Williams syndrome is a congenital multisystem disease. Cardiovascular abnormality caused by elastin deficiency is the main cause of morbidity and mortality in Williams syndrome patients. Recent studies have found that 80% of Williams syndrome patients have cardiovascular abnormalities, most of which are arterial stenosis, especially the aortic valve stenosis and pulmonary artery stenosis. Operation is the main method to treat the stenosis of the artery, and the results of the operation on the aortic valve stenosis in most centers are good, but the effect of transcatheter intervention is still not obvious, pulmonary artery reconstruction has a good effect on the treatment of peripheral pulmonary artery stenosis. Advances in genetic diagnosis, surgical techniques and treatment regimens are expected to significantly improve cardiovascular outcomes in these patients. This article reviews the latest research progress of Williams syndrome combined with cardiovascular disease.
Congenital heart disease (CHD) is a series of structural cardiac abnormalities resulting from abnormal fetal heart development. With the prolongation of survival time, their cognitive function problems begin to be concerned. From fetus period to adulthood, people with complex CHD are more likely to have abnormalities in brains. Children with complex CHD have a significantly increased risk of developmental disorders in cognitive functions, such as intelligence, attention and psychosocial disorders. These diseases persist into their adulthood. Adults with CHD have earlier neurocognitive decline, poorer performance in intelligence, executive function, attention and academic achievement, and are more likely to have mood disorders, higher incidence of mental disorders and lower quality of life. This paper reviews the studies on cognitive function of adult patients with CHD from the dimension of the whole life cycle.
Objective To evaluate the surgical characteristics, methods and clinical effect of pediatric valvoplasty through an analysis of valvoplasty in children in the age from 15 h to 14 years. Methods From January 1993 to June 2003, 376 children underwent valvoplasty. There were 349 cases (92.8%) of congenital heart disease and 27 cases(7.2%) of acquired heart disease. The procedure included mitral valvoplasty in 79 (19.4%), tricuspid valvoplasty in 159 (39.1%), aortic valvoplasty in 40 (9.8%), and pulmonary valvoplasty in 129(31.7%). Results There were 12 early deaths (3.2%) after the procedure. 297 patients were followed up from 1 month to 10 years (mean, 4.9±2.4 years). There were 2 late deaths (0.7%) after discharge. There were 5 cases (1.7%) of re-operation due to valve problem. Conclusions Valvoplasty should be the first choice in dealing with valve diseases in children. The effect of the valvoplasty works on the prognosis of the operation.
While the Fontan operation has improved the survival of a generation of children born with a functional single ventricle, it does not recreate a normal circulation. However, significant challenges remain. Early stage mortality risk seems stubbornly high. The risk of late cowplications seriously affect the longterm survival of children. As new techniques and therapies exist, more single ventricle patients survive till adulthood. Therefore, the limits of Fontan procedures is more and more evident. In recent years, the study of mechanical cavopulmonary assisting device, which addresses the limitations of Fontan circulation, has been developed and provided a more stable and effective biventricular of blood flow in the total cavopulmonary connection in existence. This would benefit not only the treatment of late Fontan complications, but also facilitating early surgical repair, which is promising.
Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although techniques such as annuloplasty, chordal elongation, and the use of pericardial patches can improve AVV function in many patients, a number of patients continue to have significant AVV insufficiency and may require AVV replacement. The present review made a comprehensive literature review concerning the outcome, optimal timing, key points of technique skills and prognosis evaluation.
Congenital heart disease refers to the structural or functional abnormality of the macrovascular in the heart or thoracic cavity caused by the failure of the formation of the heart and large blood vessels during the embryonic development or the abnormal closure of the heart or the closure of the channel after birth. In the past few years, a new and broader definition of structural heart disease has been gradually proposed. Structural heart disease narrowly refers to the pathological and physiological changes of the heart caused by abnormal anatomical structures in the heart, including congenital heart disease. A few decades ago, congenital heart disease was considered as a pediatric disease, because most patients with severe lesions rarely survive to adulthood. Due to recent advances in echocardiography, anesthesia, intensive care, percutaneous intervention, especially cardiac surgery in recent decades, the treatment and intervention strategies for congenital heart disease in children have been greatly improved, a fatal defect in childhood can now be successfully repaired or alleviated. Because of these successes, more than 90% of congenital heart disease patients are expected to survive to adulthood, which has led to emerge a new population: adult patients with congenital heart disease. Adult congenital heart disease patients are different from children. Pulmonary hypertension leads to right heart failure and eventually progresses to whole heart failure. The appearance of Eisenmenger syndrome leads to severe cyanosis and worsening of the disease. At present, the continuous development of mechanical assisted circulation support devices and heart or cardiopulmonary transplantation technology has increased the survival rate of end-stage adult congenital heart disease patients with heart failure. The high incidence of cardiovascular events in pregnant patients requires comprehensive multidisciplinary team care and early coordination planning for delivery, including early counseling for pregnancy-related risks, close monitoring of cardiac function and regular scan of fetal assessment. The prenatal and postpartum integrated diagnosis and treatment model and the development of intrauterine treatment technology reduce the incidence of congenital heart disease in adults from the source through fetal intervention. Other complications such as arrhythmia, infective endocarditis, cerebrovascular accidents, and other medical underlying metabolic diseases also challenge future diagnosis and treatment. The incidence and epidemiology of adult congenital heart disease, pulmonary hypertension and end-stage heart failure complications, as well as prenatal and postpartum integrated diagnosis and treatment and intrauterine treatment are summarized in this review.
