.

The aim of this study was to determine whether a defect at the post-receptor level of adenylyl cyclase may also contribute to the decreased effectiveness of cAMP-increasing agents in severely failing patients with congenital heart disease. The severity of congestive heart failure in 31 patients with congenital heart disease was graded by a scoring system which included a description of historical and clinical variables. Patients were divided into a group with no or mild heart failure (score < or = 6) and a group with severe heart failure (score > 6). beta-Adrenoceptor-stimulated adenylyl cyclase activity was significantly decreased by 65% in patients with severe heart failure in comparison to the group of patients with no or mild heart failure. In addition, receptor-independent adenylyl cyclase stimulation by forskolin was reduced by 52% in patients with score > 6 compared to patients with score < or = 6. This post-receptor defect of adenylyl cyclase was apparently due to a decrease in the activity of catalytic subunit of adenylyl cyclase as adenylyl cyclase stimulation by forskolin in the presence of Mn2+ which uncouples catalytic subunit from the G proteins, G(s) and G(i), was also significantly diminished in the patients with severe heart failure. In contrast, the level of inhibitory G protein alpha-subunits was apparently not different in the two groups. In summary, the data indicate that a defect at the catalytic subunit of adenylyl cyclase apparently contributes to the decreased effectiveness of cAMP-increasing agents in severely failing patients with congenital heart disease.

Adenosine Diphosphate↗
PubMed1977

[Prostaglandin E1: effect on blood gases in infants with cyanotic congenital heart disease (author's transl)].

15 infants with cyanotic congenital heart disease (pulmonary atresia, Ebstein-Syndrom) were treated with Prostaglandin E1. During treatment there was a significant rise of PO2 and SO2 in the arterial, capillary and central venous blood. There was a minor rise of pH and HCO3; the arterial pCO2 fell slightly. We used PGE1-therapy as pretreatment before the heart operation, so that the critical babies go to the operation well oxygenated and in a better state. The side effects we observed (tachypnoe, tachycardia, hyperpyrexia, augmented urine output, erythemas, apnoic spells) seemed less important than the great advantage of a better oxygenation of these hypoxic babies.

Bicarbonates↗