Respiratory Muscle Function and Exercise Performance in Adult Patients with Fontan Circulation.

At rest, a good Fontan circulation can provide a normal cardiac output (CO). However, as a consequence of its unique hemodynamic nature, the limitations of the Fontan circuit are exposed during exercise. We aimed to provide a comprehensive assessment of the pathophysiology of exercise in adult Fontan patients (FPs) and identify factors limiting their functional capacity (FC). In a single-center study conducted in 37 FPs aged ≥16 years and 19 healthy-controls (HCs) who underwent CPET on a cycle ergometer in February and March 2022, the mean peakVO2 was 21 ± 5.4 mL/kg/min, which was 55% of the predicted value. Morphologically, the left single ventricle showed a higher peakVO2% predicted value (57.4 ± 14.4% vs. 43.4 ± 8.1%, p = 0.045). The factors associated with low peakVO2 values were an early flattened or descending O2 pulse at maximal exertion (52 ± 14% vs. 62 ± 12.5, p = 0.04 and 47.6 ± 9% vs. 60 ± 14, p = 0.018, respectively) and chronotropic insufficiency (53 ± 12% vs. 69.8 ± 20%, p = 0.008). The OUES was found to be a useful parameter to assess the FC in FPs in maximal and submaximal exercise testing. A strong positive correlation was observed between the %OUES and peakVO2%predicted (r = 0.726, p > 0.001). The lung function was impaired in the FPs, mostly with a mild restrictive pattern (56.8%). The FPs showed lower inspiratory muscle strength compared to the HCs but it was not statistically associated with either the peakVO2 or VE/VCO2 slope. Regular intense physical activity improves one's FC. Although FPs have inspiratory muscle weakness, its impact on their FC is unclear. The peakVO2% predicted grew progressively higher as the level of physical activity increased (low level 49.5 ± 14%, moderate level 55 ± 12%, intense level 69 ± 20%).

Giant right atrium, what does it hide? Case presentation.

BACKGROUND: Malformation of the right atrium is a rare cardiac abnormality and is usually reported as isolated malformation in the literature. CASE PRESENTATION: Prenatal giant atrial dilatation in an asymptomatic infant was treated surgically at 18 months of age, due to potential risk of thrombosis and arrhythmias. Post-surgical echocardiographic images illustrate residual atrial elevated pressure. CONCLUSIONS: Sometimes, as seems in our case, right atrial dilatation hides an associated restrictive right ventricle.

Improved passive catheter tracking with positive contrast for CMR-guided cardiac catheterization using partial saturation (pSAT).

BACKGROUND: Cardiac catheterization is a common procedure in patients with congenital heart disease (CHD). Although cardiovascular magnetic resonance imaging (CMR) represents a promising alternative approach to fluoroscopy guidance, simultaneous high contrast visualization of catheter, soft tissue and the blood pool remains challenging. In this study, a novel passive tracking technique is proposed for enhanced positive contrast visualization of gadolinium-filled balloon catheters using partial saturation (pSAT) magnetization preparation. METHODS: The proposed pSAT sequence uses a single shot acquisition with balanced steady-state free precession (bSSFP) readout preceded by a partial saturation pre-pulse. This technique was initially evaluated in five healthy subjects. The pSAT sequence was compared to conventional bSSFP images acquired with (SAT) and without (Non-SAT) saturation pre-pulse. Signal-to-noise ratio (SNR) of the catheter balloon, blood and myocardium and the corresponding contrast-to-noise ratio (CNR) are reported. Subjective assessment of image suitability for CMR-guidance and ideal pSAT angle was performed by three cardiologists. The feasibility of the pSAT sequence is demonstrated in two adult patients undergoing CMR-guided cardiac catheterization. RESULTS: The proposed pSAT approach provided better catheter balloon/blood contrast and catheter balloon/myocardium contrast than conventional Non-SAT sequences. It also resulted in better blood and myocardium SNR than SAT sequences. When averaged over all volunteers, images acquired with a pSAT angle of 20° to 40° enabled simultaneous visualization of the catheter balloon and the cardiovascular anatomy (blood and myocardium) and were found suitable for CMR-guidance in >93% of cases. The pSAT sequence was successfully used in two patients undergoing CMR-guided diagnostic cardiac catheterization. CONCLUSIONS: The proposed pSAT sequence offers real-time, simultaneous, enhanced contrast visualization of the catheter balloon, soft tissues and blood. This technique provides improved passive tracking capabilities during CMR-guided catheterization in patients.

3D printed models in patients with coronary artery fistulae: anatomical assessment and interventional planning.

AIMS: Coronary artery fistulae represent one of the most challenging anatomical defects to define accurately. We aimed to investigate the additional benefit conferred by volume rendering of tomographic images and 3D printing for diagnosis and interventional planning. METHODS AND RESULTS: Four cases of coronary fistulae were considered for transcatheter closure. Multidetector computed tomography (three cases) or cardiac magnetic resonance (one case) images were acquired and segmented using Mimics software. Each case was reviewed after incremental consideration of diagnostic resources: two cardiologists reported source and volume-rendered images; device closure was discussed by the interventional cardiology team. All diagnoses and planned management were reviewed after inspection of a 3D model. Using source images alone, both cardiologists correctly described the course and drainage in two out of four cases. Aided by volume rendering, this improved to three out of four cases. Inspection of the 3D printed model prompted the planned interventional approach and device sizing to be altered in two out of four cases. In one out of four cases, the intervention was abandoned after inspection of the 3D printed model. CONCLUSIONS: Diagnosis and management of patients with coronary artery fistulae rely on detailed image analyses. 3D models add value when determining the feasibility of, and the approach to intervention in these cases.

