We investigated the use of the bispectral index for monitoring sedation during cardiac catheterization. The scores for the bispectral index may not reflect correct values in children, but may be helpful during titration of sedatives such as midazolam and ketamine.

We conducted a prospective randomized clinical trial in 126 patients scheduled for cardiac catheterization in a teaching hospital. They ranged in age from 4 months to 15 years. In 66 patients, sedation was performed without use of the bispectral index, while the index was used in the other 60 patients. The data collected included heart rate, mean arterial pressure, respiratory rate, saturation of oxygen, amount of sedatives, awakening time and adverse effects. We subdivided the patients into age-related groups for each parameter.

The demographic data were not statistically different. Monitoring with the bispectral index in those aged from 1 to 3 years revealed use of lower doses of midazolam, at 2.09 mg per kg per hr, with standard deviation of 0.36, and similarly lowers doses of ketamine, at 2.07 mg per kg per hr, with standard deviation of 0.22, the values in those not monitored being 2.93, with standard deviation of 0.45, and 2.96 with standard deviation of 0.51 respectively, these difference being statistically significant (p = 0.001 and p = 0.04, respectively). In those aged from 3 to 6 years of age, dosage of midazolam was 2.09, with deviation of 0.36, and of ketamine 1.78, with deviation of 0.27, following use of the bispectral index, compared to 2.89 with deviation of 0.28, and 2.62 with deviation of 0.69 respectively, when the bispectral index was not used, these again being significant differences (p = 0.033 and p = 0.04). The requirements for respiratory support and adverse effects were also significantly lower when using the bispectral index (p less than 0.05). No significant difference was found regarding dosages at the ages of 4 months to 1 year, and 6 to 15 years. The awakening time, however, was shorter with use of the index in those aged from 1 to 6 years.

When using the bispectral index for monitoring sedation during catheterization in children, we noted decreased need for doses of midazolam and ketamine, a lower need for respiratory support and less adverse effects.

To evaluate echocardiographically the function and morphology of the right heart subsequent to transcatheter closure of atrial septal defects.

We performed echocardiographic studies in 73 patients undergoing transcatheter closure of atrial septal defects 1 day prior to closure, and then 3 days and 3 months after closure. We calculated the antero-posterior diameter of the right ventricle, the transverse diameter and length of the right atrium, the pulmonary arterial pressure, and the velocities of systolic movement and early and late diastolic movement of the basal parietal walls of the right ventricle.

The atrial septal defects varied in size between 8 and 33 millimetres, and were closed using occluders measuring from 10 to 40 millimetres. At 3 days after closure, the antero-posterior diameter of the right ventricle, the transverse diameter and length of the right atrium, the pulmonary arterial pressure, and the velocities of mural motion were all significantly decreased. After 3 months, the size of the right heart had more or less normalized.

Transcatheter closure of atrial septal defects produces marked improvement in the function and geometry of the chambers of the right heart, reducing pulmonary arterial pressure as well as abolishing the interatrial shunt.

We sought to provide a new method for quantifying collateral aortopulmonary flow in patients subsequent to construction of a bidirectional cavopulmonary shunt, and to clarify the clinical advantages of the new method.

We performed lung perfusion scintigraphy and cardiac catheterization in 10 patients subsequent to construction of a bidirectional cavopulmonary shunt. First, the ratio of collateral to systemic flow was determined by whole-body images of lung perfusion scintigraphy, dividing the total lung count by the total body count minus the total lung count. Second, we integrated lung perfusion scintigraphy and cardiac catheterization data using a formula derived from the Fick principle, taking the ratio of pulmonary to systemic flow to be 1 plus the ratio calculated above and multiplied by the systemic saturation minus the inferior caval venous saturation divided by the pulmonary venous saturation minus the inferior caval venous saturation. Finally, the amount of collateral flow was obtained from the ratio of pulmonary to systemic flow. We evaluated the impact of collateral flow on the calculation of pulmonary vascular resistance.

The median age at bidirectional cavopulmonary shunt was 1.41 years, and the median age at catheterization was 2.33 years. The mean amount of collateral flow was 1.75 ± 0.46 litres/min/m2. The pulmonary vascular resistance calculated without considering the collateral flow was overestimated by an average of 57 ± 23%, compared to the resistance calculated with our new method.

The use of scintigraphy combined with catheterization allows accurate determination of aortopulmonary collateral flow, and avoids overestimation of pulmonary vascular resistance in these candidates for the Fontan circulation.

