Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings

In order to test the effect of systematic supervised physical training, we divided a total of 129 children and adolescents with congenital heart disease into a group undergoing intervention and a control group. All patients underwent exercise tests, measurements of physical activity, and a survey of psychosocial factors. An improvement in uptake of peak level of oxygen was observed after intervention. There was also an improvement in physical activity in both groups measured by a monitor, although this was significant only in those with intervention. The psychosocial scales measured by the Child Behavior Checklist showed a decrease in internalizing scores for those subjected to intervention. This was decreased due to decreased withdrawal and somatic complaints. In conclusion, we recommend systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease.

Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.

Feeding an infant is an interactive process that facilitates social, emotional and culturally based skills. Children with congenital or acquired cardiac disease frequently require supportive regimes with regard to feeding so as to maintain weight, resulting in altered experiences for both the child and family. This study evaluated the practical, emotional and social ramifications for parents, of having a child with cardiac disease who also experienced difficulties with oral feeding. The study sampled three groups of parents who had children less than 3 years of age: those with cardiac disease who had difficulty in feeding, those with cardiac disease and no such difficulty, and those with no medical diagnosis. Parents completed a questionnaire about feeding, a time diary of activities involved in feeding, and Tuckman's Mood Thermometers, which measure anger and ‘poorness-of-mood’ associated with feeding the identified child. Parents of children with cardiac disease and a feeding difficulty reported a significantly more negative mood-state, and significantly longer time associated with feeding, than parents of children in the other two groups. Emerging themes from qualitative analysis of the data suggested that having a child with congenital cardiac disease producing difficulty in feeding had a strong negative impact on the whole family.

Intrapericardial teratomas are rare primary cardiac tumors of infancy and childhood. We describe three neonates with intrapericardial teratomas diagnosed during fetal life and treated after birth. Clinical and anatomic considerations suggest that cardiopulmonary bypass provides for safe tumor dissection and complete excision of the tumor, thereby decreasing the risk of recurrence.

Innocent murmurs are common in childhood. They require accurate diagnosis to avoid unnecessary anxiety and/or restrictions. With this in mind, we studied prospectively children diagnosed by a paediatric cardiologist as having innocent murmurs to review the necessity of investigations in excluding organic abnormalities. We included 63 children in the study. The majority had the so-called Still's, or musical/vibratory murmur. None had abnormal investigations, suggesting that such investigations are largely superfluous for the purpose of diagnosis in those patients with innocent murmurs seen by a paediatric cardiologist. They are often done, nonetheless, as part of the assessment and/or expectations of the parent or the referring physician.

We describe two children with isolated origin of the left subclavian artery from the left pulmonary artery detected by echocardiography during the assessment of their congenital cardiac malformations. Both patients demonstrated pre-operative evidence of subclavian steal. This entity results from persistence of the dorsal segment of the sixth left arch, with regression of the left fourth arch and interruption of the left dorsal arch distal to the origin of the seventh left intersegmental artery. The significance of this finding relates to the potential for pulmonary overcirculation, which could have significant post-operative ramifications if not detected prior to surgical repair of an associated cardiac malformation. This entity differs from cases with a right aortic arch and aberrant left subclavian artery which has the potential to form a vascular ring, unlike cases with isolated origin of the left subclavian artery from the pulmonary artery that do not cause compression of the airway.

We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed echocardiographic and angiographic evaluations were performed in all, and biopsies of the skin in seven. All patients have elongated facies, prominent ears, micrognathia and laxity of their joints. Angiographic pictures showed a varying degree of lengthening and tortuosity of systemic, pulmonary, and coronary arteries. Pulsatile carotid arteries formed cervical masses in 2 patients, and three had severe renal arterial stenoses. All showed varying degrees of branch and peripheral pulmonary arterial stenosis, necessitating placement of stents in six. Biopsy of the skin proved normal in all seven patients studied, thus excluding cutis laxa, Ehlers-Danlos and Marfan syndromes. The constellation of abnormalities suggests a genetic syndrome of connective tissue etiology. Further genetic studies, and gene mapping, are underway.

The aim of our study was to elucidate the clinical and morphologic features of those perimembranous ventricular septal defects which extend between the ventricular outlets, particularly when found in association with anterior deviation of the muscular outlet septum and overriding of the aorta – the so called Eisenmenger ventricular septal defect. From 1990 through 1998, we have undertaken surgical correction in 203 patients with perimembranous ventricular septal defect. Of these, 15 patients had the Eisenmenger ventricular septal defect. We conducted retrospective analyses of the clinical records, catheterization data, and angiocardiographic and echocardiographic finding of these patients. Comparative studies were then made with the patients having tetralogy of Fallot, and those with simple perimembranous ventricular septal defects without overriding of the aorta. In the patients with the Eisenmenger ventricular septal defect, the extent of anterior deviation of the outlet septum was comparable with that seen in tetralogy of Fallot, but there was less rightward displacement of the aortic valvar orifice. In contrast to earlier investigators, however, we found evidence of progressive narrowing of the subpulmonary infundibulum in those with the Eisenmenger defect. These morphological features were reflected in the clinical features, since all patients showed evidence of increased pulmonary flow and congestive heart failure in early infancy, but with twothirds of them subsequently developing right-to-left shunting. We conclude, therefore, that the Eisenmenger ventricular septal defect is a discrete cardiac abnormality in which the morphologic substrate of anterior deviation of the outlet septum gives rise to a potential for progressive narrowing of the subpulmonary infundibulum. Surgical management, therefore, needs to take account of such narrowing as an additional cardinal morphologic feature.

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10–16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a rangefrom 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15–49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.