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.
Abstract: The cyanotic congenital heart defect remains a focal point to study in congenital heart diseases. A successfully developed model of cyanotic congenital heart defect can contribute to a profound advancement of clinical diagnosis and treatment. Various kinds of animal models simulating cyanotic heart diseases have been created and improved step by step , such as experimental pulmonary arteriovenous fistulas, inferior vena cava-left atrium shunt, pulmonary artery-left atrium shunt and breeding animals in mionect ic environment. As an important means, they are used to investigate the animal’s pathophysilolgocal characteristics in cyanotic and hypoxic state. However, it need a further exploration since these models are not fully perfect yet.
ObjectiveTo investigate the risk factors for prolonged postoperative mechanical ventilation patients with total anomalous pulmonary venous connection (TAPVC). MethodsWe retrospectively analyzed the clinical data of 97 survived TAPVC patients in our hospital between June 2011 and December 2013. There were 55 males and 42 females with age of 4.4 (2, 12) months. The patients ventilated longer than mean time were as a prolonged ventilated group (n=50) and the others as a normal group (n=47). Perioperative variables between the two groups were compared and selected, then put into logistic regression analysis. ResultsFor the 97 survived patients, the mean ventilation time is 49 (25, 90) hours. Age, weight, pre-operative left ventricular end-diastolic dimension, atrial septal defect (ASD) caliber, inotropic drug dosage, postoperative left ventricular end-diastolic dimension, maximum pulmonary venous velocity (P < 0.01), and cardio-pulmonary bypass (CPB) time (P < 0.05) were statistically different between the two groups. In logistic regress analysis, age (OR=0.804 with 95%CI 0.71 to 0.91) and maximum pulmonary venous velocity (OR=1.016 with 95%CI 1.00 to 1.03) were risk factors for prolonged postoperative mechanical ventilation. ConclusionAge and maximum pulmonary venous velocity are the risk factors associated with prolonged postoperative mechanical ventilation in patients with TAPVC.
ObjectiveTo determine teratogenicity of beta-blockers in early pregnancy. MethodsWe searched PubMed, EMbase, Cochrane Clinical Trials, clinicaltrials.gov, CBM, Wanfang database, and CNKI from establishment of each database to December 2014. We evaluated the quality of included literature. Statistical analysis was conducted in RevMan5.3 software. ResultsFifteen population-based case-control or cohort studies were identified. The score of included studies changed from 5-7 points. Based on meta-analysis, first trimester oral beta-blocker use showed no increased odds of all or major congenital anomalies. While in analysis examining organ-specific malformations, statistically increased odds of cardiovascular (CV) defects with OR 2.21 and 95% CI 1.63 to 3.01, cleft lip/palate (CL/P) with OR 3.11 and 95% CI 1.78 to 9.89, and neural tube (NT) defects with OR 3.56 and 95% CI 1.19 to 10.67 were observed. ConclusionCausality is difficult to interpret given small number of heterogeneous studies and possibility of biases. Given the frequency of this exposure in pregnancy, further research is needed.
Objective To explore the value of extracorporeal membrane oxygenation(ECMO) combined with hypothermia therapy for children patients with refractory cardiac arrest after congenital heart disease surgery. Methods From January 2013 to June 2016, we conducted a prospective study of 23 children (18 males, 5 females at age of 7±11 months) who underwent ECMO for refractory cardiac arrest after congenital heart disease surgery. All patients were randomly divided into two groups: a standard group (11 patients) and a hypothermia group (12 patients). The patients of the standard group received standard therapy (the core body temperature maintaining at 37.0℃) and the hypothermia group received hypothermia therapy (the core body temperature maintaining at 33.0℃). The hospital discharge rate, the rate of weaning from ECMO and the morbidity were compared between the two groups. Results Eleven of 23 patients (47.8%) were weaned from ECMO successfully and 7 of 23 patients (30.4%) discharged from hospital. The hospital discharge rate between the hypothermia group (n=6, 50.0%) and the standard group (n=1, 9.1%) had no statistical difference (χ2=4.537, P=0.069). The rate of weaning from ECMO of the hypothermia group (n=9, 75.0%) was higher than that of the standard group (n=2, 18.2%, χ2=7.425, P=0.006). The morbidity between the two groups had no statistical difference. Conclusion Extracorporeal cardiopulmonary resuscitation can improve the survival rate of the children who suffered from refractory cardiac arrest after congenital heart disease surgery. There is no evidence that ECMO combined with hyperthermia therapy is better than the only ECMO in improving the discharge rate. But ECMO combined with hypothermia therapy has higher rate of weaning from ECMO than that of the only ECMO.
Objective To analyze the clinical efficacy and mid-term outcomes of reimplantation of anomalous origin of left coronary artery from the pulmonary artery (ALCAPA), and to evaluate whether concomitant management of mitral regurgitation (MR) during ALCAPA repair is needed. Methods Between March 2005 and March 2015, 52 consecutive patients (20 males and 32 females with a median age of 10 months ranging 2-193 months) underwent reimplantation of ALCAPA at Department of Cardiac Surgery, Guangdong Cardiovascular Institute. There were 14 males and 21 females with a mean age of 35.4±42.8 months not receiving mitral valvuloplasty (a Non-MVP group), and 6 males and 11 females with a mean age of 13.5±11.0 months receiving mitral valvuloplasty (a MVP group). In order to facilitate the analysis, degree of MR was graded by number: 0.0=none, 1.0=trivial, 2.0=mild, 2.5=mild-moderate, 3.0=moderate, 3.5=moderate-severe and 4.0=severe. Results The left ventricular fractional shortening (LVFS) and left ventricular end diastolic diameter (LVEDD) demonstrated significant improvement between preoperation and discharge (28.6%±9.6% vs. 32.1%±10.1%, P=0.023; 38.4±5.6 mm vs. 30.5±5.7 mm, P<0.001), and there was also significant improvement in the mean MR grade between preoperation and discharge (2.9±1.2,vs. 2.4±1.2, P=0.001). There were 4 in-hospital deaths (7.7%). The median follow-up was 21.0 months (ranging 1.5-111.0 months). Three patients (5.8%) were lost to follow-up, 1 patient required reoperation for mitral valve replacement and there was no death during follow-up. Significant improvement was seen in LVFS between discharge and final follow-up (32.1%±10.1% vs. 38.0%±6.0%, P=0.001); however, there was no significant difference in the degree of MR between discharge and final follow-up (2.4±1.2 vs. 2.3±1.2, P=0.541). There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, mechanical ventilation time or hospital stay between the two groups. Conclusion Creation of a dual coronary system with reimplantation of the left coronary artery results in complete recovery of left ventricular function. However, concomitant management of MR during ALCAPA repair remains controversial. Concomitant mitral valve repair for ALCAPA patients with moderate-severe and severe MR is helpful to early function recovery of mitral valve.
Our country has made great progress in the surgical treatment of congenital heart disease, but after entering the new era, congenital cardiac surgery also presents some new features and trends. The quality of data of Guangdong congenital heart defects monitoring network was recognized by international organizations. We analyzed the data of the incidence of congenital heart disease, the rate of surgical treatment, the mortality of surgical procedures and the quality of surgical treatment in Guangdong province from Guangdong congenital heart defects monitoring network, and then accurately understood the development characteristics of congenital cardiac surgery. It is very helpful to clarify the regional status of congenital cardiac surgery, which is conducive to the development of a more reasonable surgical strategy for congenital heart disease, and finally promote the further development of congenital cardiac surgery in China.