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.

Introduction: Aspects of the management of ventricular septal defects which remain uncertain include the role of screening, and the need for closure of defects that remain patent. Aim: To ascertain the natural history of clinically significant ventricular septal defects, and to examine uncertainties in strategies of management. Methods: We studied prospectively a regional cohort of children born with isolated ventricular septal defects, noting age at presentation; sex; morphology and size of the defect; age at closure, if it occurred, and the means of closure. Results: We identified 290 children with isolated ventricular septal defects, of whom 147 (51%) were male. The mean follow up period was 65 months, with five cases being lost to follow up. Surgical closure was required in 41 (14%) cases, and 155 (54%) defects closed spontaneously. Spontaneous closure occurred in 123 (68%) out of 180 cases with completely muscular borders, and in 31 (29%) of the 107 patients with perimembranous defects. There were two cases with associated aortic regurgitation and no cases of endocarditis. Size and morphology of the defect were significant predictors of spontaneous and surgical closure. In addition, young age at diagnosis was a significant predictor of spontaneous closure. Conclusions: The size and morphology of a ventricular septal defect are important determinants of spontaneous closure and to the need for surgical intervention. Early age at presentation, in contrast, is not predictive of the need for surgical intervention. In early childhood, there appears to be very little risk of endocarditis or aortic valvar prolapse. Neither screening of populations to identify ventricular septal defects, nor surgical closure of asymptomatic defects in childhood, are supported by our findings.

Sudden death occurs in patients after repair of congenital heart disease. In those with tetralogy of Fallot, or a similar lesion, ventricular tachycardia has been hypothesized as the major arrhythmic mechanism for sudden death. It would be desirable to identify individuals at risk for sudden death, to determine which arrhythmia would be likely to cause sudden death, and to treat those individuals with an appropriate antiarrhythmic to prevent sudden death. For the last 10 years, physicians have been treating patients with antiarrhythmic drugs, based on a number of criteria, the most common of which is the presence of premature ventricular contractions.1,2 The practice has recently been called into question by the CAST trial. It is the purpose of this paper to review the evidence that repair causes ventricular arrhythmias, that ventricular arrhythmias cause sudden death, and that ventricular arrhythmias should be treated prophylactically.

We aimed to determine the effect of supplementation with coenzyme Q10 on conventional therapy of children with cardiac failure due to idiopathic dilated cardiomyopathy. In a prospective, randomized, double-blinded, placebo-controlled trial, we randomized 38 patients younger than 18 years with idiopathic dilated cardiomyopathy to receive either coenzyme Q10, chosen for 17 patients, or placebo, administered in the remaining 21. Echocardiographic systolic and diastolic function parameters were determined for every patient at baseline, and after 6 months of supplementation. The index score for cardiac failure in children as established in New York was used for assessing the functional class of the patients. After 6 months supplementation, 10 patients randomized to receive coenzyme Q10 showed improvements in the grading of diastolic function, this being significantly more than that achieved by those randomized to the placebo group (p value = 0.011). The mean score for the index of cardiac failure index for those receiving coenzyme Q10 was also lower than the control group (p value = 0.024).

Our results, therefore, indicate that administration of coenzyme Q10 is useful in ameliorating cardiac failure in patients with idiopathic dilated cardiomyopathy through its significant effect on improving diastolic function.

In paediatric pulmonary embolism, cardiac findings and thromboembolic outcomes are poorly defined. We conducted a mixed retrospective-prospective cohort study of paediatric pulmonary embolism at the Children's Hospital Colorado between March, 2006 and January, 2011. A total of 58 consecutive children – age less than or equal to 21 years – with acute pulmonary embolism were enrolled. Data collection included clinical and laboratory characteristics, treatments, serial echocardiographic and electrocardiographic findings, and outcomes of pulmonary embolism non-resolution and recurrence. The median age was 16.5 years ranging from 0 to 21 years. The most prevalent clinical risk factors were oral contraceptive pill use (52% of female patients), presence of a non-infectious inflammatory condition (21%), and trauma (21%). Thrombophilias included heterozygous factor V Leiden in 21%; antiphospholipid antibody syndrome was established in 31% overall. Proximal pulmonary artery involvement was present in 34%. At presentation, nearly half of the patients had hypoxaemia and 37% had tachycardia. The classic electrocardiographic finding of S1Q3T3 was present in 12% acutely; tricuspid regurgitation greater than 3 metres per second, septal flattening, and right ventricular dilation were each present on acute echocardiogram in 25%. Nearly all patients received therapeutic anticoagulation, with initial systemic tissue plasminogen activator administered in 16% for occlusive iliofemoral deep venous thrombosis and/or massive pulmonary embolism. Pulmonary embolism resolution was observed in 82% by 6 months. Recurrent pulmonary embolism occurred in 9%. There were no pulmonary embolism-related deaths. Right ventricular dysfunction was rare in follow-up. These data indicate that acute heart strain is common, but chronic cardiac dysfunction is rare, following aggressive management of acute pulmonary embolism in children.

