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PubMed1996

Cerebrovascular events in adult patients with cyanotic congenital heart disease.

OBJECTIVES: We sought to determine the frequency of spontaneous cerebrovascular events in adult patients with cyanotic congenital heart disease and to evaluate any contributing factors. BACKGROUND: Cerebrovascular events are a serious complication of cyanotic congenital heart disease in infants and children but are said to be uncommon in adults. METHODS: Between 1988 and 1995, 162 patients with cyanotic congenital heart disease (mean age 37 years, range 19 to 70) were retrospectively evaluated for any well documented cerebrovascular events that occurred at > or = 18 years of age. Events related to procedures, endocarditis or brain abscess were excluded. RESULTS: Twenty-two patients (13.6%) had 29 cerebrovascular events (1/100 patient-years). There was no significant difference between those with and without a cerebrovascular event in terms of age, smoking history, degree of erythrocytosis, ejection fraction or use of aspirin or warfarin (Coumadin). Patients who had a cerebrovascular event had a significantly increased tendency to develop hypertension, atrial fibrillation, microcytosis (mean corpuscular volume < 82) and history of phlebotomy (p < 0.05). Even when patients with hypertension or atrial fibrillation were excluded, there was an increased risk of cerebrovascular events associated with microcytosis (p < 0.01). CONCLUSIONS: Adults with cyanotic congenital heart disease are at risk of having cerebrovascular events. This risk is increased in the presence of hypertension, atrial fibrillation, history of phlebotomy and microcytosis, the latter condition having the strongest significance (p < 0.005). This finding leads us to endorse a more conservative approach toward phlebotomy and a more aggressive approach toward treating microcytosis in adults with cyanotic congenital heart disease.

Adult↗
PubMedDate not supplied

Congenital heart disease among school children in Shimla hills.

A stratified random sample of 15,080 school going children out of 40,950 children in the age group of 5 to 16 years were screened for the prevalence of congenital heart disease and rheumatic fever/rheumatic heart disease. Thirty children were found to be suffering from definite congenital heart disease giving a prevalence of 2.25 per thousand. Atrial septal defect was the commonest lesion (38.2%) with a prevalence of 0.87 per thousand followed by ventricular septal defect (32.2%) with a prevalence of 0.73 per thousand. The prevalence of congenital heart disease was found to be significantly more in female (3.3 per thousand) than in male children (1.4 per thousand) (p < 0.05). Three (8.8%) cases had a family history of congenital heart disease. Rheumatic fever/rheumatic heart disease was found to have a significant prevalence among cases with congenital heart disease (8.8%) as compared to children without congenital heart disease (0.3%) (p < 0.001). The findings suggest the need for screening of family members of those suffering from congenital heart disease and that special attention be paid to the occurrence of rheumatic fever/rheumatic heart disease in cases of congenital heart disease.

Adolescent↗
PubMed2005

Delivery room and early postnatal management of neonates who have prenatally diagnosed congenital heart disease.

Advances in fetal echocardiography are providing highly accurate diagnoses of congenital heart disease prior to delivery, making it possible to plan the delivery-room management of these newborns. Knowledge of the expected transitional circulation occurring with birth and the pathophysiologic implications of congenital heart disease increases the likelihood of providing efficient and effective therapies. The majority of neonates who have congenital heart disease will not require delivery room resuscitation in excess of routine care; however, a small number of prenatally diagnosed cardiac lesions are more likely to require urgent postnatal intervention immediately following delivery. These cardiac lesions include transposition of the great arteries with intact ventricular septum and restrictive atrial septum, hypoplastic left heart syndrome with intact atrial septum, obstructed total anomalous pulmonary venous return, and complete congenital heart block. Prenatal diagnosis allows for coordination of care surrounding delivery and during the early postnatal hours.

Delivery Rooms↗
PubMed2003

Modified ultrafiltration in surgical correction of congenital heart disease with cardiopulmonary bypass.

