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Alagille syndrome, caused by mutations in the gene encoding Jagged1 (JAG1), a ligand in the Notch signaling pathway, is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, face and skeleton. The aim of the present study is try to disclose the clinical features, management and outcomes of pulmonary artery stenosis associated with Alagille syndrome. By comprehensive literature retrieval, 38 articles involving 401 patients were recruited for this study. The pertinent variables closely related to pulmonary artery stenosis in patients with Alagille syndrome were comprehensively analyzed by following the PRISMA guidelines. The management of pulmonary artery pathologies, especially a severe type of pulmonary artery stenosis in Alagille syndrome, is a concerned matter. Publications of literature retrieval of recent 3 decades were the study material of this article. The pulmonary artery pathologies, especially the severe type of pulmonary artery stenosis in Alagille syndrome, warrant surgical or interventional treatments. After the procedures, the right ventricular to left ventricular pressure ratio was reduced by 25%. There were no intergroup differences in terms of recovery, reintervention and mortality rates between interventionally and surgically treated patients. Transcatheter treatment is preferable due to less trauma. Surgical treatment of pulmonary artery stenosis can be performed currently with intracardiac defect repair.

The aim of the present study is to describe the indications, treatment effects, and patient outcomes of percutaneous management of left ventricular pseudoaneurysm (LVPA). The study materials were based on comprehensive literature retrieval since 2004. The mechanisms of LVPA formation can be divided into surgical, percutaneous, and medial disease related. Of the surgical mechanisms, coronary artery bypass grafting prevailed. The formation time was the longest in medical disease-related LVPAs up to 44.4 months. The percutaneous procedures succeeded on the first try in 79 (84.9%) patients, whereas failures were encountered during the percutaneous manoeuvres in 14 (15.1%) patients. Percutaneous closure of LVPA was especially indicated for patients carrying a high surgical risk. The iatrogenic traumas, such as left ventricular venting, should be avoided to prevent this complication. The preliminary cut-off valves of oversize 3.3 mm and oversize ratio 1.6 should be followed for reference for device choice.

Left ventricular pseudoaneurysm (LVPA) is a rare complication of myocardial infarction. The clinical characteristics and treatment of choice in the current era remain to be elaborated. The present article aims to give an overview of post-infarct LVPA and discuss the management strategy and outcomes. The study was based on comprehensive retrieval of literature of the recent 10 years. Myocardial infarctions as the underlying causes of LVPAs were mostly acute myocardial infarctions. The mean time for LVPA formation was 51.7 months after myocardial infarction. Postoperative and follow-up ejection fraction values were significantly higher than preoperative. In the interventional group patients, the oversize ratio of patients in whom devices remained in position was a little larger than that of those with a migrated one, but lack of a statistical significance (1.32±0.25 vs. 1.25±0.21, p=0.707). The mortality rate was significantly higher in the conservative group than in the surgical and interventional groups. Post-infarct LVPAs are curable to surgical aneurysmectomy and left ventricular reconstruction, thereby avoiding unexpected LVPA ruptures and other fatal complications. Elderly patients and patients at a high operative risk may resort to interventional therapy. The conservatively treated patients inevitably carry a considerable risk of death. An oversize ration of >1.3 might be a reference value for preventing device migration in the interventional group patients.

Abstract Background A parachute tricuspid valve is a very rare congenital cardiac anomaly. Its morphological features and clinical implications have not been sufficiently described so far. The purpose of the present systematic review is to disclose the morphological and clinical characteristics of parachute tricuspid valve, and to discuss its diagnostic methods, treatments and patients’ outcomes. Main body The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement guidelines were followed in this systematic review. Publications were systematically searched in the PubMed, Highwire Press, and the Cochrane Library databases. By comprehensive retrieval of the pertinent literature published between 1979 and 2019, 13 reports were collected with 14 patients recruited into this study. Their ages ranged from neonate to 52 years old with a median age of 23 years. Tricuspid valve regurgitation of a less-than-severe degree was seen in 6 (60%) patients, tricuspid valve stenosis was present in 3 (30%) patients and normally functioning tricuspid valve was noted in 1 (10%) patient. All patients had a single papillary muscle in the right ventricle. The chordae tendineae could be normal in length and thickness, or elongated, or shortened and thickened. Forty percent of the patients were asymptomatic or with only mild symptoms and did not need a surgical or interventional therapy, and 6 (60%) patients were indicated for a surgical/interventional treatment due to their severe presenting symptoms, associated congenital heart defects, and the resultant severe right ventricular inflow obstruction and (or) tricuspid stenosis. Patients’ outcomes varied depending on the substantial status of the patients with a survival rate of 70% and mortality rate of 30%. Conclusion A few patients with a parachute tricuspid valve are asymptomatic or only with mild symptoms and a surgical or interventional treatment is not required. The surgical/interventional indications for parachute tricuspid valve patients are their severe presenting symptoms, associated congenital heart defects, and the resultant severe right ventricular inflow obstruction and (or) tricuspid stenosis. The survival rate of this patient setting is satisfactory.

