Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.

We compared the levels of lipoprotein (a) in 48 Caucasian patients with pulmonary arterial hypertension, comprising 32 females and 16 males, aged 28.0 ± 12.0 years, with a range from 4 through 52 years, with 48 normal Caucasian subjects matched for age and sex. Pulmonary hypertension was secondary in 41 patients with Eisenmenger's syndrome, these comprising 27 females and 14 males aged 27.0 ± 12.0 years, with a range from 4 through 51 years, and primary in the other 7 patients, 5 females and 2 males, whose age was 30.0 ± 14.0 years, with a range from 9 through 52 years. Lipoprotein (a) was measured using an immunoprecipitation and turbidimetric assay after a 12 hour fast. Levels of the protein, expressed as the median (% 25; % 75), were higher in those with Eisenmenger's syndrome than in normal controls (p = 0.003). In addition, there was a greater prevalence of levels of lipoprotein greater than 30.0 mg/dl in those with secondary pulmonary arterial hypertension patients than in our normal population (p = 0.03). We have found no differences, however, in the levels of lipoprotein(a) in those who had primary pulmonary arterial hypertension when compared with their matched controls, albeit that the number of patients studied was small. We conclude that increased levels of lipoprotein (a) may be secondary to pulmonary arterial hypertension as a marker of tissue damage or may be genetically determined. In either way, the increase in lipoprotein (a) could be an additional factor predisposing to the vascular alterations known to occur in this disease.

Increasingly, paediatric cardiologists are called upon to diagnose cardiac malformations prenatally. In the main, the types of malformation seen during fetal life will be similar to those documented postnatally, but the frequency with which they are encountered, as well as the views that can be used for diagnosis, will be different. This review aims to describe the anatomic spectrum of malformations seen in 917 fetal hearts examined consecutively following prenatal diagnosis. The distribution of anomalies is illustrated in terms of a simple sweep through the fetal thorax passing from the four-chamber plane to the outflow tracts, and then to more cranial views of the mediastinum. Two-thirds of the anomalies described would have been detectable in the four-chamber plane. Some, such as tricuspid valvar abnormalities, will alter the normal appearances of the four-chambers dramatically. In terms of the overall spectrum, however, such obvious abnormalities only form a minor part of the total number. Others, such as atrioventricular septal defect, will often require closer inspection of intracardiac anatomy, but will make up a large proportion of the entire cohort. Up to one third of the anomalies in the series would have required views more cranial to the four-chamber plane of section. In these, it would have been necessary to examine the nature of the left ventricular outflow tract, the crossing of the two outflows, or else the arterial arches in order to secure detection. In the fetus, these and other planes must be considered by the echocardiographer in order completely to detect and document the entire spectrum of cardiac abnormalities likely to be encountered.

With the ever-increasing sophistication of the Internet, and the wealth of information that can now be mined through this source, it may be thought that the age of the book has finished, and hence there is no further need for reviews of newly published books. There is much to be said, nonetheless, for the greater facility of acquiring information from the pages of a book compared to the computer screen. In this review, therefore, I discuss the advantages of obtaining information from the printed page when presented in such splendid form as shown by new books devoted, on the one hand, to the clinical anatomy of the coronary arteries, and on the other hand, to paediatric congenital cardiac surgery. At the same time, I also attempt to provide some insights into the reasons why the authors and editors produced these excellent volumes, which deserve to be on the bookshelves of all those concerned with the diagnosis and treatment of children with congenitally malformed hearts.

The concept of sequential analysis of congenitally malformed hearts is now widely accepted, although some aspects remain controversial. In this review, we analyse the evolution of the scgmental approach, showing how our own concepts of analysis have chaanged little since our initial description, heart dicrates the presence of the atrial, ventriclar, and arterial segments, Analysis depends upon recognizing the morphologic differences of the components within each segment, and then how these parts are joined together across the atrioventicular and ventriculo-arterial Junctions. The distinction of chambers and arterial trunks follows the principle of the morphologic method, namely that scructures be identified on the basis of their own intrinsic morphology, and not according to other features which themselves might be variable. Differentiation of junctional arrangements as opposed to segmental stucture resolves ongoing controversics such as the nature of “univentricular hearts”.Analysis in the fashion proposed shows that there is nothing intrinsically complicated with malformed hearts.

Over the last 30 years, 94 episodes of infectious endocarditis have been identified in 86 children seen at the Children's Hospital of Pittsburgh. In most of these children no precipitating event could be documented. There was no change in underlying cardiac anomalies over the three decades of this study, and left ventricular outflow tract obstruction, ventricular septal defect and tetralogy of Fallot were the most common anomalies. Streptococci and staphylococci were the most common infecting organisms, and there was a slight increase in the percentage of streptococci in the last decade. Cardiovascular damage occurred in about one-third of the cases, and aortic regurgitation was most common. Mitral regurgitation was also an important complication, as was the development of an aortic aneurysm. Important systemic and pulmonary emboli were rare. Surgical intervention was necessary in one-fifth of the children. Valvar replacement was the most common procedure done. The mortality rate declined markedly over the period of the study, dropping from 36% in the first decade to 8% in the last.

