A novel simultaneous clot-fibrinolysis waveform analysis for assessing fibrin formation and clot lysis in haemorrhagic disorders

Simultaneous evaluation of coagulation and fibrinolysis facilitates an overall understanding of normal and pathological haemostasis. We established an assay for assessing clot formation and fibrinolysis simultaneously using clot waveform analysis by the trigger of a mixture of activated partial thromboplastin time reagent and an optimized concentration of tissue-type plasminogen activator (0·63 μg/ml) to examine the temporal reactions in a short monitoring time (<500 s). The interplay between clot formation and fibrinolysis was confirmed by analysing the effects of argatroban, tranexamic acid and thrombomodulin. Fibrinogen levels positively correlated with coagulation and fibrinolytic potential and initial fibrin clot formation was independent of plasminogen concentration. Plasminogen activator inhibitor-1-deficient (-def) and α2-antiplasmin-def plasmas demonstrated different characteristic hyper-fibrinolytic patterns. For the specificity of individual clotting factor-def plasmas, factor (F)VIII-def and FIX-def plasmas in particular demonstrated shortened fibrinolysis lag-times (FLT) and enhanced endogenous fibrinolysis potential in addition to decreased maximum coagulation velocity, possibly reflecting the fragile formation of fibrin clots. Tranexamic acid depressed fibrinolysis to a similar extent in FVIII-def and FIX-def plasmas. We concluded that the clot-fibrinolysis waveform analysis technique could sensitively monitor both sides of fibrin clot formation and fibrinolysis, and could provide an easy-to-use assay to help clarify the underlying pathogenesis of bleeding disorders in routine clinical practice.     

Results of clot waveform analysis and thrombin generation test for a plasma‐derived factor VIIa and X mixture (MC710) in haemophilia patients with inhibitors—phase I trial: 2nd report

We reported the results of a clinical pharmacological study of MC710 (a mixture of plasma‐derived FVIIa and FX) in haemophilia patients with inhibitors during a non‐haemorrhagic state. This report provides the results of a clot waveform analysis (CWA) and thrombin generation test (TGT) using blood samples obtained in this study. CWA and TGT were conducted using blood samples obtained from a pharmacokinetic and pharmacodynamic study in which MC710 (five dose rates: 20, 40, 80, 100 and 120 μg kg^?1) was compared with NovoSeven (120 μg kg^?1) and FEIBA (two dose rates: 50 and 75 U kg^?1) as control drugs in 11 haemophilia patients with inhibitors without haemorrhagic symptoms. CWA showed that MC710 provided significantly greater improvement than the control drugs in activated partial thromboplastin time (APTT) at 80 μg kg^?1; maximum clot velocity and maximum clot acceleration were more enhanced by MC710 than by control drugs. TGT revealed that MC710 significantly shortened the initiation time of thrombin generation in comparison to FEIBA and induced greater thrombin generation potency than NovoSeven. It was not clear whether or not MC710 caused significant dose‐dependent changes in the two measurements; however, differences between MC710 and the control drugs were clarified. MC710 was confirmed to have superior coagulation activity and thrombin productivity and is expected to have superior bypassing activity.     

四腔心平面头侧偏斜法筛查胎儿心脏复杂畸形的临床研究

目的探讨四腔心平面头侧偏斜法快速检测胎儿心脏复杂畸形的临床价值。方法应用彩色多普勒超声仪对37 360例妊娠16～40周的孕妇采用胎儿心脏四腔心平面头侧偏斜法进行胎儿心脏检测,对出现异常者进一步做胎儿超声心动图详细检查确诊。结果共检出胎儿复杂心脏畸形51例,漏诊6例,敏感性为89.5%,漏诊率10.5%。结论四腔心平面头侧偏斜法快速检测胎儿心脏复杂畸方法简单,敏感性高,值得在基层医院推广。     

Mechanical thrombectomy using the AngioJect in a child with congenital heart disease.

Acute thrombosis of a vascular channel can be a serious and perhaps fatal complication. We describe a patient who developed complete thrombosis of the left pulmonary artery following catheter placement of a stent in this vessel shortly after open heart surgery. The thrombosis was successfully removed using the Possis AngioJet mechanical thrombectomy catheter.     