Abstract Objective: To give an overview of the Ortner’s syndrome caused by an aortic arch aneurysm. Methods: By comprehensive retrieval of the pertinent literature published in the past two decades, 75 reports including 86 patients were collected and recruited into this study along with a recent case of our own. Results: The aortic arch aneurysms causing hoarseness were most commonly mycotic aneurysms. In this patient setting, in addition to the left recurrent laryngeal nerve, trachea was the most commonly affected structure by the aortic arch aneurysm. Surgical/interventional/hybrid treatments led to a hoarseness-relieving rate of 64.3%, much higher than that of patients receiving conservative treatment. However, hoarseness recovery took longer time in the surgically treated patients than in the interventionally treated patients. Conclusion: The surgical and interventional treatments offered similar hoarseness-relieving effects. Surgical or interventional treatment is warranted in such patients for both treatment of arch aneurysms and relief of hoarseness.

Abstract Introduction Fetal arrhythmias are a common phenomenon with rather complicated etiologies. Debates remain regarding prenatal diagnosis and treatment of fetal arrhythmias. Methods The literature reporting on prenatal diagnosis and treatment of fetal arrhythmias published in the recent two decades were retrieved, collected and analyzed. Results Both fetal magnetocardiogram and electrocardiogram provide information of cardiac time intervals, including the QRS and QT durations. M-mode ultrasound detects the AV and VA intervals, fetal heart rate, and AV conduction. By using Doppler ultrasound, simultaneous recording of the atrial and ventricular waves can be obtained. Benign fetal arrhythmias, including premature contractions and sinus tachycardia, do not need any treatment before and after birth. Sustained fetal arrhythmias that predispose to the occurrence of hydrops fetalis, cardiac dysfunction or eventual fetal demise require active treatments. Intrauterine therapy of fetal tachyarrhythmias has been carried out by the transplacental route. If maternal transplacental treatment fails, intraumbilical, intraperitoneal, or direct fetal intramuscular injection of antiarrhythmic agents can be attempted. Conclusions The outcomes of intrauterine therapy of fetal tachyarrhythmias depend on the types or etiology of fetal arrhythmias and fetal conditions. Most are curable to a transplacental treatment by the first-line antiarrhythmic agents. Fetal cardiac pacings are effective methods to restore sinus rhythm in drug-resistant or hemodynamically compromised cases. Immediate postnatal pacemaker implantation is warranted in refractory cases.

Fetal arrhythmias warrant sophisticated surveillance and management, especially for the high-risk pregnancies. Clinically, fetal arrhythmias can be categorized into 3 types: premature contractions, tachyarrhythmias, and bradyarrhythmias. Fetal arrhythmias include electrocardiography, cardiotocography, echocardiography and magnetocardiography. Oxygen saturation monitoring can be an effective way of fetal surveillance for congenital complete AV block or SVT during labor. Genetic surveillance of fetal arrhythmias may facilitate the understanding of the mechanisms of the arrhythmias and provide theoretical basis for diagnosis and treatment. For fetal benign arrhythmias, usually no treatment but a close follow-up is need, while persistant fetal arrhythmias with congestive heart dysfunction or hydrops fetalis, intrauterine or postnatal treatments are required. The prognoses of fetal arrhythmias depend on the type and severity of fetal arrhythmias and the associated fetal conditions. Responses of fetal arrhythmias to individual treatments and clinical schemes are heterogeneous, and the prognoses are poor particularly under such circumstances. Keywords: arrhythmia, fetus, fetal therapies, prenatal care

