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PubMed1978

Hyperuricemia in congenital heart disease.

Gout is rarely noted as a clinical problem in secondary polycythemia-- even if profound polycythemia exists, as in cyanotic congenital heart disease. A retrospective study of 81 patients with congenital heart disease was done to assess the incidence of hyperuricemia. Twenty of 46 patients with cyanotic congenital heart disease had serum levels of uric acid greater than 8 mg/dl. Thirteen of 16 (81%) cyanotic male patients more than 15 years old had serum levels greater than 8 mg/dl. For cyanotic patients, serum levels of uric acid were related directly to the degree of polycythemia (r = .44; P less than .02). Impaired renal function or drug therapy did not seem to account for the hyperuricemia. Because levels of uric acid greater than 10 mg/dl probably are nephropathic, many of these patients may be incurring subclinical uric acid nephropathy.

Adolescent↗
PubMedDate not supplied

[Inflammatory response mediators in newborns and infants with congenital heart diseases during surgical treatment under extracorporeal circulation].

Surgical treatment of complex congenital heart disease under extracorporeal circulation is accompanied by a systemic inflammatory reaction occurring in neonatal infants and babies. There were drastic increases in the concentration of tumor necrosis factor, interleukin-8, neutrophilic elastase and a predominance of a proinflammatory response over an inflammatory one at the warming stages and after administration of protamine sulfate. The rate of an inflammatory response depended on the duration of extracorporeal circulation. Modified ultrafiltration could remove cytokines; however, their concentration in the body remains high. A relationship was found between the course of a postoperative period and the rate of a developing reaction to extracorporeal circulation in the surgical treatment of congenital heart diseases.

Cardiac Surgical Procedures↗
PubMed1978

Clinical applications of real-time two-dimensional scanning in congenital heart disease.

Real-time two-dimensional visualization of cardiac anatomy has been a major advance in the noninvasive evaluation of congenital heart disease. Performed from precordial or suprasternal notch sites, the techniques allow noninvasive evaluation of the cardiac chambers, atrioventricular valves, and great arteries for the diagnosis of common and complex forms of congenital heart disease. Two-dimensional real-time echocardiography, therefore, can be expected to become an integral part of the noninvasive diagnosis and serial followup of children and infants with congenital heart disease.

Aortic Valve Stenosis↗
PubMed2005

[Surgical management of preterm infants and low birth weight neonates with congenital heart disease].

UNLABELLED: Nowadays, due to the development of cardiac surgery, pediatric cardiology and anesthesia, almost every congenital heart disease can be corrected totally or partially. The increasing number of surgical corrections will lead to better life quality. The surgical mortality has decreased significantly, even in the most complex cases. Only few cases can not be treated surgically. The aim of this study was to examine these positive changes, what they mean in the treatment of premature babies with congenital heart disease. These patients are endangered because of their age as well, they require special treatment and the surgical treatment has always meant high risk, urgent interventions. PATIENTS: between 01. 01. 1975. and 31. 12. 2003. 447 premature babies were operated on. The patients were divided into 3 subgroups by their weight: I: 470-1500 gr. - 19 patients, II: 1500-2000 gr. - 93 pts., IIl: > 2000 gr. - 335 pts. There were 69 corrective surgical procedures performed mainly after 1998. RESULTS: Overall early mortality: 81 patients (18.1%). Detailed mortality: group I.: 0%, group 11: 23 (24.7%), group III.: 58 (17.3%). The early mortality reduced from 21.6% to 6.3% (closed procedure), and 27.7% to 10.8% (ECC operation). The successful operations in the smallest weight groups were as follows: On ECC: Total Anomalous Pulmonary Venous Return (1600 g), Transposition of the Great Arteries--Arterial Switch (1800 g), Ventricular Septal Defect (1800 g), Aortopulmonary fenestration (2000 g), Hypoplastic Left Heart Syndrome-Norwood procedure (2200), AV septal defect (2300 g), Interrupted aortic arch + Ventricular Septal Defect (2300 g), Truncus arteriosus (2500 g). Without ECC: Coarctation of the aorta (930 g), Patent Ductus Arteriosus (470 g). CONCLUSION: Nowadays the possibilities and the chances of the corrective procedures of congenital heart diseases in those patients with bodyweight of over 2000 g and in those that are mature babies are the same. In those patients with bodyweight 1500-2000 g procedures without ECC had good results, on-pump procedures had higher mortality rate, but the long-term results were acceptable. At the moment only procedures without ECC are performed on babies with weight under 1500 g--but with higher risk. Our effort is to perform ECC operations in patients with bodyweight less than 1500 g.

