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Background: Patency of the coronary arteries is an issue after reports of sudden cardiac death in patients with transposition of the great arteries (TGA) operated with arterial switch (ASO). Recent studies give rise to concern regarding the use of ionising radiation in congenital heart disease, and assessment of the coronary arteries with coronary MR angiography (CMRA) might be an attractive non-invasive, non-ionising imaging alternative in these patients. Theoretically, the use of 3.0T CMRA should improve the visualisation of the coronary arteries. The objective of this study was to assess feasibility of 3.0T CMRA at the coronary artery origins by comparing image quality with non-contrast CMRA in ASO TGA patients to healthy age-matched controls, and by comparing image quality with non-contrast CMRA to contrast enhanced CMRA in the patient group. Material and methods: Twelve patients, 9-15 years (mean 11.9 years, standard deviation 1.5 years), and 12 age-matched controls (mean 12.7 years, standard deviation 1.7 years) were examined with 3D balanced steady-state free precession (SSFP). Nine of twelve patients had Gadolinium-enhanced fast low-angle shot (Gd-FLASH) performed after SSFP. Image quality at the coronary artery origins was evaluated subjectively with a 10 cm figurative visual analogue scale (fVAS) and objectively by signal-to-noise and contrast-to-noise ratio (SNR, CNR). Results: All, but one, coronary artery origins were identified. No significant difference in image quality scores was found between patients and controls with SSFP (mean values 6.5 cm—9.1 cm in patients and 7.0 cm—8.0 cm in controls, p-values > 0.1). With SSFP, intra-observer fVAS mean score was 6.7 cm—8.6 cm and with Gd-FLASH 7.7 cm—8.7 cm. CNR was higher with Gd-FLASH (p < 0.03). Intra-observer agreement index (AI) with SSFP was moderate-to-good (0.43–0.71) and with Gd-FLASH good (0.64–0.79) in all origins. Inter-observer AI was good in the left main stem (LMS) with SSFP (0.65). With Gd-FLASH inter-observer AI was good in LMS (0.78) and moderate (0.5) in the left anterior descending artery, but lacking in the other origins though with a good agreement on Bland-Altman plots. Conclusions: Our findings indicate a better, more reproducible image quality with Gd-FLASH than with non-contrast SSFP CMRA on 3.0T for evaluation of the coronary artery origins in ASO TGA children and adolescents.

Objectives A technique that uses two-dimensional images to create a knowledge-based, three-dimensional model was tested and compared to magnetic resonance imaging. Background Measurement of right ventricular volumes and function is important in the follow-up of patients after pulmonary valve replacement. Magnetic resonance imaging is the gold standard for volumetric assessment. Echocardiographic methods have been validated and are attractive alternatives. Methods Thirty patients with tetralogy of Fallot (25 ± 14 years) after pulmonary valve replacement were examined. Magnetic resonance imaging volumetric measurements and echocardiography-based three-dimensional reconstruction were performed. End-diastolic volume, end-systolic volume, and ejection fraction were measured, and the results were compared. Results Magnetic resonance imaging measurements gave coefficient of variation in the intraobserver study of 3.5, 4.6, and 5.3 and in the interobserver study of 3.6, 5.9, and 6.7 for end-diastolic volume, end-systolic volume, and ejection fraction, respectively. Echocardiographic three-dimensional reconstruction was highly feasible (97%). In the intraobserver study, the corresponding values were 6.0, 7.0, and 8.9 and in the interobserver study 7.4, 10.8, and 13.4. In comparison of the methods, correlations with magnetic resonance imaging were r = 0.91, 0.91, and 0.38, and the corresponding coefficient of variations were 9.4, 10.8, and 14.7. Echocardiography derived volumes (mL/m2) were significantly higher than magnetic resonance imaging volumes in end-diastolic volume 13.7 ± 25.6 and in end-systolic volume 9.1 ± 17.0 (both P < .05). Conclusions The knowledge-based three-dimensional right ventricular volume method was highly feasible. Intra- and interobserver variabilities were satisfactory. Agreement with magnetic resonance imaging measurements for volumes was reasonable but unsatisfactory for ejection fraction. Knowledge-based reconstruction may replace magnetic resonance imaging measurements for serial follow-up, whereas magnetic resonance imaging should be used for surgical decision making.