Acute kidney injury (AKI) is a common complication of pediatric cardiac surgery and is associated with increased morbidity and mortality. Literature of AKI after pediatric cardiac surgery is comprehensively reviewed in terms of incidence, risk factors, biomarkers, treatment and prognosis. The novel RIFLE (pediatric RIFLE for pediatrics), Acute Kidney Injury Network (AKIN) and Kidney Disease Improving Global Outcomes (KDIGO) criteria have brought about unified diagnostic standards and comparable results for AKI after cardiac surgery. Numerous risk factors, either renal or extrarenal, can be responsible for the development of AKI after cardiac surgery, with low cardiac output syndrome being the most pronounced predictor. Early fluid overload is also crucial for the occurrence of AKI and prognosis in pediatric patients. Three sensitive biomarkers, neutrophil gelatinase-associated lipocalin, cystatin C (CysC) and liver fatty acid-binding protein, are regarded as the earliest (increase at 2–4 h), and another two, kidney injury molecule-1 and interleukin-18 represent the intermediate respondents (increase at 6–12 h after surgery). To ameliorate the cardiopulmonary bypass techniques, improve renal perfusion and eradicate the causative risk factors are imperative for the prevention of AKI in pediatric patients. The early and intermediate biomarkers are helpful for an early judgment of occurrence of postoperative AKI. Improved survival has been achieved by prevention, renal support and modifications of hemofiltration techniques. Further development is anticipated in small children. Key Words: acute kidney injury, cardiac surgical procedures, pediatrics

ABSTRACT Background Mosaicism poses challenges for genetic counseling and preimplantation genetic testing for monogenic disorders (PGT‐M). NGS‐based PGT‐M has been extensively used to prevent the transmission of monogenic defects, but it has not been evaluated in the application of PGT‐M resulting from mosaicism. Methods Four women suspected of mosaicism were confirmed by ultra‐deep sequencing. Blastocyst trophectoderm cells and polar bodies were collected for whole genome amplification, followed by pathogenic variants detection and haplotype analysis based on NGS. The embryos free of the monogenic disorders were transplantable. Results Ultra‐deep sequencing confirmed that the four women harbored somatic mosaic variants, with the proportion of variant cells at 1.12%, 9.0%, 27.60%, and 91.03%, respectively. A total of 25 blastocysts were biopsied and detected during four PGT cycles and 5 polar bodies were involved in one cycle additionally. For each couple, a wild‐type embryo was successfully transplanted and confirmed by prenatal diagnosis, resulting in the birth of four healthy infants. Conclusions Mosaic variants could be effectively evaluated via ultra‐deep sequencing, and could be prevented the transmission by PGT. Our work suggested that an NGS‐based PGT approach, involving pathogenic variants detection combined with haplotype analysis, is crucial for accurate PGT‐M with mosaicism.

Pulmonary artery hypertension (PAH) is very rare in childhood, and it can be divided into heritable, idiopathic drug- and toxin-induced and other disease (connective tissue disease, human immunodeficiency virus infection, portal hypertension, congenital heart disease, or schistosomiasis)-associated types. PAH could not be interpreted solely by pathophysiological theories. The impact of the transforming growth factor-β superfamily-related genes on the development of PAH in children remains to be clarified. Pertinent literature on the transforming growth factor-β superfamily-related genes in relation to PAH in children published after the year 2000 was reviewed and analyzed. Bone morphogenetic protein receptor type II gene mutation promotes cell division or prevents cell death, resulting in an overgrowth of cells in small arteries throughout the lungs. About 20% of individuals with a bone morphogenetic protein receptor type II gene mutation develop symptomatic PAH. In heritable PAH, bone morphogenetic protein receptor type II mutations may be absent; while mutations of other genes, such as type I receptor activin receptor-like kinase 1 and the type III receptor endoglin (both associated with hereditary hemorrhagic telangiectasia), caveolin-1 and KCNK3, the gene encoding potassium channel subfamily K, member 3, can be detected, instead. Gene mutations, environmental changes and acquired adjustment, etc. may explain the development of PAH. The researches on PAH rat model and familial PAH members may facilitate the elucidations of the mechanisms and further provide theories for prophylaxis and treatment of PAH. Key Words: bone morphogenetic proteins, mutation, pulmonary hypertension

