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PubMedDate not supplied

Accelerated ventricular rhythm in children: a review and report of a case with congenital heart disease.

We report a child with accelerated ventricular rhythm (AVR) and congenital heart disease. Three children with congenital heart defect associated with AVR were previously reported, but in each AVR occurred only postoperatively. Because our patient's 24-hour electrocardiograph recording showed AVR rates, and differences between sinus and AVR rates, exceeding published childhood limits, we reviewed the topic. On the basis of our review, we suggest guidelines for diagnosing AVR and differentiating it from ventricular tachycardia.

Accelerated Idioventricular Rhythm↗
PubMed1996

Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management.

BACKGROUND: Management of pulmonary hypertension, a potentially fatal complication of operations to correct congenital heart disease, has evolved through the last 15 years. Monitoring of pulmonary arterial pressure and mixed venous saturation became available, and prophylactic use of alpha-blockers and other vasodilators increased. This study examines risk factors for morbidity and mortality from pulmonary hypertension after operations to correct congenital heart disease and evaluates the impact of management changes on outcomes. METHODS: By means of multivariable logistic regression analysis, 880 high-risk patients with congenital heart disease (of 2484 patients undergoing cardiopulmonary bypass between January 1980 and December 1994) were analyzed to determine which were at risk for postoperative pulmonary hypertension and its associated morbidity and mortality. RESULTS: Patients with atrioventricular canal (n = 182), truncus arteriosus (n = 47), total anomalous pulmonary venous connection (n = 90), transposition of great arteries (n = 97), hypoplastic left heart syndrome (n = 50), and ventricular septal defect (n = 414) demonstrated a higher risk of postoperative pulmonary hypertension. By multivariable logistic regression, preoperative pulmonary hypertension (p < 0.0001), absence of mixed venous saturation monitoring (p < 0.0001), and absence of prophylactic alpha-blockade (p = 0.0004) significantly increased postoperative pulmonary hypertension. Preoperative pulmonary hypertension (p < 0.001) and absence of prophylactic alpha-blockers (p = 0.0004) were significant risk factors for in-hospital death related to pulmonary hypertension. Repair at older age (except in the case of total anomalous pulmonary venous connection) was a significant risk for postoperative pulmonary hypertension (p = 0.03). CONCLUSION: Mixed venous saturation monitoring and alpha-receptor blockade reduced the incidence of pulmonary hypertension after operations for congenital heart disease. Early definitive repair reduced morbidity and mortality from postoperative pulmonary hypertension.

Cardiac Surgical Procedures↗
PubMed1981

Contribution of pulsed Doppler echocardiography to ultrasound diagnosis of congenital heart diseases.

The authors document the importance of echocardiograhy (ECHO) with special respect to the pulsed Doppler technique (PDE) in the diagnosis of congenital heart diseases. Typical ECHO and PDE findings in atrial and ventricular septal defects, patent ductus arteriosus, valvular stenosis and insufficiency, absence of pulmonary valves, tricuspid and pulmonary atresia, Fallot's tetralogy, arterial trunk, double-outlet right ventricle, transposition of the great arteries, total anomalous pulmonary venous drainage and hypoplastic left-heart syndrome are described. The authors conclude that a combination of M-mode, cross-sectional and pulsed Doppler echocardiography permits a comparatively detailed non-invasive diagnosis of the entire spectrum of congenital heart diseases.

Aortic Valve Stenosis↗
PubMedDate not supplied

Measurement of tricuspid annular diastolic velocities by Doppler tissue imaging to assess right ventricular function in patients with congenital heart disease.

We assessed the clinical utility of using diastolic tricuspid annular velocities obtained by Doppler tissue imaging as a noninvasive index of right ventricular function in patients with congenital heart disease. Doppler tissue imaging at the tricuspid annulus and pulsed Doppler echocardiography of the right ventricular inflow were performed in 71 children with congenital heart disease, with and without elevated right ventricular pressure. Cardiac catheterization was performed in all patients with congenital heart disease, and the hemodynamic determinants of the tricuspid annular and inflow velocities were determined. In patients with congenital heart disease, the ratio of the late-to-early diastolic tricuspid annular velocity (Aa/Ea) showed a highly significant correlation with right ventricle pressure/left ventricle pressure (r = 0.79, p < 0.0001), right ventricular end diastolic pressure (r = 0.46, p < 0.0001), and the first derivatives of the change in right ventricle pressure during diastole (r = 0.72, p < 0.0001). However, the late-to-early diastolic tricuspid inflow velocity (A/E) did not correlate with any invasively measured index of right ventricular function. Aa/Ea, derived from tricuspid annular velocities as measured by Doppler tissue imaging, is a valuable, noninvasive tool for detecting an elevated right ventricular pressure in patients with congenital heart disease.