Bioelectrical impedance analysis in the management of heart failure in adult patients with congenital heart disease

Objective: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.
Design: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]), laboratory values, and HF-related admission prevalence.
Results: Patients with New York Heart Association (NYHA) functional classes III-IV had a higher EI than those with NYHA classes I-II (mean ± SD, 0.398 ± 0.011 vs 0.384 ± 0.017, P < .001). EI was significantly correlated with brain natriuretic peptide level (r = 0.51, P < .001). During the mean follow-up period of 7.1 months, KaplanMeier analysis showed that a discharge EI > 0.386, the median value in the present study, was significantly associated with a future increased risk of HF-related admission (HR = 4.15, 95% CI = 1.70-11.58, P < .001). A body weight reduction during hospitalization was also related to EI reduction.
Conclusions: EI determined using BIA could be a useful marker for HF severity that could predict future HF-related admissions in adult patients with CHD.     

Evaluation of depression,anxiety, and stress status in parents of patient with congenital clubfoot treated with Ponseti method: A prospective study

Congenital diseases have been reported to increase the incidence of depression, anxiety, and stress among parents. In this study, we aimed to investigate the depression, anxiety, and stress status in parents of patients with congenital clubfoot before and after treatment with the Ponseti method. A total of 20 patients diagnosed with congenital clubfoot at our clinic and treated with the Ponseti method were included in this study. The Depression Anxiety Stress Scale-21 (DASS-21) was used to evaluate the depression, anxiety, and stress status of the parents before and after treatment. We considered the following parameters to investigate the effects of these on the parents: the educational level of the parents; economic status of the family; gender; birth order of the child in the family; time of diagnosis (prenatal or postnatal). The mean DASS and subdomain scores after treatment were significantly lower than those before treatment (P < .05). Moreover, there was a significant difference in the pre- and posttreatment mean DASS and depression scores of the participants in terms of the education level (P < .05). The pre- and posttreatment DASS and depression scores of the participants with an education level of primary school and below were lower than those of the participants with an education level of secondary and high school. Parents may be less concerned during this process if they are fully informed by the orthopedic surgeons about the treatment protocol and the near-perfect results of the Ponseti method as well as are counseled by healthcare professionals.     

双向腔肺分流术在复杂先心病治疗中的应用

目的探讨运用双向腔静脉-肺动脉分流术（BCPS）治疗复杂先心病的疗效及安全性。方法47例复杂先心病患者,21例在体外循环下,26例在非体外循环下完成BCPS手术。共行单侧BCPS术30例,双侧BCPS术17例。结果术后同术期死亡3例,病死率6．38％。随访35例,有7例术后2年左右进行二期根治手术,2例术后2年死亡,余26例心彩超复查无吻合口狭窄,发绀得到不同程度的缓解,心功能改善,生活质量明显提高。结论双向腔静脉-肺动脉分流术对于不能一期矫治的功能性单心室类复杂先心病患者是一种有效的姑息手术方法,非体外循环下行腔肺吻合术有助于心、肺功能的早期恢复,还可减少患者的住院开支,是安全、可行且有利的。     

Narrative analysis of adults with complex congenital heart disease: Childhood experiences and their lifelong reverberations

Background: With access to surgical care, >90% of today’s infants with congenital heart disease (CHD) will reach adulthood. During childhood, survivors accrue a wealth of health care experience and develop strategies for navigating life with a chronic disease.
Methods: Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis—an established qualitative research method for studying how individuals derive meaning from their personal stories. Audio recordings of 2‐4 hour free‐form interviews were transcribed and iteratively analyzed to identify common themes and detect similarities or differences in language, viewpoint and interpretation. Recruitment continued until saturation was reached (n = 10).
Results: While each narrative was unique, CHD had a pervasive effect on the autobiography of all participants. Seven themes were discussed consistently: (1) parental/sibling relationships, (2) physical limitations, (3) embarrassment/denial, (4) memories of pediatric health care, (5) transition to adult care, (6) education and career choices, and (7) relationship and reproductive choices. While some of the recalled experiences were negative, all participants also spoke positively about the effect of CHD on their lives.
Conclusions: Adults with CHD provide the voices of expert witnesses; illuminating how the pediatric journey influences their identity, choices, personal relationships and adult health care interactions. These narratives could inform and improve the contemporary care of children with heart disease.     

