Морфофункциональные особенности левых отделов сердца после коррекции тотального аномального дренажа легочных вен

Highlights. The geometry of the left atrium and the growth of the left heart after correction of total anomalous pulmonary venous connection are evaluated for the first time.Aim. To assess the morphological and functional characteristics of the left heart after correction of total anomalous pulmonary venous connection.Methods. 40 patients referred to the correction of total anomalous pulmonary venous connection were enrolled in a pilot, two-center, simple, blind, prospective randomized study. Patients were assigned to the sutureless repair group (n = 20) and conventional repair group (n = 20).Results. In the early postoperative period, the indexed left atrial volume in the group of sutureless repair was 15 (13.65; 17.25) versus 12.85 (10.95; 15.15) in the group of conventional repair, p = 0.057. The end diastolic volume index in the sutureless repair group was 38 (28.5; 45), while in the conventional repair group - 37.1 (31; 47.75), p = 0.48. At the follow-up, the indexed left atrial volume in the group of sutureless repair was 37 (34.5; 38.9) versus 31 (23.6; 35) in the group of conventional repair, p = 0.01. The end diastolic volume index (EDVI) in the group of sutureless repair was 50 (43; 57), while in the group of conventional repair - 50.2 (28.8; 60.9), p = 0.49.Conclusion. The growth of the left atrium depended on the chosen technique for correcting total anomalous pulmonary venous connection and was higher in the group of sutureless repair (37) compared to the group of conventional repair (31). The proportionality of the growth of the left ventricle did not depend on the surgical technique and was equivalent in both groups.Основные положения. Впервые оценены геометрия левого предсердия и рост левых отделов сердца после коррекции тотального аномального дренажа легочных вен.Цель. Оценить морфофункциональные особенности левых отделов сердца после коррекции тотального аномального дренажа легочных вен (ТАДЛВ).Материалы и методы. В представленном пилотном двуцентровом рандомизированном проспективном простом слепом исследовании выполнена оценка качества жизни 40 пациентов в возрасте до года, подвергшихся хирургической коррекции ТАДЛВ: процедура Sutureless (бесшовная коррекция, n = 20) и конвенциональная биатриальная коррекция (n = 20).Результаты. В раннем послеоперационном периоде индексированный объем левого предсердия в группе бесшовной методики составил 15 (13,65; 17,25) мл/м2, в группе конвенциональной коррекции - 12,85 (10,95; 15,15) мл/м2; p = 0,057. Индекс конечного диастолического объема в группе бесшовной методики составил 38 (28,5; 45) мл/м2, в группе конвенциональной биатриальной коррекции - 37,1 (31; 47,75) мл/м2; p = 0,48. В отдаленном периоде индексированный объем левого предсердия в группе бесшовной методики составил 37 (34,5; 38,9) против 31 (23,6;35) мл/м2 соответственно; p = 0,01. Индекс конечного диастолического объема - 50 (43; 57) против 50,2 (28,8; 60,9) соответственно; p = 0,49.Заключение. Индексированный объем левого предсердия зависел от способа коррекции ТАДЛВ и был выше в группе бесшовной методики: 37 против 31 мл/м2 в группе конвенциональной биатриальной коррекции. Пропорциональность увеличения левого желудочка не зависела от тактики вмешательства и не различалась между исследуемыми пациентами

ИНВАГИНАЦИЯ УШКА ЛЕВОГО ПРЕДСЕРДИЯ У РЕБЕНКА РАННЕГО ВОЗРАСТА ПОСЛЕ КОРРЕКЦИИ ДЕФЕКТА МЕЖЖЕЛУДОЧКОВОЙ ПЕРЕГОРОДКИ

The inversion of the left atrium appendage in infants is a very rare anomaly, which may occurs either after cardiac surgery. We introduce a case report of infant with left atrium appendage invagination after ventricular septal defect closure. Инвагинация ушка левого предсердия у детей раннего возраста является крайне редкой аномалией, которая возникает как после кардиохирургических операций, так и спонтанно при нарушениях ритма сердца. Мы представляем клинический случай у ребенка раннего возраста с инвагинацией ушка левого предсердия после коррекции дефекта межжелудочковой перегородки.

Multiple heart malformations in a patient with Holt—Oram syndrome

Holt—Oram syndrome is a rare genetic disease characterized by an abnormality of the upper limb, congenital heart disease and / or conduction abnormalities. The disease is caused by the mutations in the Tbox5 gene (allocation 12q24.21), inherited in an autosomal dominant manner. Heart septal defects and isolated thenar hypoplasia are typical congenital malformations. The article describes a clinical case of a 7-month-old girl with a family history of Holt—Oram syndrome: the absence of the first metacarpal bone of the left hand and multiple heart defects (atrial septal defect, multiple defects of the ventricular septum of the Swiss cheese type, aortic valve stenosis). The authors present a detailed clinical diagnosis of Holt—Oram syndrome, as well as genetic analysis and genetic testing of the child and immediate relatives

Effect of the dynamic curve type from the perifocal infiltration zone obtained by magnetic resonance imaging on prognosis in pelvic pathologies in women

The aim of the study is to evaluate the role and prognostic significance of magnetic resonance imaging (MRI) perfusion methods in prognosis of disease course and outcome based on evaluation of the perifocal infiltration zone in pelvic disorders in women.Materials and methods. Retrospective analysis of pelvic MRI data obtained using MRI system with induced magnetic field of 1.5 T was performed. The study included 530 protocols of pelvic scans in women. Two equal groups (n = 265) of patients with oncological and nononcological pathologies of pelvic organs were formed; after application of propensity score matching, each group contained 165 patients. All examination protocols included dynamic contrast enhancement. The obtained data were statistically analyzed using the Stata 13 software.Results. In the study, types of dynamic curves obtained using dynamic contrast enhancement from the perifocal infiltration zone were analyzed. There was no data showing a correlation between the type of dynamic curve from the zone of perifocal changes and disease prognosis or possibility of differential diagnosis.Conclusions. Evaluation of the perifocal infiltration zone per MRI results with intravenous contrast does not affect prognostic accuracy of the method (p >0.05); contrast can be used only for differential diagnosis or evaluation of local advancement of the process

EXTRACORPORAL CARDIOPULMONARY RESUSCITATION (ECPR) FOLLOWING PULMONARY ARTERY RUPTURE IN AN INFANT

The rupture of the pulmonary artery during balloon valvular dilatation is rare and the severest complication. In this clinical case we described the rupture of the pulmonary artery in a 10-month-old baby with cardiac tamponada and an extreme hemodilution due to blood loss. CPR has been using for 40 minutes but being ineffective. For 3 days we have been applying ECMO. This patient was discharged without any neurological disorders

Stenting of the right ventricular outflow tract after thrombosis of the modified Blalock-Taussig shunt in a 8 month old infant with tetralogy of Fallot and right pulmonary artery agenesis

Pulmonary artery agenesis combined with tetralogy of Fallot is the most rarely seen congenital heart disease. Children with this anomaly are an especially problematic category of patients undergoing staged surgical repair. The postoperative period quite often is complicated with shunt thrombosis while a redo open surgery is associated with a very high risk; therefore, endovascular repair is a preferred procedure. We describe a case of right ventricular outflow tract stenting in a 8 month old girl with tetralogy of Fallot and right pulmonary artery agenesis. The patient was admitted at 3 month after performing of a left-sided modified Blalock-Taussig shunt with severe signs of heart failure and desaturation caused by shunt thrombosis. Assessments performed at 2 months after stenting of the outflow tract demonstrated good oxygen saturation in arterial blood (80% and above) and improvement of heart failure symptoms to NYHA II class