Blood Flow Velocity↗
PubMed1975

Congenital heart disease in low birth weight infants.

In this study of low birth weight infants (less than 2500 g), we compared the birth weight distribution of patients with patent ductus arteriosus associated with prematurity with that of patients having congenital heart disease other than patent ductus arteriosus. Among 1436 low birth weight infants 37 infants had congenital heart disease other than patent ductus arteriosus and 198 had isolated ductus arteriosus. Infants with congenital heart disease had a mean birth weight of 2018 grams (standard deviation = 370 g) which was significantly greater than that of infants with patent ductus arteriosus. Four of the 37 infants with congenital heart disease other than ductus arteriosus were among 1150 low birth weight infants born in this institution, an incidence of 3.5/1000 live low birth weight infants. Seventy-eight of the 198 infants with patent ductus arteriosus were among 1150 low birth weight infants born in this institution, an incidence of 70/1000 live low birth weight infants. Twenty-one of the 37 infants with congenital heart disease were of appropriate weight for gestational age and 16 were small for gestational age.

Aorta↗
PubMed2006

Familial recurrence of congenital heart disease: an overview and review of the literature.

Familial recurrence is one of the classic patterns for the clinical presentation of congenital heart disease (CHD). In recent years, many groups studied empiric recurrence risk in these heart defects, and the aim of this review was to analyse the patterns of inheritance detected in different types of CHD, including atrioventricular canal defect, tetralogy of Fallot, transposition of the great arteries, left-sided obstructions and atrial septal defect. These studies may influence clinical practise guidelines through accurate genetic counselling, but also, they may have important implications in the knowledge of genetic and pathogenetic mechanisms of CHD.

Family↗
PubMed1996

[The clinical value of fetal congenital heart disease diagnosed by color Doppler echocardiography].

OBJECTIVE: To investigate the clinical value of color Doppler echocardiography for the diagnosis of fetal congenital heart disease (CHD). METHODS: 368 cases of high risk fetuses of CHD, aged from 20-40 gestational weeks, were examined by color Doppler echocardiography with Acuson 128 x P/10 color Doppler flow imaging system. The prenatal echocardiographic diagnosis were confirmed by fetal autopsy and echocardiographic examine after birth as well as follow-up. RESULTS: 11 cases of fetal CHD were detected by prenatal echocardiography, of those, 5 cases of CHD were confirmed by fetal autopsy after induction of labor and 5 cases of CHD were confirmed by color Doppler echocardiography after birth, 1 case of false-positive and 1 case of false-negative. CONCLUSIONS: The study suggests that four-chamber view is an important view for detecting fetal CHD by echocardiography, but multiple views are necessary for diagnosis of fetal complex CHD. Color Doppler echocardiography lists as the first choice for prenatal diagnosis of fetal CHD.

Adult↗
PubMed1985

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).

Acetaminophen↗
PubMed1989

Delayed sternal closure: a lifesaving maneuver after early operation for complex congenital heart disease in the neonate.

Early repair of complex congenital heart malformations may lead to life-threatening respiratory and hemodynamic embarrassment on sternal closure. To avoid a fatal outcome in these situations, we postponed sternal closure in nine critically ill neonates by suturing silicone elastomer sheeting to the skin edges. This maneuver, in a setting of optimal inotropic and ventilatory support, allowed eight of the nine neonates to survive. The mean age at operation was 10.2 days (range 3 to 31). The mean preoperative weight was 3004.4 gm (range 1550 to 3780). The sternal wound was protected with an impervious silicone elastomer sheeting for a mean of 5.6 days (range 2 to 12). There was no instance of wound infection. The judicious application of this technique after operations for complex congenital heart disease can provide the necessary compliance vital for immediate cardiopulmonary performance and ultimate survival.

Edema↗
PubMed2003

Transnasal placement of biplane transesophageal echocardiography probe intraoperatively in an adolescent with congenital heart disease.

UNLABELLED: Intraoperative transesophageal echocardiography (TEE) is frequently used in children with congenital heart disease (CHD). Although transnasal TEE is being used in various settings in the adult population, there are no descriptions of its use intraoperatively in patients with CHD. This report describes the successful use of transnasal TEE after multiple unsuccessful transoral attempts in an adolescent male undergoing subaortic stenosis repair. IMPLICATIONS: Transnasal transesophageal echocardiography (TEE) is being used in various settings in the adult population. The author describes its use intraoperatively in an adolescent undergoing surgery for congenital heart disease after unsuccessful transoral attempts.