Increasingly, the interventional cardiologist is seeking to close interatrial communications by inserting devices by means of catheterisation. So as to optimise these procedures, it is adavantageous to have a firm grasp of the anatomy of the normal atrial septal structures, this then providing the basis to understand the morphology of the holes which can exist between the chambers, not all of which are true septal defects.A true septal structure can be removed without exiting from the cavities of the heart. It is the flap valve of the oval fossa, along with the anterior rim of the fossa, which fulfill this criterion. The remainder of the extensive rim of the normal fossa is no more than an infolding between the walls of the right and left atriums and their venous tributaries, and has different dimensions at various points around the ircumference. The so-called muscular atrioventricular “septum” is a sandwich incorporating a layer of epicardial fibro-adipose tissue. True defects of the atrial septum, therefore, exist because of deficiency, perforation, or absence of the flap valve. Most of these defects will prove suitable for interventional closure, but potential caveats include multiple defects, aneurysm of the flap valve, or adjacency of the fossa to the venous orifices. The other interatrial communications, namely the sinus venosus, coronary sinus, and “ostium primum” defects are outside the confines of the oval fossa. Recognition of this feature is the key to their diagnosis, and their ifferentiation from true atrial septal defects. Of these defects, only the coronary sinus defect is likely to be suitable for device closure, and then only in the very rare circumstances when it is seen in isolation

Background: Surgical closure of ventricular septal defects has been performed for many years, and is considered as the gold standard for treatment. It remains associated with morbidity and mortality. Transcatheter techniques have been developed in the last 10 years as a possible alternative to conventional surgery. Methods: The procedure is performed under general anaesthesia, and with continuous fluoroscopic and transesophageal echocardiographic guidance. Devices of the Amplatzer family, two in particular, have achieved a large popularity in clinical practice, and are currently the devices most commonly used to close muscular and perimembranous ventricular septal defect percutaneously. Results: Data from literature show that successful closure of muscular defects is obtained in around 96% of patients, with a rate of major complication of around 2%. Pooling data from the literature shows that successful closure of perimembranous defects is also obtained in 96% of patients, again with major acute complications in around 2%. The major problem is the occurrence of complete atrioventricular block, reported in 1.7% of subjects. Acquired defects can occur as residual leaks after surgical closure, or as consequence of myocardial infarction. There are very few data concerning percutaneous closure of postoperative residual defects. As for the surgical approach, in patients with post-myocardial defects the success rate of percutaneous closure is around 88%, with a mortality of 22%. Conclusions: Nowadays, in experienced hands, percutaneous closure is a safe and effective procedure. In selected patients, closure of congenital or acquired muscular and perimembranous ventricular septal defects can be considered a real alternative to the standard surgical approach, with the advantage of a significantly reduced rate of mortality and complications.

Since Takotsubo cardiomyopathy was first described by Sato in 1990, multiple cases have been reported, but only few in children, among whom this type of cardiomyopathy is to some extent underappreciated. A series of children with this syndrome were therefore reviewed, drawing comparison with cases reported by others. The review addresses the current challenges in diagnosis, presentation, triggers, clinical course, management, and possible pathogenic mechanisms of the entity.

Although overall outcomes for children undergoing heart surgery have improved, there is a significant variation in outcomes across hospitals. This review discusses the variation in cost and outcomes across centres performing congenital heart surgery, potential underlying mechanisms, and efforts to reduce variation and improve outcome.

