体外循环对紫绀型先天性心脏病病人血小板的影响

检测１８例体外循环紫绀型先天性心脏病手术病人术前，术中及术后３，８天外周血血小数量和 附，聚集功能，探讨体外循环对血小板质和量的影响。结果显示，血小板数量和聚集功能在术后显著下降并持续至术后８天，血小板粘附功能显著下降，术后３天恢复。提示体外循环气血界面，人工材料非内皮表面可导致血小板激活，粘附，聚集面在量消耗，数量和功能显著下降。     

Molecular markers of cardiomyopathy in cyanotic pediatric heart disease

Cyanotic congenital heart disease (CHD) accounts for approximately 25% of all types of CHD, encompassing a variety of cardiac anomalies. Children with cyanotic CHD are at risk for heart failure, cardiomyopathy, and arrhythmias. In addition to the hemodynamic burden, recent data suggest that hypoxia may contribute to heart failure. Previous studies have shown that neonatal hypoxia results in significant myocardial gene expression alterations that persist in adulthood after the termination of the hypoxic stimulus in the neonatal period or early infancy. In this article we review the current knowledge on molecular biomarkers of cyanotic CHD pathobiology, and expand on how the current knowledge establishes the basis for future studies to further define the role of molecular tools in cyanotic CHD to improve diagnostic, prognostic and therapeutic strategies.     

肾区域组织氧饱和度预测紫绀型患儿心脏手术后急性肾损伤的效果

目的探讨紫绀型先天性心脏病患儿心脏手术围术期肾区域组织氧饱和度(RrSO_2)对术后急性肾损伤(AKI)的预测价值。方法选取心肺转流(CPB)下接受心脏手术的紫绀型先天性心脏病患儿36例,男22例,女14例,年龄≤1岁,分别于气管插管后5 min(T_1)、升主动脉夹闭后5 min(T_2)、升主动脉开放后5 min(T_3)、CPB结束(T_4)、超滤结束(T_5)、CPB结束后3 h(T_6)、CPB结束后8 h(T_7)、CPB结束后24 h(T_8)记录MAP、RrSO_2、鼻咽温,同时抽取动脉血和中心静脉血进行血气分析,记录动脉血氧饱和度(SaO_2)、动脉血Hb、乳酸(Lac)、中心静脉血氧饱和度(ScvO_2),根据改善全球肾脏病预后组织(KDIGO)诊断标准将患儿分为两组:急性肾损伤组(D组)和正常组(N组)。单因素分析两组患儿的临床资料,将不同时点MAP、RrSO_2、鼻咽温、Hb、SaO_2、Lac、ScvO_2值、有意义变量纳入二元Logistic回归分析,采用受试者工作特征(ROC)曲线分析RrSO_2对AKI的预测价值。结果 T_3—T_6时D组RrSO_2明显低于N组(P0.01)。两组不同时点MAP、鼻咽温、SaO_2、Hb、Lac和ScvO_2差异无统计学意义。二元Logistic回归分析显示,T_3—T_6时RrSO_2是紫绀型先天性心脏病患儿心脏术后AKI的危险因素(P0.05)。ROC曲线分析结果显示,T_3—T_6时RrSO_2临界值分别为60.2%、57.2%、55.0%、54.0%,敏感性分别为66.7%、83.3%、66.7%、83.3%,特异性分别为100%、92%、100%、75%,曲线下面积分别为0.896、0.861、0.792、0.840。结论 RrSO_2可作为紫绀型先天性心脏病患儿心脏AKI的一项无创预测指标。     

Brain abscesses in cyanotic heart disease

Sixty cases of brain abscesses in patients with cyanotic heart disease is reported. Forty four (73.3%) belonged to pediatric age group. There was only single case of infratentorial abscess located in the cerebellar paravermian region. The abscesses were multiloculated in 42%, multiple in 10% and solitary in 48% cases. Streptococci was the commonest (77%) micro-organism isolated from pus culture. Forty eight patients were treated by aspiration through twist drill, 7 patients conservatively and 5 had primary excision of abscesses. Forty four patients improved in their neurological status, three had fixed neurological deficits and 5 patients deteriorated further. There were 8 deaths. Complication like cyanotic spells was recorded in 17% cases treated by aspiration. None of the patients treated by twist drill aspiration had seizure. It is suggested that aspiration of abscesses through twist drill hole and antibiotic therapy could be the treatment of choice. Vascular slowing in a localised area resulting in infraction and thus preparing the focus for abscess formation is the possible mechanism in the evolution of these abscesses.     

