Abstract: Eisenmenger’s syndrome is a kind of pathophysiologic syndrome that occurs in the end stage of congenital heart disease. The diagnosis of Eisenmenger’s syndrome is clear, but its early clinical presentations are nonspecific and its prognosis is poor. Recently, the therapeutic regimen is steadily improved by the endless efforts that lots of scientists have made. Treatments including new drugs, modified surgery, extracorporeal membrane oxygenation, transplantation and gene therapy can not only relieve symptoms, correct abnormal pulmonary hemodynamics, but also can improve prognosis to some extent. We will review and introduce the advance of research on Eisenmenger’s syndrome, including its pathogenesis, diagnosis and treatment.
ObjectiveTo observe the mutation and expression of Nkx2.5 in congenital heart disease patients with diminutive pulmonary blood. We preliminarily explored the association between Nkx2.5 gene and pathogenesis of congenital heart disease patients with diminutive pulmonary blood. MethodsFifty six patients of congenital heart disease with diminutive pulmonary blood in the first affiliated hospital of Bengbu medical college and Anhui province children, s hospital between May 2012 and May 2014 were as an experimental group. Sixty three patients of ventricular septal defect were as a control group. In the trial group, there were 30 males and 26 females averagely aged 5.82± 4.23 years ranking from 6 months to 14 years. In the control group, there were 36 males and 27 females averagely aged 6.93± 4.56 years ranking from 6 months to 14 years. Before operation, peripheral venous blood of all the patients were collected. We used polymerase chain reaction combined with DNA sequencing technology to detect Nkx2.5 gene exon sequence and to analyze the association between Nkx2.5 gene mutation and congenital heart disease with diminutive pulmonary blood. And we got some hypertrophic myocardial tissue from right ventricular outflow tract in the operation, whose size was 0.5× 0.5× 0.5 cubic centimeter. And we extracted myocardial tissue RNA. The expression changes of Nkx2.5 gene mRNA were detected by real-time fluorescence quantitative polymerase chain reaction technique. ResultsThere was no mutations tested out in the peripheral venous blood in both two groups. The expression of mRNA in Nkx2.5 gene of the trial group was lower than that in the control group with a statistical difference. ConclusionNkx2.5 gene mutation may be associated with multiple factors. The occurrence of congenital heart disease with diminutive pulmonary blood may be related with a decline of Nkx2.5 gene expression in the myocardial tissue.
Abstract: Objective To summarize the immediate effects and the near and midterm followup results of transthoracic balloon valvuloplasty for newborns and infants with severe and critical pulmonary valve stenosis to find out an effective plan for onestop balloon valvuloplasty. Methods From March 2006 to March 2010, 32 patients including 23 males and 9 females with severe and critical pulmonary valve stenosis were treated in Fu Wai Hospital. Their age ranged from 5 days to 11 months (4.59±3.21 months). Weight of the patients ranged from 2.3 to 10.5 kg (6.48±2.05 kg). Dilatation was performed under general anesthesia with intubation and the guidance of echocardiography. During the follow-up period, all survivors had serial echocardiographic assessment to measure the transpulmonary pressure gradient (TPG) and the degree of pulmonary regurgitation. Results All operations were successful with no severe postoperative complications. Hemodynamic indexes were stable after operation with TPG lowered from from 82±27 mm Hg preoperatively to 23±12 mm Hg postoperatively (t=15.28, Plt;0.05). Only 4 patients had a TPG of more than 40 mm Hg on echocardiography before leaving the hospital. Tricuspid regurgitation was decreased significantly with 17 cases of nonregurgitation, 13 cases of light regurgitation and 2 cases of moderate regurgitation. Saturation of peripheral oxygen in all the patients increased to higher than 95%. Followup time ranged from 1 month to 4 years (16±11 months). The results of the follow-up were satisfying for all the patients. The average TPG was 17±10 mm Hg with only one above 40 mm Hg. Pulmonary valve regurgitation was found in 24 patients including 23 with light pulmonary regurgitation and 1 with moderate regurgitation. Conclusion Transthoracic balloon valvuloplasty for newborns and infants with severe and critical pulmonary valve stenosis is safe and effective.
