IN 1999 AND EARLY 2000, THE ASSOCIATION FOR European Paediatric Cardiology published the European Paediatric Cardiac Code as independent but linked Short and Long Lists, containing 650 and 3876 primary terms respectively. The historical background and rationale for development of this coding system has been previously detailed, but essentially it followed a series of meeting of the coding committee of the Association between 1997–1999, during which a pre-existing Long List was adopted and then used to create the condensed Short List. The system was published as the recommended standard coding system for use across Europe, covering the diagnosis and therapy of children with congenital and acquired cardiac disease. The scope of the lists was to encompass the needs of all those involved with such patients, from the fetal cardiologist through to the specialist in adult congenital heart disease; and from the general paediatric cardiologist and cardiac surgeon, to those specialising in transcatheter interventions, paediatric electrophysiology, and paediatric echocardiographers. In addition, the code was crossmapped to the 9th and 10th revisions of the International Classification of Diseases (“ICD-9” and “ICD-10”) provided by the World Health Organisation in order to facilitate returns to central government, a requirement in most countries. In so doing, it was hoped to address the concerns of many centres that such information submitted by professional coding staff was often inaccurate due to the complex nature of congenital cardiac disease, together with the limited scope and vague terminology of the International listings.

The Long List

The Long List is a comprehensive hierarchical system of coding and classification for the diagnosis and treatment of cardiac disease. The list was first published in 2000, as part of the standard coding system recommended by the Association for European Paediatric Cardiology for use across Europe. It embraces the entirety of the diagnosis and therapy of children with congenital and acquired cardiac disease. The Long List, as a whole, was adopted by the Association in 1998. Its development and expansion has been detailed previously. In essence, it grew from a list of 507 purely diagnostic terms published in 1985, through to a 1,777 item, hierarchical 12 tree structure in the Netherlands by 1988, and then to a single hierarchical structure of over 4,300 items in London by 1994. Although originally aimed at the clinician or surgeon treating cardiac disease first appearing in infancy or childhood, during this time it was enlarged to encompass abnormalities first diagnosed in fetal life, as well as cardiac disease first acquired during adult life. The list published in 2000 was composed of 4,777 items. These were made up of 3,906 individual terms, most of whom were mutually exclusive and unambivalent, given the constraints of clinical ambiguities and differing cultures of practice.

Triiodothyronine has been introduced as a promising new supplement for patients undergoing openheart surgery. This manuscript will review the existing literature regarding supplementation with triiodothyronine in children.

Infants and children undergoing cardiac surgery experience a profound decrease in levels of free and total triiodothyronine. These decreases are of sufficient magnitude to effect cardiac function. Supplementation with triiodothyronine has been shown to enhance myocardial performance, improve recovery of circulating levels of triiodothyronine, and to result in an increase in mixed venous oxygen saturation. As encouraging as these results are, there is no definitive study which demonstrates that triiodothyronine improves clinical outcome in children. Thus, while triiodothyronine appears to have promise as a supplement for children undergoing openheart surgery, more studies are needed to establish the efficacy of such therapy scientifically.

In a retrospective study, we analysed the data from 101 adults with echocardiographic follow-up after surgical repair of defects within the oval fossa at a mean age of 35 ± 17 years; 56% of the cohort being above the age of 30 years. Mean age at follow-up was 44 ± 18 years, and length of follow-up was up to 40 years (11 ± 12 years). At follow-up, atrial fibrillation or flutter was present in one quarter. Dilation of the right atrium, found in 64%, of the left atrium, found in 44%, and of the right ventricle, found in 29%, were also frequent, as well as pulmonary arterial hypertension, which was found in 30%. Diminished right ventricular ejection fraction, in contrast, was very rare, found only in 1%, and abnormal left ventricular ejection fraction was not encountered. By multivariate analysis, predictors for right or left atrial, or right ventricular, dilation were age at follow-up, degree of tricuspid regurgitation, pulmonary hypertension, and/or atrial fibrillation. In a subset of 21 patients in sinus rhythm, we correlated prospectively the diastolic and systolic function of both ventricles with levels of brain natriuretic peptide, comparing values to those of 20 age-matched controls with a mean age of 46 ± 14 years. Levels of brain natriuretic peptide were significantly higher in patients than in controls (p = 0.006), and correlated significantly with diastolic dysfunction (p = 0.007) and left atrial size (p < 0.0001). In the long-term follow-up after surgical repair of defect within the oval fossa, therefore, complete normalization of heart size and function is rare. Despite preserved systolic function, persistent diastolic dysfunction is common and is associated with elevated levels of brain natriuretic peptide, which may explain the late occurrence of atrial arrhythmias.

From January 1996 to April 2001, we treated eight patients with subdivided left atrium. Their ages at diagnosis ranged from newborn to 6.4 years. Prominent clinical symptoms were failure to thrive, respiratory symptoms, demand for oxygen, and congestive cardiac failure. Diagnosis was made by transthoracic echocardiography in all cases. Cardiac catheterization was necessary only in those patients who had associated cardiac anomalies, or suspected signs of pulmonary hypertension. In 7 patients, surgery was performed immediately after diagnosis, but one preterm infant died before operation due to neonatal sepsis and respiratory distress syndrome. In the postoperative period, one patient developed a severe capillary leak syndrome, and died due to irreversible congestive cardiac failure. The other 6 patients have all been followed up, with good results in the short- and intermediate-term at a mean of 34.3 ± 20.2 months. The infants were thriving, had a reduction in the frequency of infections of the respiratory tract, no significant arrhythmias, and showed early recovery from pulmonary hypertension and right ventricular hypertrophy.

A left superior caval vein frequently occurs in the malformed, as well as in the structurally normal, heart. Its physiological impact varies, and is determined by its connections and whether there are associated cardiac lesions. In this review, we describe 3 patients with such a vein, 1 without other lesions and 2 with the anomalous venous channel as a component of a complex cardiac malformation. In all cases, transcatheter techniques were used to treat the physiological dysfunction caused by the presence of the vein. The connections of the vein, and the complexity of the associated cardiac lesions, determine the options for treatment. We define the role of surgical as opposed to transcatheter intervention, and discuss strategies for transcatheter treatment.