Pediatric cardiomyopathies are a group of myocardial diseases with complex taxonomies. Cardiomyopathy can occur in children at any age, and it is a common cause of heart failure and heart transplantation in children. The incidence of pediatric cardiomyopathy is increasing with time. They may be associated with variable comorbidities, which are most often arrhythmia, heart failure, and sudden death. Medical imaging technologies, including echocardiography, cardiac magnetic resonance, and nuclear cardiology, are helpful in reaching a diagnosis of cardiomyopathy. Nevertheless, endomyocardial biopsy is the final diagnostic method of diagnosis. Patients warrant surgical operations, such as palliative operations, bridging operations, ventricular septal maneuvers, and heart transplantation, if pharmaceutical therapies are ineffective. Individual therapeutic regimens due to pediatric characteristics, genetic factors, and pathogenesis may improve the effects of treatment and patients' survival. Key Words: cardiomyopathy, classification, pediatrics

Ebstein's anomaly is uncommon. Genetic bases of this congenital heart defect may be related to the mutations in myosin heavy chain 7 and NKX2.5, among others. Asymptomatic patients with Ebstein's anomaly can be conservatively treated and kept under close follow-up, whereas surgical operation is indicated for those patients with evidence of right heart dilation and progressively impaired ventricular systolic function. A biventricular repair consisting of the reconstruction of a competent monocuspid tricuspid valve, right ventriculorrhaphy, subtotal atrial septal defect closure, and aggressive reduction atrioplasty is suitable for most patients, and 1.5-ventricular repair (bidirectional Glenn shunt) is indicated for patients with poor right ventricular function; by contrast, heart transplantation is used in patients with severe left ventricular dysfunction.

Fetal primary cardiac tumors are rare, but they may cause complications, which are sometimes life threatening, including arrhythmias, hydrops fetalis, ventricular outflow/inflow obstruction, cardiac failure, and even sudden death. Among fetal primary cardiac tumors, rhabdomyomas are most common, followed by teratomas, fibromas, hemangiomas, and myxomas. Everolimus, a mammalian target of rapamycin inhibitor, has been reported to be an effective drug to cause tumor remission in three neonates with multiple cardiac rhabdomyomas. Neonatal cardiac surgery for the resection of primary cardiac tumors found by fetal echocardiography has been reported sporadically. However, open fetal surgery for pericardial teratoma resection, which was performed successfully via a fetal median sternotomy in one case report, could be a promising intervention to rescue these patients with large pericardial effusions. These recent achievements undoubtedly encourage further development in early management of fetal cardiac tumors. Owing to the rarity of fetal primary cardiac tumors, relevant information in terms of prenatal diagnosis, treatment, and prognosis remains to be clarified.

Abstract Aortic dissection (AD) has been recognized to be associated with an inflammatory process. Clinical observations demonstrated that patients with AD had an elevated interleukin (IL)-6 level in comparison to hypertensive or healthy controls. Adverse events such as acute lung injury, postimplantation syndrome, and death are associated with an elevated IL-6 level. Thus, circulating IL-6 could be a reliable biomarker for the diagnosis of AD and for the eveluation of the therapeutic outcomes and the prognosis of AD patients. Therapeutic interventions aiming at attenuating the inflammatory status by IL-6 neutralization could effectively decrease the IL-6 level and thus reverse the progression of the disorder of AD patient. Endovascular aortic repair can effectively control the inflammatory cytokines. Selective antegrade cerebral perfusion with deep hypothermic circulatory arrest during aortic arch replacement shows better neuroprotectve effect with an improved IL-6 level of the cerebrospinal fluid. These results facilitate the understanding of the etiology of AD and guide the directions for the treatment of acute AD in the future. More effective therapeutic agents developed based on the theories of IL-6 signaling involved in the mechasims of AD are anticipated.