Objective To investigate the iron deficiency (ID) in children with congenital heart disease (CHD) and find high risk factors of ID. Methods The clinical data of 227 pediatric patients with CHD from February to June 2016 were retrospectively analyzed. The incidence of ID according to the result of iron metabolism examination (serum ferritin <12 μg/L as the diagnostic criteria) was investigated. According to their basic CHD types, patients were divided into a cyanotic group and an acyanotic group. We tried to find the high risk factors of ID in those pediatric CHD patients by comparing their age, gender, growth condition and blood routine test results. Results There were 19.8% pediatric CHD patients complicated by ID. The incidence of ID in the cyanotic patients was higher than that in the acyanotic patients (31.0% vs. 17.3%, P=0.045). In both groups, ID patients presented the characteristics of younger age, higher anemia rate, lower mean corpuscular volume (MCV), lower mean corpuscular hemoglobin (MCH), lower mean corpuscular-hemoglobin concentration (MCHC) and longer red blood cell distribution width (RDW). Conclusion Cyanosis, younger age (infant), anemia, decreased MCV, decreased MCH, decreased MCHC and increased RDW are high risk factors of ID in CHD children.
Objective To study the feasibility, indication, technique of emergency operation and the perioperative management on critical congenital heart disease in infants. Methods From May 1998 to May 2003, 67 emergency operations of critical congenital heart disease were performed in infants . The age was 14d-32 months (mean , 11.8±8.9 months), the weight was 2.6-14.8 kg( mean, 8.4±3.0 kg). The diagnosis included ventricular septal defect in 39, tetralogy of Fallot in 13, complete atrioventricular septal defect in 5, total anomalous pulmonary venous drainage in 4, atrial septal defect with pulmonary valve stenosis in 3, D-transposition of the great arteries in 2, and postoperative pulmonary valve vegetation in 1. Corrective operation in 64 and palliative operation in 3 were done. Results The preoperative managing time was 0-9 d(mean , 3.7±2.6 d), the lasting time of intubation was 1-14d( mean, 3.8±3.2 d), and ICU staying time was 2-18 d (mean, 5.7±2.8 d). The operative mortality was 7.5% (5/67), the postoperative complications in other infants include 14 low cardiac output syndrome, 14 atelectasis, 8 lung infection, 2 worsened pulmonary hypertension, 1 pneumothorax , and 1 phrenic nerve paralysis. Following up 2-60 months with 1 case late death, the cardiac function of the others were class Ⅰ-Ⅱ/Ⅵ, the operative result was satisfactory. Conclusions The emergency operation on critical congenital heart disease in infants is safe, and can save most of their lives, the good result can be obtained from surgical treatment. Accurate choice of indications, and perioperative good nutritional and cardiorespiratory function support are important.
ObjectiveTo systematically review the association of interleukin-33 (IL-33) expression and coronary heart disease (CHD). MethodsWe searched The Cochrane Library, PubMed, EMbase, CBM, VIP, CNKI and WanFang Data up to June 30th, 2014, to collect case-control studies concerning the association of IL-33 expression with CHD. Two reviewers independently screened literature according the inclusion and exclusion criteria, extracted data and assessed the methodological quality of included studies; and then, meta-analysis was performed using the RevMan 5.2 software. ResultsSix case-control studies were included. The results of meta-analysis showed that:there were no significant differences in the levels of IL-33 between stable angina pectoris or ST-elevation myocardial infarction patients and healthy population (MD=-25.15, 95%CI -51.08 to 0.77, P=0.06; MD=-28.97, 95%CI -62.89 to 4.95, P=0.09). However, there were significant differences in the levels of IL-33 between unstable angina pectoris or non-ST-elevation myocardial infarction patients and healthy population (MD=-24.79, 95%CI -50.00 to 0.42, P=0.05; MD=-14.60, 95%CI -20.09 to -9.12, P<0.000 01). ConclusionIL-33 expression may be associated with unstable angina pectoris and non-ST-elevation myocardial infarction patients.