Objective. The hypothesis was that the levels of circulating inflammatory mediators are related to the degree of volume and pressure stress on the pulmonary vasculature in children with congenital systemic to pulmonary shunts. Design. Prospective, cross-sectional study. Setting. Tertiary center covering all pediatric heart surgery and interventions in Norway. Patients. Seventy-four children, aged 0–12 years, admitted for surgical or interventional treatment of congenital systemic to pulmonary shunts. Outcome Measures. Plasma levels of eight mediators of vascular inflammation and endothelial activation, sampled from different vascular compartments. Results. Patients with the most pronounced pulmonary flow and pressure stress demonstrated no elevation of inflammatory mediator levels when compared with healthy controls. No pulmonary production or uptake of the measured markers was found. Hemodynamic explanatory factors showed weak correlations to the inflammatory marker levels by univariate analysis. Age was the only factor that significantly explained inflammatory response in the multivariate model. The presence of Down syndrome, irrespective of hemodynamic category, was associated with elevated plasma levels of soluble tumor necrosis factor receptor I, when controlling for age. Conclusions. Inflammatory mediators show no significant relationship to pulmonary hemodynamics in children with systemic to pulmonary shunts. Children with Down syndrome may have an increased inflammatory response.

Objectives. Abnormal right ventricular systolic pressure response (RVPR) during exercise has previously been demonstrated in patients with septal defects of the heart. Our study investigated whether moderate altitude affects RVPR and oxygen saturation during rest and exercise in patients with surgically closed septal defects. Design. Ten patients with surgically closed heart septal defects (six secundum atrial septal defects, four ventricular septal defects) were examined by cardiopulmonary exercise testing and by echocardiography at rest and during supine cycling at sea level. After 2 hours in a hypobaric chamber at 2500 m/8200 ft altitude, exercise echocardiography was repeated. Results. During sea level exercise four patients showed abnormal RVPR (>50 mm Hg). Acute hypoxic exposure led to right ventricular systolic pressure increase above 40 mm Hg in two patients. During altitude exercise seven patients showed abnormal RVPR. Average maximal right ventricular systolic pressure was 56.5 ± 12.7 mm Hg and average for the lowest oxygen saturation was 80.0 ± 5.7%. Two patients had simultaneous oxygen desaturation below 80% and right ventricular systolic pressure above 50 mm Hg. Conclusions. Moderate altitude affects right ventricular systolic pressure and oxygen saturation in adolescents with surgically closed ventricular or atrial septal defects. Moderate altitude may induce or aggravate abnormal RVPR and oxygen desaturation during exercise in these patients.

OBJECTIVE We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic-to-pulmonary shunts and the change in these markers during treatment with bosentan. BACKGROUND Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic-to-pulmonary shunts is largely unknown. PATIENTS AND METHODS Plasma levels of several inflammatory mediators were determined by enzyme immunoassays in 14 children and adolescents with pulmonary hypertension related to congenital systemic-to-pulmonary shunts before and after 12 months treatment with bosentan, and compared with levels in 54 healthy controls. RESULTS The patients were characterized by increased plasma levels of von Willebrand factor ( approximately 2.5-fold), C-reactive protein ( approximately 3.5-fold), and soluble CD40 ligand ( approximately 2.5-fold) as compared with controls, representing markers of endothelial cell activation, systemic inflammation, and platelet-mediated inflammation, respectively. Patients also had significantly elevated plasma levels of osteoprotegerin ( approximately 1.6-fold). Within the study group, N-terminal pro-brain natriuretic peptide levels correlated significantly with the concentrations of C-reactive protein (r= 0.61, P < .027) and von Willebrand factor (r= 0.74, P= .004). Except for a decline in monocyte chemoattractant protein-1 and receptor activator of nuclear factor-kappaB ligand, bosentan therapy did not attenuate the systemic inflammation. CONCLUSION Children and adolescents with pulmonary hypertension related to congenital systemic-to-pulmonary shunts are characterized by enhanced systemic inflammation involving increased endothelial cell activation and platelet-mediated inflammation. These inflammatory responses seem essentially to be unmodified by bosentan, potentially representing new targets for therapy in this disorder.