Cardiac MRI in Congenital Heart Disease – Our Experience

A significant recent advance that has occurred world over in the continuously evolving field of Magnetic Resonance Imaging (MRI) practice is the introduction of Cardiac applications. Cardiac MRI has moved to the centre stage of clinical management strategy by non-invasively imaging the structure as well as function of the heart. It has a wide range of specific applications such as delineation of morphological anatomy, quantification of flow and pressure across cardiac valve dysfunction, evaluation of myocardial function, assessment of infarcts, mapping coronary arteries and so on. Evaluation of congenital heart disease (CHD) is an important application of Cardiac MRI since the morphological details of chambers, septum, defects and anomalous connections are depicted accurately. Besides, flow information across valves, chambers, outflow tracts and shunts are also provided. This article describes our experience in the use of cardiac MRI in congenital heart disease.Key Words: Cardiac MRI, Congenital heart disease, Cyanotic and Acyanotic heart disease     

‘Hair-on-end’ skull changes resembling thalassemia caused by marrow expansion in uncorrected complex cyanotic heart disease

Hair-on-end skull changes resembling thalassemia were rarely described in the 1950s and 1960s in children with cyanotic congenital heart diseases; these changes were described almost entirely in patients with tetralogy of Fallot or D-transposition of the great arteries. As these lesions have become correctable, the osseous changes, never common, seem now only to exist in a small number of patients with uncorrectable complex cyanotic congenital heart disease who survive in a chronic hypoxic state. We present two cases: a case of marked marrow expansion in the skull of a 5-year-old boy with uncorrectable cyanotic heart disease studied by CT, and a second case of an 8-year-old with tetralogy of Fallot and pulmonary atresia studied by plain skull radiographs. The true incidence of these findings is unknown.     

Approach to congenital heart disease in the neonate

Congenital heart disease in the newborn is a unique and complex problem faced by both pediatricians and cardiologists as it requires skillful handling and balancing of both neonatal issues as well as cardiac physiology. Babies with cyanotic and acyanotic heart disease are physiologically different from each other and management has to be tailored to the individual diagnosis and clinical status. Rapid diagnosis and appropriate management is the key to reducing mortality and morbidity in this fragile patient population. The following review deals with the common presentations of heart problems in a newborn baby and methods of diagnosis, investigative modalities, recent advances and approach to management     

Brain abscess in children, experience Of Mashad University

Objective： Brain abscesses continue to pose a problem in neurosurgery, and it is a serious life-threatening complication for several diseases. The objective of this research was to lock at the clinical profile of children suffering from brain abscess, predisposing conditions, microbiology and outcome of these patients. Methods： Ninety children aged less than 15 years were reviewed. The clinical presentation, predisposing factors, diagnosis, management and outcome were noted. There were 56（ 62.2% ） males and 34 （ 37.7% ） females. The mean age of presentation was 5.7 ± 4.2 years. Results： The mean duration of illness at the time of presentation was 10.6 ± 12.4 days. Typically patients presented with fever, vomiting, headache, neurological deficit, and seizures. The predisposing conditions found were cyanotic congenital heart disease in 18 （20 % ） of children, meningitis in 6 （6.7 % ）, Otitis or mastoiditis in 32 （35.5 % ）, Head trauma in 6 （ 6.7 % ）, previous intraeranial surgery in 8 （8.8 % ） and no underlying cause was found in 22 （24.4 % ） children. The most common microbe in children with cyanotic congenital heart disease was of the Streptococcus group （54 % ）. Computerized tomography confirmed the diagnosis and the most common location of the abscess was the parietal lobe of the cerebral hemisphere. That abscesses were larger than 2 cm in diameter where aspirated surgically. Excision was performed for eight children. Six children expired, two due to intracranial bleeding and the others due to severe cerebral edema and tentorial herniation. Complications were observed in 11 children out of which six had squeal and five had bemiparesis. Conclusion： Attention to hygiene of mouth and teeth in infant and child patients with cyanotic heart disease is very important in prevention of infectious brain diseases. We must also pay attention to ear-nose-throat diseases of children. That is very important the patients be examined again after treatment because of finding and trea     

Perioperative monitoring of thromboelastograph on blood protection and recovery for severely cyanotic patients undergoing complex cardiac surgery

In this study, we assessed the clinical effect of a new transfusion therapy guided by thromboelastograph (TEG) on blood protection. Thirty-one children with severe cyanosis (hematocrit ≥54%), who were diagnosed as having transposition of the great arteries or double outlet right ventricle with or without pulmonary valve stenosis, and underwent arterial switch operation or double roots transplantation, were involved and were divided into two groups. In group F (n=17), the transfusion therapy after cardiopulmonary bypass was performed with fibrinogen administration combined with traditional transfusion, guided by TEG. In group C (n=14), traditional transfusion guided by clinical experiences only was performed. We observed the blood protection effects and recovery conditions of these patients. In surgery, compared with group C, the chest closure time, fresh-frozen plasma (FFP), and platelet (PLT) volume used at closure time had no significant reductions in group F (P>0.05, respectively), and the patients in group F had no significant reductions in the amount of chest drainage (P>0.05). The total PLT and total red blood cells usage were also the same (P>0.05). But during the first 24h, FFP usage in the intensive care unit (ICU) and total perioperative FFP usage had significantly dropped in group F (P<0.05); the mechanical ventilator time, ICU stay, and hospitalization time in group F were much shorter than those in group C (P<0.05). So, TEG was effective in perioperative blood protection. Fibrinogen could be a substitute for FFP to restore hemostasis and improve the prognosis for these patients.