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PubMed1992

Oxygen desaturation during sleep in infants and young children with congenital heart disease.

Oxygen saturation (SaO2) during sleep and pulmonary functions were evaluated in 19 infants with congenital heart disease, aged 6 +/- 4 months, and in 11 normal infants, aged 8 +/- 5 months, to determine whether infants with congenital heart disease have more frequent oxygen desaturation during sleep and, if so, its relationship to underlying pulmonary function. Infants with congenital heart disease were classified as acyanotic (n = 11) or cyanotic (n = 8) on the basis of their aortic SaO2 at the time of cardiac catheterization (greater or less than 90% SaO2). Pulmonary function tests included respiratory rate, functional residual capacity, total respiratory system compliance, and maximal flows at functional residual capacity. Significant differences were found in the values for the lowest SaO2 of each 5-minute epoch (SaO2L) averaged during the entire sleep time (normal 94% +/- 2%, acyanotic 90% +/- 3%, and cyanotic 74% +/- 4%; p less than 0.01). The three groups also differed significantly in frequency distributions of percentage of total sleep time with SaO2L less than 90% (SaO2%T) (normal 10% +/- 17%, acyanotic 36% +/- 34%, and cyanotic 97% +/- 4%; p less than 0.05). Compared with the control group, the acyanotic group had a higher respiratory rate (66 +/- 19 breaths/min vs 35 +/- 6 breaths/min; p less than 0.01), a lower tidal volume (65% +/- 29% predicted vs 105% +/- 18% predicted; p less than 0.01), and a lower total respiratory compliance (59% +/- 18% predicted vs 106% +/- 30% predicted; p less than 0.01). A negative correlation existed between SaO2%T and aortic SaO2 (R2 = 0.64; p less than 0.01). We conclude that oxygen desaturation occurs during sleep in infants with congenital heart disease; the presence of desaturation appears to be related to the initial degree of hypoxemia and the presence of abnormal pulmonary function.

Cyanosis↗
PubMed2006

Quality of life and health status in adults with congenital heart disease: a direct comparison with healthy counterparts.

INTRODUCTION: Despite having increased life expectancy, patients with congenital heart disease are sometimes presumed to have a diminished quality of life. This study therefore assessed the quality of life and perceived health of adults with congenital heart disease and compared these two measures with those reported by healthy control subjects. METHODS: Using a comparative study design, we examined quality of life and perceived health in 404 patients who were matched for age, sex, educational level, and employment status with 404 healthy counterparts. Quality of life was measured using a linear analog scale and the Satisfaction with Life Scale. Problems and concerns relevant to patients' quality of life were assessed by the Congenital Heart Disease-TNO-AZL Adult Quality of Life scale. Perceived health was also assessed with a linear analog scale. RESULTS: Patients perceived their quality of life (median linear analog scale score 80; median Satisfaction with Life Scale score 28) and health status to be good (median linear analog scale 80). Quality of life was significantly better in patients with congenital heart disease than in healthy peers, with a mean standardized difference of 0.22 on the linear analog scale and 0.34 on the Satisfaction with Life Scale. No group difference was found for perceived health. Problems and concerns in adults with congenital heart disease overlapped to a large extent with those identified by control subjects. Patients reported significantly higher distress scores for 16 of 77 items, whereas control subjects perceived more distress for 20 items. CONCLUSION: Adults with congenital heart disease perceived their quality of life to be better than did their healthy counterparts. This finding refutes the presumed lower quality of life in patients with cardiac anomalies.

Adolescent↗
PubMed1998

Ultrasound diagnosis of fetal diaphragmatic hernia and complex congenital heart disease at 12 weeks' gestation--a case report.

Congenital diaphragmatic hernia is commonly associated with congenital heart disease. Their coexistence indicates a poor prognosis. Prenatal diagnosis of these conditions in early pregnancy allows the option of pregnancy termination. We present a case of left-sided fetal diaphragmatic hernia and complex congenital heart disease diagnosed by ultrasound examination at 12 weeks' gestation.

Abortion, Induced↗
PubMed1996

Maternal congenital heart disease and pregnancy outcome.