The prevalence of mitral valvar prolapse has been reported to be between 0.6 and 21%. The goal of our study was to evaluate its prevalence in young athletes who underwent hand-held echocardiography as a screening mostly in southern California.

We retrospectively analyzed 1742 echocardiograms that were performed as a part of a cardiac screening of teenage athletes. The total prevalence of mitral valvar prolapse was calculated and stratified based on gender.

We screened a total of 1172 male and 570 female high school athletes. The echocardiographic prevalence of mitral valvar prolapse was 0.9%. The prevalence was similar in both genders, at 1.2% in male and 0.7% in female athletes.

The prevalence of mitral valvar prolapse in young athletes mostly in southern California was found to be less than 1%, and was similar in both genders.

Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in whom the anomalous artery originated from the pulmonary trunk. This technique, however, is applicable only when the anomalous artery arises from the right posterior pulmonary sinus. We report a new technique for re-implantation using combined autogenous aortic and pulmonary arterial flaps in situations when a direct connection was not possible.

We have treated 4 patients, aged 3 months, 6 months, 18 months, and 27 years respectively, who presented with anomalous origin of the left coronary artery from the left posterior pulmonary sinus. We used our proposed technique for transfer because lack of coronary arterial length, diminished vessel elasticity, and extensive collaterals around the pulmonary sinuses prevented direct attachment.

There was no early or late death. Postoperatively, all patients are in functional class I, with good biventricular function at a median follow-up of 74 months, with a range from 9 to 96 months. Postoperative coronary angiography in our 4th patient showed good arterial flow, without any distortion.

The potential benefits of this modification of the trapdoor technique are excellent operative exposure, use of autogenous and viable tissue capable of further growth, avoidance of injury to the aortic and pulmonary valvar apparatus and production of obstruction within the right ventricular outflow tract, complete elimination of use of pericardium for augmentation of the neo-aortic tube, achievement of the anastomosis with correct angling and length, and the possibility of implantation in all patients, including adults, regardless of the distance from the aorta or the coronary arterial configuration.

Infants with congenitally malformed hearts who require early open-heart surgery are at high risk for developmental, psychosocial, and academic difficulties. Our objective was to describe the pattern of use of educational supports and rehabilitation services in these children at early school age.

Parents of children who participated in a prospective study of developmental progress following open-surgery were contacted to participate in a telephone survey. The questionnaire included questions regarding current educational and rehabilitation resources their child was receiving, as well as the needs perceived by the parents for services, and obstacles to accessing services.

The survey was completed by 60 families, the mean age of the children being 8.1 years, with standard deviation of 1.1 years. Of the children, 22% received educational supports, which primarily included supplemental tutoring. Rehabilitation services were received by 23%, speech therapy for 9 children, psychologic support for 6, occupational therapy for 3, and physical therapy for 1. Children receiving these services were significantly more likely to have had low developmental scores in the expected domains, when compared to those not receiving services. The majority of developmentally delayed children were not receiving adequate, if any, resource support. Medical and surgical history was not associated with greater likelihood of receipt of services.

Children with congenitally malformed hearts who are now of school age are at risk for developmental challenges and academic difficulties, yet many do not receive services to optimize performance. Modification of current practice to include systematic, periodic screening, as well as the availability of a resource person for information and referral, may be warranted to meet the ongoing needs of these children and their families, and to optimize their health and well-being.

One of the most common uses of stents in patients with congenitally malformed hearts is treatment of pulmonary arterial stenosis. Although there are reports of fractured pulmonary arterial stents, little is known about the risk factors for, and implications of, such fractures.

We reviewed angiograms to identify fractures in stents previously inserted to relieve stenoses in pulmonary arteries from 1990 through 2001 in patients who also underwent follow-up catheterization at least 3 years after placement of the stent. We undertook matched cohort analysis, matching a ratio of 2 fractured to 1 unfractured stent.

Overall, 166 stents meeting the criterions of our study had been placed in 120 patients. We identified fractures in 35 stents (21%) in 29 patients. All fractured stents were in the central pulmonary arteries, 24 (69%) in the central part of the right pulmonary artery, and all were complete axial fractures, or complex fractures along at least 2 planes. Stent-related factors associated with increased risk of fracture identified by multivariable logistic regression included placement in close apposition to the ascending aorta (p = 0.001), and a larger expanded diameter (p = 0.002). There was obstruction across 28 of 35 fractured stents, which was severe in 11. We re-stented 21 of the fractured stents, and recurrent fracture was later diagnosed in 3 of these. A fragment of the fractured stent embolized distally in 2 patients, without clinically important effects.