Adolescent↗
PubMed1996

Pulmonary balloon valvuloplasty in the palliation of complex cyanotic congenital heart disease.

OBJECTIVE: To assess the value of pulmonary balloon valvuloplasty in the interim palliation of complex congenital heart disease and pulmonary stenosis in children, who often require numerous palliative operations before definitive surgical repair. METHODS: Evaluation of pulmonary balloon valvuloplasty procedures performed over a five year period in 18 patients (age 8 days--29 years; mean 5.5 years) with complex cyanotic congenital heart disease. RESULTS: After pulmonary balloon valvuloplasty oxygen saturation increased from a mean (SD) of 69 (7.5)% to 83 (7.0)% (P < 0.001). Mean pulmonary artery pressure increased from a mean (SD) of 11.3 (3.8) mm Hg to 15.7 (3.9) mm Hg (P < 0.001). Transient complete atrioventricular block occurred in one patient. No other complications were encountered. In 5 patients (28%) there was an inadequate improvement in cyanosis compared with pre-procedure values (72 (4.7)% v 66 (8.1)%). Reasons for failure were increasing infundibular stenosis in three and inadequate mixing in one child. In 13 patients (72%) pulmonary balloon valvuloplasty gave adequate interim palliation over a mean follow up of 1.1 (1.3) years. Oxygen saturation was 81 (5.6)% at last follow up compared with 70 (7.3)% before pulmonary balloon valvuloplasty (P < 0.001). CONCLUSION: Pulmonary balloon valvuloplasty is a safe and effective technique in the palliation of patients with complex cyanotic congenital heart disease associated with pulmonary valve stenosis.

Adolescent↗
PubMed2003

The right heart in congenital heart disease.

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.

Journal Article↗
PubMed1994

Congenital heart disease and pregnancy.

In Europe and North America, a dramatic fall in the incidence of rheumatic fever and rheumatic heart disease has coincided with advances in medical and surgical management of congenital heart disease and has resulted in a shift in the relative incidence of these two categories of cardiac disorders in women of child-bearing age. This review deals with pregnancy and congenital heart disease--unoperated and operated. Central to this topic is the intricate interplay between maternal circulatory and respiratory physiology and maternal congenital heart disease, and the effects of this interplay upon the fetus which is exposed to risks that threaten its intrauterine viability and to risks that subsequently express themselves as developmental defects or transmitted congenital malformations of the heart or circulation.

Delivery, Obstetric↗
PubMed2005

Comparison of health related quality of life with cardiopulmonary exercise testing in adolescents and adults with congenital heart disease.

OBJECTIVES: To compare self reported quality of life with measured exercise capacity in patients with congenital heart disease. DESIGN: Prospective cross sectional clinical study. SETTING: Tertiary referral centre for congenital cardiology. PATIENTS AND METHODS: 149 patients (60 female, 14-60 years old) with various congenital heart defects completed a health related quality of life questionnaire (medical outcomes study 36 item short form). Then they performed a cardiopulmonary exercise test on a bicycle in a sitting position. RESULTS: Peak oxygen uptake correlated significantly with the physical functioning (r = 0.521, p < 0.0005) and general health scales (r = 0.313, p < 0.0005) but not with role-physical, bodily pain, vitality, social functioning, role-emotional, mental health, and health transition scales. Closer examination of the data showed that despite an overall good correlation a substantial number of patients overestimated their physical capabilities. CONCLUSIONS: Exercise tests and quality of life instruments should be used together to get an appropriate overview of the health status of patients with congenital heart disease.

Adolescent↗
PubMed2004

Dental caries experience in children with congenital heart disease: a case-control study.