The surgical correction of congenital heart disease using haemodilution and hypothermia with cardiopulmonary bypass (CPB) may expose patients to tissue ischaemia and initiate a systemic inflammatory response, increasing the total body water and inducing impairment, especially of heart, lung and brain function. It is possible to use ultrafiltration during CPB in the rewarming phase to remove water accumulation in the third space (conventional ultrafiltration). The reduced volume of prime used in children and the ability only to filter the reservoir blood during CPB led the Great Ormond Street Group to modify the method of ultrafiltration with regards to the placement of the filter and the timing of filtration (post-CPB). The main advantage of the modified technique is the ultrafiltration of the patient. A prospective nonrandomized study has been conducted to compare conventional with conventional + modified ultrafiltration. From January 1996 to March 1998, 41 patients underwent correction of congenital heart disease and were submitted to a comparative study (homogeneous groups), using either the conventional or the conventional + modified ultrafiltration techniques. There were no technical complications, no patient required mediastinal re-exploration due to bleeding and it was possible to close all the chests. There were significant differences in the ultrafiltrate volume balance (143.3 +/- 54.3 versus 227 +/- 71.4 mL; P < 0.001) but there were no significant differences in clinical postoperative evolution between the conventional and the conventional + modified ultrafiltration.

Blood Transfusion↗
PubMed2006

Improved perceived health status persists three months after a special sports camp for children with congenital heart disease.

INTRODUCTION: Although summer and sports camps for children with congenital heart disease are organized in many countries and regions, empirical data on the effects of such camps is limited. OBJECTIVES: The aim of the present study was to investigate changes in the perceived health status and habitual physical activities in children attending a special sports camp. MATERIALS AND METHODS: In this longitudinal study, 25 children with congenital heart disease who participated in a three-day multi-sports camp were included. The perceived health status was measured using the Child Health Questionnaire-Child Form, CHQ-CF87, completed by the child at the start of the camp (T1), at the end of the camp (T2), and 3 months after the camp concluded (T3). Habitual physical activities were assessed by means of a modified version of the Baecke questionnaire, which was completed by one of the parents at T1 and T3. RESULTS: During the sports camp, we observed significant improvements in the children's perception of their physical functioning, role-physical functioning, general health, role-emotional functioning, self-esteem, mental health, and general behavior. For physical functioning, role-emotional functioning, and family activities, high scores persisted three months after the sports camp concluded. The habitual physical activities (sport and leisure time) of the children remained unchanged. DISCUSSION AND CONCLUSION: In conclusion, we propose that a special sports camp for children with congenital heart disease may improve specific dimensions of subjective health status. Our study confirms a previous report on the benefits of such camps for afflicted children. If these findings can be further corroborated in other settings, participation in sport camps should be advocated as a simple, noninvasive means to promote healthier children.

Adolescent↗
PubMed1993

Congenital heart disease in CHARGE association.

This study reviews the spectrum of congenital heart disease and associated anomalies in 59 patients with the CHARGE association. We have analyzed our clinical experience in managing the cardiovascular anomalies and have reviewed outcome and risk factors for mortality. This study also highlights problems of cardiac management in children born with multiple system involvement. Twenty patients have died; actuarial survival was 78% at 1 year and 60% at 10 years. In only four of the nonsurvivors could their demise be ascribed to their underlying congenital heart disease. We found the outlook for survival was poor if more than one of the following three features were present; cyanotic cardiac lesions, bilateral posterior choanal atresia, or tracheoesophageal fistula. However, mortality was largely due not to the structural heart or choanal abnormalities, but instead reflected the underlying pharyngeal and laryngeal incoordination which resulted in aspiration of secretions. Furthermore, outcome is likely to be improved if collaboration between specialist surgical teams allows necessary procedures to be performed using the minimum of anesthetics. Examination of both the short- and long-term management of these children has stressed the importance of a multidisciplinary approach to their care. The pattern of cardiac defects was not random; lesions within the Fallot spectrum accounted for 33% of their congenital heart disease. Atrioventricular septal defects were also overrepresented. Not all cardiovascular defects could be explained by hypothesizing a neural crest etiology.

Abnormalities, Multiple↗
PubMed1999

First-cousin matings and congenital heart disease in Saudi Arabia.