Fetal cardiac interventions for congenital heart diseases may alleviate heart dysfunction, prevent them evolving into hypoplastic left heart syndrome, achieve biventricular outcome and improve fetal survival. Candidates for clinical fetal cardiac interventions are now restricted to cases of critical aortic valve stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum and evolving hypoplastic right heart syndrome, and hypoplastic left heart syndrome with an intact or highly restrictive atrial septum as well as fetal heart block. The therapeutic options are advocated as prenatal aortic valvuloplasty, pulmonary valvuloplasty, creation of interatrial communication and fetal cardiac pacing. Experimental research on fetal cardiac intervention involves technical modifications of catheter-based cardiac clinical interventions and open fetal cardiac bypass that cannot be applied in human fetuses for the time being. Clinical fetal cardiac interventions are plausible for midgestation fetuses with the above-mentioned congenital heart defects. The technical success, biventricular outcome and fetal survival are continuously being improved in the conditions of the sophisticated multidisciplinary team, equipment, techniques and postnatal care. Experimental research is laying the foundations and may open new fields for catheter-based clinical techniques. In the present article, the clinical therapeutic options and experimental fetal cardiac interventions are described.

Abstract Aberrant origin of vertebral artery is rare. The anatomical features and clinical significance of this lesion remain to be clarified. A comprehensive collection of the pertinent literature resulted in a cohort of 1286 cases involving 955 patients and 331 cadavers. There were more left than right and more unilateral than bilateral aberrant vertebral arteries. Patients with aberrant origin of vertebral artery were often asymptomatic and in only 5.5% of the patients their symptoms were probably related to the aberrant origin of vertebral artery. The acquired cardiovascular lesions were present in 9.5% of the patients, 20.9% of which were vertebral artery-associated lesions. Eight (0.8%) patients had a vertebral artery dissection. Logistic regression analysis showed significant regressions between bovine trunk and left vertebral artery (P=0.000), between the dual origins of vertebral artery and cerebral infarct/thrombus (P=0.041), between associated alternative congenital vascular variants and cervical/aortic dissection/atherosclerosis (P=0.008). Multiple logistic regression demonstrated that side of the aberrant origin of vertebral artery (left vertebral artery) (P=0.014), arch branch pattern (direct arch origin) (P=0.019), presence of the common trunk (P=0.019), associated acquired vascular disorder (P=0.034) and the patients who warranted management (P=0.000) were significant risk predictors for neurological sequelea. The patients with neurological symptoms and those for neck and chest operations/ interventions should be carefully screened for the possibility of an aberrant origin of vertebral artery. The results from the cadaver metrology study are very helpful in the design of the aortic stent. The arch branch pattern has to be taken into consideration before any maneuver in the local region so as to avoid unexpected events in relation to aberrant vertebral artery.

ABSTRACT α-smooth muscle actin, encoded by ACTA2 gene, is an isoform of the vascular smooth muscle actins, typically expressed in the vascular smooth muscle cells contributing to vascular motility and contraction. ACTA2 gene mutations cause a diversity of diffuse vasculopathies such as thoracic aortic aneurysms and dissections as well as occlusive vascular diseases, including premature coronary artery disease and ischemic stroke. Dynamics of differentiation-specific α-smooth muscle actin in arterial smooth muscle cells and proliferation of the proteins have been well described. Although a variety of research works have been undertaken in terms of modifications of α-smooth muscle actin and mutations of ACTA2 gene and myosin, the underlying mechanisms towards the pathological processes by way of gene mutations are yet to be clarified. The purpose of the present article is to describe the phenotypes of α-smooth muscle actin and implications of ACTA2 mutations in vasculopathies in order to enhance the understanding of potential mechanisms of aortic and coronary disorders.

ABSTRACT OBJECTIVE: This study aims to present an updated clinical picture of the infected cardiac myxoma. Revankar & Clark made a systematic review of infected cardiac myxoma based on the literature before 1998. Since then, there has not been any updated information describing its recent changing trends. METHODS: A comprehensive literature search of infected cardiac myxoma was conducted on MEDLINE, Highwire Press and Google between 1998 and 2014. RESULTS: In comparison with Revankar & Clark's series, the present series disclosed a significantly decreased overall mortality. It is believed that refinement of the prompt diagnosis and timely management (use of sensitive antibiotics and surgical resection of the infected myxoma) have resulted in better outcomes of such patients. CONCLUSION: The present series of infected cardiac myxoma illustrated some aggravated clinical manifestations (relative more occasions of high-grade fever, multiple embolic events and the presence of refractory microorganisms), which should draw enough attention to careful diagnosis and treatment. In general, the prognosis of infected cardiac myxoma is relatively benign and the long-term survival is always promising.

