Paediatric and adult congenital cardiology education and training in Europe.

BACKGROUND: Limited data exist on training of European paediatric and adult congenital cardiologists. METHODS: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. RESULTS: 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). CONCLUSION: 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.

QRS Duration During Follow-Up of Tetralogy of Fallot: How Valuable is it? Analysis of ECG Changes in Relation to Pulmonary Valve Implantation.

Long-term results after tetralogy of Fallot (TOF) repair are determined by the extent of right ventricular remodeling to chronic pulmonary regurgitation entailing progressive RV dysfunction and a risk of developing ventricular arrhythmia. Pulmonary valve replacement (PVR) can alleviate this burden. As a predictor of ventricular arrhythmia, QRS duration remains a strong parameter in this decision. We performed a retrospective analysis of all PVR patients between 2005 and 2018, studying the time evolution of electrocardiographic parameters before and after PVR through linear mixed model analysis. 42 TOF patients underwent PVR. The median timespan between primary repair and PVR was 18 years (IQR 13-30). The indication for PVR was primarily based on the association of exercise intolerance (67%) and significant RV dilation on cMRI (median RVEDVi 161 ml/m2 IQR 133-181). Median QRS length was 155 ms (IQR 138-164), 4 (10%) patients had a QRS > 180 ms. QRS duration increased significantly before PVR, but barely showed regression after PVR. Changes of QRS duration after PVR were independent of RV dilation. In conclusion, when the decision for PVR in TOF patients is primarily based on RV volume and/or function threshold, QRS duration > 180 ms is rarely observed. In contrast with the significant increase of QRS duration before PVR, QRS length regression appears to be independent of the extent of RV dilation or QRS > 160 ms. Considering that the decision for PVR is based on mechanical RV characteristics, the utility of serial follow-up of QRS duration in contemporary operated TOF patients becomes questionable in absence of clinical arguments for ventricular arrhythmia.

When coronary arteries need systolic pressure: surgical considerations.

OBJECTIVES: Patients with pulmonary atresia and intact ventricular septum (PA-IVS) may have coronary sinusoids connected to a hypertensive right ventricle. Coronary perfusion may then completely depend on the right ventricular systolic pressure wave; decompression of the right ventricle can be deleterious in these patients. This study was set to investigate the treatment strategies and outcomes of patients with coronary sinusoids. METHODS: National multicentre retrospective analysis over 15 years (1985-2010) in a population of ~10.2 million people. All patients with PA-IVS and coronary sinusoids were identified from local databases. All angiograms, echocardiograms, surgical reports and outcome data were reviewed. RESULTS: Thirty patients were identified. Right ventricular-dependent coronary circulation was present in nine patients (30%). A systemic-to-pulmonary artery shunt was created in 23 patients (77%) at a median age of 13.0 days (range: 1-479). A bidirectional Glenn was performed in 20 children at a median age of 7.8 months (range: 2.1-112.9) and 11 children proceeded to a Fontan repair at a median age of 3.6 years (range: 2.1-19.6). Pulmonary valve perforation and angioplasty were performed in six children and in three a simultaneous decompression procedure was also done. Ten patients died (33%). Four died at the time of systemic-to-pulmonary artery shunt and three during the bidirectional Glenn shunt. In all these patients myocardial ischaemia was reported. CONCLUSIONS: Mortality in PA-IVS with sinusoids is high. Right ventricular coronary dependence may lead to early death if the right ventricular systolic pressure wave is interrupted. Adequate understanding of the underlying pathology and pathophysiology is essential before attempting any procedure in this subgroup of children.

Influence of combined aerobic and resistance training on metabolic control, cardiovascular fitness and quality of life in adolescents with type 1 diabetes: a randomized controlled trial.