OBJECTIVE: To study the obstetrics and neonatal outcome of women with congenital heart disease. METHOD: This is a retrospective study of 85 women with congenital cardiac disease. Data collected include maternal characteristics, New York Heart Association Class, cardiac complications and obstetric and neonatal outcome. RESULTS: The maternal and neonatal outcomes were excellent with no maternal and perinatal mortality. The main cardiac lesions were mitral valve prolapse (60.8%), atrial septal defect (8.6%), ventricle septal defect (6.5%) and aortic regurgitation (4.3%) and 2.1% each of tricuspid regurgitation, pulmonary regurgitation, Ebstein's anomaly, coarctation of aorta, patent ductus arteriosus, and Eisenmenger's syndrome. Six deliveries were associated with New York Heart Association deterioration. Compared to the general obstetric population, more women in the study group were primigravida, had pregnancy induced hypertension, underwent instrumental vaginal deliveries and caesarean sections and had more babies with lower birth weights. CONCLUSION: Despite potential difficulties and complications associated with congenital heart disease, careful cardiac and obstetric management in a tertiary referal centre resulted in good maternal and fetal outcomes.

Adolescent↗
PubMedDate not supplied

Prevalence and pattern of congenital heart disease in Hazara.

BACKGROUND: The incidence and particularly the pattern of congenital heart disease may vary in different geographical locations, but the extent to which such reported variations are attributable to differences in genetic predisposition, environmental factors, or in study methodology and diagnostic precision remains uncertain. There is, therefore, a continuing need for studies on various aspects of congenital heart disease, in different communities and races. METHODS: This study was done on 188 consecutive cardiac patients reporting at Ayub Teaching Hospital, Abbottabad, Pakistan from June 1998 to June 2000. RESULTS: 114 of 188 cardiac patients studied had congenital heart disease. Both sexes were equally affected. Ventricular septal defects were the most common lesions (relative frequency 61.4%), followed by Tetrology of Fallots, ASD and PDA with a relative frequency of 8.77%. The detection rate under one month was 28.07% and at one year 75.43%. CONCLUSION: There is therefore, a need for increased awareness, especially among primary health and other front line doctors for earlier case detection.

Female↗
PubMed1988

Assessment of hypertrophy in myocardial biopsies taken during correction of congenital heart disease.

Myocardial biopsies were obtained from 27 patients undergoing corrective cardiac surgery for congenital heart disease. Normal hearts of 18 autopsied patients were used as reference. The biopsy material was assessed for desoxyribonucleic acid (DNA) concentration and ploidy profile of cell nuclei in order to quantitate myocardial hypertrophy at the time of operation. DNA-concentration decreased significantly with age (r = -0.76; p less than 0.001). Ploidy profile of myocardial nuclei correlated with age: the relative number of diploid nuclei decreased (r = -0.67; p less than 0.001), the relative numbers of tetraploid and octoploid nuclei increased with age (r = 0.58; p less than 0.01 and r = 0.77; p less than 0.001 respectively). At 8 years of age the patients with congenital heart disease reached myocardial DNA-concentrations comparable with those in normal adult hearts. At higher age the patients with congenital heart disease exceeded normal adult values for myocardial DNA-concentration. These findings are interpreted to represent rapid development of hypertrophy with an early onset, reaching at 8 years of age values observed in normal adult hearts. Quantitation of myocardial hypertrophy by DNA-concentration and ploidy profile of nuclei may offer a means to explain some of the factors of influence on the outcome of corrective cardiac surgery for congenital heart disease in relation to its timing. Our data stress the need for preventing irreversible myocardial damage by timely (surgical) therapy.

Adult↗
PubMed1994

Magnetic resonance imaging in adult congenital heart disease.

Some patients with congenital cardiac anomalies develop their first symptoms as adults, and many more will survive to adulthood with congenital lesions that have been treated surgically. Magnetic resonance imaging (MRI) currently provides sufficient morphological information to allow the anatomical diagnosis of congenital abnormalities involving the heart and the great arteries. Newer MR techniques have also been developed that provide functional information such as measurements of valve gradients, stroke volumes, regurgitant volumes, and shunt volumes. Cardiac evaluation utilizing MR techniques may soon replace cardiac catheterization for the preoperative diagnosis of congenital heart disease and its long-term follow-up.

Adult↗
PubMed2002

Myocardial inflammatory activation in children with congenital heart disease.