AbstractObjective:Cardiac myxoma in pregnancy is rare and the clinical characteristics of this entity have been insufficiently elucidated. This article aims to describe the treatment options and the risk factors responsible for the maternal and feto-neonatal prognoses.Methods:A comprehensive search of the literature of cardiac myxoma in pregnancy was conducted and 44 articles with 51 patients were included in the present review.Results:Transthoracic echocardiography was the most common diagnostic tool for the diagnosis of cardiac myxoma during pregnancy. Cardiac myxoma resection was performed in 95.9% (47/49); while no surgical resection was performed in 4.1% (2/49) patients (P=0.000). More patients had an isolated cardiac myxoma resection in comparison to those with a concurrent or staged additional cardiac operation [87.2% (41/47) vs. 12.8% (6/47), P=0.000]. A voluntary termination of the pregnancy was done in 7 (13.7%) cases. In the remaining 31 (60.8%) pregnant patients, cesarean section was the most common delivery mode representing 61.3% and vaginal delivery was more common accounting for 19.4%. Cardiac surgery was performed in the first, second and third trimester in 5 (13.9%), 14 (38.9%) and 17 (47.2%) patients, respectively. No patients died. In the delivery group, 20 (76.9%) neonates were event-free survivals, 4 (15.4%) were complicated and 2 (7.7%) died. Neonatal prognoses did not differ between the delivery modes, treatment options, timing of cardiac surgery and sequence of cardiac myxoma resection in relation to delivery.Conclusion:The diagnosis of cardiac myxoma in pregnancy is important. Surgical treatment of cardiac myxoma in the pregnant patients has brought about favorable maternal and feto-neonatal outcomes in the delivery group, which might be attributable to the shorter operation duration and non-emergency nature of the surgical intervention. Proper timing of cardiac surgery and improved cardiopulmonary bypass conditions may result in even better maternal and feto-neonatal survivals.

Abstract Objective: The diagnosis and treatment of sternal wound infections with mycobacteria are challenging. Such an infection is often associated with a delayed diagnosis and improper treatment that may lead to a worsened clinical outcome. The present study is designed to highlight its clinical features so as to facilitate a prompt diagnosis and timely treatment. Methods: MEDLINE, Highwire Press, and Google search engine were searched for publications in the English language, with no time limit, reporting on sternal wound infection caused by tuberculosis after cardiac surgery. Results: A total of 12 articles reporting on 14 patients were included in this study. Coronary artery bypass grafting was the underlying surgical procedure in more than half of the cases. Purulent discharge and cold abscess were the two main presenting symptoms. Diagnosis of sternal wound infection was evidenced in all 14 patients by different investigations, with culture of samples being the most sensitive method of identifying the pathogen. Good response to first-line anti-tuberculous agents was noted. Almost all patients required surgical debridement/resection and, sometimes, sternal reconstruction. A delayed diagnosis of sternal wound infection may lead to repeated recurrences. A comparison between patients with sternal wound infection due to tuberculosis and non-tuberculous mycobacterial infections showed that the former infections took an even longer period of time. Comparisons also revealed patients with sternal tuberculosis infection had a significantly higher mortality than patients with sternal non-tuberculous infection (29.2% vs. 0%, P=0.051). Conclusion: Sternal infection caused by tuberculosis after cardiac surgery has a longer latency, better response to first-line drugs, and better outcomes in comparison with non-tuberculous sternal infection. Early diagnosis and early anti-tuberculous treatment can surely improve the patients' prognosis.

The present article aims to highlight fetal cardiac interventions (FCIs) in terms of indications, strategies, and fetal prognoses. FCIs of the early years were predominantly pharmacological therapies for fetal arrhythmia or heart block. A transplacental transmission of therapeutic agents has now become the main route of pharmacological FCIs. There have been various FCI strategies, which can be categorized into three types: pharmacological, open FCIs, and closed FCIs. Rather than as a routine management for materno-fetal cardiac disorders, however, FCIs are only applied in those fetal cardiac disorders that are at an increased risk of mortality and morbidity and warrant an interventional therapy. Pharmacological FCIs have been well applied in fetal arrhythmias but require further investigations for novel therapeutic agents. The development of open FCI in humans is an issue for the long run. Closed FCIs may largely rely on advanced imaging techniques. Hybrid FCIs might be the future goal in the treatment of fetal heart diseases.