Utilidad de la angiografía rotacional 3D en el diagnóstico de origen anómalo de arteria coronaria izquierda en tronco pulmonar / Use of 3D-rotational angiography in the diagnosis of the anomalous origin of the left coronary artery in the pulmonary artery

No disponible

Resultados a largo plazo de la valvuloplastia transluminal percutánea en la estenosis valvular pulmonar en población pediátrica / Long-term Results of Percutaneous Balloon Valvuloplasty in Pulmonary Valve Stenosis in the Pediatric Population

Introducción y objetivos La valvuloplastia pulmonar percutánea se ha convertido en el tratamiento de elección en los casos de estenosis valvular pulmonar. El objetivo de este estudio es evaluar la efectividad, determinar los factores predictores de éxito y analizar los resultados a largo plazo en población pediátrica. Métodos El estudio comprende a 53 pacientes con estenosis valvular pulmonar sometidos a valvuloplastia percutánea en el periodo entre diciembre de 1985 y diciembre de 2000. En el control realizado a largo plazo, se analizaron parámetros ecocardiográficos de tamaño y función de ventrículo derecho, presencia de insuficiencia pulmonar y el gradiente transvalvular pulmonar residual. Resultados El gradiente transvalvular pulmonar disminuyó de 74 [intervalo intercuartílico, 65-100] a 20 [intervalo intercuartílico, 14-34] mmHg. El procedimiento fue fallido en 2 pacientes (3,77%). La tasa de éxito inmediato se situó en el 73,58%. El tiempo de seguimiento mostró una mediana de 15 [intervalo intercuartílico, 10-24] años. Todos los pacientes en el seguimiento mostraban algún grado de insuficiencia pulmonar, en el 58,4% de los casos de grado II y en el 31,2%, de grado III . Hubo un único caso de reestenosis a largo plazo (2,1%), y tenían dilatación ventricular derecha grave el 27,1% de los pacientes. Según los parámetros estudiados, no hubo casos de disfunción ventricular derecha significativa. No fue preciso el recambio valvular pulmonar en ninguno de los casos. Conclusiones La valvuloplastia transluminal percutánea con catéter balón es una técnica efectiva en el tratamiento de la estenosis valvular pulmonar, con buenos resultados a largo plazo (AU)

Introduction and objectives Percutaneous pulmonary valvuloplasty is the preferred interventional procedure for pulmonary valve stenosis. The aim of this study was to evaluate the effectiveness of this technique, assess the factors leading to its success, and determine the long-term results in the pediatric population. Methods The study included 53 patients with pulmonary valve stenosis undergoing percutaneous balloon valvuloplasty between December 1985 and December 2000. Right ventricular size and functional echocardiographic parameters, such as pulmonary regurgitation and residual transvalvular gradient, were assessed during long-term follow-up. Results Peak-to-peak transvalvular gradient decreased from 74 mmHg [interquartile range, 65-100 mmHg] to 20 mmHg [interquartile range, 14-34 mmHg]. The procedure was unsuccessful in 2 patients (3.77%). The immediate success rate was 73.58%. Follow-up ranged from 10 years to 24 years (median, 15 years). During follow-up, all patients developed late pulmonary regurgitation which was assessed as grade II in 58.4% and grade III in 31.2%. There was only 1 case of long-term restenosis (2.1%). Severe right ventricular dilatation was observed in 27.1% of the patients. None of the patients developed significant right ventricular dysfunction. Pulmonary valve replacement was not required in any of the patients. Conclusions Percutaneous balloon valvuloplasty is an effective technique in the treatment of pulmonary valve stenosis with good long-term results (AU)

Long-term results of percutaneous balloon valvuloplasty in pulmonary valve stenosis in the pediatric population.

INTRODUCTION AND OBJECTIVES: Percutaneous pulmonary valvuloplasty is the preferred interventional procedure for pulmonary valve stenosis. The aim of this study was to evaluate the effectiveness of this technique, assess the factors leading to its success, and determine the long-term results in the pediatric population. METHODS: The study included 53 patients with pulmonary valve stenosis undergoing percutaneous balloon valvuloplasty between December 1985 and December 2000. Right ventricular size and functional echocardiographic parameters, such as pulmonary regurgitation and residual transvalvular gradient, were assessed during long-term follow-up. RESULTS: Peak-to-peak transvalvular gradient decreased from 74 mmHg [interquartile range, 65-100 mmHg] to 20 mmHg [interquartile range, 14-34 mmHg]. The procedure was unsuccessful in 2 patients (3.77%). The immediate success rate was 73.58%. Follow-up ranged from 10 years to 24 years (median, 15 years). During follow-up, all patients developed late pulmonary regurgitation which was assessed as grade II in 58.4% and grade III in 31.2%. There was only 1 case of long-term restenosis (2.1%). Severe right ventricular dilatation was observed in 27.1% of the patients. None of the patients developed significant right ventricular dysfunction. Pulmonary valve replacement was not required in any of the patients. CONCLUSIONS: Percutaneous balloon valvuloplasty is an effective technique in the treatment of pulmonary valve stenosis with good long-term results.