Many of the controversies surrounding the description of ventricular septal defects arise from differences in the perspectives from which they are viewed. In this review, we analyse these defects as seen in cross-sectional echocardiographic images, correlating them with morphologic specimens. The classification we advocate, which now has a suitable pedigree, is a simple descriptive method distinguishing between perimembranous, muscular, and doubly committed types of defects. The approach is to categorise the defects as seen from the right ventricle, the usual port of access for surgeons. The term ‘perimembranous’ highlights the proximity of the atrioventricular conduction axis to the margin of the defects in which the remnant of the membranous septum forms a direct border. This system is applicable to all interventricular communications, no matter how malformed the heart may be in which they are enclosed.

Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial hypertension are related to those with the idiopathic form of the disease, but may also improve quality of life and survival in patients with pulmonary arterial hypertension associated with congenital cardiac disease. Despite the evident similarities in pulmonary vascular disease, it is important also to recognise the differences between patients in whom pulmonary arterial hypertension is the consequence of systemic-to-pulmonary shunts as opposed to those with other conditions. Patients with pulmonary hypertension associated with congenital cardiac disease themselves constitute a heterogeneous population, in which generalisation may be hazardous. Specific considerations need to be given to the type of cardiac diagnosis, the prognosis and evolution of pulmonary vascular disease, and the circulatory physiology before embarking on new strategies for medical treatment in the individual patient. In this review, we highlight the features that require specific attention in these patients. In addition, we discuss briefly the data currently available on the effectiveness of the new anti-proliferative drugs in patients with the Eisenmenger syndrome.

Little is known about the implications of left ventricular noncompaction in children with additional congenital cardiac malformations. With this in mind, we conducted a retrospective review of every left ventricular angiogram performed in a single tertiary referral centre for paediatric cardiology, in Melbourne, Australia, between 1994 and 2000 in children with congenital heart disease, looking specifically for patients with angiographic evidence of noncompaction of the left ventricle. The outcome of patients identified as having noncompaction was compared with that of patients from the same population, stratified by their primary congenital cardiac malformation.

Of 1515 children undergoing left ventricular angiography, 31, with 13 being male, were found to have angiographic evidence of left ventricular noncompaction, giving a prevalence of 2% (95% CI: 1.3%–2.8%). Of 69 (22%) children with a functionally single left ventricle, 15 fulfilled the criterions for noncompaction, compared to 16 of 1446 (1.1%) children with a balanced ventricular arrangement (p is less than 0.0001). The presence of noncompaction and a functionally single left ventricle were each associated with a doubling of mortality, the effect being cumulative. Of surviving patients with left ventricular noncompaction, 19% (4%–34%) have left ventricular dysfunction at their latest follow-up. We suggest that the important late sequels of noncompaction justify careful scrutiny for this entity in children with congenital cardiac disease.

Perhaps because it guards the inlet to the lesser circulation, the morphologically tricuspid valve has received less attention in terms of its anatomy than the well-explored mitral valve, which will receive attention in a subsequent review in this supplement.1 As we will show in our initial review, nonetheless, the approach to morphological analysis is the same for both valves, irrespective of whether the specific morphology is displayed in the autopsy room or the echocardiographic laboratory. It is essential that the valve be analysed so as to reveal the precise structure of each if its components – the so-called valvar complex.2 Equally important, in the current era, with the burgeoning use of three-dimensional displays that place the heart firmly within the context of the body, it is essential that the components of the valve be described as seen relative to the bodily axis,3 rather than following the present custom of describing the heart as though it is removed from the body and positioned on its own apex.

Objective: Intraoperative ultrasound was introduced to evaluate the adequacy of repair after surgical repair of congenital cardiac malformations. Our purpose was to review the evolution of this technique at our centre. Methods: We evaluated all intraoperative ultrasound studies undertaken between 1997 and 2002, reviewing the data from 1997 through 2001 retrospectively, but undertaking a prospective audit of studies undertaken from 2002 onwards. In all, we carried out a total number of 639 intraoperative ultrasound studies, from a possible 2737 cardiac operations (23.3%), using the epicardal approach in 580 (90.7%), and transoesophageal ultrasound in the other 59 patients (9.3%). Median age was 0.6 years, with an interquartile range from 0.06 to 3.6 years. Results: The findings obtained using intraoperative ultrasound influenced the surgical management in 63 of the 639 patients (9.9%), suggesting the need for additional surgery in 26, adjustment of the band placed round the pulmonary trunk in 16, preoperative assessment of the cardiac malformation in 5, and confirming the need for prolonged support with cardiopulmonary bypass for impaired ventricular function in 16. There were 18 early reoperations, 5 of which may have been predicted by intraoperative ultrasound. Of the 183 studies reviewed prospectively in 2002, it was not possible to obtain the complete range of views in 8 (4.4%), while in 27 patients (14.7%), the postoperative findings using transthoracic interrogation differed from the findings obtained immediately following bypass. Conclusion: Intraoperative ultrasound is an important technique for monitoring the results of complex congenital cardiac surgery. The immediate recognition of significant lesions, together with multidisciplinary discussion, allows for improved management and prevention of early surgical reintervention.