OBJECTIVES: To compare the dental health of a group of children with complex congenital heart disease with that of age and gender matched healthy controls. DESIGN: Case-control study. SETTING: Faculty of Medicine and Odontology/Pediatric cardiology and Pedodontics, Umeå University, Sweden. SAMPLE AND METHODS: All the cases and their controls lived in the county of Västerbotten in northern Sweden. Each group comprised 41 children with a mean age of 6.5 years. Data were collected from medical and dental records while all bitewing radiographs were read separately by one of the authors. RESULTS: Children with congenital heart disease had significantly more caries in their primary teeth than the control group. The mean dmfs-value was 5.2 +/- 7.0 in the cardiac group compared to 2.2 +/- 3.5 in the control group (P < 0.05). Twenty-six of the children had all four 6-year-molars, and their mean DMFS-values were 0.9 +/- 1.9 in the cardiac group compared to 0.3 +/- 0.6 in the control group (P > 0.05). The children with congenital heart disease had received more caries prevention based on the use of fluorides than the control group. There was a significant correlation between the number of fluoride varnish treatments and the dmfs value of the child (r = 0.411, P < 0.01). Fifty-two per cent of the children in the cardiac group had been prescribed fluoride tablets on one or more occasions compared to 17% in the control group (P < 0.01). Number of months on digoxin medication and the dmfs-value had a significant correlation (r = 0.368, P < 0.05). Ten of the children had been on digoxin medication between 6 and 87 months; this subgroup had a mean dmfs-value of 10.1 +/- 8.5. CONCLUSION: Swedish children with complex congenital heart disease have poorer dental health than healthy age and gender matched controls in spite of intensive preventive efforts. In many cases, intervention had been given when caries were present. A closer cooperation between paediatric cardiology and paediatric dentistry is needed.

Cardiotonic Agents↗
PubMed1987

Congenital heart disease in pregnancy: short- and long-term implications.

One hundred forty-four pregnancies that occurred in 74 patients with congenital heart disease at the Toronto General Hospital between 1975 and 1986 were reviewed. The patients were divided into two groups, those with acyanotic lesions and those with cyanotic lesions. Maternal and fetal outcomes in these two groups were compared. Patients with cyanotic lesions developed significantly more congestive heart failure and deteriorated more often in functional cardiac status than those with acyanotic lesions (p less than 0.005). Women with obstructive lesions had a higher incidence of pregnancy-induced hypertension than our general population (p less than 0.005). There was one maternal death in the puerperium in a woman with Eisenmenger's syndrome. Preterm births were more frequent in the cyanotic group (p less than 0.01) as were small for gestational age infants (p less than 0.005). In the cyanotic group the mean birth weight was 900 gm less than that in the acyanotic group (p less than 0.005). The major benefits of corrective cardiac surgery were in the cyanotic group and consisted of fewer spontaneous abortions (p less than 0.01) and small for gestational age infants (p less than 0.02) and more term births (p less than 0.01). Long-term follow-up revealed that 19.2% of women with acyanotic lesions had significant cardiac developments, compared with 90% in the cyanotic group (p less than 0.005). One of 87 infants (1.1%) had congenital heart disease and two had Marfan's syndrome.

Female↗
PubMed1997

Prenatal diagnosis of congenital heart disease.

Owing to the widely different levels of experience of examiners, there is a large discrepancy in study results of second trimester ultrasound screening for fetal malformations, which is a result of varying levels of obstetric scanning expertise prevalent at the reporting centre. This holds particularly true for the prenatal diagnosis of congenital heart disease where detection rates ranging from 0 per cent to 60 per cent are being reported. On the other hand, congenital heart disease affects about 4-8 per 1000 live births and is a leading cause of infant mortality, whereas prenatal diagnosis could possibly prevent death and long-term morbidity in some of these neonates. Various screening concepts for more effective detection of congenital heart diseases are analysed in this article, including the more recent technique of early echocardiography between 13 and 15 weeks of gestation. High-risk groups are defined and the group of fetuses with increased thickness of nuchal translucency seems to be of particular interest.

Echocardiography↗
PubMed1990

[Prenatal diagnosis of congenital heart diseases and cardiac arrhythmias by Doppler echocardiography].

PURPOSE: To detect in prenatal life fetal arrhythmia and congenital heart disease. We performed fetal echocardiography following classical indications according to the literature. PATIENTS AND METHODS: Two-dimensional echocardiography associated with M-mode and Doppler was performed in 200 patients according to the indications: maternal diabetes, fetal arrhythmia, maternal congenital heart disease, previous fetus with cardiac defect, intrauterine growth retardation, nonimmune fetal hydrops, rubeola, isoimmunization, fetal malformation by ultrasound, polyhydramnios, gemelarity, oligohydramnios, maternal ingestion of drugs, lupus and others. RESULTS: Structural abnormalities of the heart were found in 6 cases and associated with bradiarrhythmia in 5 cases (total of 5.5%). Fetal arrhythmias without cardiac malformation were found in 35 cases (17.5%) and premature atrial contractions were the most frequent. CONCLUSION: The findings in this study indicate that it is possible the accurate definition of the cardiac anatomy and function. We have emphasized the method trying to spread it, objecting to improve assistance to the fetus and newborn with arrhythmias and/or congenital heart disease.

Arrhythmias, Cardiac↗