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.
ObjectiveTo summarize and analyze the experience of subarterial ventricular septal defect (VSD) repaired with simple pulmonary artery approach.MethodsWe retrospectively anlyzed the clinical data of 102 patients with subarterial VSD repaired with simple pulmonary artery approach in our hospital from August 2015 to October 2018. There were 67 males and 35 females at median age of 3 years (ranging 4 months to 49 years).ResultsThe median operation time was 82 (54-136) min. Median cardiopulmonary bypass time was 36 (21-62) min. The median aortic cross-clamping time was 13 (7-32) min. Thirty two patients of tracheal intubation were removed from the fast-track operating room immediately after surgery. Of the 102 patients, 67 patients underwent a small incision in the lower sternum. The median postoperative ICU stay time was 26 (13-36) h. There was no planned reoperations and no early death.ConclusionSimple pulmonary artery approach for subarterial ventricular septal defect repair with less intracardiac procedures, short operation time, less trauma, quick postoperative recovery has certain advantages in the application of specific groups.
Objective To modify the sewing technique of a hand-made bicuspid pulmonary valve using the expanded polytetrafluoroethylene in right ventricle outflow tract reconstruction for summarizing the short-term experience. Methods The patients with complex congenital heart diseases and concurrent contaminant pulmonary regurgitation that underwent right ventricle outflow tract reconstruction through the bicuspid pulmonary valve were enrolled. The postoperative artificial valve function and right ventricle function indexes were evaluated. Results A total of 17 patients were collected, including 10 males and 7 females, with an average age of 18.18 years and an average weight of 40.94 kg. Of 17 patients, 16 used valved conduit for the reconstruction of the right ventricle outflow tract with the size ranging from 18 to 24 mm. There was no patient requiring mechanical circulatory support and no in-hospital death. During the follow-up with a mean period of 12.89 months, only one vale dysfunction occurred without any complications and adverse events (P<0.001). Postoperative right atrium diameter, right ventricle diameter, and tricuspid regurgitation area significantly decreased in contrast to those preoperatively (P<0.05). Conclusion Sewing the bicuspid pulmonary valve utilizing 0.1 mm expanded polytetrafluoroethylene is a feasible, effective, and safe technique of right ventricle outflow tract reconstruction in the field of complex congenital heart diseases.
Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). MethodsA retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). ConclusionCompared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.