Occurrence Of Congenital Heart Disease Research Articles

A Clinicoepidemiologic Study of Heart Disease in Schoolchildren of Menoufia, Egypt

This study was conducted to determine the prevalence of rheumatic heart disease (RHD) and congenital heart disease (CHD) in primary schoolchildren of Menoufia, Egypt and to study the relationship between these two problems and socioeconomic conditions. A total of 8000 children were screened for cardiac disease in their schools. Children with confirmed RHD and CHD in addition to 200 healthy children (controls) were visited at their homes to assess their social environment and other factors according to a designed questionnaire. We found prevalence rates of 3.4/1000 and 2.6/1000 for the RHD and CHD respectively. The most common cardiac defects were double mitral and pulmonary stenosis in the RHD and CHD groups respectively. Nineteen (39.6%) of the patients were diagnosed for the first time during the study. Penicillin prophylaxis was received by only four (14.8%) of the RHD children and none of the CHD children. The number of other affected siblings, increased multiparity, repeated abortion, and intake of contraceptives by mothers were significantly higher in families of the children with CHD. The proportion of illiterate, unskilled, and heavy smoking fathers was higher in the two patient groups. Family income was lowest in the RHD group, while increased crowding index and low whole social environment were significantly related to both RHD and CHD. Effective programs at the community and health service levels are needed in Menoufia to solve the problem of heart disease in schoolchildren in the immediate future.

Epidemiology of congenital heart disease in the United States

Data from the National Center for Health Statistics were examined to aid in assessing the burden of congenital heart disease in the United States. In 1979 through 1988 combined, 46,450 deaths were attributed to CHD, 26,319 occurring in the first year of life. Another 14,283 deaths were attributed to other congenital anomalies of the circulatory system. In 1988 through 1990 combined, there were >300,000 hospital discharges with any diagnosis of CHD, with 2.7 million days of care provided. For infants aged <1 year, the rate was 13.7 per 1000 live births per year. Another 242,049 discharges had any diagnosis of other congenital circulatory anomalies. In 1983 through 1985, >700,000 persons reported a history of CHD. In 1989, heart malformations were reported on the birth certificate of 4621 live births in 45 states and the District of Columbia. In 1988 through 1990 there were an estimated 37,445 repairs of atrial and ventricular septa. In addition, 45,635 cardiac catheterizations were performed in patients aged <5. Average annual percentage declines in infant mortality rates for the period 1979 through 1988 were consistent with an effect of improvements in surgical technique and intensive care. The average annual infant mortality rate for hypoplastic left heart syndrome increased 1.3%, and rates for five causes amenable to treatment such as transposition of great vessels declined. Infant mortality rates in nonmetropolitan areas were higher than in metropolitan areas, suggesting that limited access to care might be associated with mortality. Congenital heart disease is a significant cause of mortality and morbidity and health care costs in children and adults in the United States. Continued efforts are needed to develop and apply strategies to prevent the occurrence of congenital heart disease.

Risk of congenital heart defects in relatives of patients with atrioventricular canal.

To investigate the overall occurrence of congenital heart disease in 103 pedigrees with a proband affected with atrioventricular canal. Family study of patient series. Department of Pediatric Cardiology, Bambino Gesu' Hospital, Rome, Italy. One hundred three consecutive patients with atrioventricular canal and normal karyotype and their first- and second-degree relatives. None. In 12 pedigrees (11.7%), one or more relatives had concordant or discordant congenital heart disease. Congenital heart disease occurred in four (1.9%) of the 206 parents of probands, in four (3.6%) of the 111 siblings, and in five (0.8%) of the 644 uncles or aunts. None of the grandparents had congenital heart disease. Atrioventricular canal affected several family members in five pedigrees. Familial aggregation of atrioventricular canal in several pedigrees requires careful collection of family histories, accurate cardiologic evaluation of the first- and second-degree relatives of the probands, and fetal echocardiography in all pregnant women in at-risk families.

Teratology, Genetics, and Recurrence Risks

This brief review has described historic highlights of etiologic knowledge, current concepts in the categorization of cardiovascular anomalies based upon ongoing advances in teratology, and epidemiologic evaluations of biologic and xenobiotic risk factors with emphasis on the teratogenic roles of maternal diabetes, hyperphenylalaninemia, and parental exposures to alcohol, drugs, solvents, pesticides, lead, and other toxic substances. Evidence is presented for a strong genetic basis of cardiovascular maldevelopment requiring further studies to define at-risk families. Counseling and personal and societal preventive interventions may reduce the occurrence of some forms of CHD.

Congenital heart disease in the offspring and maternal habits and home exposures during pregnancy.

