Our purpose was to examine the cognitive processes that influence involvement in physical activity among 100 adolescents, 55 boys and 45 girls, ranging in age from 12 to 18 years, with trivial, mild, or moderate forms of congenital cardiac disease. We hypothesized, first, that the severity of the congenital cardiac malformation itself has an indirect effect on self-efficacy regarding physical activity, and that the relationship between the two is mediated by the recommendations of the cardiologist and the attitude of the mother. Second, we argued that self-efficacy serves as a mediating variable between the recommendations of the cardiologist and the attitude of the mother, on the one hand, and involvement in physical activity, on the other. The results confirmed both hypotheses. In a population of adolescents with trivial to moderate congenital cardiac malformations, beliefs in self-efficacy, rather than severity of the disease, were the most influential factors in determining whether or not adolescents will engage in sports or other physical activities. We also demonstrated the importance of the role played by the recommendations of the cardiologist in determining both the attitudes of the mother and the belief in self-efficacy of the adolescents.

Twisted atrioventricular connections occur almost exclusively in the hearts with biventricular atrioventricular connections. Only one example of double inlet left ventricle has been illustrated in which the axes of the two atrioventricular valves crossed each other. We describe herein three patients, and one autopsied specimen, with double inlet right ventricle in which magnetic resonance imaging clearly demonstrated twisted atrioventricular connections.

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.

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.

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.

The middle aortic syndrome, with diffuse narrowing of the thoracic and abdominal aorta, was present in 10 of 18 patients with Williams' syndrome (55%). There were 3 thoracic coarctations, and 2 abdominal coarctations, with gradients greater than 20 mmHg across the zone of narrowing. Seven patients had mild renal arterial stenosis, and 6 had visceral arterial stenoses. Ten were hypertensive. Measured dimensions of the aortic lumen failed to increase with age in 3 males who had serial angiographic studies. One developed mesenteric arterial stenosis, with mild bilateral renal arterial stenoses, between the ages of 9 and 19 years. Aortic intravascular ultrasound performed in 2 patients confirmed abnormally thick vessel walls with small lumens. Diffusely narrowed and thick-walled stiff arteries, lacking elastin, are a feature of Williams' syndrome. The arteriopathy tends to progress with age, and systemic hypertension is common in teenagers and beyond. The middle aortic syndrome was present in more than half our patients, and does not necessarily reflect a bias because of cardiologic referral. Aortography with measurement of aortic diameters and delineation of the visceral branches is an important requirement for complete evaluation of patients with Williams' syndrome.

Deletions of chromosome 22q11 are common in patients with tetralogy of Fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.

A woman, having already delivered one child, underwent fetal echocardiography during thre subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process.

In recent years, post-operative intensive care of the child with congenital cardiac disease has placed an emphasis on earlier weaning from mechanical ventilation. We describe our experience of postoperative fast-tracking of children undergoing cardiac surgery during a charitable mission in Venezuela, where resources and equipment were severely limited. During our stay, 11 children, with a median age of 2 years, underwent total correction of tetralogy of Fallot. The median duration of ventilation was 2.5 hours, and all patients were extubated within 12 hours of surgery. Effective analgesia was achieved without the need for continuous intravenous infusions of opiates. This experience shows that early extubation can safely be carried out in well-selected patients after surgery to correct congenital cardiac malformations. This allows faster throughput of patients, and helps provide an efficient and cost-effective service.

We describe a profoundly retarded infant girl with multiple anomalies caused by trisomy 13. Due to heart failure, which was resistant to medical treatment, we closed successfully a ventricular septal defect at three months of age. She died at 10 months of age. Despite the short survival, we believe that the patient benefitted significantly from the surgical repair of her cardiac defect.

An infant developed severe desaturation within a few hours of construction of a modified Blalock-Taussig shunt. Echocardiography revealed that the shunt had become occluded, and this was confirmed angiographically. At catheterisation, therefore, we passed a 0.014”percutaneous transluminal coronary angioplasty wire through the occluded shunt into the right pulmonary artery and then dilated the shunt successfully using a 5 mm coronary angioplasty balloon. Six weeks later, the shunt remained patent.

We describe a neonate with critical coarctation of the aorta. Since treatment with Prostaglandin El had failed to reopen the arterial duct, and surgery was deemed to be associated with an unacceptably high risk in this unstable neonate, it was decided to perform balloon dilation of the coarctation as a palliative procedure to stabilize the patient. Balloon angioplasty failed to reduce the pressure gradient across the coarctation, so a stent was implanted retrogradely as an emergency procedure. Subsequently, the stented segment was resected surgically and end-to-end anastomosis created without complications at seven months of age.