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Limited data exist on training of European paediatric and adult congenital cardiologists.
A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.
Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87–9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63–10.72 million), and one training centre per 4.29 million population (range 1.63–10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1–17), and duration of training was 3 years (range 2–5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41).
Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.
Persistence of the embryonic “fifth aortic arch” in postnatal life is a rare, enigmatic – and at times controversial – condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the “great pretender” by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of “persistent fifth aortic arch” while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.
The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management.
Methods and Results
Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug.
Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.
We present a neonate with an antenatal diagnosis of Scimitar syndrome and aortic arch hypoplasia. After delivery, computerised tomography scan additionally revealed an anomalous origin of the circumflex coronary artery from the main pulmonary artery. The management of this rare combination is discussed.
The long-term haemodynamic effects of a restrictive ventricular septal defect permitting moderate left-to-right shunting are not known.
Patients and methods
Echocardiographic measure ments of left heart dimensions and function were compared between a group of 9 older children and adults (median age 21 years, range 12–24.5 years) having restrictive ventricular septal defects (median Qp/Qs 1.7, range 1.4–2.1) and a group of 10 age matched controls.
Left ventricular mass indexed to body surface area was significantly greater in subjects than in controls (102±29 vs. 75±13 g/m2, p=0.02), although there was no significant difference in the ratio of mass to volume. There were no significant differ ences between indexes of left ventricular systolic function. Ratios of peak early to late diastolic ventricular filling were lower in those with septal defects (1.5±0.3 vs. 1.8±0.3, p=0.013), but there were no differences in other indexes of diastolic function.
Resting left ventricular function does not appear to have deteriorated by early adult life in patients with restrictive ventricular septal defects and moderate volume loading. This would support a continued conservative approach to management in these patients.
A 3-month-old infant developed signs of cardiac failure, which was initially attributed to cardiomyopathy. At 8 months, further investigations showed evidence of myocardial ischaemia with reversal of the flow of blood in the left coronary artery, which received no demonstrable inflow from the aorta. An anomalous connection of this artery with the pulmonary trunk was diagnosed but, at surgery, it was found that the arterial orifice was completely atretic, although the main stem was of normal size. A left internal thoracic arterial graft to the anterior descending coronary artery was performed, but he died on the third day after the operation. Postmortem examination showed a small dimple within the aorta at the site of the orificial atresia, extensive myocardial infarction, and two zones of myocardial bridging of the anterior descending coronary artery. We discuss the relationship of coronary orificial atresia with single coronary artery. Although they are related, they typically have different and contrasting clinical presentations. The possible role of the myocardial bridging is also considered.
Congenital anomalies of the tricuspid valve, and/or its supporting apparatus, leading to severe tricuspid regurgitation are rare. Although well tolerated in early childhood, long-standing and progressive volume loading of the right heart leads to symptoms of decreased exercise tolerance, and may predispose to arrhythmias in the long term. We report three cases of severe tricuspid regurgitation related to anomalies of the cords supporting the antero-superior leaflet of the tricuspid valve. Shortened cords leading to tethering of the leaflet were seen in two cases, and hypoplasia of the leaflet in the other. In all cases, the regurgitant jet was directed posteriorly towards the coronary sinus and atrial septum. Surgical repair was possible in one case, while it proved necessary to replace the valve in a second. The third child is asymptomatic and under regular review.
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