To report our experience with balloon dilation of critical aortic valvar stenosis in neonates via the umbilical artery using currently available catheters.

There is no agreement regarding the optimal vascular approach for balloon dilation of critical aortic valvar stenosis in neonates.

Since June of 2005, we have attempted to obtain umbilical arterial access within the first week after birth in all neonates with critical aortic valvar stenosis. In patients in whom umbilical artery access was obtained, we proceeded with an attempt at balloon dilation.

We were presented with 5 patients with critical aortic valvar stenosis within the first week after birth, and the umbilical arterial approach was obtained in all, with effective relief of the stenosis achieved in 4.

The umbilical arterial approach should always be considered for balloon dilation of neonatal critical aortic valvar stenosis. Using currently available catheters, the procedure is safe, simple, and effective even in patients weighing less than 2.5 kilograms. Further experience using this approach is warranted.

To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia.

In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management.

We carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings.

Of 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced.

The patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.

We report the experience in a single institution with balloon aortic valvoplasty for congenital aortic stenosis. Unlike most other reported series, we included patients with associated lesions involving the left side of the heart.

Between November, 1986, and November, 2006, we performed 161 interventions on 143 patients, of whom 33 were neonates, 33 infants, and 77 children, just over one-quarter (28.6%) having associated lesions.

The overall reduction in peak-to-peak gradient of 60 ± 24% (p < 0.01), was more effective in primary versus secondary intervention (63 ± 24% versus 47 ± 23%; p < 0.03), and in those with fused bifoliate as opposed to truly bifoliate valves (66 ± 17% versus 53 ± 30%; p = 0.01). Patients with associated lesions were younger (40.89 ± 60.92 months versus 81.9 ± 72.9 months; p = 0.001), and were less likely to achieve a final pressure gradient of less than 20 mmHg (35.0% versus 61.2%; p < 0.01). Overall mortality was higher in cases with associated lesions (27.5% versus 1.9%; p < 0.0001) but not catheter-related death (2.5% versus 1.9%; p = 1.0). Reintervention was more frequently required in infants (p = 0.02) but not in cases with associated lesions (p = 0.35).

Balloon valvoplasty is a safe and effective method for the treatment of congenital aortic stenosis. Prior surgery to the aortic valve, reintervention, associated cardiovascular lesions, and the anatomy of the valve predict a less effective reduction in the gradient. Major complications and catheterization-related death are mainly secondary to very young age, but not to associated cardiac lesions.

The so-called Gerbode ventriculo-atrial defect is a rare defect that permits shunting from the left ventricle to the right atrium. It takes 2 forms, either a deficiency of the atrioventricular membranous septum, or shunting initially through a ventricular septal defect, with atrial shunting occurring through a deficiency in the septal leaflet of the tricuspid valve. In this review, we describe the natural history and outcomes of surgical repair for the variant with shunting through a deficiency at the site of the atrioventricular membranous septum.

From 1990 to 2008, we identified 6 patients from our departmental database who had undergone surgical closure of a congenital defect of the atrioventricular component of the membranous septum. Median age at repair was 1.6 years, with a range, from 0.4 to 19 years. All patients were symptomatic, with 3 having congestive cardiac failure, 2 failing to thrive, and 2 having intolerance to exercise. All had a dilated right atrium demonstrated by echocardiogram, with a mean preoperative gradient measured by echocardiogram to be 109 millimetres of mercury, with a range from 65 to 150 millimetres of mercury.

There was no operative or late mortality. The mean size of the defect was 6.2 ± 2.0 millimetres, with a range from 4 to 8 millimetres. All were closed by insertion of a patch. The mean period of cardiopulmonary bypass was 90.5 ± 11.3 minutes, the mean time of aortic cross-clamping 54.8 ± 6.9 minutes, and the mean length of stay in hospital 4.3 ± 1.0 days. No patient had a residual defect, and only trivial regurgitation of the tricuspid valve was evident by postoperative echocardiography. There were no complications or reoperations.

The membranous ventriculo-atrial defect can be recognized echocardiographically on the basis of dilation of the right atrium in the setting of an unusually high Doppler echocardiogram gradient compared to the ventricular septal defect with shunting only at ventricular level. Since all patients in our series were symptomatic, we recommend surgical closure of all these defects.

To evaluate the diagnosis, clinical features, management and post-natal follow-up in consecutive fetuses identified with tachycardia.

We reviewed consecutive fetuses with tachycardia identified in a single tertiary institution between January, 2001, and December, 2008. We considered several options for management, including no treatment but close surveillance, trans-placental antiarrhythmic therapy in fetuses presenting prior to 36 weeks of gestation, and delivery and treatment as a neonate for fetuses presenting after 36 weeks of gestation. Data was gathered by a review of prenatal and postnatal documentation.

Among 29 fetuses with tachycardia, 21 had supraventricular tachycardia with 1 to 1 conduction, 4 had atrial flutter, 3 had atrial tachycardia, while the remaining fetus had ventricular tachycardia. Of the group, 8 fetuses (27.6%) were hydropic. Transplacental administration of antiarrhythmic drugs was used in just over half the fetuses, delivery and treatment as a neonate in one-quarter, and no intervention but close surveillance in one-sixth of the case. Twenty-six of 29 fetuses (89.7%) were born alive. Only patients with fetal hydrops suffered mortality, with 37.5% of this group dying, this being statistically significant, with the value of p equal to 0.03, when compared to non-hydropic fetuses. Only 3 patients (11.5%) were receiving antiarrhythmic prophylaxis beyond the first year of life.