A technique that uses two-dimensional images to create a knowledge-based, three-dimensional model was tested and compared to magnetic resonance imaging.Measurement of right ventricular volumes and function is important in the follow-up of patients after pulmonary valve replacement. Magnetic resonance imaging is the gold standard for volumetric assessment. Echocardiographic methods have been validated and are attractive alternatives.Thirty patients with tetralogy of Fallot (25 ± 14 years) after pulmonary valve replacement were examined. Magnetic resonance imaging volumetric measurements and echocardiography-based three-dimensional reconstruction were performed. End-diastolic volume, end-systolic volume, and ejection fraction were measured, and the results were compared.Magnetic resonance imaging measurements gave coefficient of variation in the intraobserver study of 3.5, 4.6, and 5.3 and in the interobserver study of 3.6, 5.9, and 6.7 for end-diastolic volume, end-systolic volume, and ejection fraction, respectively. Echocardiographic three-dimensional reconstruction was highly feasible (97%). In the intraobserver study, the corresponding values were 6.0, 7.0, and 8.9 and in the interobserver study 7.4, 10.8, and 13.4. In comparison of the methods, correlations with magnetic resonance imaging were r = 0.91, 0.91, and 0.38, and the corresponding coefficient of variations were 9.4, 10.8, and 14.7. Echocardiography derived volumes (mL/m(2)) were significantly higher than magnetic resonance imaging volumes in end-diastolic volume 13.7 ± 25.6 and in end-systolic volume 9.1 ± 17.0 (both P.05).The knowledge-based three-dimensional right ventricular volume method was highly feasible. Intra and interobserver variabilities were satisfactory. Agreement with magnetic resonance imaging measurements for volumes was reasonable but unsatisfactory for ejection fraction. Knowledge-based reconstruction may replace magnetic resonance imaging measurements for serial follow-up, whereas magnetic resonance imaging should be used for surgical decision making.

The hypothesis was that the levels of circulating inflammatory mediators are related to the degree of volume and pressure stress on the pulmonary vasculature in children with congenital systemic to pulmonary shunts.Prospective, cross-sectional study.Tertiary center covering all pediatric heart surgery and interventions in Norway.Seventy-four children, aged 0-12 years, admitted for surgical or interventional treatment of congenital systemic to pulmonary shunts.Plasma levels of eight mediators of vascular inflammation and endothelial activation, sampled from different vascular compartments.Patients with the most pronounced pulmonary flow and pressure stress demonstrated no elevation of inflammatory mediator levels when compared with healthy controls. No pulmonary production or uptake of the measured markers was found. Hemodynamic explanatory factors showed weak correlations to the inflammatory marker levels by univariate analysis. Age was the only factor that significantly explained inflammatory response in the multivariate model. The presence of Down syndrome, irrespective of hemodynamic category, was associated with elevated plasma levels of soluble tumor necrosis factor receptor I, when controlling for age.Inflammatory mediators show no significant relationship to pulmonary hemodynamics in children with systemic to pulmonary shunts. Children with Down syndrome may have an increased inflammatory response.

Abnormal right ventricular systolic pressure response (RVPR) during exercise has previously been demonstrated in patients with septal defects of the heart. Our study investigated whether moderate altitude affects RVPR and oxygen saturation during rest and exercise in patients with surgically closed septal defects.Ten patients with surgically closed heart septal defects (six secundum atrial septal defects, four ventricular septal defects) were examined by cardiopulmonary exercise testing and by echocardiography at rest and during supine cycling at sea level. After 2 hours in a hypobaric chamber at 2500 m/8200 ft altitude, exercise echocardiography was repeated.During sea level exercise four patients showed abnormal RVPR (50 mm Hg). Acute hypoxic exposure led to right ventricular systolic pressure increase above 40 mm Hg in two patients. During altitude exercise seven patients showed abnormal RVPR. Average maximal right ventricular systolic pressure was 56.5 ± 12.7 mm Hg and average for the lowest oxygen saturation was 80.0 ± 5.7%. Two patients had simultaneous oxygen desaturation below 80% and right ventricular systolic pressure above 50 mm Hg.Moderate altitude affects right ventricular systolic pressure and oxygen saturation in adolescents with surgically closed ventricular or atrial septal defects. Moderate altitude may induce or aggravate abnormal RVPR and oxygen desaturation during exercise in these patients.