To test the effect of maternal habits and home exposures during early pregnancy on the occurrence of congenital heart disease in the offspring, 406 cases and 756 controls were studied. The cases included all cardiovascular malformations detected in Finland during 1982-1983, while the healthy controls were randomly selected from all babies born during the same period. Case and control mothers were interviewed after delivery using a structured and pre-tested questionnaire. Maternal overall drug consumption during the first trimester was as prevalent among case mothers (13.3%) as controls (14.6%). Neither was the risk of congenital heart disease associated with maternal use of contraceptive pills, salicylates, diazepam, or sweetening agents separately. Maternal exposures to disinfectants, dyes, lacquers, paints, pesticides, or glues at home were equally prevalent in case and control groups. Several earlier miscarriages was a predictor of an infant born with congenital heart disease (OR = 2.7, CI95 = 1.4-5.3). Maternal ultrasound examination was performed during the first 16 weeks of pregnancy more often among the case group (28.3%) than among the control group (22.0%). However, the association between ultrasound examination and the risk of congenital heart disease in the offspring was not statistically significant (OR = 1.2, 95% confidence interval 0.9-1.7) when adjusted for confounding factors such as the threat of miscarriage in logistic regression analysis. It is concluded that maternal ultrasound examination, intake of some common drugs, and exposure to a number of environmental factors at home during early pregnancy are probably not harmful for the developing fetal heart.

Effect of fathers' age and birth order on occurrence of congenital heart disease.

The aim was to examine if there is an effect of fathers' age and of birth order on the occurrence of congenital heart disease. This was a hospital based case-referent study including use of birth defects surveillance data. Subjects were 497 cases of congenital heart disease aged between 3 months and 5 years, born in Beijing and Hebei Province, China; 6222 children without congenital heart disease serve as reference baseline. With stratified analysis and logistic regression analyses, congenital heart disease was found to be associated with fathers' age less than 25 years (odds ratio 2.63), independent of mothers' age and of birth order. There was also evidence to show a higher birth order effect on the occurrence of congenital heart disease independent of parental ages. Higher birth order and fathers aged less than 25 years were both independently associated with some categories of congenital heart disease and with congenital heart disease overall.

Tetralogy of Fallot in triplet siblings

The occurrence of congenital heart disease in siblings of multiple births, particularly in twins, has been documented. 1–6 Because of the rarity of triplet gestations, few triplets have been included in even the largest population-based studies. Triplet infant boys were recently referred for evaluation of precordial murmurs. All 3 infants had tetralogy of Fallot, each with a different type and degree of pulmonary stenosis.

Cardiac valve replacement for stenotic bicuspid aortic valve in husband and for purely regurgitant prolapsed mitral valve in wife

Congenital heart disease is recognized to occur in families, i.e., in a parent or grandparent and in 1 or more siblings. The occurrence of congenital heart disease in both husband and wife must indeed be rare, and even more so is the occurrence of cardiac valve replacement in both husband and wife. Such was the case, however, in 2 recently studied patients. Each had the most common congenital heart disease that occurs in their sex, namely, mitral valve prolapse in women and bicuspid aortic valve in men. 1

Congenital heart disease in relation to maternal use of Bendectin and other drugs in early pregnancy.

To clarify the association of Bendectin and other drugs used in early pregnancy with the occurrence of congenital heart disease, we interviewed 298 mothers of children with congenital heart disease and 738 mothers of healthy controls. Differential recall of drug use by mothers of affected children and mothers of controls was evaluated by comparison of information collected by interview with that recorded in the prenatal record. Data derived from maternal interviews were generally consistent with the record data. Reported Bendectin use was minimally associated with congenital heart disease (prevalence odds ratio, 1.1; 90 per cent confidence interval, 0.8 to 1.5). The data from this study were consistent with previously reported associations of other drugs with congenital heart disease. In particular, aspirin use in early pregnancy was associated with about a twofold increase in the frequency of defects in septation of the truncus arteriosus (prevalence odds ratio, 2.1; 90 per cent confidence interval, 1.1 to 3.9).

Studies of the HLA complex in families of children with congenital heart disease.

In order to test the hypothesis that HLA-linked developmental determinants are involved in the pathogenesis of congenital heart disease, we performed tissue typing on 10 families in which two or more siblings had cardiac malformations. No association between the occurrence of congenital heart disease and the HLA complex was found.

Rubella in Mother and Congenital Cataracts in Child

A prospective study of virus diseases occurring in pregnant women from 1957 to 1964 and a concurrent survey of hospital records revealed a fourfold increase in the number of cases of congenital cataracts following the widespread epidemic of 1964, as compared to the incidence in previous years in which no epidemic of rubella occurred. This increase was related to a greater number of cases of rubella in pregnant women and to a higher rate of occurrence of cataracts among the offspring of those mothers affected within the first eight weeks of gestation. The increased risk that congenital cataracts would occur suggests that there may have been an alteration in the capacity of the rubella virus in 1964 to injure human fetal lens tissue. Preliminary findings on the occurrence of congenital heart disease suggest that the incidence of other major defects in offspring of women who contracted rubella during pregnancy in a year in which a severe epidemic occurred may also rise.