Across the globe, the implementation of quality improvement science and collaborative learning has positively affected the care and outcomes for children born with CHD. These efforts have advanced the collective expertise and performance of inter-professional healthcare teams. In this review, we highlight selected quality improvement initiatives and strategies impacting the field of cardiovascular care and describe implications for future practice and research. The continued leveraging of technology, commitment to data transparency, focus on team-based practice, and recognition of cultural norms and preferences ensure the success of sustainable models of global collaboration.

We report a fatal dissection of the descending aorta as a complication after a two-staged implantation of a stent to relieve aortic coarctation in a young female with Turner's syndrome. Implantation of the stent, with dilation up to 70 percent, and half a year later re-dilation to 100 percent, was without any complication. A week after the re-dilation, however, the patient suffered acute dissection of the descending aorta. We initially stabilized the situation, but some days later her condition deteriorated, and she died. We discuss the various options for treating coarctation and re-coarctation. As far as we know, this is the first description of implantation of a stent in the setting of coarctation in Turner's syndrome, albeit with an unfortunate conclusion.

In order prospectively to investigate the frequency and evolution of subclinical valvitis, we selected 40 consecutive patients suffering their initial attack of rheumatic fever, seen in our clinic from 1992 to 1994, and followed-up until 2001, with a mean period of follow-up of 8.1 years, and a standard deviation of 0.6 year. We also assembled a matched control group of 37 healthy children and adolescents. We discovered a murmur of mitral regurgitation in 28 (70.0%) of the patients. In 3 (7.5%) of these patients, there was also a murmur of aortic regurgitation. In the group of 28 symptomatic patients, Doppler echocardiography showed mitral regurgitation in all, and aortic regurgitation in 17. In the group of 12 patients without clinical evidence of cardiac involvement, Doppler echocardiography identified mitral regurgitation in 2, isolated in one and associated with aortic regurgitation in the other. Thus, the frequency of subclinical valvitis was 16.7%. In patients with subclinical valvitis only the aortic regurgitation regressed during the period of follow-up. In the group of 28 symptomatic patients, mitral regurgitation disappeared in 6 (21.4%), aortic regurgitation in 7 of the 17 having this feature (41.2%), while 2 patients (7.1%) developed mitral stenosis. The sensitivity and specificity of cardiac auscultation were, respectively, 93.3%, with 95% confidence intervals between 72.3% and 97.4%, and 100%, with 95% confidence intervals between 65.5% and 100%, for the diagnosis of mitral regurgitation, and 16.7%, with 95% confidence intervals between 4.4% and 42.3%, and 100%, with 95% confidence intervals between 81.5% and 100%, for that of aortic regurgitation. We conclude that the Doppler echocardiogram is an important means of diagnosing and assessing the evolution of subclinical rheumatic valvar lesions, which are not always transient. We suggest that Doppler echocardiography should be performed in all patients with acute rheumatic fever. Subclinical valvitis should be considered as mild carditis, provided that strict criterions are observed in the differential diagnosis from physiological regurgitation, and Doppler echocardiographic findings are analyzed in the context of the other manifestations of the disease.

We carried out a retrospective case control analysis to evaluate the outcome, and the need for treatment, of problems with atrioventricular conduction occurring during an acute attack of rheumatic fever, assessing the occurrence of second and third atrioventricular block versus first degree block.

We reviewed and analysed the clinical, electrocardiographic and echocardiographic records of all children diagnosed in a single institute as having acute rheumatic fever during a period of seven consecutive years.

During the period from October, 1994, through October, 2001, 65 children meeting the modified Jones criterions for acute rheumatic fever were hospitalized in the Soroka University Medical Center, Israel. First-degree atrioventricular block was identified in 72.3% of the children, and resolved with no specific treatment other than non-steroidal anti-inflammatory medications. Second-degree atrioventricular block of Mobitz type I, was observed in one child (1.5%), which progressed from first-degree block, and subsequently resolved. Complete atrioventricuar block was found in 3 children (4.6%), one progressing from Mobitz type I second-degree block, and two being seen as the first presentation. Of the three children with complete atrioventricular block, one patient was not treated, the second was treated with aspirin, and the final one with combined aspirin and steroids. The disturbances of conduction resolved in all three.

We conclude that advanced atrioventricular block is rare during acute rheumatic fever. If occurring, block appears to be temporary, and resolves with conventional anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when syncope or clinical symptoms persist.