In patients with coronary arterial disease, stress imaging is able to demonstrate abnormalities in the motion of the ventricular walls, and abnormalities in coronary arterial perfusion not apparent at rest. It can also provide information on prognostic factors. In patients with congenitally malformed hearts, stress imaging is used to determine contractile reserve, abnormalities of mural motion, and global systolic function, but also to assess diastolic and vascular function. In most of these patients, stress is usually induced using pharmacological agents, mainly dobutamine given in varying doses. The clinical usefulness of abnormal responses to the stress induced in such patients has to be addressed in follow-up studies. The abnormal stress might serve as surrogate endpoints, predicting primary endpoints at an early stage, which are useful for stratification of risk in this population of growing patients. We review here the stress imaging studies performed to date in patients with congenitally malformed hearts, with a special emphasis on echocardiography and cardiac magnetic resonance imaging.

Our aim was further to clarify the diagnostic usefulness of N-terminal pro-B-type natriuretic peptide for detecting ventricular dysfunction in children, and its correlation with myocardial performance index and New York University Pediatric Heart Failure Index score. We also hypothesized that the level of this natriuretic peptide in the serum could predict the severity of diastolic abnormalities in children with cardiac failure. We enrolled 99 patients, aged from 3 months to 16 years, who had been referred for echocardiography to evaluate ventricular function. Echocardiographic evidence of left ventricular systolic and diastolic dysfunction was found in 20 and 42 patients, respectively. We classified these patients as having impaired relaxation, seen in 12 patients, pseudonormal patterns seen in 19 patients, and restrictive-like patterns of filling seen in 11 patients. The mean of the log-transformed values for N-terminal pro-B-type natriuretic peptide increased significantly according to the severity of diastolic dysfunction (p = 0.003, p = 0.022, p < 0.0001). A value of 178 pg/ml had a sensitivity of 88% and specificity of 81% for detecting abnormal diastolic function (p < 0.0001). Furthermore, the log-transformed values correlated with myocardial performance index (p < 0.0001) in a positive manner, and the levels increased significantly according to New York University Pediatric Heart Failure Index score, showing a linear correlation with a robust r value for regression (r = 0.89, p < 0.0001). Our findings suggest that higher levels of the peptide, having a good correlation with New York University Pediatric Heart Failure Index score and myocardial performance index, might be a suitable marker to rule out ventricular diastolic dysfunction in children.

Trans fatty acids are unsaturated fatty acids produced by the partial hydrogenation of polyunsaturated oils. Over the last few years, an increasing interest on these fatty acids has been shown because of their role in the pathogenesis of cardiovascular diseases. To date, major scientific associations strongly recommend consuming a low intake of trans fatty acids for the prevention of cardiovascular diseases, but data on the consumption of these fatty acids in the general population are still lacking. We conducted this observational study on a population of Italian teenagers in order to evaluate the consumption of trans fatty acids in the diet. We studied 81 Italian teenagers, 45 males and 36 females, with a median age of 16 years. To assess their consumption of trans fatty acids, we used the “High School Survey”, a questionnaire prepared by the Harvard Medical School. Total calories of the studied population were 2359.2 ± 591.5 kcal/day with a mean intake of trans fatty acids of 3.24 ± 1.48 g/day, corresponding to 1.23% of the total energy. A relevant proportion of subjects, namely 51 (62.9%), exceeded the limit contribution of 1% of energy from trans fatty acids. Their intake of total calories and trans fatty acids significantly increased according to their increasing age (p = 0.0003 for trend). Our data, therefore, obtained in a limited population of Italian adolescents, showed that a consistent proportion of adolescents does not follow the nutritional recommendations for intake of trans fatty acids, likely increasing their risk of cardiovascular diseases.

Totally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.

Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.

Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.

Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need.

We studied the duration and dispersion of the P wave in patients after a Senning operation, assessing its value in detecting the risk of atrial tachycardias.