The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success of modern medicine. With the development of diagnostic techniques, surgical procedures and interventional techniques, more than 90% of CHD children can survive to adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients aged above 60 years. Standardized guidelines can provide good theoretical support for the comprehensive management of adult CHD. Ten years after the European Society of Cardiology guidelines for the management of grown-up CHD released in 2010, the new version was officially released in August 2020. The new version of guidelines updated the classification and stratification of diseases, comprehensive intervention methods and intervention timing, and put forward some new concepts, new intervention standards and methods. For adult CHD that has not been repaired or needs to be repaired again, the indication and mode of surgical intervention and perioperative management have a great impact on the prognosis. The new version of the guidelines provides a detailed description of the surgical and intervention indications and methods for different diseases, and clarifies the management methods for high-risk groups. This article attempts to interpret this newly updated guideline from the perspective of a surgeon, sort out several key diseases introduced by the guideline, and strives to provide a concise and actionable guideline for domestic counterparts.
Objective To summarize the clinical experience of cardiac surgery during pregnancy in a single center for the past 11 years. MethodsThe clinical data of 26 pregnant patients (mean age 28.6±4.9 years) complicated with heart diseases who underwent non-emergency cardiac surgery with cardiopulmonary bypass from 2010 to 2020 in Guangdong Provincial People's Hospital were retrospectively analyzed. Patients were divided into two groups according to the gestational age at the time of surgery: a change group (gestational age<21 weeks) and a stable group (gestational age≥21 weeks). The hospitalization data and follow-up data of the patients were collected. ResultsMean gestational age at surgery was 23.4±4.2 weeks. Eleven patients had congenital heart diseases and fifteen had valvular heart diseases. Meanpostoperative ICU stay was 2.5±2.4 d, and mean total hospital stay was 22.5±9.5 d. There were 5 postoperative fetal losses. There was no maternal death during follow-up. No statistical difference in the maternal postoperative outcomes between two groups. ConclusionThe number of patients undergoing cardiac surgery during pregnancy is increasing. The maternal mortality rate is low and the prognosis is good, but the fetal loss remains concern. Cardiac surgery performed before or after the establishment of cardiopulmonary adjustment in pregnancy does not change the maternal postoperative outcomes.
ObjectiveTo explore a standardized model for cardiothoracic surgery resident training program based on a combined clinical and research oriented team approach. Methods We conducted this study in a nationally ranked Class AAA hospital among the eleven residents who were accepted into the program. Throughout their training periods, clinical surgical skills were taught by one-on-one mentoring by individually assigned doctoral degree advisors, other attending surgeons and doctoral degree candidates in the program. To foster their scientific curiosity and research skills, regular didactic lectures in basic science and statistical skills by the doctoral thesis advisors, supplemented by monthly journal clubs during which trainees were required to present and discuss a previously assigned topics based on recent clinical cases within the department, thus combining acquisition of clinical skills and theory/research at the same setting. ResultsEleven physicians were selected for this training path. With respect to clinical surgical skills, 4 residents were judged to be sufficient at the end of the first cycle. The other seven residents at the end of the second cycle. One trainee won the third prize in the Medical Skill Competition in the hospital. As for scientific research skills, the team produced 11 scientific articles, one of which was accepted by the annual national conference in cardiothoracic surgery and the first author was invited to present the article. The team submitted four research projects, one won funding at the provincial competition and three within the medical school. ConclusionOur proposed standardized model of cardiothoracic surgery resident training based on a combined clinical and research oriented approach appears to be practical with moderate success. Adaptation of this model by other cardiothoracic surgery training programs in the nation will further attest to its value and functionality.