OBJECTIVE: In several cardiac-related diseases, there is a strong association between systemic endotoxemia, myocardial cytokine production, and cardiac failure. Because pre- and postoperative endotoxemia recently was reported in children with congenital heart disease, we sought direct evidence of myocardial inflammatory activation in a cohort of children undergoing congenital heart surgery on cardiopulmonary bypass. Inflammatory activation was prospectively defined as the presence of nuclear factor-kappaB nuclear translocation in myocardial tissue samples. DESIGN: Prospective observational study. SETTING: Tertiary care pediatric intensive care unit. PATIENTS: Fifteen children with congenital heart disease undergoing operative repair on cardiopulmonary bypass. INTERVENTIONS: All patients underwent operative repair of congenital heart disease on cardiopulmonary bypass and had plasma samples obtained for endotoxin and tumor necrosis factor-alpha, both pre- and postoperatively. Myocardial tissue samples were obtained intraoperatively, both before and during cardiopulmonary bypass. MEASUREMENTS AND MAIN RESULTS: Elevated plasma endotoxin concentrations were documented in all 15 patients during the study period. In 12 patients, plasma endotoxin was elevated before cardiopulmonary bypass. The median preoperative tumor necrosis factor-alpha concentration was 16.4 pg/mL, which is higher than concentrations reported in adults with New York Heart Association class III congestive heart failure. Examination of myocardial tissue samples revealed nuclear factor-kappaB nuclear translocation (predominantly p50/p65 heterodimers) in nine of 15 patients (60%). Four of these nine patients had nuclear factor-kappaB nuclear translocation before initiation of cardiopulmonary bypass, with p50/p50 homodimers present in two of the four. CONCLUSIONS: These data provide the first evidence of nuclear factor-kappaB activation in children with congenital heart disease and the first evidence of myocardial nuclear factor-kappaB translocation in human hearts before explant for transplantation. Furthermore, these data suggest that, similar to adults with advanced congestive heart failure, the myocardial inflammatory cascade may contribute to the pathophysiology of congenital heart disease in infants and children.

Active Transport, Cell Nucleus↗
PubMed1981

Long-term results of operated and non-operated patients with congenital heart diseases.

The long-term results of 1023 adult patients with congenital heart diseases, operated and non-operated, with atrial septal defect (ASD), patent ductus arteriosus (PDA), pulmonary stenosis (PS) and tetralogy of Fallot, were followed for up to 24 years, and the long-term results of operated and non-operated congenital heart diseases were compared. The survival rate of patients with operated isolated ASD was significantly higher than in patients with non-operated isolated ASD, and that of operated patients with VSD associated with other anomalies was also significantly higher than in non-operated VSD patients with such involvement. The survival rate showed no significant difference in the operated and non-operated patients with PDA, PS and tetralogy of Fallot. In all congenital heart diseases, there was a close correlation between the mean pulmonary artery pressure and the mortality rate both in operated and non-operated patients and a marked improvement of physical capacity of operated patients at the time of the last follow-up examination.

Adolescent↗
PubMed2003

Brain injury in children with congenital heart disease.

The incidence of neurodevelopmental impairment in children with congenital heart disease is high. Its aetiology is multiple and complex. Prevention and treatment must start during the preoperative period and continue through the intra- and postoperative periods. Research has resulted in a clearer understanding of the relationship between congenital heart disease and the brain, and of the effects of cardiopulmonary bypass, hypothermia and circulatory arrest. This has led to modifications in management which may improve neurological outcome in the future.

Brain Injuries↗
PubMed2006

Early complications of stenting in patients with congenital heart disease: a multicentre study.

AIMS: Stenting has become an established interventional cardiology procedure for congenital heart disease. Although most stent procedures are completed successfully, complications may occur. This multicentre study evaluated early complications after stenting in patients with congenital heart disease, including potential risk factors. METHODS AND RESULTS: In this combined Dutch-Belgian retrospective study, 309 consecutive patients had undergone 366 catheterizations and received 464 stents in 13 different anatomical positions (418 sites). Seventy-two stenting-related complications (19%) occurred, of which 24 (5.7%) were major. Seven procedure-related deaths were documented (2.3%). Stent malpositioning and embolization were most common (7.7%). The use of non-premounted stents tended to be associated with higher complication rates. Centre inexperience with stenting and stenting of native vs. post-surgical stenosis tended to be associated with increased major complication rates. CONCLUSION: After stenting, complications are common for congenital heart disease. The vast diversity of stenotic sites combined with relatively small patient populations makes these procedures sensitive to complications. Combining operator experience may reduce the risks of stenting in congenital heart disease. The availability of premounted stents for greater vessel diameters will likely reduce incidences of stent migration and embolization.

