By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle.

Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect.

The operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia.

Surgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.

Postconditioning by brief episodes of ischaemia performed just at the time of reperfusion have been shown to reduce the size of infarcts in animal models, and in the clinical setting of percutaneous cardiac intervention. The clinical applicability of postconditioning in cardiac surgery remains to be determined. We investigated the effect of postconditioning on myocardial protection in children undergoing cardiac surgery.

We randomly assigned 40 patients scheduled for surgical correction of congenitally malformed hearts under cold blood cardioplegic arrest to postconditioning or control treatment. Postconditioning was performed by two cycles of 30 seconds ischaemia and 30 seconds reperfusion using aortic reclamping, and declamping started 30 seconds after cardioplegic arrest. We assayed creatine kinase-MB, troponin I, transcardiac release of lactate and neutrophil counts.

The types of procedure, age, bypass and aortic cross-clamping times were similar in both groups. The postoperative peaks of creatine kinase-MB and troponin I were lower after aortic de-clamping in the postconditioned patients compared with their controls (128 ± 48 units per liter as opposed to 199 ± 79 units per liter, p = 0.016, and 0.34 ± 0.21 nanograms per milliliter as opposed to 0.61 ± 0.53 nanograms per milliliter, p = 0.05), with reduced inotropic scores in those submitted to postconditioning compared with their controls (4.8 ± 3.1 versus 2.3 ± 1.5, p = 0.036). Transcardiac release of lactate was reduced in the postconditioned patients compared with their controls (0.10 ± 0.27 as opposed to 0.37 ± 0.43 millimols per liter, p = 0.048). No differences between groups were found for transcardiac neutrophil count during reperfusion (10.8 ± 6.3% for postconditioning versus 14.0 ± 8.7% for controls, p = 0.48).

Our study demonstrates that postconditioning may protect the myocardium of children undergoing cold blood cardioplegic arrest. These data support the need for a larger clinical trial of postconditiong in children undergoing cardiac surgery.

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.

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.

Many studies have suggested that more women then men present with physical symptoms. There is no data available, however, on the differences in reporting of physical symptoms between teenage male and female athletes. Our objective was to evaluate the differences according to gender in physical symptoms in healthy teenage athletes.

A total of 1,465 high school athletes, between the ages of 13 and 19 years participated in a mass echocardiographic screening programme for detection of cardiac abnormalities. Screening was conducted using a hand-carried cardiac ultrasound device (OptiGo, Philips). All participants were actively involved in a high school sport programme. Each athlete was required to fill out a questionnaire before the screening. The athletes were asked to report the occurrence of physical symptoms with activity or exercise. A physical examination was not performed during screening.

There were 1,031 (70.4%) male and 434 (29.6%) female participants. Significantly more female teenage athletes reported physical symptoms (190/434, 43.8% versus 267/1,031, 25.9%, odds ratio: 2.28, confidence interval: 1.76–2.81, p less than 0.001). Symptoms did not correlate with any echocardiographically identified cardiac abnormalities in either gender. The differences in the reporting of symptoms were significant for all physical symptoms addressed by the questionnaire.

There is a high prevalence of reporting physical symptoms in young healthy athletes without any relation to cardiac abnormalities. Young female athletes report physical symptoms nearly twice as often as their male counterparts.

Cardiac anomalies are among the most frequent congenital malformations, but the basic underlying causes for most cardiac defects remains undetermined. Some 40 years ago, a higher incidence of blood group B was reported in a small number of African-American children with congenital cardiac defects. In this study, we sought to re-evaluate this association using a larger population.

We collected data from 1985 patients undergoing cardiac surgery from July, 2000, through December, 2004. We divided the patients into 6 subgroups according to their diagnosis. We then compared the prevalence of ABO phenotypes between the patients and the general population of the United States of America by chi-square analysis. There were no significant differences in the distribution of the ABO phenotypes amongst the subgroups of those with congenital cardiac disease, or any for subgroup compared to the general population.

