Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called “200%” origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle.

This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.

Sudden death in young competitive athletes can be avoided by implementation of pre-participation screening programmes. A screening programme should be performed only by trained physicians and should include the athlete’s personal and family history, physical examination results, and the readings from a 12-lead-electrocardiogram. The athlete should undergo this screening programme every second year to detect progressive diseases. In addition, the programme should include detailed instructions to the athletes to pause training during infections in order to prevent sudden death due to myocarditis.

It has long been contentious as to whether the presence of bilateral infundibulums, or conuses, is a prerequisite for the diagnosis of double-outlet right ventricle. As the use of such a criterion would abrogate the so-called “morphological method”, which correctly states that one variable entity should not be defined on the basis of another entity that is itself variable, it is now accepted that double outlet can exist in the setting of fibrous continuity between the leaflets of the atrioventricular and arterial valves. Although this debate has now been resolved, there are other contentious areas still requiring clarification in the setting of hearts unified because of the presence of this particular ventriculo-arterial connection – for example, it is questionable whether the channel between the ventricles should be described as a “ventricular septal defect”, whereas it is equally arguable that the mere presence of fibrous continuity between the leaflets of the arterial valves does not necessarily place the channel in a doubly committed location. In this review, we describe a series of autopsied hearts in which the anatomical features serve to illuminate these various topics. We then discuss recent findings regarding cardiac development that point to the individuality of the building blocks of the ventricular outflow tracts, specifically the outlet septum, the inner heart curvature, or ventriculo-infundibular fold, and the septomarginal trabeculation, or septal band.

The 1st World Congress of Paediatric Cardiology was held in London, United Kingdom, in 1980, organised by Dr Jane Somerville and Prof. Fergus Macartney. The idea was that of Jane Somerville, who worked with enormous energy and enthusiasm to bring together paediatric cardiologists and surgeons from around the world. The 2nd World Congress of Paediatric Cardiology took place in New York in 1985, organised by Bill Rashkind, Mary Ellen Engle, and Eugene Doyle. The 3rd World Congress of Paediatric Cardiology was held in Bangkok, Thailand, in 1989, organised by Chompol Vongraprateep. Although cardiac surgeons were heavily involved in these early meetings, a separate World Congress of Paediatric Cardiac Surgery was held in Bergamo, Italy, in 1988, organised by Lucio Parenzan. Thereafter, it was recognised that surgeons and cardiologists working on the same problems and driven by a desire to help children would really rather meet together. A momentous decision was taken to initiate a Joint World Congress of Paediatric Cardiology and Cardiac Surgery. A steering committee was established with membership comprising the main organisers of the four separate previous Congresses and additional members were recruited in an effort to achieve numerical equality of cardiologists and surgeons and a broad geographical representation. The historic 1st “World Congress of Paediatric Cardiology and Cardiac Surgery” took place in Paris in June, 1993, organised by Jean Kachaner. The next was to be held in Japan, but the catastrophic Kobe earthquake in 1995 forced relocation to Hawaii in 1997. Then followed Toronto, Canada, 2001, organised by Bill Williams and Lee Benson; Buenos Aires, Argentina, 2005, organised by Horatio Capelli and Guillermo Kreutzer; Cairns, Australia, 2009, organised by Jim Wilkinson; Cape Town, South Africa, 2013, organised by Christopher Hugo-Hamman; and Barcelona, Spain, 2017, organised by Sertac Cicek. With stops in Europe (1993), Asia-Pacific (1997), North America (2001), South America (2005), Australia (2009), Africa (2013), and Europe again (2017), in 2021, The World Congress of Paediatric Cardiology and Cardiac Surgery will be held for the first time in the continental United States.1 The 8th World Congress of Paediatric Cardiology and Cardiac Surgery will be held in Washington DC, United States of America, 19–24 September, 2021, and will be organised by Jeffrey P. Jacobs and Gil Wernovsky. Mitchell I. Cohen served as the Scientific Program Co-Chair for the 2017 World Congress of Paediatric Cardiology and Cardiac Surgery, and he will again serve as the Scientific Program Co-Chair for the 2021 World Congress of Paediatric Cardiology and Cardiac Surgery along with Kathyrn Dodds RN, MSN, CRNP. Information about the upcoming 8th World Congress of Paediatric Cardiology and Cardiac Surgery can be found at www.WCPCCS2021.org

Genetic and environmental factors may be similar in certain CHD. It has been widely accepted that it is the cumulative effect of these risk factors that results in disease. Pulmonary atresia is a rare type of complex cyanotic CHD with a poor prognosis. Understanding the molecular mechanism of pulmonary atresia is essential for future diagnosis, prevention, and therapeutic approaches. In this article, we reviewed several related copy number variants and related genetic mutations, which were identified in patients with pulmonary atresia, including pulmonary atresia with ventricular septal defect and pulmonary atresia with intact ventricular septum.

Treatment of arrhythmias by catheter ablation targeting the anatomical foci of arrhythmias by radiofrequency has evolved dramatically in recent years. A road map for the relevant heart structures is an important asset for the success of the procedure and should be obtained before the intervention. This can be achieved by intra-cardiac echocardiography, conventional angiographic methods, multidetector CT, or MRI. The electrophysiological technique comprises a diagnostic procedure and an interventional – therapeutic – procedure. Electrocardiographic-gated multidetector CT is important in the diagnostic session to evaluate the anatomical details in combination with electric activity mapping. This combined protocol provides a unique view of the propagation of electrical activity, either normal or abnormal, over cardiac structures and allows a precise functional and anatomical evaluation to be obtained. In this review, we evaluate the role of electrocardiographic-gated multidetector CT in roadmapping arrhythmias in the paediatric age group, focussing on its strengths; we also evaluated some additional aspects that need further improvement in the future.

We hypothesised that infants with ventricular dysfunction after cardiac surgery have impaired haemodynamic response to arginine–vasopressin therapy. We retrospectively reviewed the medical records of neonates and infants treated with arginine–vasopressin within 48 hours of corrective or palliative cardiac surgery who underwent echocardiographic assessment of ventricular function before initiation of therapy. Patients were classified as “responders” if their systolic blood pressure increased by ⩾10% without increase in catecholamine score or if it was maintained with decreased catecholamine score. Response was assessed 1 hour after maximum upward titration of arginine–vasopressin. A total of 36 children (15 neonates) were reviewed (17 male). The median (interquartile) age was 10.4 weeks (1.1–26.9), and the median weight was 4.3 kg (3.2–5.8). Diagnoses included single ventricle (eight), arch abnormalities (five), atrioventricular septal defect (four), double-outlet right ventricle (three), tetralogy of Fallot (three), and others (13). In all, 12 patients (33%) had ventricular dysfunction. Only 15 (42%) responded favourably according to our definition 1 hour after the “target” arginine–vasopressin dose was achieved. Ventricular dysfunction was not associated with poor response. The overall mortality was 25%, but mortality in patients with ventricular dysfunction was 42%. Favourable response was associated with shorter ICU stay (9.5 days versus 19.5 days, p=0.01). We conclude that arginine–vasopressin fails to increase blood pressure in ~50% of hypotensive children after cardiac surgery. The response rate does not increase with duration of therapy. Ventricular function does not predict haemodynamic response. The mortality in this group is very high. Prospective comparison of vasopressin with other vasoactive agents and/or inotropes is warranted.