In situ fracture of pulmonary arterial stents is relatively common, and in most cases is related to compression by the aorta. There is usually recurrent obstruction across the fractured stent, but fractured stents rarely embolize, and are not associated with other significant complications.

To identify the presence of ventricular diastolic dysfunction by tissue Doppler in fetuses of diabetic mothers, with or without septal hypertrophy, in comparison to fetuses of nondiabetic mothers.

A contemporary transverse study in fetuses with a gestational age between 25 weeks to term, studying diastolic function by assessment using tissue Doppler and pulsed wave Doppler of the atrioventricular diastolic flow. The mothers of the fetuses all had previous or gestational diabetes, and were referred to the Fetal Cardiology Unit of the Institute of Cardiology in Porto Alegre, Brazil. We analysed variance with the Student-Neumann-Keuls post hoc test. An alfa of 0.05 was considered significant for statistical analysis.

The mean myocardial velocities of the E′and A′ waves at the mural mitral annulus, in fetuses of diabetic mothers with myocardial hypertrophy, were, respectively, 7.00 plus or minus 1.6 centimetres per second, and 10.24 plus or minus 3.3 centimetres per second. In the fetuses of diabetic mothers group without myocardial hypertrophy, the comparable values were 7.19 plus or minus 2.4 centimetres per second and 10.77 plus or minus 3.77 centimetres per second, respectively. In the control group, they were 4.81 plus or minus 0.85 centimetres per second and 8.01 plus or minus 2.2 centimetres per second. The difference between the velocities in fetuses of diabetic mothers and in fetal normal mothers was statistically significant (p less than 0.05). Statistically significant differences were also observed in E′ and A′ diastolic waves at the aortic mitral annulus, as well as for the tricuspid annulus when tissue Doppler assessment was carried out in the same sample. The mean ratio between the E and E′ of mitral and tricuspid waves in the control fetuses of normal mothers was significantly higher than in fetuses of diabetic mothers.

Pulsed tissue Doppler, when used in fetuses of diabetic mothers and compared with fetuses of nondiabetic mothers, shows evidence of impaired diastolic function, independently of the presence of myocardial hypertrophy.

The purpose was to examine the relationship between the complexity of structural heart disease and psychological well-being in adults with congenital cardiac disease.

A total of 380 patients registered at the Adult Congenital Heart Clinic in Calgary, Alberta, Canada were surveyed. The patients were mailed a socio-demographic questionnaire and an instrument to measure psychological well-being. The instrument of psychological well-being measures six dimensions: positive relations with others, autonomy, environmental mastery, personal growth, purpose in life, and self-acceptance. Of the 380 patients surveyed, 85 of 205 patients with simple structural malformation responded, giving a response rate of 41%, and 78 of 175 patients with complex malformations, thus giving a response of 45%.

There was no statistically significant difference in the mean scores of each of the six dimensions of psychological well-being between those patients with simple and complex malformations. Two-way analysis of variance, and multivariate analysis of variance, found significant differences in mean scores for dimensions when other socio-demographic variables were included in the analysis. For example, significantly higher mean scores for the dimension of purpose in life was found in patients who obtained a higher level of education (p = 0.009), and in patients who were employed (p < 0.001). We present the socio-demographic variables that statistically impact the mean scores for the dimension of psychological well-being.

Psychological well-being is not affected by the complexity of the structural congenital cardiac disease. Certain socio-demographic variables that impact psychological well-being, nonetheless, must be considered when developing multidisciplinary programmes to care for young adults with congenitally malformed hearts.

To assess neurohormonal activation of cardiac failure in adults with congenitally corrected transposition, and to determine the most sensitive marker for recognition of the cardiac failure.

The onset of morphologically right ventricular dysfunction is unpredictable in patients with congenitally corrected transposition, the combination of discordant atrioventricular and ventriculo-arterial connections, and its markers are unknown.

We measured amino terminal pro brain natriuretic peptide in 19 patients, aged 35 plus or minus 13.1 years, and in 19 control subjects. Morhologically right ventricular function was assessed by echocardiography, including tissue Doppler echocardiography and magnetic resonance imaging or multislice computed tomography.