Cardiac Surgical Procedures↗
PubMed1994

Orthotopic heart transplantation in children with congenital heart disease.

The early experience (February 1982 to June 1988) with transplantation for the treatment of congenital heart disease at the University of Pittsburgh was disappointing due to an excessively high perioperative mortality. From July 1988 to June 1992, a further 21 children with congenital heart disease underwent orthotopic transplantation. Thirteen had undergone multiple prior palliative procedures (mean, 2.8 per patient). In 12 of these patients, prior procedures involved the pulmonary arteries on one or more occasions. In contrast to our earlier experience, there were no deaths stemming from inadequate surgical reconstruction or pulmonary hypertension. The actuarial survival was 71% at both 1 and 3 years. This did not differ significantly from the survival among 18 patients who underwent transplantation for the management of cardiomyopathy over the same period (1-year and 3-year survival, 83%). The perioperative mortality and short-term survival are now similar for children undergoing transplantation for the treatment of either congenital heart disease or cardiomyopathy. These improved results probably reflect more careful patient selection and an increasing surgical experience with complex reconstructive procedures.

Adolescent↗
PubMed2001

Mortality from congenital abnormality in Malaysia 1991-1997: the effect of economic development on death due to congenital heart disease.

An analysis was done of available data from the Department of Statistics Malaysia, on the type of congenital abnormality contributing to death, to determine whether progress in health care over recent years was associated with any decline in mortality from congenital abnormality. A significant decline in death due to congenital abnormality was observed between 1991 and 1996. This was attributable to a decline in deaths due to congenital heart disease occurring because of improvements in cardiac surgical services for infants. In 1997 death due to congenital heart disease increased significantly. This could be attributed to improvements in the diagnosis of congenital heart disease in the neonate.

Congenital Abnormalities↗
PubMed1998

Psychopathology in young adults with congenital heart disease. Follow-up results.

AIMS: The aim of the present study was to assess the occurrence of a wide range of behavioural and emotional problems long-term (> 9 years) after surgical correction for congenital heart disease in infancy and childhood. METHODS: The problem scores on the Young Adult Self-Report of 166 19-25-year-old adults with congenital heart disease were compared with those of subjects of similar age from the general population. RESULTS: On most Young Adult Self-Report scales no differences were found between the mean problem scores of the congenital heart disease-adults and reference peers. On only two Young Adult Self-Report scales (i.e. Somatic Complaints and Strange) and the total problem score were significant though small differences found between the mean problem scores of the congenital heart disease adults and reference peers. No significant relationship was found between cardiac diagnosis and problem behaviours in congenital heart disease adults. No relationship was found between IQ scores and problem behaviours in congenital heart disease adults. CONCLUSION: Overall, it can be concluded that the results of the congenital heart disease adults were not unfavourable, since the differences in mean (total) problem scores between the congenital heart disease sample and reference group were small and limited to only two specific problem areas.

Adult↗
PubMed1988

Congenital heart disease: incidence in the first year of life. The Alberta Heritage Pediatric Cardiology Program.

The Heritage Pediatric Cardiology Program is a regional study of patients with congenital heart disease in northern and central Alberta, Canada. Cases of congenital heart disease were confirmed by clinical examination by a pediatric cardiologist plus echocardiography (noninvasive criteria) with or without cardiac catheterization, cardiac surgery, or autopsy (invasive criteria). From 1981 to 1984 inclusive, 573 confirmed cases of congenital heart disease occurred in a population of 103,411 livebirths (5.54 and 3.36/1,000 livebirths using the noninvasive and the invasive criteria, respectively). This is significantly higher (p less than 0.0001) than the rates found in the Baltimore-Washington Infant Study (3.69 and 2.38/1,000 livebirths for the noninvasive and invasive methods, respectively) or the New England Regional Infant Cardiac Program (2.20/1,000 livebirths using invasive methods). Although some lesions occur at rates similar to those found in these other registries, there are 4.4 times more double outlet right ventricles and 2.9 times more atrial septal defects in northern and central Alberta than were found in the New England Regional Infant Cardiac Program (p less than 0.001). The overall congenital heart disease rate has increased 47% between 1981 and 1984, and the rate of ventricular septal defects has doubled during the same period in northern and central Alberta. No methodological bias was found to explain these higher and increasing rates.