Adolescent↗
PubMed1999

Neonatal ECG screening for congenital heart disease in Down syndrome.

We studied the value of routine neonatal electrocardiography (ECG) in the 1st 48 hours of life to diagnose congenital heart disease in 37 neonates with Down syndrome. Twenty-four infants had no clinical evidence of congenital heart disease, had normal ECGs and normal cardiac anatomy on echocardiography. Thirteen children (35.2%) had congenital heart disease. The ECG was normal in seven infants with congenital heart disease: four with atrial septal defect (ASD), two with tetralogy of Fallot (TOF) and one with ventricular septal defect (VSD). A left QRS axis deviation was found in six infants: five with complete atrioventricular septal defect (AVSD) and one with VSD and mitral valve prolapse. However, only the five infants with complete AVSD had a superior QRS axis. Although neonatal ECG detected the presence of complete AVSD in neonates with Down syndrome, it missed an equal number with other heart defects. Echocardiography remains the way reliably to diagnose or exclude congenital heart disease in these infants.

Down Syndrome↗
PubMed2005

Accuracy of fetal echocardiography in the routine detection of congenital heart disease among unselected and low risk populations: a systematic review.

OBJECTIVE: To determine among unselected and low risk populations the accuracy with which fetal echocardiography during the second trimester detects congenital heart disease. DESIGN: A systematic review of studies that assess the accuracy of fetal echocardiography. SETTING: District General Hospital and Tertiary referral centres. POPULATION: Women during the second trimester attending for ultrasound assessment. METHODS: General bibliographic databases (e.g. MEDLINE, EMBASE) and specialist computerised databases (e.g. Cochrane Library, National Research Register), grey literature, manual searching of reference lists of primary and review articles and personal contact with experts were used to identify studies. Studies were included if fetal echocardiography among unselected or low risk pregnant women was compared against a postnatal reference standard. Data were extracted on quality, study design and characteristics, and accuracy data to construct 2 x 2 tables. Data were synthesised qualitatively, and sensitivity and specificity with 95% confidence intervals were calculated. MAIN OUTCOME MEASURE: Sensitivity and specificity. RESULTS: Five primary studies met the inclusion criteria and comprised 60,901 subjects. One study assessed the accuracy of fetal echocardiography among an unselected population and four studies among low risk populations. All eligible studies found that fetal echocardiography helped to correctly diagnose normal fetus among unselected and low risk populations. Correct diagnosis of babies for congenital heart defects was higher among the unselected population (85%) than among the low risk populations (range from 35% to 86%); however, the potential for ascertainment bias and the choice of reference standard limits the validity of this finding. The variation in sensitivity across studies was not explainable by clinical factors such as scanning regime, operator skill and equipment. CONCLUSIONS: The evidence from this review about the accuracy of fetal echocardiography does not lend support to its routine use among unselected and low risk populations during the second trimester to detect congenital heart disease.

Echocardiography↗
PubMed2006

An alternate technique to pacing in complex congenital heart disease: assessment of the left thoracotomy approach.

BACKGROUND: Pacing in children with congenital heart disease often requires alternate approaches to standard transvenous pacing. The surgical approach used to implant the pacemaker leads has been shown to impact lead survival. There is a paucity of pediatric literature describing the experience using a left thoracotomy approach. OBJECTIVES: To report on short- and mid-term experiences with pacemaker implant via the left thoracotomy approach in children with complex congenital heart disease. METHODS AND RESULTS: Data were abstracted retrospectively from patients' hospital charts. To date, the left thoracotomy technique has been used in 11 patients with complex heart disease, with a median of three prior cardiac operations. The median patient age was five years (range of two months to 23 years of age). The pacing indications were acquired postoperative atrioventricular block (n=5), sinus node dysfunction (n=5) and long QT syndrome (n=1). There were no intraoperative complications or long-term complications from this approach. The pacing thresholds at implant and follow-up were acceptable in all patients. One patient died in follow-up for reasons unrelated to the pacemaker or arrhythmia. CONCLUSIONS: The placement of epicardial pacemaker leads via the left thoracotomy approach is a safe and effective alternative to transvenous pacing in pediatric patients with complex congenital heart disease.

Adult↗