ObjectiveTo explore the prognostic value of serum cystatin C (Cys C) in patients with congenital heart disease-associated pulmonary arterial hypertension (PAH-CHD).MethodsA retrospective cohort study was conducted on adult PAH-CHD patients who were hospitalized for the first time in the First Affiliated Hospital of Xinjiang Medical University from January 2010 to January 2020. The serum Cys C and other related data of patients were collected. The median follow-up time was 57 months. The main end event was all-cause death. According to the prognosis, the patients were divided into a survival group and a death group. Cox regression was used to analyze the risk factors for all-cause death in patients with PAH-CHD.ResultsA total of 456 patients were enrolled, including 160 males and 296 females, aged 38.99±14.72 years. The baseline data showed that there were statistical differences in resting heart rate, serum Cys C, creatinine, NT-proB-type natriuretic peptide (NT-proBNP), high-sensitivity cardiac troponin T (hs-cTnT), high-sensitivity C reactive protein (hs-CRP), New York Heart Association (NYHA) cardiac function classification and serum potassium between the survival group and the death group. Univariate Cox regression analysis showed that serum Cys C, NT-proBNP, hs-cTnT, creatinine and NYHA cardiac function classification were related risk factors for all-cause death in patients with PAH-CHD. Multivariate Cox regression analysis showed that serum Cys C (HR=3.820, 95%CI 2.053-7.108, P<0.001), NYHA grade Ⅲ (HR=2.234, 95%CI 1.316-3.521, P=0.010), NYHA grade Ⅳ (HR=4.037, 95%CI 1.899-7.810, P=0.002) and NT-proBNP (HR=1.026, 95%CI 1.013-1.039, P<0.001) were independent risk factors for all-cause death in patients with PAH-CHD and had a good predictive value.ConclusionAs a new cardiac marker, serum Cys C can predict all-cause death in patients with PAH-CHD and is an independent risk factor.
Objective To systematically review the methodological quality of guidelines concerning pharmacological intervention for complicated hypertension. Methods The databases and relevant guideline websites such as MEDLINE, EMbase, CBM, WangFang Data, National Guideline Clearinghouse (NGC), Guidelines International Network (GIN), National Institute for Health and Clinical Excellence (NICE) and Clinical Practice Guideline Network (CPGN) were searched to collect the clinical guidelines concerning pharmacological intervention for complicated hypertension. By adopting the Appraisal of Guidelines for Research and Evaluation (AGREE), the methodological quality of guidelines was assessed. Meanwhile the similarities, differences and features of drug recommendation in guidelines for different areas and diseases were analyzed by means of analogy comparison.Results A total of 21 guidelines concerning pharmacological intervention for complicated hypertension were included. The number of guidelines concerning hypertension complicated with coronary heart disease (CHD), stroke, diabetes mellitus (DM) and kidney disease (KD) was 5, 5, 7 and 4, respectively. The publication year ranged from 2000 to 2011. According to the AGREE instrument, 19 and 2 guidelines were graded as Level B and C, respectively. The overall guidelines got low average scores in the domain of “Stakeholder involvement” and “Applicability”, including 9 evidence-based guidelines. There were totally 4 and 3 classes in terms of the level of evidence and recommendation, respectively; moreover, 10 and 6 expression forms were adopted in the level of evidence and recommendation, respectively. For hypertension with angina pectoris, -blocker (BB) and calcium channel blocker (CCB) were recommended unanimously. For hypertension with myocardial infarction, angiotensin converting enzyme inhibitor (ACEI) and BB were recommended unanimously. For hypertension with heart failure, ACEI, angiotensin-receptor blocker (ARB) and BB were recommended unanimously. For hypertension with later stage of post-stroke, 76.47% guidelines recommended diuretic (D) and ACEI. For hypertension with acute stroke, recommendations were mainly based on the guidelines developed by American Heart Association/American Stroke Association (AHA/ASA). For hypertension with DM or KD, the guidelines basically recommended that systolic/diastolic pressure should be controlled in the range of less than 130/80 mmHg. For hypertension with DM, ACEI were recommended unanimously, followed by D and CCB. For hypertension with KD, ACEI/ARB was recommended, while 3 of the 5 guidelines recommending CCB were from Asian. Conclusion The overall methodological qualities of complicated hypertension guidelines differs, with high proportion of evidence-based guidelines. The classification criteria of the levels of evidence and recommendation are still suboptimal. For hypertension with CHD, DM, KD and later stage of stroke, results from high quality clinical evidence are consistent, and the recommendations are basically unanimous, with no regional and quality difference. But in some clinical researches beyond reaching a consensus at present, the recommendation discrepancy exists, and there still remains controversy for hypertension with acute stroke.