GENETIC ASPECTS OF CARDIOVASCULAR DISEASES IN ANIMALS.

When the incidences and types of cardiovascular disease present in various species, breeds, and strains, or families of animals are compared, certain differences are apparent. It is often difficult, however, to separate hereditary from environmental influences. Studies of vascular disease in zoo animals have shown that changing environmental conditions can alter the incidence of certain types of lesions in various species. Species differences, however, in resistance to diet-induced atherosclerosis appear to be genetically determined. The prevalence and types of congenital cardiac malformations appear to differ from species to species, but further systematic study is required. Arterial blood pressure is higher in the giraffe and turkey than in other species, and normal variants in cardiac rhythm are characteristic of the dog, horse, and mole. Relatively high incidences of specific cardiovascular diseases are found in certain breeds of animals. The White Carneaux, Autosexing King, and Silver King breeds of pigeons have a high incidence of atherosclerosis. Congenital heart disease appears to be more common in purebred than in mongrel dogs, and an unusual aggregation of cases of subaortic stenosis in the Boxer and German Shepherd breeds has been found. In a survey of heart disease in dogs, the prevalence of chronic congestive heart failure in the male Cocker Spaniel greatly exceeded that in the male and female of all other breeds. Arterial blood pressure is higher in Broad Breasted Bronze turkeys than in the Jersey Buff breed. This is associated in the former breed with a relatively high incidence of spontaneous aortic rupture. The occurrence of cardiovascular disease is unusually high in certain families and strains of animals. Among swine, litter and strain differences in serum cholesterol levels and in susceptibility to atherogenic diets occur. The White Carneaux breed of pigeons is actually a highly inbred strain with a remarkable predisposition to the development of atherosclerosis. Strains of chickens and rats with high incidences of interventricular septal defects have been developed by selective breeding. The familial occurrence of congenital heart disease in dogs and swine has been observed, and an inherited vascular anomaly in cattle has been described. Through selective breeding of laboratory rodents, strains with various types of cardiomyopathies have been developed. Certain diseases thought to be similar to the heritable disorders of connective tissue in man have been identified in domestic species. The level of arterial blood pressure is a heritable characteristic, and strains of rabbits, rats, and chickens with relative hypertension have been produced by selective breeding. Many of these observations indicate the importance of inheritance in determining susceptibility to various types of acquired cardiovascular disease. Genetic factors appear to operate in determining the occurrence of certain congenital malformations. Breeding experiments provide the most convincing evidence of genetic influence on the development of specific cardiovascular lesions. This experimental approach holds the greatest promise for furthering knowledge and understanding of the role of inheritance in the etiology of disease of the heart and blood vessels.

THE ASSOCIATED OCCURRENCE OF THE NEPHROTIC SYNDROME AND CONGENITAL HEART DISEASE

Two patients with congenital heart disease who developed the nephrotic syndrome are discussed. Neither had evidence of heart failure or increased venous pressure. The possible pathophysiologic mechanisms leading to the nephrotic syndrome are discussed. The interesting possibility is raised that the associated occurrence of congenital heart disease and the nephrotic syndrome in such patients may represent different expressions of an inherited or developmental abnormality.

Familial Congenital Complete Heart Block

In general, the occurrence of congenital heart disease may be considered to be due to genetic factors, environmental factors, or a combination of the two. When an identical defect is found in more than one member of the same family, a genetic etiology is suggested. The medical literature reveals numerous reports of a wide variety of congenital heart lesions occurring in families. Taussig, 1 for example, reported on a family in which patent ductus arteriosus appeared in 3 successive generations. This experience provides strong exidence that at least certain types of congenital heart lesions may have a genetic basis. Complete congenital heart block is an uncommon lesion, having been reported only slightly over 100 times in the world literature. The etiology of this condition remains unsettled. Autopsy reports 2-9 which include detailed histologic study of the conduction system have revealed that most often the atrioventricular node was separated from the

Congenital heart disease among mental defectives and an assessment of cardiac survey methods.

THE frequency of congenital and acquired heart disease has been investigated by a variety of methods in many different population groups, but mental defectives have not received comparable attention. Mautner1 recently made a clinical study at a state institution, but confined his observations to mongolism and gave no figures of prevalence. The purpose of the present study was to assess the over-all frequency of heart disease in mental deficiency with particular reference to the presence of congenital lesions. We were also interested in the occurrence of congenital heart disease among persons with different types of mental retardation, especially mongolism, because . . .

Coarctation of the aorta in three members of a family

Three cases of coarctation of the aorta occurring in one family and affecting the mother, daughter, and son are reported. This paper provides a follow-up on these cases, which were previously noted as examples of the familial occurrence of congenital heart disease.