BACKGROUND: Consanguinity may be a risk factor that contributes to congenital heart disease in an inbred population, particularly among first cousins; with high rates of intermarriage between relatives, consanguinity is associated with congenital heart disease particularly among first cousins. PATIENTS AND METHODS: This study examines a group of 1,028 consecutive congenital heart disease (CHD) patients identified through the Congenital Heart Disease Registry at King Faisal Specialist Hospital in Riyadh, Saudi Arabia. Families were interviewed by an Arabic-speaking data collector for information on consanguinity and demographic data. After exclusions for nonavailability, data were collected on 949 cases and the proportions of first-cousin consanguinity in the study sample were compared to national population data. A z test of proportions was utilized to test the hypothesis that there are higher proportions of first-cousin consanguinity in the study sample of congenital heart patients than in the general population. FINDINGS: Data indicate that the proportion of first-cousin matings among CHD patients is significantly higher than that of first-cousin intermarriages reported in the general population (p < 0.001). Regional breakdowns of the data substantiate significant differences (p < 0.001) in the proportions of first-cousin consanguinity in the study sample compared to the general population. INTERPRETATION: In a population with a high degree of inbreeding, consanguinity may exacerbate underlying genetic risk factors, particularly for offspring of first-cousin matings. This finding has public health implications for genetic counseling and prevention of some cardiac malformations.

Journal Article↗
PubMed1995

Aspects of the aetiology of congenital heart disease.

A concise overview of current knowledge on the aetiology of congenital heart disease is provided. At present, only 10 to 20% of the cases occurring in neonates can be attributed to known risk factors. Recurrence within relatives, chromosomal anomalies, genetic disorders, maternal disease and teratogen exposure are addressed briefly; contemporary research models and methods, e.g. embryology and genetics and molecular biology, are referred to. A major innovation has been the introduction of the concept of common pathogenetic pathways. Thus, different teratogenic factors or risk-factors may affect normal development at an identical stage and cause similar malformations. Also, the importance of timing of an event is stressed. If the time frame of exposure does not coincide with embryogenesis any teratogenic effect may be missed. Large-scale epidemiological studies on fetuses and neonates with congenital heart disease are introduced as a third mode of research on the aetiology, although this approach is not used efficiently at present; cases of intra-uterine death can be considered a valuable source of information that needs further attention. Combined, the above three lines of research may prove productive, but the design of a comprehensive research project would need to be handled carefully. Possibilities for prevention of the occurrence of cardiovascular malformations are reported. Through lack of knowledge of causality, at present, only secondary prevention may be possible and hence deserves attention. However, there appears to be no provision for thorough pre-natal screening tests for congenital heart disease in an unselected population.

Heart Defects, Congenital↗
PubMed2005

Young adults experiences of living with congenital heart disease.

BACKGROUND: Advances in care have improved the longevity and quality of life for children with congenital heart disease; however, many of them need lifelong highly qualified specialist care. The cardiac lesion involved may not always be the patient's main problem; issues related to quality of life may dominate. AIM: To explore and gain a deeper understanding of young adults experiences of living with congenital heart disease in order to enhance the quality of care provided by the health care system. METHOD: Transcribed in-depth interviews were analysed using a phenomenological-hermeneutic method. Six adults with congenital heart disease, aged 22-39 years old, were included in the study. RESULTS: Analyses yielded two themes, having the disease and living with illness, both originating from the main theme of ambivalence. The interviewees were ambivalent in how they viewed themselves, how they faced their daily life and how they dealt with their encounters with the health care system. They had to strike a balance between being different and not being different; being sick and being healthy; revealing their congenital heart disease or hiding it and living with a hidden handicap. They also had to cope with the disease and with the health care system. CONCLUSION: Young adults with congenital heart disease are ambivalent. They have a strong wish to be healthy and they might hide their symptoms from the healthcare personnel and sometimes even from themselves. A psychosocial preparedness when meeting these patients is necessary.

Adult↗
PubMed1984

Congenital heart disease and cow's milk intolerance.

Four infants with congenital heart disease showed in their first months of life poor weight gain in spite of the absence of cardiac failure; a diagnosis of intolerance to cow's milk proteins was made on the basis of laboratory findings and subsequently confirmed by the success of the avoidance diet and the positivity of the challenge tests; all four children had previously received nasogastric feeding, and this may represent a favouring factor for sensitization. Intolerance to cow's milk proteins should be considered as a possible cofactor for failure to thrive in infants with congenital heart disease.