Alberta↗
PubMed1998

[Changes in serum levels of total amino acids in young children with congenital heart diseases].

OBJECTIVE: To study the implication of early surgical operation on the improvement of protein malnutrition in young children with congenital heart diseases. METHODS: Sixteen kinds of serum free amino acids were determined with an automatic analyzer for 72 cases with congenital heart diseases before and after surgical operation and 51 healthy children. RESULTS: Serum total amount of essential amino acids reduced in children with congenital heart diseases, mainly of branch-chained amino acids, especially significant in young ones. Serum amino acids restored to normal level one month after surgical operation. CONCLUSION: There existed protein malnutrition in children with congenital heart diseases, especially in young ones. Surgical operation for them in a suitable time will improve their protein nutritional status.

Adolescent↗
PubMed2006

Sustained effects of cardiac rehabilitation in children with serious congenital heart disease.

OBJECTIVE: Past studies have documented the acute benefits of cardiac rehabilitation in children with congenital heart disease. It is not known whether these benefits persist. PATIENTS AND METHODS: Fifteen patients, ages 8 to 17 years, with complex congenital heart disease, whose exercise function immediately after a 12-week cardiac rehabilitation program was superior to that present on a precardiac rehabilitation exercise test, were restudied 6.9 +/- 1.6 months after completion of the cardiac rehabilitation program (approximately 1 year after the precardiac rehabilitation study). Changes in exercise function relative to baseline, precardiac rehabilitation exercise tests were also compared with changes observed in a group of 18 control subjects, with similar diagnoses, who also had 2 exercise tests separated by a year but did not undergo cardiac rehabilitation. RESULTS: The cardiac rehabilitation patients' exercise function did not change significantly over the 6.9-month period after the completion of the cardiac rehabilitation program; percentage of predicted peak oxygen consumption and peak work rate remained significantly superior to baseline, precardiac rehabilitation values. These changes were also associated with improvements in self-esteem, behavior, and emotional state. In contrast, among the control subjects, small, but statistically insignificant declines in peak oxygen consumption and peak work rate were observed on the final exercise test compared with values obtained at baseline, 1 year earlier. The improvements realized by the cardiac rehabilitation patients differed significantly from the concurrent changes observed among the control subjects and appeared to be a result of an increase in the oxygen pulse at peak exercise; significant changes in peak heart rate were not observed. CONCLUSIONS: In patients with congenital heart disease, cardiac rehabilitation produces significant, sustained improvements in exercise function, behavior, self-esteem, and emotional state.

Adolescent↗
PubMedDate not supplied

Oxygen saturation as a screening test for critical congenital heart disease: a preliminary study.

The aim of this study was to assess the utility of arm and leg oxygen saturation as a candidate screening test for the early detection of ductal-dependent left heart obstructive disease. We measured arm and leg oxygen saturation in 2876 newborns admitted to well baby nurseries and 32 newborns with congenital heart disease. Fifty-seven newborns in the well baby nurseries (0.02%) had an abnormal test (leg saturation less than 92% in room air or 7% lower saturation in the leg than in the arm). Four of the 57 had critical congenital heart disease, including 1 with coarctation of the aorta. Of the 32 newborns with congenital heart disease, 11/13 (85%) with left heart obstructive disease had abnormal oxygen saturation tests, as did 15/19 (79%) with other forms of congenital heart disease. Pulse oximetry deserves further study as a screening test for critical congenital heart disease.

Baltimore↗
PubMed2004

Styles of coping and social support in a cohort of adults with congenital heart disease.

OBJECTIVE: To determine styles of coping, that is personal ways of dealing with problems, and social support, or support from the social environment, in a cohort of adults with congenital heart disease. METHODS: We subjected 362 patients with congenital heart disease, aged from 20 to 46 years, belonging to five diagnostic groups, to extensive medical and psychological examination from 20 to 33 years after their first open heart surgical procedure. During psychological examination, 342 patients filled in questionnaires concerning styles of coping, specifically the Utrecht Coping List, and social support, using the Social Support List. RESULTS: Overall, styles of coping in the total sample are comparable to those of peers in the general population, except for lower active problem solving, which can be attributed to female patients. Males with congenital heart disease showed more favourable styles of coping compared to their peers, such as higher seeking of social support, lower passive reaction patterns, and lower expression of negative emotions. Compared to the reference group, the total cohort of patients reported to receive less social support, but also to experience less discrepancies between desired and received social support, indicating feelings of independence in these adults. Females with congenital heart disease were found to seek and receive more social support compared to their male counterparts. CONCLUSION: Overall, few differences in styles of coping were found between the patients and their reference groups. Perceived social support in the sample of patients was favourable.