ObjectiveTo summarize clinical results and experience of subarterial ventricular septal defect (sVSD) closure through a minimal right vertical infra-axillary incision in children, and evaluate the feasibility of this technique. MethodsClinical data of 27 sVSD patients who underwent surgical repair through a minimal right vertical infra-axillary incision from March 2009 to January of 2013 in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed. There were 20 male and 7 female patients with their age of 1.1-11.0 (4.4±2.8) years and body weight of 7.6-28.0 (14.6±5.3) kg. After induction of anesthesia, the patients were placed in a 90 degree left lateral position. The incision was performed along right midaxillary line vertically between the superior border of the third rib and inferior border of the fifth rib. The thoracic cavity was entered through the fourth intercostal space. The pericardium was opened 2 cm anterior to the phrenic nerve and suspended to elevate the heart and great vessels by the pericardial traction sutures. Cardiopulmonary bypass (CPB) was established after cannulation of the ascending aorta, superior vena cava and inferior vena cava. Closure of sVSD was performed through longitudinal pulmonary incision. After procedures under CPB finished, the aortic cannula was removed and the pericardium was closed with interrupted sutures. The thoracic drainage tube was placed through the sixth intercostal space. All the patients were followed up 3, 6 and/or 12 months after surgery. Satisfactory degree of the patients and their relatives were evaluated. Chest X-ray, electrocardiography and echocardiography were examined. ResultsSurgical repair of sVSD was performed in all the patients through this incision without any difficulty, incision prolongation or conversion to another incision. There was no in-hospital death, reexploration for bleeding, postoperative atelectasis, pulmonary infection, pleural effusion, impairment of central nervous system, phrenic nerve damage, poor wound healing, wound infection or pericardial effusion. Incision length was 4.4-7.0 (5.07±0.66) cm. Echocardiography before discharge showed no residual shunt in all the patients. All the patients were followed up 3, 6 and/or 12 months at the outpatient department or via the phone. During follow-up, there was no late death, residual shunt or chest asymmetry. The short incision under the armpit was obscured and provided favorable cosmetic results. All the patients and their relatives were satisfied with the cosmetic results. ConclusionRepair of sVSD through a minimal right vertical infra-axillary incision is feasible and safe for children. The advantages of this technique are favorable for children with sVSD.
Objective To evaluate the effectiveness and safety of simvastatin 40 mg daily use in treatment of coronary heart disease. Methods The study was designed as before-after study in the same patients. One hundred and sixty seven patients with coronary heart disease were prescribed simvastatin 40 mg daily for 3 and 6 months. Total cholestero (TC), low-density lipoproteins cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), triglycerldes (TG), ALT and creatine kinase (CK) in serum before therapy and at the end of 3 months and 6 months treatment were dectected. Continuous data were analyzed by standard difference of blocked randomization and described by mean±SD. Dunnet-t test was used for multiple comparison of trial and control groups. Statistical difference was set up at P＜0.05. Success rate was assessed by chi square test at the end of 3 and 6 months treatment. Results Simvastatin 40 mg/d significantly decreased the level of TC (P＜0.000 5), LDL-C (P＜0.000 5), TG (P＜0.05), and could elevate HDL-C (P＜0.05). There were 39.5% of patients whose LDL-C reduced below 70 mg/dl. One patient whose CK raised 5.6 times of upper line of normal range and 4 patients whose ALT raised more than 2 times of upper line of normal range withdrew. The reliability of simvastatin 40 mg/d was relatively good. Conclusions Simvastatin 40 mg/d could significantly improve the lipid profile, and is relatively reliable in treatment of coronary heart disease.