We measured the duration and dispersion of the wave in surface 12 lead electrocardiograms obtained from 18 patients with sinus rhythm, having a mean age of 12.8 years, with 13 being males and 5 females, who had undergone a Senning operation, comparing the values obtained in 35 age and gender-matched healthy people. The patients had undergone repair at a mean age of 13.4 months, and had a mean duration of follow-up of 12.8 years after the procedure. We also made 24 hour Holter recordings.

The maximal duration of the P wave, at a mean of 129.3 milliseconds, and dispersion with a mean of 78 milliseconds, were both significantly increased in the patients compared with their controls, the mean values for the normal subjects being 103.7 and 54 milliseconds. Supraventricular tachycardia was detected in 1 of 3 patients with dispersion greater than 100 milliseconds, and in 2 of 15 patients (13%) with dispersion less than 100 milliseconds as measured from the Holter recordings (p > 0.05).

Thus, the maximum duration and dispersion of the P wave were increased in patients after a Senning operation, but we were unable to establish any relationship between these measurements and atrial tachycardias as observed using Holter monitoring.

Hypertrophic cardiomyopathy is a genetic myocardial disorder. In such patients, myocardial bridging is the most frequent encountered coronary arterial anomaly. Patients may, however, on occasion, present with other much rarer malformations of the coronary arteries. Duplication of the right coronary artery is a very rare anomaly. We have now encountered a patient with hypertrophic cardiomyopathy in whom we found myocardial bridging of the anterior interventricular coronary artery and duplication of the right coronary artery. To the best of our knowledge, this association has not previously been described.

The most common benign cardiac tumours are the myxomas, the rhabdomyomas, and the fibromas, with the latter 2 variants being the most common tumours encountered in children. The size and location of tumours within the heart create a variety of clinical findings, such as murmurs, chest pain, tachyarrythmias, and congestive cardiac failure. Nowadays, the tumours are usually diagnosed by echocardiography, magnetic resonance imaging and cardiac catheterization. Surgical excision is the treatment of choice if the tumour causes either arrhythmia or cavitary obstruction. In this report, we describe a giant ventricular fibroma co-existing with an atrial septal defect in a girl aged 15 years.

Derivatives of Imidazoline usually act to stimulate peripheral alpha2 receptors causing vasoconstriction. In young children, however, they can also stimulate alpha2receptors in the cardiovascular and central nervous systems, possibly causing cardiovascular, neurological, and respiratory depression. These medications do not require medical prescriptions, so often parents use them, bypassing paediatricians. We report here a case of cardiovascular and neurological depression induced by oxymetalzoline in a toddler.

Kawasaki disease is a systemic vasculitis occurring in children of all ages. Coronary arterial aneurysms are one of the main fatal complications of the disease, and are usually observed with the onset of coronary arterial disease in adults. We report a young male presenting with myocardial infarction due to coronary arterial aneurysms, but in the absence of previous symptoms of Kawasaki disease.

Coronary arterial involvement is rare in Takayasu’s arteritis. We describe successful coronary arterial bypass grafting in a 15 year teenager with Takayasu’s arteritis and unstable angina because of stenosis of the main stem of the left coronary artery.

We report replacement of the aortic valve in a patient aged 9 years with mucolipidosis III and severe aortic insufficiency. Histopathology demonstrated abnormalities of the matrix and lysosomal inclusion bodies. As life expectancy increases for patients with lysosomal storage disorders, approaches to intervention for valvar disease become increasingly important.

We describe our experience with 2 patients having Costello syndrome, aged 11 and 36 months, who suffered systolic anterior motion of the aortic leaflet of the mitral valve and obstructive cardiac hypertrophy requiring surgery, comparing their cardiac characteristics to those described previously. We conclude that the heterogeneous nature of the cardiac hypertrophy in this syndrome can be considered and managed as the sum of a diffuse hypertrophy accessible to beta-blockade, and an asymmetric hypertrophy accessible to surgical myomectomy.