Background: Definition of normative data of the great arteries from neonatal to adult ages may aid in assessment of the growth of cardiovascular structures, thus guiding the timing and type of intervention in patients with congenital cardiac disease. Methods: We calculated the cross-sectional areas of the arterial roots at the basal attachment of the valvar leaflets, the sinuses, and standardized distal sites using cineangiograms of 59 normal children and adolescents with mean age of 5.4 plus or minus 4.7 years and a range from 0.1 to 16 years, the children having a mean weight of 21.2 plus or minus 15.7 kilograms, with a range from 2.2 to 68 kilograms, and mean height of 108 plus or minus 35 centimetres, with a range from 43 to 184 centimetres. Values at each site were calculated averaging end-diastolic and end-systolic measurements, and indexed to body surface area. Results are expressed as the mean plus or minus the standard deviation. Results: The diameter of the aortic root at the basal attachment of the leaflets was 249 plus or minus 26, the midpoint of the sinuses 379 plus or minus 59, the sinutubular junction 290 plus or minus 58, the isthmus 158 plus or minus 36, the postisthmic region 152 plus or minus 33, and the descending aorta at the level of diaphragm 130 plus or minus 18 millimetres squared per metre squared. The pulmonary root measured at the basal attachment of the leaflets was 253 plus or minus 28, the midpoint of the sinuses 352 plus or minus 58, the sinutubular junction 293 plus or minus 58, the right pulmonary artery 176 plus or minus 25, the left pulmonary artery 153 plus or minus 20, and sum of right and left pulmonary arteries 330 plus or minus 37 millimetres squared per metre squared. All indexes were consistent over a wide range for body surface areas. Conclusions: Definition of normative data of the great vessels may aid in the evaluation of congenital or acquired abnormalities, serving as guidelines for intervention during medical or surgical management and follow-up.

Traditionally, medical professionals have entrusted the parents of children with chronic illness with the task of imparting knowledge about the illness to the children. This practice assumes that parents understand the illness, and that they pass on their knowledge in a manner appropriate for the individual child. The aim of our study was to assess the knowledge that parents of children with a cardiac defect have about the malformation in the heart of their child. We sent a modified version of the Leuven questionnaire to 350 families. The questionnaire was filled in and sent back to our centre by 148 families. Parents showed a good knowledge of the name and anatomical characteristics of the cardiac disease suffered by their child. Parents with a child taking drugs were not very knowledgeable about the regime, side effects, and interaction with other drugs or food. Only one-quarter of the parents knew the definition of endocarditis, although almost two-fifths were aware that unexplained fever for more than 5 days was the most typical symptom. Less than half of the parents knew that endocarditis could recur. About two-fifths of parents knew the real possibility of their child being involved in competitive sports; but almost half of parents were unable to answer this question. The poor knowledge about particular aspects of the disease, treatment, and preventive measures revealed by our parents may have major consequences. The results of our study are relevant to general daily clinical practice.

Increasing numbers of children who have undergone intracardiac repair of tetralogy of Fallot have survived to adulthood, but often have residual haemodynamic and/or electrical abnormalities. We report the unusual observation of 4 instances of cerebrovascular accidents in young adults subsequent to surgical repair of tetralogy of Fallot.

Aims: Firstly to assess maternal perceptions of the impact of congenital or acquired cardiac disease on the child, parents, and siblings, and secondly to determine whether there were differences between different diagnostic groups, or between those with and without other health problems, with a view to informing the development of a cardiac liaison nursing service for children. Methods: A postal survey of 447 families of children with congenital or acquired cardiac disease. Results: Completed questionnaires were received from 209 (46.8 percent) families. The cardiac lesion was perceived to have a negative impact on many areas of family life for about one fifth of the sample, particularly in those families where the child was perceived to be more ill. Family relationships, however, were affected in a very different way, with 43 percent reporting that family members had become closer, and only 8 percent that they had been “pulled apart” by the condition of their child. There were a number of differences in the perceived impact of the cardiac malformation on school and family life between children with different diagnoses, with this being particularly evident for families of the patients who had undergone transplantation. When the sample was divided according to the presence or absence of other problems with health, however, many of these differences between the diagnostic groups disappeared. Conclusions: Irrespective of the severity of the disease, the presence of a cardiac malformation has an impact on everyday life for a significant number of children and families, particularly if associated with other problems with health. Implications for targeting resources to reduce morbidity in these children and families are discussed.

Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract.

The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve.

We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups.

The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of −1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of −0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle.

The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.

The aim of the study was to evaluate segmental and global right ventricular function at rest and during stress in patients having a systemic morphologically right ventricle. We studied 17 patients after atrial correction for transposition, 13 with congenitally corrected transposition, and 11 age-matched controls using cardiovascular magnetic resonance at rest and during stress with dobutamine given at 15 micrograms per kilogram per minute. Blood was drawn to obtain levels of brain natriuretic peptide. Right ventricular ejection fraction was calculated, and wall-motion and wall-thickening were assessed, in 4 segments of a midventricular slice. The findings for the systemic right ventricle were compared to the left ventricle in controls. Patients with transposition showed a decreased ejection fraction at rest (57 percent versus 69 percent, p equal to 0.005), decreased wall motion of the anterior, lateral and septal wall (p less than 0.01, p less than 0.01, and p less than 0.01) and decreased thickening of the lateral wall (p less than 0.01). Patients with congenitally corrected transposition showed normal ejection fraction, wall thickening, and wall motion at rest. During dobutamine stress, we found no significant differences in wall motion and thickening between the two groups. Ejection fraction, also increased to comparable values during stress, at 67 percent versus 66 percent, p not being significant. In both groups, we observed similarly increased levels of brain natriuretic peptide (p equal to 0.02 and 0.03, respectively). We conclude that only patients with transposition showed segmental wall motion and wall thickening abnormalities at rest. After dobutamine stress, however, segmental and global right ventricular dysfunction was similar in both groups.

Deteriorating ventricular function is a major concern after the Senning operation. A Doppler-derived non-geometric measurement, the so-called myocardial performance index, has been described for use in adults and children. We aimed to assess the utility of this index as a method for quantification of right ventricular function in patients in the late follow-up of the Senning procedure, and to correlate the right ventricular ejection fraction and the first derivative of right ventricular pressure as derived using echocardiography with the ejection fraction determined using magnetic resonance imaging.

We studied 44 patients within a mean postoperative period of 15.3 years. We calculated the right ventricular myocardial performance index by pulsed wave Doppler interrogation of tricuspid inflow and aortic outflow, the ejection fraction by Simpson's rule, and the first derivative of right ventricular pressure by continuous wave Doppler from tricuspid regurgitation.

Mean values of right ventricular myocardial performance index, ejection fraction, and the first derivative of right ventricular pressure were 0.50, 39 percent and 1,398 millimetres of mercury per second, respectively. A cut-off value of 0.47 for the right ventricle myocardial performance index was determined, with a sensitivity of 75 percent and a specificity of 62.5 percent. We found no correlation between ejection fraction and the first derivative of right ventricular pressure as estimated by echocardiography and the ejection fraction as shown by magnetic resonance imaging (r2 equal to 0.29 and 0.04 respectively).

We concluded, first, that patients with preserved right ventricular function had values for the right ventricular myocardial performance index lower than 0.47, and second, that ejection fraction and the first derivative of right ventricular pressure as determined echocardiographically did not correlate with values derived using magnetic resonance imaging.

