As I have emphasized in previous supplements, Florida is the fourth largest state in the United States of America. The programme for care of children with congenital cardiac malformations at Children's Hospital of Philadelphia is one of the largest, and most prestigious and comprehensive in the world. The Congenital Heart Institute of Florida is the largest programme providing services for patients with congenital cardiac disease in Florida. “Heart Week in Florida”, the joint collaborative project sponsored by the Children's Hospital of Philadelphia together with the Congenital Heart Institute of Florida, has now become recognized as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of heart disease in neonates, infants, children, and young adults. In 2006, however, we broke from our previous mould, since the component of our “week” organized by the group from Philadelphia was organized in Phoenix, Arizona, thanks to the support provided by our colleagues working at Children's Hospital in Phoenix. It was a huge success, diminished only slightly by the inclement weather facing those who needed to journey back from sunny Arizona and Florida to the frozen and snowy northeast coast of the United States. All institutions involved with the organization of the events of 2006, nonetheless, are very grateful to Bob Anderson, and the team at Cardiology in the Young, for their support of “Heart Week in Florida”, and for the opportunity to publish this Supplement.

As I have described in the introductions to our previously published supplements, Florida is the fourth largest state in the USA. Our “Heart Week” is a joint collaborative project sponsored by The Children's Hospital of Philadelphia and The Congenital Heart Institute of Florida. The cardiac programme at Children's Hospital of Philadelphia is one of the largest and most prestigious in the world. Our programme at the Congenital Heart Institute is the largest providing services for patients with congenital cardiac disease in Florida. Both institutions are very grateful to Bob Anderson, and the team at Cardiology in the Young, for their support of “Heart Week”, and for the opportunity to publish this particular Supplement. In February 2005, Bob was a valued faculty member, and tremendous supporter, of both the Fifth Annual International Symposium on Congenital Heart Disease held at All Children's Hospital and University of South Florida, and the Eighth Annual Update on Pediatric Cardiovascular Disease arranged by Children's Hospital of Philadelphia.

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.

Hearts which, at first sight, seem to have a solitary chamber within their ventricular mass have long been the subject of controversy. As difficult as it is to manage these cardiac malformations medically and surgically, it has been at least as challenging, to date, merely to describe and classify them. Even the most commonly used terms, “single ventricle” and “univentricular heart”, spark heated debate. In distant times, when congenitally malformed hearts were pathological curiosities, these entities were described as “cor triloculare biatriale”. Therein lies the beginning of the problem, since when hearts of this type were examined by more enlightened pathologists, such as the great Maude Abbott,1 it became plain that the apparently solitary ventricular mass in reality possessed a second, albeit much smaller, chamber. Abbott described this second structure as the “outlet chamber”. This convention of describing a “single ventricle”, albeit with a co-existing “outlet chamber”, that presumably lacked ventricular status, continued throughout the first half of the twentieth century, although it had been recognised by then that hearts could rarely be found with truly solitary ventricles, and these were typically deemed to be common structures. Van Praagh et al.2 neatly summarised the problem with this approach when they pointed out that the so-called “single ventricle” possessed two ventricular chambers, whilst the “common ventricle” described the truly solitary arrangement. In their seminal investigation of 1964, Van Praagh et al.2 analysed only those hearts unified because of double inlet atrioventricular connection, or alignment. They excluded arbitrarily from their investigation all hearts with atrioventricular valvar atresia, despite the similarity in morphology between many of these latter lesions and the hearts with double inlet.3

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair.

The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.

Testing using the head-up tilt table is performed regularly as a diagnostic tool in the evaluation of syncope. Recommendations for protocols, and interpretation of the results, however, are mainly based on experience in adults. We evaluated the results of tilt table testing in 100 consecutive children and adolescents aged from 6 to 18 years and referred for investigation of syncope. Over half the patients, 55%, proved impossible to classify using the criterions established by the European Society of Cardiology. Based on our data, we propose a modified classification for responses to tilt table testing in the young.

Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet (Fig. 1). The second feature is anterior and apical rotational displacement of the functional annulus (Fig. 2). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebstein's malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.