A significant proportion of fetal tachycardias recognized before 36 weeks of gestation can be treated successfully by transplacental administration of antiarrhythmic drugs. Fetuses presenting after 36 weeks of gestation can be effectively managed postnatally. The long-term prognosis for fetuses diagnosed with tachycardia is excellent, with the abnormal rhythm resolving spontaneously during the first year of life in most of them.

To assess the outcomes in children and adolescents with rheumatic fever of the implantation of mechanical as opposed to biological heart valves.

We assessed 73 patients with rheumatic heart disease under the age of 18 years, who underwent replacement of heart valves between January, 1996, and December, 2005, at the National Institute of Cardiology in Rio de Janeiro, Brazil. Of the group, 71 patients survived, and were divided into a group of 52 receiving mechanical prostheses, and 19 with biological prostheses. We compared endpoints between the groups in terms of mortality, reoperation, haemorrhage, and stroke. Survival curves were estimated using the Kaplan-Meier method and were compared by the Mantel (log-rank) test.

Overall mortality was 8.2%. In those receiving mechanical prostheses, 2 (3.8%) patients died, 5 (9.6%) underwent reoperation, 2 (3.8%) suffered severe haemorrhage, and 3 (5.8%) had strokes. In those receiving biological valves, 2 (10.5%) patients died, and 4 (21%) underwent reoperation. After 2, 4, and 8 years, overall survival was 96%, 93% and 86%, respectively, with a borderline difference between the groups (p = 0.06). The probabilities of remaining free from reoperation (p = 0.13), and from combined endpoints, showed no statistically significant difference between the groups (p = 0.28).

Patients with mechanical prostheses had lower mortality and required fewer reoperations, but when all combined endpoints were considered, the groups did not differ. The biological prosthesis proved to be a good option for cardiac surgery in children and adolescents with difficulties or risks of anticoagulation.

to analyse retrospectively the data of fetuses diagnosed with isolated complete atrioventricular block and efficacy of treatment of the fetus by maternal therapy.

Between 1992 and 2004, we diagnosed complete atrioventricular block in 26 singleton and 2 twins fetuses of 27 pregnant women known to have anti Ro/La antibodies, 11 with autoimmune disease, one patient analysed in 2 pregnancies. At presentation, 20 of the fetuses were compensated and non-hydropic, while 8 had hydrops. Twenty patients were treated with dexamethasone, 2 with associated salbutamol and one mother with isoproterenol.

Age at presentation was not different between the hydropic and non-hydropic fetuses. The fetuses with hydrops, however, had a lower mean heart rate at presentation, 48.5 ± 9.25 with a range from 32 to 60, compared to 59.95 ± 7.9 beats per minute, with a range from 50 to 80, in the non-hydropic fetuses (p less than 0.002). Equally, after birth the mean heart rate in hydropic fetuses was 42.6 ± 5.1, with a range from 38 to50, as opposed to 56.05 ± 11.8 beats per minute, with a range from 29 to 110, in the non-hydropic fetuses (p less than 0.015), The hydropic fetuses were delivered at 31.7 ± 3.8 weeks’ gestation, with a range from 29 to 38 weeks (p less than 0.003) compared to 35.5 weeks’ gestation ±2.04, with a range from 31 to 38, in the non-hydropic fetuses. Mortality was 37.5% in the hydropic fetuses, versus 5% of those without hydrops (p less than 0.02). Pacemakers were implanted in 22 of 26 infants born alive, at a median of 45 days, with a range from 1 day to 5 years, in those without hydrops during fetal life, and 3 days, with a range from 1 day to 8 months in those afflicted by hydrops, of whom 2 died despite the implant of the pacemaker. The presence and degree of hydrops had a significantly negative predictive value. No significant differences were observed between the treated and non treated cases, albeit that administration of steroids ameliorated rapidly the hydrops in 3 of 5 cases.

The outcome in our cases was mainly dependent on the presence and degree of fetal cardiac failure. Treatment of the fetus by maternal administration of steroids did not result in any regression of the conduction disorder, but had a favourable effect on fetal hydrops.

Echocardiographic measurements are widely used as outcomes of different studies. The aim of this study was to assess intraobserver and interobserver reliability of echocardiographic measurements in healthy children.

We studied 28 children, with a median age of 7.5 years, and inter-quartile range from 3 to 11 years. Intraobserver and interobserver reliability were assessed by repeated measurements of the diameters of the aortic root, the left atrium, and left ventricular end-diastolic structure. We also measured the ventricular end-diastolic septal thickness and the end-diastolic thickness of the left ventricular posterior wall. We calculated intraclass correlation coefficients, with corresponding 95% confidence intervals, and computed Bland and Altman plots, permitting us to derive limits of agreement plus or minus 2 standard deviations for the mean differences in cardiac measurements.

We found high intraobserver and interobserver intraclass correlation coefficient, ranging from 0.91 for ventricular septal thickness, with 95% confidence intervals from 0.78 to 0.96, to 0.99 for the diameter of the aortic root, 95% confidence interval from 0.97 to 1.00. Limits of agreement in the Bland and Altman plots ranged from zero millimetres for left ventricular end-diastolic posterior wall thickness to 1.60 millimeters (6.3%) for left atrial diameter.

Our study demonstrated good repeatability and reproducibility for ultrasonic measurements of left cardiac structures in children, showing that values obtained for measurement of these structures in both clinical and epidemiological research projects can be confidently accepted.

Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables.

Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively.

We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively.

An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.