©М.О. Гончарь СТАН ФУНКЦІОНАЛЬНОЇ АДАПТАЦІЇ СЕРЦЕВО-СУДИННОЇ СИСТЕМИ У ДІТЕЙ ПІСЛЯ ХІРУРГІЧНОЇ КОРЕКЦІЇ ВРОДЖЕНИХ ВАД СЕРЦЯ Харківський національний медичний університет СТАН ФУНКЦІОНАЛЬНОЇ АДАПТАЦІЇ СЕРЦЕВО-СУДИННОЇ СИСТЕМИ У ДІТЕЙ ПІСЛЯ ХІРУРГІЧНОЇ КОРЕКЦІЇ ВРОДЖЕНИХ ВАД СЕРЦЯ. В результаті обстеження 148 школярів у 73,7% пацієнтів, які були прооперовані з приводу вроджених вад серця, у віддалені терміни спостереження виявлено зниження рівня фізичноїпрацездатності за даними велоергометрі!. У 22,1% дітей встановлено добру толерантність до фізичного навантаження, у 4,2%-задовільну, у 70,7%-знижену, низьку-у 3,0% хворих. Незадовільну толерантність до фізичного навантаження мають діти з комбінованими вадами серця, що мали ускладнення до оперативної корекції. СОСТОЯНИЕ ФУНКЦИОНАЛЬНОЙ АДАПТАЦИИ СЕРДЕЧНО-СОСУДИСТОЙ СИСТЕМЫ У ДЕТЕЙ ПОСЛЕ ХИРУРГИЧЕСКОЙ КОРРЕКЦИИ ВРОЖДЕННЫХ ПОРОКОВ СЕРДЦА. В результате обследования 148 пациентов у 73,7% детей, прооперированных по поводу врожденных пороков сердца, в отдаленные сроки наблюдения по данным велоэргометрии выявлено снижение уровня физической работоспособности. У 22,1% детей выявлена хорошая толерантность к физической нагрузке, у 4,2%-удовлетворительная, у 70,7 %-сниженная, низкая-у 3,0% больных. Неудовлетворительную толерантность кфизической нагрузке имеют дети с комбинированными пороками сердца, имевшие осложнения при оперативной коррекции. CONDITION OF CARDIOVASCULAR FUNCTIONAL ADAPTATION IN CHILDREN AFTER SURGICAL TREATMENT OF CONGENITAL HEART DISEASE. 148 patients after surgical treatment of congenital heart disease have been observed. 73,7% children had impaired tolerance to physical exertion during bicycle ergometry, among them 70,7% patients with decreased and 3,0% with low level. 22,1% children have shown good and 4,2%-satisfactory exercise tolerance. Poor tolerance to physical exertion was typical in children with combined congenital heart disease who had complications during surgery. Ключові слова: вроджені вади серця, міокардиальна дисфункція, велоергометрія. Ключевые слова: врожденные пороки сердца, миокардиальная дисфункция, велоэргометрия.

Background-Isolated cardiac conduction block is a relatively common condition in young and elderly populations. Genetic predisposing factors have long been suspected because of numerous familial case reports. Deciphering genetic predisposing factors of conduction blocks may give a hint at stratifying conduction block carriers in a more efficient way. Methods and Results-One Lebanese family and 2 French families with autosomal dominant isolated cardiac conduction blocks were used for linkage analysis. A maximum combined multipoint lod score of 10.5 was obtained on a genomic interval including more than 300 genes. After screening 12 genes of this interval for mutation, we found a heterozygous missense mutation of the TRPM4 gene in each family (p.Arg164Trp, p.Ala432Thr, and p.Gly844Asp). This gene encodes the TRPM4 channel, a calcium-activated nonselective cation channel of the transient receptor potential melastatin (TRPM) ion channel family. All 3 mutations result in an increased current density. This gain of function is due to an elevated TRPM4 channel density at the cell surface secondary to impaired endocytosis and deregulation of Small Ubiquitin MOdifier conjugation (SUMOylation). Furthermore, we showed by immunohistochemistry that TRPM4 channel signal level is higher in atrial cardiomyocytes than in common ventricular cells, but is highest in Purkinje fibers. Small bundles of highly TRPM4-positive cells were found in the subendocardium and in rare intramural bundles. Conclusions-the TRPM4 gene is a causative gene in isolated cardiac conduction disease with mutations resulting in a gain of function and TRPM4 channel being highly expressed in cardiac Purkinje fibers. (Circ Cardiovasc Genet. 2010;3:374-385.)Key Words: bundle-branch block Ⅲ heart block Ⅲ ion channel Ⅲ sudden death Ⅲ genetics C onduction block is a condition in which the depolarization wave initiated in the sinus node of the heart is slowed down or even blocked on the way to ventricular cardiomyocytes. The position of the block, its completeness (partial or complete), and the number of blocks are largely variable from one individual to the other. Conduction blocks are not rare in the general population either among young people or late in life. Right bundle-branch block (RBBB) has a prevalence of about 0.1% in normal children, with a male predominance. 1 Complete RBBB with normal left ejection fraction and no diagnoses of cardiac disease was observed in 0.3% of individuals in a population of adults with a median age of 64 years in a community-based study lead by Miller et al. 2 The natural evolution of isolated conduction blocks is unpredictable, and deciphering the genetic predisposing factors might help in unmasking those that are dangerous. Rare cases of familial conduction blocks are secondary to mutations in SCN5A 3 or NKX2.5. 4 In the latter gene, the conduction block is often associated with a congenital heart defect (typically an atrial septal defect). Recently, a South African family with cardiac conduction block was reported to carry a ...

Objective: To assess the changing profile of infective endocarditis in patients with congenital heart disease. Methods: All cases diagnosed from 1966 to 2001 (revised Duke criteria) were retrospectively reviewed and categorised in periods I (, 1990) and II (> 1990). Results: 153 episodes occurred, 81 in period I and 72 in period II. Mean age of affected patients was higher in period II. Non-operated ventricular septal defect, Rastelli correction and palliated cyanotic heart disease increased. Infective endocarditis in corrective surgery changed to patients with prosthetic material. Post-surgical cases decreased. Dental problems were the leading cause (period I 20% v II 33% of cases) with a large variety of pathological organisms (multiple species of Streptococcus). Cutaneous causative infections increased (5% to 17%) with different species of Staphylococcus. Negative blood cultures lessened (20% to 7%, p = 0.03). Streptococci were the most common causative organisms in both periods. Severe heart failure and cardiac complications lessened (20% to 4% and 31% to 18% during periods I and II, respectively). Early surgery was more frequent in period II (32% v 18.5%, p = 0.02). One-and 10-year survival was 91% v 97% in period I and 89% v 97% in period II, respectively (NS). Conclusion: Current targets include complex cyanotic disease, congenital heart disease corrected with prosthetic material and small ventricular septal defect. Postoperative cases lessened; dental and cutaneous causes increased. Survival was unchanged. Prophylactic measures targeted at dental and cutaneous sources should be emphasised.