Adaptation, Psychological↗
PubMed1975

Monozygotic twins with concordance for Down's syndrome and congenital heart disease.

Cytogenetic, clinical, and other findings are presented on a pair of same-sex, monochorial MZ twins concordant for both trisomy 21 and congenital heart disease. The literature on Down's syndrome in twins and on congenital heart disease in twins is reviewed and the exceptional occurrence of concordant congenital heart disease in MZ twins is stressed.

Abnormalities, Multiple↗
PubMed2001

Covert fears and anxiety in asthma and congenital heart disease.

AIM: To compare anxiety, fears and behavioural problems in children with asthma and children with congenital heart disease, and with the normative population. To also review the influence of maternal anxiety, time since diagnosis and severity of disease. DESIGN: Children administered Fear Survey Scale (FSSC-R) and Child Manifest Anxiety Scale (R-CMAS). Mothers given Child Behaviour Checklist (CBCL) and State Trait Anxiety Scale (STAI-S and STAI-T). Normative means and SDs compared with means and SDs for both medical groups. The mother's scores on the STAI-S and STAI-T scales were correlated with the child's scores on the FSSC-R and the R-CMAS. SETTING: Outpatient Asthma and Cardiology multidisciplinary Clinics at a tertiary care paediatric facility, Alberta Children's Hospital. SUBJECTS: Forty children with asthma (aged 6--17 years) were compared with 39 children with congenital heart disease. Intake questionnaires and interviews determined these children to be without obvious psycho-social problems. RESULTS: Children with asthma and children with congenital heart disease had more medical fears, and more physiological anxiety than normative samples. Increased maternal anxiety was correlated in both groups with increased child anxiety, medical fears and behavioural problems in the child. Similarly, increased severity of asthma or cardiac problems was associated with more physiological anxiety and more fears. Less time since diagnosis of the disease adversely affected social interactions in both groups of children. CONCLUSION: Physiological anxiety, medical fears and maternal anxiety are important issues requiring attention in asthma and cardiac disease, even in the absence of obvious psychosocial problems. There may be specific problems with a recent diagnosis of a chronic illness.

Adult↗
PubMed2003

Congenital heart disease in infants of diabetic mothers: echocardiographic study.

Our objective was to study neonates born to insulin-dependent diabetes mellitus (IDDM) mothers to detect the spectrum of congenital heart disease (CHD). Between July 2000 and June 2001, a prospective study of 100 consecutive infants of diabetic mothers (IDMs) at King Khalid University Hospital in Riyadh was undertaken. Family and maternal history, physical examination with special attention to the cardiovascular system, and echocardiography were performed. A total of 100 consecutive IDMs examined. The most common echocardiographic findings were patent ductus arteriosus (PDA; 70%), patent foramen ovale (68%), atrial septal defect (5%), small muscular ventricular septal defect (4%), mitral valve prolapse (2%), and pulmonary stenosis (1%). Hypertrophic cardiomyopathy (HCMP) was observed in 38% of cases, mainly hypertrophy of the interventricular septum. Severe forms of CHD encountered were D-transposition of great arteries, tetralogy of Fallot, and hypoplastic left heart syndrome (1% each). Isolated aortic stenosis and coarctation of aorta were not encountered in this series. Overall incidence of congenital heart disease was 15% after excluding PDA and HCMP. Maternal IDDM is a significant risk factor for CHD. Careful evaluation and early diagnosis of CHD in this high-risk group are highly indicated. There is a need for development of prenatal screening programs for CHD in our population.

Abnormalities, Multiple↗
PubMed1969

Diagnosis of congenital heart disease in the first two weeks of life.

The differential diagnosis of congenital heart disease which presents cyanosis or respiratory distress, or both, in the first two weeks of life, is difficult. Close correlation of clinical features, electrocardiogram and chest roentgenogram is most helpful. The diagnosis of congenital heart disease should lead to immediate cardiac catheterization, angiocardiography and appropriate therapy.

Cyanosis↗