AbstractObjective:It was supposed that troponins in cardiac myxoma patients might be in a same fashion as in the conditions without myocardial injury. In order to verify this hypothesis, troponins in cardiac myxoma patients were discussed by presenting a comprehensive retrieval of the literature with incorporating the information of a recent patient.Methods:Postoperative detections of troponin I, creatine kinase isoenzyme MB (CK-MB) and N-terminal pro-B-type natriuretic peptide revealed elevated troponin I and CK-MB and normal N-terminal pro-B-type natriuretic peptide. Postoperative troponin I and CK-MB shared a same trend, reaching a peak value at postoperative hour 2, gradually decreased on postoperative day 1, and reached a plateau on postoperative days 7 and 13. A significant correlation could be noted between the postoperative values of the two indicators (Y=0.0714X + 0.6425, r2=0.9111, r=0.9545, P=0.0116). No significant linear correlation between troponin I and N-terminal pro-B-type natriuretic peptide were found. Literature review of troponins in cardiac myxoma patients revealed the uncomplicated patients had a normal or only slightly elevated troponin before open heart surgery. However, the complicated patients (with cerebral or cardiac events) showed a normal preoperative troponin in 3 (23.1%) and an elevated troponin in 10 (76.9%) patients (χ2=7.54, P=0.0169, Fisher's exact test). The overall quantitative result of troponin I was 2.45±2.53 µg/L, and that of troponin T was 3.10±4.29 mg/L, respectively.Conclusion:Troponins are not necessarily elevated in patients with a cardiac myxoma without coronary syndrome. By contrast, patients with a cardiac myxoma with an elevated troponin may herald the presence of an associated coronary event. An old cerebral infarct does not necessarily cause an elevation of troponin or B-type natriuretic peptide, or new neurological events, but might lead to a delayed awakening.

AbstractObjective:The clinical features of cardiac myxoma stroke have not been sufficiently described. Debates remain concerning the options and timing of treatment and the clinical outcomes are unknown. This article aims to highlight the pertinent aspects of this rare condition.Methods:Data source of the present study came from a comprehensive literature collection of cardiac myxoma stroke in PubMed, Google search engine and Highwire Press for the year range 2000-2014.Results:Young adults, female predominance, single cerebral vessel (mostly the middle cerebral artery), multiple territory involvements and solitary left atrial myxoma constituted the outstanding characteristics of this patient setting. The most common affected cerebral vessel (the middle cerebral artery) and areas (the basal ganglion, cerebellum and parietal and temporal regions) corresponded well to the common manifestations of this patient setting, such as conscious alteration, ataxia, hemiparesis and hemiplegia, aphasia and dysarthria. Initial computed tomography scan carried a higher false negative rate for the diagnosis of cerebral infarction than magnetic resonance imaging did. A delayed surgical resection of cardiac myxoma was associated with an increased risk of potential consequences in particular otherwise arterial embolism. The mortality rate of this patient population was 15.3%.Conclusion:Cardiac myxoma stroke is rare. Often does it affect young females. For an improved diagnostic accuracy, magnetic resonance imaging of the brain and echocardiography are imperative for young stroke patients in identifying the cerebral infarct and determining the stroke of a cardiac origin. Immediate thrombolytic therapy may completely resolve the cerebral stroke and improve the neurologic function of the patients. An early surgical resection of cardiac myxoma is recommended in patients with not large territory cerebral infarct.

Objective: A systematic analysis was made in view of the epidemiology, clinical features, diagnosis, treatment and main outcomes of mycobacterial endocarditis. Methods: The data source of the present study was based on a comprehensive literature search in MEDLINE, Highwire Press and Google search engine for publications on mycobacterial endocarditis published between 2000 and 2013. Results: The rapidly growing mycobacteria become the predominant pathogens with Mycobacterium chelonae being the most common. This condition has changed significantly in terms of epidemiology since the 21st century, with more broad patient age range, longer latency, prevailed mitral valve infections and better prognosis. Conclusion: Mycobacterial endocarditis is rare and the causative pathogens are predominantly the rapidly growing mycobacteria. Amikacin, ciprofloxacin and clarithromycin are the most frequently used targeted antimicrobial agents but often show poor responses. Patients with deep infections may warrant a surgical operation or line withdrawal. With periodic multidrug therapy guided by drug susceptibility testing, and surgical managements, patients may achieve good therapeutic results.