Animals↗
PubMed1978

Parental reactions to children with congenital heart disease.

Informal discussions with 260 families of children with congenital heart disease are reported. Parents raised questions concerning etiology, timing of the diagnosis, pathophysiology and symptomatology, and need for restriction. Depending upon the stage and seriousness of the disease, common parental behavior was observed. A psychological process similar to mourning is required at the time of diagnosis and at the time of corrective surgery in order to promote the family's adaptation to the child with congenital heart disease. Management suggestions are included.

Adaptation, Psychological↗
PubMed2002

Health related quality of life and health status in adult survivors with previously operated complex congenital heart disease.

OBJECTIVE: To examine the impact of previously operated complex congenital heart disease on health related quality of life and subjective health status and to determine the relation between these parameters and physical status. DESIGN: Cross sectional; information on medical follow up was sought retrospectively. SETTING: Patients were randomly selected from the archives of the paediatric cardiology department, Leiden University Medical Centre, Leiden, The Netherlands, and approached irrespective of current cardiac care or hospital of follow up. PATIENTS: Seventy eight patients with previously operated complex congenital heart disease (now aged 18-32 years) were compared with the general population. MAIN OUTCOME MEASURES: Health related quality of life was determined with a specifically developed questionnaire (Netherlands Organisation for Applied Scientific Research Academic Medical Centre (TNO-AZL) adult quality of life (TAAQOL)) and subjective health status was assessed with the 36 item short form health survey (SF-36). Physical status was determined with the objective physical index, Somerville index, and New York Heart Association functional class. RESULTS: Health related quality of life of the patients was significantly worse than that of the general population in the dimensions gross motor functioning and vitality (p < 0.01). Correlations between health related quality of life and physical status were poor. Patients had significantly worse subjective health status than the general population in the dimensions physical functioning, role functioning physical, vitality, and general health perceptions (p < 0.01). Correlations between subjective health status and physical indices were weak. CONCLUSION: Adult survivors with previously operated complex congenital heart disease experienced limitations only in the physical dimensions of health related quality of life and subjective health status. Objectively measured medical variables were only weakly related to health related quality of life. These results indicate that, when evaluating health related quality of life, dedicated questionnaires such as the TAAQOL should be used.

Adolescent↗
PubMed1996

New understandings in the genetics of congenital heart disease.

In spite of the amazing success during the past half century in diagnosis and treatment of congenital heart disease, very little is known of its cause. However, a genetic cause has been clearly established for many forms of cardiovascular disease, and new understandings in the molecular genetics of congenital heart disease provide further insight. Progress has been quite impressive for some cardiovascular abnormalities, whereas in other areas the findings are more preliminary. For example, the molecular genetic cause of supravalvular aortic stenosis and the heart disease associated with Marfan syndrome has been clearly established. Impressive progress has been made in conotruncal defects, Holt-Oram syndrome, Alagille syndrome, and total anomalous pulmonary venous connection. In other areas, such as patent ductus arteriosus and atrioventricular septal defect, the findings are more preliminary. Taken as a whole, the prospect of understanding the genetic basis of congenital heart disease has never been better. Understanding the cause of congenital heart disease will provide new insights into both normal cardiac development and the pathophysiologic basis of congenital heart defects.

Abnormalities, Multiple↗
PubMedDate not supplied

Hemodynamic abnormalities in fetuses with congenital heart disease.

The purpose of this investigation was to assess left and right ventricular function, volume ejection fraction, combined stroke volume, and combined ventricular output in the human fetus with congenital heart disease compared to the normal healthy fetus. Seventy-two fetuses with a variety of in utero diagnosed congenital cardiac defects were compared with a control group of fetuses with structurally normal hearts matched for race, maternal age, and gestational age. We demonstrated significant hemodynamic changes in the fetus with congenital heart disease. There was a significant (p < 0.0001) decrease in the volume ejection fractions, biventricular stroke volume, and cardiac output in the congenital heart disease group compared to matched controls. Our findings suggest that hemodynamic abnormalities in the fetus with congenital heart disease are present before birth and we speculate that myocardial reserve may not be adequate to respond to hemodynamic stressors such as birth or heart surgery.