The patients showed a highly significant increase in the levels of amino terminal pro brain natriuretic peptide, the levels being significantly elevated even in asymptomatic patients. Left atrial dimensions were larger in patients, and significantly lower tissue Doppler echocardiographic velocities were measured at the lateral site of the tricuspid annulus and at the basal segment of the interventricular septum. The ejection fraction of the morphologically right ventricle correlated significantly with the levels of brain natriuretic peptide, and with left atrial dimensions.

Neurohormonal activation is present in patients with congenitally corrected transposition even when they are asymptomatic. It is correlated with left atrial dimensions and tissue Doppler echocardiographic parameters. Levels of brain natriuretic peptide, and peak tricuspid early diastolic annular velocity, are the earliest and most sensitive markers of morphologically right ventricular dysfunction.

The modification of placing the shunt from the right ventricle to the pulmonary arteries, also known as Sano procedure, has allegedly improved results over the short term in surgical palliation of hypoplastic left heart syndrome with the Norwood procedure. With this in mind, we reviewed autopsied specimens from neonates and children who did not survive after either a classic arterio-pulmonary shunt, or the modified procedure with the shunt placed from the right ventricle to the pulmonary arteries, so as to evaluate the pathological substrates of the remodelling of the systemic right ventricle, assessing any differences induced by the 2 techniques.

We obtained the hearts from 11 patients with neonatal diagnosis of hypoplastic left heart syndrome who died after the first or second stages of the Norwood sequence of operations, comparing them with 6 normal hearts matched for age and weight. Macroscopic, microscopic and morphometric analysis were performed on each specimen, evaluating the diameter of the myocytes, extracellular matrix remodelling in terms of fibrosis and type of collagen, and vascularization in terms of capillary density.

Hypertrophy of the myocytes was significantly increased in the hearts from patients having either a classic arterio-pulmonary or the ventriculo-pulmonary modification of the shunt compared to controls (p < 0.05). Myocardial fibrosis was increased in those having a shunt placed from the right ventricle to the pulmonary arteries when compared to the other 2 groups. The ratio of collagen I to collagen III was similar in those undergoing a classic arterio-pulmonary shunt compared to controls (0.94), but was lower in those having a shunt placed from the right ventricle to the pulmonary arteries (0.61), with an increase in collagen type III. The density of capillaries was lower in those who had undergone a classic arterial shunt when compared to the others.

We have shown greater remodelling of the ventricular myocardial extracellular matrix in patients having a shunt from the right ventricle to the pulmonary arteries when compared to those having a classic arterio-pulmonary shunt, with this remodelling progressing even after the neonatal period. This may influence a later suboptimal ventricular performance.

The strategies of repair of tetralogy of Fallot change with the age of patients. In children older than 4 years and adults, the optimal strategy may be to use different method of reconstruction of the right ventricular outflow tract from those followed in younger children, so as to avoid, or reduce, the pulmonary insufficiency that is increasingly known to compromise right ventricular function.

From April, 2001, through May, 2008, we undertook complete repair in 312 patients, 180 male and 132 female, with a mean age of 11.3 years ±0.4 years, and a range from 4 to 48 years, with typical clinical and morphological features of tetralogy of Fallot, including 42 patients with the ventriculo-arterial connection of double outlet right ventricle. The operation was performed under moderate hypothermia using blood cardioplegia. The ventricular septal defect was closed with a Dacron patch. When it was considered necessary to resect the musculature within the right ventricular outflow tract, or perform pulmonary valvotomy, we sought to preserve the function of the pulmonary valve by protecting as far as possible the native leaflets, or creating a folded monocusp of autologous pericardium.

The repair was achieved completely through right atrium in 192, through the right ventricular outflow tract in 83, and through the right atrium, the outflow tract, and the pulmonary trunk in 36 patients. A transjunctional patch was inserted in 169 patients, non-valved in all but 9. There were no differences regarding the periods of aortic cross-clamping or cardiopulmonary bypass. Of the patients, 5 died (1.6%), with no influence noted for the transjunctional patch. Of those having a non-valved patch inserted, three-tenths had pulmonary regurgitation of various degree, while those having a valved patch had minimal pulmonary insufficiency and good right ventricular function postoperatively, this being maintained after follow-up of 8 to 24-months.

Based on our experience, we suggest that the current strategy of repair of tetralogy of Fallot in older children and adults should be based on minimizing the insertion of transjunctional patches, this being indicated only in those with very small ventriculo-pulmonary junctions. If such a patch is necessary, then steps should be taken to preserve the function of the pulmonary valve.