儿童先天性心脏病介入治疗436例临床分析

目的总结儿童先天性心脏病介入治疗的临床经验,分析其疗效。方法2007年1月至2013年6月新疆维吾尔自治区人民医院北院共施行0～14岁儿童先天性心脏病介入封堵治疗436例,其中继发孔型房间隔缺损213例,室间隔缺损83例,动脉导管未闭139例,房间隔缺损合并动脉导管1例。房间隔缺损、室间隔缺损及动脉导管未闭封堵术均采用Amplatzer法。结果全组患儿无手术死亡病例,封堵成功423例（97．4％）,房间隔缺损组、室间隔缺损组和动脉导管未闭组的成功封堵率分别为97．7％（208／213）,94．0％（79／83）,98．6％（137／139）。室间隔缺损封堵术后早期心律失常发生率较高（15．2％）。全组门诊心脏超声心动图、心电图、胸部X线片随访3-36个月,无残余分流、死亡及严重心脏事件。结论经导管介入治疗先天性心脏病创伤小、安全、可靠、成功率高,部分患者可替代外科手术。     

Recommendations to organize care for adults with congenital heart disease in the Czech Republic

This paper was formed by the Expert committee for congenital heart disease (CHD) in adults – a division of Czech Society of Cardiology (ČKS). It was designed as an appendix to National cardiovascular programme ČKS created in 2013 and was based on Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology created in 2014 [1].Aims of this paper are: To optimize medical care in all its aspects for adults with CHD in the Czech Republic, to facilitate easy transition between paediatric and adult medical care, to enable research in the field in order to create evidence based care, to support training of regional cardiologists and other specialists who are involved in monitoring adult patients with CHD, to help with communication with national institutions, to provide information for other medical workers and patients, to consolidate resources.     

The impact of interventional cardiology for the management of adults with congenital heart defects.

The objective of this study is to assess the impact of interventional cardiology procedures for the management of ACHD. The interventional approach to the management of CHD in the adult population is becoming increasingly recognized as the preferred treatment option for a wide number of congenital cardiac conditions. The files of all consecutive patients over 18 years of age who were hospitalized in our department from January 2000 to December 2004 were reviewed. Over the study period, 1,115 ACHD (583 women; mean age 41 +/- 13.8, years, range 18-72 years) were hospitalized in our department; 752 patients underwent cardiac catheterization and 82.4% of them had an interventional procedure carried out. ASD (329/620) and PFO (159/620) closure account for 78% of all the procedures carried out, with a 2.7% of major complications incidence (all of them closing ASDs). Other procedures such as stenting aortic coarctation (40/620), ventricular septal defect closure (33/620), patent ductus arteriosus embolization (30/620), pulmonary valvuloplasty (12/620), stenting pulmonary artery branches (8/620), etc (5/620) were carried out. The most important complication was one death, which occurred in the case of a 22-year-old woman after stent implantation for a recurrent aortic coarctation. A trivial residual shunt was detected in only 5% of the patients who had a 6-month follow-up after VSD closure; no residual shunt was found after PDA embolization during the 12-month follow-up. In conclusion, we believe that the interventional approach is a safe and successful treatment option for a wide number of congenital cardiac conditions. The increasing use of catheter interventions for these patients will be responsible for an increase of complex cases in surgery.     

Efficiency of the home cardiac rehabilitation program for adults with complex congenital heart disease

Objective: We aimed to study the efficiency and safety of once‐a‐week outpatient rehabilitation followed by home program with tele‐monitoring in patients with com‐ plex cyanotic congenital heart disease.
Design: Prospective nonrandomized study.
Method: Patients who have been diagnosed either Eisenmenger’s syndrome or inop‐ erable complex cyanotic heart disease and able to attend 12‐week cardiac rehabilita‐ tion program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once‐a‐week in the first 6 weeks followed by home‐based exercise program (bicycle and walking) with a target at 40%‐70% of maximum heart rate (HRmax) at pretraining peak exercise for another 6 weeks was performed in the intervention group. Video and telephone calls were scheduled for evaluation of compliance and complication. Data from cardiopulmonary exercise testing (CPET) on cycle ergometry including peak oxygen consumption (peakVO2), oxygen pulse (O2 pulse), ventilatory equivalent for carbon dioxide (VE/CO2 at an‐ aerobic threshold), constant work‐rate endurance time (CWRET) at 75% of peak VO2, and 6‐minute walk distance (6MWD) were compared between baseline and after training by paired t test.
Result: Of the 400 patients in our adult congenital heart disease clinic, 60 patients met the inclusion criteria. Eleven patients who could follow program regularly were assigned home program. There was a statistically significant improvement of CWRET, O₂ pulse, and 6MWD after finishing the program (P = .003, .039, and .001, respec‐ tively). The mean difference of 6MWD change in the home‐program group was sig‐ nificantly higher than in the control group (69.3 ± 47.9 meters vs. 4.1 ± 43.4 meters, P = .003). No serious adverse outcomes were reported during home training.
Conclusion: Once‐a‐week outpatient hospital‐based exercise program followed by supervised home‐based exercise program showed a significant benefit in improve‐ ment of exercise capacity in adults with complex cyanotic congenital heart disease without serious adverse outcomes.