Adult↗
PubMed2005

[Prenatal diagnostics of congenital heart disease--own results].

OBJECTIVES: Congenital heart defects (CHD) are the most common congenital malformation. They are still often overlooked in fetuses during the routine obstetric scanning. DESIGN: The aim of our study was to present our results in detecting CHD in high risk pregnancies. MATERIAL AND METHODS: The echocardiograms of 135 fetuses and case history of neonates who had prenatal diagnosis of CHD were revied retrospectively. RESULTS: We diagnosed CHD in 135 fetuses. The most common CHD were: atrio-ventricular canal (32 fetuses-23,7%), hypoplastic left heart syndrome (17 fetuses--12.6%), ventricular septal defect (11 fetuses--8.2%), and aortic stenosis (10 fetuses--7.4%). There were 15 intrauterine deaths (11.1%) and 8 neonatal deaths. CONCLUSIONS: Prenatal echocardiography enables early detecting and adequate treatment of congenital heart defects.

Echocardiography↗
PubMedDate not supplied

Thiamine deficiency in children with congenital heart disease before and after corrective surgery.

BACKGROUND: Malnutrition is common in children with congenital heart disease, while thiamine deficiency (TD) is common in malnutrition, in critically ill children, and in adults with congestive heart failure treated with loop diuretics. Our goal was to determine whether children with congenital heart disease had TD and whether treatment with loop diuretics is related to TD in these patients. METHODS: Twelve children with ventricular septal defect (VSD) treated with furosemide, and 10 children with tetralogy of Fallot (TOF) referred for corrective surgery were consecutively enrolled into a prospective study. Data were collected 24 hours before surgery and 5 days after surgery for nutrition evaluation, medications used, anthropometric measurements, and laboratory markers of malnutrition. Thiamine and pyridoxine deficiencies were evaluated using activated enzyme assays. RESULTS: Seven children (32% of patients) did not meet the recommended daily allowance (RDA) for calories and 18% of patients did not meet the RDA for thiamine intake. Anthropometric measurements were low in both groups, more so in those with VSD, although the difference did not reach statistical significance. Overall, 18% (1/12 with VSD and 3/10 with TOF) of children with congenital heart disease had thiamine deficiency before surgery. Three of the four children with TD had adequate intake of thiamine. Six children (27%) had TD 5 days postsurgery (3 children with VSD and 3 children with TOF). CONCLUSIONS: TD is common in children with congenital heart disease (CHD) referred for corrective surgery both before and after surgery. Our results suggest that neither diuretic treatment nor malnutrition can fully explain the development of TD in these children.

Anthropometry↗
PubMed1999

[Prevalence and distribution of congenital heart diseases in Indre-et-Loire. Evaluation of prenatal diagnosis (1991-1994)].

UNLABELLED: Congenital heart diseases are the most frequent malformation at birth. New technologies have improved diagnosis procedures (echocardiography and Doppler). The aim of our study was to evaluate the prevalence of congenital heart diseases, their different types, and the detection rate of antenatal diagnosis. METHODS: A retrospective study was performed for all infants with congenital heart disease (CHD), born between January 1st 1991 and December 31st 1994, and for all fetuses which died after disruption of pregnancy, in Indre-et-Loire (a French country). In all cases, CHD diagnosis was confirmed with echocardiography and Doppler. RESULTS: CHD prevalence in newborns was 9.8% and 10.4% for the total population including dead fetal material. A high proportion of septal defects (64.8%) was observed with muscular, isolated and small forms (< 3 mm) in 70.2% of cases. The prevalence of great vessels transposition (0.15%), left ventricular hypoplasia (0.11%), and atrioventricular septal defect (0.11%), were lower than in previous studies. The performance of antenatal diagnosis was estimated at 40.5% for the four years; the prevalence of detectable CHD was only 1.4/1000. The atrioventricular septal defect was the most frequently detected. CONCLUSION: Relative high prevalence of congenital heart disease in this French county is due to the high level of small septal defects. Prevalence of detectable CHD remains low, which explains in part the difficulties of improving the antenatal diagnosis.

Abortion, Induced↗