OBJECTIVE: To evaluate the effect of combined exercise training on metabolic control, physical fitness and quality of life in adolescents with type 1 diabetes. DESIGN: A double-blind randomized controlled trial with patients receiving combined aerobic and strength or no training. SETTING: University Hospital Ghent (Belgium). SUBJECTS: Sixteen children with type 1 diabetes were randomized into a control group (n = 8) and an intervention group (n = 8). INTERVENTIONS: Patients participated twice a week for 20 weeks in the combined aerobic and strength group. The control group continued their normal daily activities. MAIN MEASURES: Before and after the intervention anthropometric variables (weight, length, BMI, body composition), metabolic control (glycaemia, HbA1c, daily insulin injected), aerobic capacity (peak Vo(2), peak power, peak heart rate, 6-minute walk distance), strength (1 repetition maximum of upper and lower limb, hand grip strength, muscle fatigue resistance, sit-to-stand) and quality of life (SF-36) were assessed. RESULTS: At baseline, none of the measured parameters differed significantly between the two groups. There was no significant evolution in the groups concerning anthropometric indices, glycaemia and HbA1c. However, the daily doses of insulin injected were significantly lowered in the training group (0.96 IU/kg.day pre versus 0.90 IU/kg.day post; P < 0,05), while it was increased in the control group. Physical fitness increased significantly in the training group. General health, vitality and role emotional had a tendency to improve. CONCLUSION: Combined exercise training seemed to lower daily insulin requirement and improve physical fitness, together with better well-being.

The effect of bosentan in patients with a failing Fontan circulation.

OBJECTIVES: To investigate the effect of bosentan in patients with a failing Fontan circulation. DESIGN: A multicentric open label, non-controlled study. SETTING: 5 tertiary care centres for congenital cardiology. PATIENTS: We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded. RESULTS: Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued. CONCLUSIONS: Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

Midterm assessment of the reconstructed arteries after the arterial switch operation.

BACKGROUND: The arterial switch operation is the preferred treatment for transposition of the great arteries (TGA), but there are concerns on the fate of the neoarterial trunks. METHODS: Ninety-three children were reviewed for functional and morphologic assessment of both reconstructed arteries after the arterial switch operation. Longitudinal analysis focused on neoaortic valve function, neoaortic obstruction, and neopulmonary stenosis as well as on the time-related size changes of both roots, with its clinical implications. RESULTS: Within a mean follow-up of 4.8 +/- 3.9 years, aortic regurgitation of 2 or greater developed in 10% in TGA with intact ventricular septum (IVS) versus 23% in TGA with ventricular septal defect (VSD). A VSD and major pulmonary to aortic annulus size discrepancy were main precursors of early neoaortic valve dysfunction, whereas development of aortic regurgitation of 2 or greater was additionally promoted by the duration of follow-up. Presence of a VSD enhanced neoaortic root enlargement, resulting in a mean root z-score of 3.25 in TGA/VSD versus 1.96 in TGA/IVS. Root dilation was more severe in case of aortic regurgitation of 2 or greater (z = 3.38). Neoaortic obstruction occurred in 8%, mostly at the neosinotubular anastomosis, and correlated with prior pulmonary to aortic ratio greater than 1.5. Concerning the neopulmonary tract, increased flow velocity was observed in 24%, primarily at the supravalvular level. Two patients with pulmonary annulus hypoplasia (z < -2) required early reintervention. Regarding clinical outcome, freedom from reintervention at 1, 5, and 10 years was, respectively, 98%, 96%, and 96% for TGA/IVS, versus 65%, 63%, and 63% for TGA/VSD. A VSD and aortic arch obstruction were significant predictors for reintervention. CONCLUSIONS: After arterial switch operation, the neoaortic root is usually enlarged, but with a growth pattern comparable to that of a normal population. The association of a VSD and major arterial root size discrepancy predisposes to both neoaortic valve dysfunction and root enlargement. Severe root dilation appears to be closely related to significant neoaortic valve regurgitation, mainly as a result of a time-depending and reciprocal process. Neopulmonary stenosis is a frequent finding, but rarely has clinical consequences. Because the factor "time" is the principal determinant of late neoaortic valve dysfunction and root dilation, strict serial surveillance after arterial switch operation is mandatory.