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Williams syndrome is a multisystem, congenital disorder which is commonly associated with arterial stenoses: supravalvar aortic stenosis and peripheral pulmonary artery stenosis. Venous abnormalities have not been previously reported in children with Williams syndrome. We present a case of a 3-year-old girl with Williams syndrome and diffuse venous ectasia as detected by MRI.
Diagnosing prosthetic valve infective endocarditis in children is challenging. Transthoracic and transesophageal echocardiography can yield false-negative results. Data are lacking in paediatric multimodality imaging in prosthetic valve infective endocarditis. We present two children with repaired CHD where initial echocardiogram was non-diagnostic, while CT angiogram and 18F-fluorodeoxyglucose positron emission tomography in combination with CT angiography, respectively, confirmed the diagnosis of endocarditis affecting clinical management.
Ventricular pseudoaneurysm is a rare but well-known complication after valvular endocarditis. The lesion was localised exactly where pre-operative CT scan showed lack of enhancement in the postero-lateral wall of the left ventricle. This case demonstrates how much attention must be paid to myocardial CT images and emphazises the need of close follow-up in patients with infective endocartitis.
We present a case of pulmonary venous baffle obstruction in a child with a history of congenitally corrected transposition status post double switch repair. We highlight two forms of volume rendering three-dimensional reconstructions from computed tomographic data which allowed for detailed pre-surgical planning. These reconstructions emphasise the concept of maximizing previously obtained two-dimensional data in a time-efficient and cost-effective manner. The benefits of these reconstructions are reviewed, highlighting the relatively novel virtual dissection reconstruction technique that appeared identical to what the surgeon encountered in the operating theatre. This technique allowed the surgeon to quickly advance a preconceived detailed surgical repair.
We describe percutaneous repair of severe pulmonary regurgitation and a right ventricular outflow tract pseudoaneurysm in a 19-year-old patient after repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. A 3D printed model of his heart was used to simulate percutaneous repair with a closure device in the aneurysm neck and a Venus P-valve in the right ventricular outflow tract. The encouraging findings from the simulation allowed us to plan the complex procedure effectively with a successful outcome and avoidance of surgery.
Coronary-pulmonary artery fistula is a rare anomaly in which an aortopulmonary collateral artery arises from a coronary artery, often seen in patients with pulmonary atresia with ventricular septal defect. In the presented case, a coronary-pulmonary artery fistula arose from the left main coronary artery and supplied blood flow to a left upper lobe segment. The life-sized three-dimensional printed model was helpful in pre-surgical planning for unifocalisation of the aortopulmonary collateral arteries.
We present a pilot case using an innovative fusion of echocardiogram and MRI achieved with a MATLAB-based imaging programme to explore the feasibility of this imaging strategy in the functional and anatomic assessment of a patient with repaired tetralogy of Fallot requiring pulmonary valve intervention. Echocardiogram and MRI neutralises the disadvantages and limitations of each individual imaging modality and yields important anatomic and haemodynamic information crucial to the treatment decision-making process. Future image fusion strategies can apply to three-dimensional images and image-directed therapy for CHD.
The purpose of the study was to analyse whether prolonged and regular physical training in children leads to changes in myocardial systolic deformation and rotational mechanics. For that purpose, cardiac MRI feature tracking was performed retrospectively in 35 pre-adolescent male soccer players and 20 matched controls. There were no changes in global strain, but left ventricular twist and apical rotation were greater in soccer players, which adds to the features of paediatric athlete’s heart.