Aims: To identify factors predisposing to abnormal left ventricular geometry and mechanics in 52 patients after successful repair of aortic coarctation. Methods and results: We evaluated left ventricular remodelling, systolic midwall mechanics, and isthmic gradient by echo-Doppler, systemic blood pressure at rest/exercise and by ambulatory blood pressure monitoring, and the aortic arch by magnetic resonance imaging. Echocardiographic findings were compared with those of 142 controls. The patients with aortic coarctation showed an increased indexed left ventricular end-diastolic volume, increased mass index, increased ratio of mass to volume and systolic chamber function. The contractility, estimated at midwall level, was increased in 21 percent of the patients. In 26 (50 percent) of the patients, we found abnormal left ventricular geometry, with 9 percent showing concentric remodelling, 33 percent eccentric hypertrophy, and 8 percent concentric hypertrophy. These patients were found to be older, underwent a later surgical repair, and to have higher systolic blood pressures at rest and exercise as well as during ambulatory monitoring. The relative mural thickness and mass index of the left ventricle showed a significant correlation with different variables on uni- and multivariate analysis. Age and diastolic blood pressure at rest are the only factors associated with abnormal left ventricular remodelling. Conclusions: Patients who have undergone a seemingly successful surgical repair of aortic coarctation may have persistently abnormal geometry with a hyperdynamic state of the left ventricle. This is more frequent in older patients, and in those with higher diastolic blood pressures.

Objective: To examine the current subjective state of health, and the longitudinal course of psychological well-being, in adult patients with congenital cardiac malformations. Methods: Our study concerns the second follow-up of a cohort of patients with congenital cardiac malformations. We examined 362 consecutive patients, aged from 20 to 46 years, who underwent surgical procedures for treatment of congenital cardiac disease between 1968 and 1980, specifically for treatment of atrial and ventricular septal defects, tetralogy of Fallot, transposition, and pulmonary stenosis. The patients were submitted to extensive medical and psychological examination. During psychological examination, in which all patients were seen by the same psychologist (EvR), patients filled in questionnaires concerning their current subjective state of health, using the 36-item Short Form Health Survey (SF-36), and psychological well-being, answering the Heart Patients Psychological Questionnaire. The data we acquired at their first follow-up was used to measure the longitudinal course of psychological well-being over the intervening period of 10 years. Results: When compared to a reference group, the patients assessed their state of health less favourably concerning physical functioning, but more favourable with regard to social functioning, bodily pain, and limitations of role due to emotional problems. Younger female patients reported more limitations of role due to physical functioning than did the female patients who were older. Patients with transposition showed a negative trend for their subjective state of health, but reported the least bodily pain. Within the overall group of patients, displeasure had increased, while social inhibition had decreased, over the intervening period of 10 years. Conclusion: Extra attention should be paid to the subjective experiences of young female patients with congenital cardiac disease. The patients with transposed arterial trunks seem overall to experience poorer physical health.

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions.

The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. Patients and methods: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. Results: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prologed intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessuful balloon dilation, underwent surgical reoperation. Conclusions: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.

We treated successfully using the Ross procedure a 14-year old with a congenitally stenotic bifoliate aortic valve associated with anomalous origin of the right coronary artery. The anomalous artery arose from the same aortic sinus that gave rise to the main stem of the left coronary artery, and reached the right atrioventricular groove by traversing the tissue plane between the aortic root and the subpulmonary infundibulum. Both coronary arteries were reimplanted using a single arterial button.

An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.

Aorto-left ventricular tunnels are anomalous communications between the ascending aorta and the left ventricle. Prenatal diagnosis, followed by successful surgical management, is rare. We describe a fetus diagnosed with aorto-left ventricular tunnel at 35 weeks who achieved a favourable outcome after immediate postnatal surgical repair.

We describe an infant with an atrioventricular septal defect and separate valvar orifices for the right and left ventricles, the so-called primum defect, who presented with serious pulmonary hypertension. Treatment with sildenafil was changed to intravenous epoprostenol due to lack of any measurable effects of the initial therapy as shown by echocardiography. The girl died three days after the change in treatment. We suggest that a mismatch between ventilation and perfusion contributed significantly to her death.

Cardiac involvement is a rare complication of infection by the influenza B virus. It usually presents with ventricular dysfunction, arrhythmias, or both. We report a 13-year-old boy with clinical, electrocardiographic, and laboratory findings of myocardial ischaemia during an otherwise silent acute infection with influenza B. Coronary endothelial injury constituted a potential underlying mechanism, and microthrombosis was promoted by high levels of lipoprotein(a) in the serum.