Axillary arterio-venous fistula can be used as the stage of surgical treatment and a bridge to Fontan and/or heart transplantation for this category of patients.
Aim Comprehensive evaluation of blood flow in the thoracic aorta using a software for 4D processing of magnetic resonance (MR) images of the heart and blood vessels (4D Flow) in patients with aortic coarctation in the late postoperative period.Materials and methods The MR study of the heart was performed for 10 patients (7 boys and 3 girls) aged 8 to 13 years (median, 9.5 [8.3; 10.8] years) who underwent resection with end-to-end anastomosis for aortic coarctation at age of 2 weeks to 10 months. MR tomography was performed on a 1.5 T MR scanner using a multichannel surface coil for scanning, electrocardiographic synchronization, and a specialized package of pulse sequences for scanning of the heart. Blood flow was evaluated with a 4D data handling software for processing of MR images of heart and blood vessels (4D Flow). The following blood flow parameters were analyzed: blood flow volume per second, peak blood flow velocity, peak and minimum blood flow area at the levels of ascending aorta, arch, isthmus, and descending aorta, and pressure gradient at the level of maximum narrowing of the aorta. 3D-MR images were used for evaluation of aortic geometry. Blood flow formation, distribution, and trajectories were analyzed by maps of vectors, particle trace, and stream lines. Statistical analysis was performed with a Statistica (v. 6.0 StatSoft Inc.) package.Results Accelerated flow in the region of residual aortic stenosis in systole was observed in all patients; 4 patients had an additional vortex flow below the aortic stenosis and a spiral flow in the descending aorta. The pressure gradient on the aortic isthmus was directly correlated with the left ventricular myocardial mass index (r=0.65; р=0.04) and indexes of blood flow in the ascending and descending aorta (р=0.03; р=0.026). No significant correlation was found for blood flow indexes and geometry of the aortic arch (H / L). Delayed contrast enhancement MR imaging did not detect any fibrotic changes in the myocardium in only one patient. The fibrosis severity inversely correlated with the right ventricular ejection fraction (r=0.65; р=0.04) and directly correlated with the pressure gradient at the aortic isthmus (r=0.63; p=0.05).Conclusion The 4D MR image processing software for the heart and blood vessels allows studying the blood flow in detail under natural conditions, provides potential advantages in comprehensive evaluation of patients with aortic coarctation during a dynamic follow-up. For a definitive conclusion about the relationship between the altered blood flow in the thoracic aorta and markers of residual, post-correction pathology, larger studies are required as well as long-term follow-up of patients with documented pathological patterns of blood flow (changes in blood flow velocity and volume throughout the entire thoracic aorta in combination with disorders in the normal flow geometry during the cardiac cycle).
Palliation of congenital single ventricle heart defects suggests multi-stage surgical interventions that divert blood flow from the inferior and superior vena cava directly to the right and left pulmonary arteries, skipping the right ventricle. Such system with cavopulmonary anastomoses and single left ventricle is called Fontan circulation, and the region of reconnection is called the total cavopulmonary connection (TCPC). Computational blood flow models allow clinicians to predict the results of the Fontan operation, to choose an optimal configuration of TCPC and thus to reduce negative postoperative consequences. We propose a two-scale (1D3D) haemodynamic model of systemic circulation for a patient who has underwent Fontan surgical operation. We use CT and 4D flow MRI data to personalize the model. The model is tuned to patient’s data and is able to represent measured time-averaged flow rates at the inlets and outlets of TCPC, as well as pressure in TCPC for the patient in horizontal position.We demonstrate that changing to quiescent standing position leads to other patterns of blood flow in regional (TCPC) and global haemodynamics. This confirms clinical data on exercise intolerance of Fontan patients.
Частота первичных новообразований сердца в детской популяции относительно редка и, по данным литературы, не превышает 0,32%, при этом фиброма сердца отмечается в 13-18%. Кардиальная фибромадоброкачественная опухоль, которая встречается преимущественно в детском возрасте. Может проявляться нарушениями ритма сердца, сердечной недостаточностью, внезапной смертью, при этом немалая доля пациентов остаются асимптомными, и опухоль выявляется случайно. Диагностировать новообразование сердца помогают электрокардиография и эхокардиография, однако точный диагноз позволяют установить такие дополнительные методы как компьютерная или магнитно-резонансная томография и гистологическое исследование. Тем не менее, в ряде ситуаций окончательную природу опухоли возможно установить лишь после ее резекции. В данном сообщении представлено описание этапов диагностики и последующей успешной резекции большой фибромы межжелудочковой перегородки, создававшей обструкцию выводного тракта правого желудочка, у пациента раннего возраста. Примечательно, что опухоль имела практически асимптомное течение. Новообразование впервые было выявлено в возрасте 1 месяца при выполнении плановой эхокардиографии, и в последующем ребенок в течение нескольких месяцев наблюдался кардиологом; при этом имела место тенденция к росту опухоли, однако какие-либо специфические жалобы ребенка отсутствовали.Ключевые слова: дети раннего возраста, опухоли сердца, фиброма, межжелудочковая перегородка, резекция Frequency of primary heart tumors in children is relatively rare and according to the literature data does not exceed 0.32%, while heart fibroma is registered in 13-18%. Cardiac fibroma is a benign tumor that occurs mainly in childhood. It can be manifested by heart rhythm disturbances, heart failure, sudden death, while large number of patients remain asymptomatic and tumor is revealed accidentally. Electrocardiography and echocardiography help to diagnose this heart neoplasm, but an accurate diagnosis can be made using additional methods such as computer or magnetic resonance imaging and histological examination. Nevertheless, in some situations, nature of tumor can be established only after its resection. This case report describes the stages of diagnosis and subsequent successful resection of a large fibroma of the interventricular septum, which has created the obstruction of right ventricular outflow tract, in a baby patient. It should be noticed that the course of the disease was almost asymptomatic. The tumor was first detected at the age of 1 month when performing routine echocardiography and subsequently the child was observed for several months by cardiologist; there was a tendency to the tumor increase, but there were no specific complaints from the child.
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