While statistical significance is influenced by the size of the population within the United States of America and the small numbers within each of our subgroups of patients with congenital cardiac disease, we have been unable to show any relationship between the distribution of ABO phenotypes and the existence of congenital cardiac disease.

To connect the hepatic veins to the azygos venous system through a lateral thoracotomy, and without the use of extracorporeal circulation, so as to relieve arteriovenous fistulas after a previous Kawashima operation.

Description of the operative technique by which the hepatic veins are anastomosed to the hepatic venous system.

The surgical approach was successfully applied in 3 patients, all of whom showed an excellent rise of saturations of oxygen after redirection of the hepatic venous blood.

The operative method presented is an elegant means of redirecting the hepatic venous blood to the pulmonary circulation without the disadvantages of extracorporeal circulation and resternotomy.

Paediatric cardiac services are poorly developed or totally absent in underdeveloped countries. Institutions, foundations and interested individuals in those nations in which sophisticated paediatric cardiac surgery is practised have the ability to alleviate this problem by sponsoring paediatric cardio-surgical missions to provide care, and train local caregivers in developing, transitional, and third world countries. The ultimate benefit of such a programme is to improve the surgical abilities of the host institution. The purpose of this report is to present the impact of our programme over a period of 14 years.

We specifically reviewed our database of patients from our missions, our team lists, surgical results, and the number and type of personnel trained in the institutions that we have assisted. In order for the institution to be entered into the study, the foundation had to provide at least 2 months of training. In addition, the institution had to respond to a simple questionnaire concerning the number and types of surgery performed at their facility before and after intervention by the foundation.

We made 140 trips to 27 institutions in 19 countries, with 12 of the visited institutions qualifying for inclusion. Of these, 9 institutions reported an increase in the number and complexity of cases currently being performed in their facility since the team intervened. This goal had not been accomplished in 3 institutions. The reasons for failure included the economic situation of the country, hospital and national politics, personality conflicts, and continued lack of hardware and disposables.

Paediatric cardiac service assistance can improve local services. A significant commitment is required by all parties involved.

In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called “Perfect Fontan” operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a “perfect” outcome is routinely achievable in the current era when using a standardized surgical procedure.

Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation.

An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a “perfect” outcome. Freedom from replacement or revision of the tube was 97% at 10 years.

Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the “perfect” outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.

We report the multicentric French experience with transcatheter closure in children weighing 15 kilograms or less, with the aim of assessing the efficacy of the procedure in this age group.

We included all children weighing 15 kilograms or less, and seen between January, 1997, and June, 2004, who had successful transcatheter closure of an interatrial communication within the oval fossa.

Transcatheter closure was performed in 35 patients weighing 15 kilograms or less, of whom 14 were male and 21 female. The procedures were undertaken in 8 different centres, the patients having a median age of 3 years, with a range from zero to 6.2 years, and a mean weight of 13 kilograms, with a range from 3.6 to 15 kilograms. All the patients were symptomatic, with associated cardiac malformations present in 4 cases, and extracardiac anomalies in 4 patients, including Down’s syndrome in 3, and Adams Oliver syndrome in the other case. In 1 patient, emergency cardiac surgery was needed 24 h after the procedure to correct a previously undiagnosed divided right atrium. No other complication occurred. After a median follow-up of 2 years, with a range from 0.5 to 5.2 years, all the patients are asymptomatic, except for one long-standing patient with bronchodysplasia. In 1 other patient, a small residual bidirectional shunt was detected by echocardiography. No patient presented significant arrhythmia. In the patients followed-up for more than 12 months, we found a significant gain in weight gain.

Transcatheter closure of an interatrial communication within the oval fossa is efficient in children weighing 15 kilograms or less, and can be proposed as a first line of treatment in symptomatic patients. Children with retarded growth tend to have complete recovery within one year of closure.