Abstract Myocardial bridging is rare. Myocardial bridges are most commonly localized in the middle segment of the left anterior descending coronary artery. The anatomic features of the bridges vary significantly. Alterations of the endothelial morphology and the vasoactive agents impact on the progression of atherosclerosis of myocardial bridging. Patients may present with chest pain, myocardial infarction, arrhythmia and even sudden death. Patients who respond poorly to the medical treatment with β-blockers warrant a surgical intervention. Myotomy is a preferred surgical procedure for the symptomatic patients. Coronary stent deployment has been in limited use due to the unsatisfactory long-term results.

Postperfusion lung syndrome is rare but lethal. Secondary inflammatory response was the popularly accepted theory for the underlying etiology. Respiratory index (RI) and arterial oxygen tension/fractional inspired oxygen can be reliable indices for the diagnosis of this syndrome as X-ray appearance is always insignificant at the early stage of the onset. Evaluations of extravascular lung water content and pulmonary compliance are also helpful in the definite diagnosis. Multiorgan failure and triple acid-base disturbances that might develop secondary to postperfusion lung syndrome are responsible for the poor prognosis and increased mortality rather than postperfusion lung syndrome itself. Mechanical ventilation with low tidal volume (TV) and proper positive end-expiratory pressure can be an effective treatment strategy. Use of ulinastatin and propofol may benefit the patients through different mechanisms.

Objective: The outcomes in pregnant patients with bicuspid aortic valves (BAVs) are rarely reported, despite the potentially critical nature of the condition. The aim of this study is to present the clinical complications of BAV in pregnancy. Materials and methods: A MEDLINE database search and a Google internet search were conducted to find literature on BAV in pregnancy published between 1980 and 2012. Results: BAV in pregnancy can lead to critical cardiovascular events including aortic dissection, aortic valve disorders, and infective endocarditis; some of these complications may lead to poor maternal outcomes or fetal demise. No differences were noted in either maternal or fetal mortality between syndromic and nonsyndromic pregnant patients with BAV (maternal: 50% vs. 28.6%; p = 0.4959; fetal: 25% vs. 0%; p = 0.1987). The peak and mean pressure gradients across the aortic valve increased significantly with advancing gestational trimester; a remarkable decrease in peak pressure gradients was seen postpartum. The calculated aortic valve area showed a significant decrease in the third trimester compared with the prepregnancy value, as well as a considerable postpartum decrease. Conclusion: Syndromic and nonsyndromic BAVs may have similar importance for maternal and fetal mortality. Aortic valve stenosis may become more severe with advancing pregnancy, with attenuation after delivery. Patients may require surgical intervention for the complications of BAV during pregnancy.

Objective: Anomalous origin of coronary artery is uncommon. The taxonomies of anomalous origin of coronary artery are inconsistent and complex. Conceptual and therapeutic debates remain. The aim of the present study is to reappraise the concept of anomalous origin of coronary artery and to discuss the potential hazards and treatment rationale of this anomaly on basis of literature review. Methods: A comprehensive literature review was made in terms of the taxonomies including “simple”, “multiple” and “complex” types of anomalous origin of coronary artery. Results: Anomalous origin of coronary artery can be simply categorized according to the ectopically originated coronary artery. There are a couple of complex anatomical variants: “multiple” type, involving more than one coronary artery or branch, which can be subdivided into 2 subtypes, A) more than one coronary arteries or branches arising from one place; and B) two coronary arteries/branches arising from separate ectopic sites; and “complex” type, associated with acquired heart disease, or congenital heart defects. Conclusion: Sudden cardiac death in anomalous origin of coronary artery is associated with the anatomical features including abnormal coursing, acute angle take-off and ostial abnormalities. Atherosclerosis is prone to be in the right-sided ectopic and retroaortic coursing coronary artery. Surgical treatment is a definitive therapy. Simple coronary artery bypass grafting is not recommended due to the potential hazards of coronary steal phenomenon and poor patency of mammary arterial grafts, and modified maneuvers such as coronary ostial reimplantation, impinged coronary segment unroofing and coronary stent deployment are advocated instead.

Objective: This study aims to present the graft pathology at the time of harvest and its impact on long-term survival. Methods: The remnants of the bypass grafts from 66 consecutive patients with coronary artery disease receiving a coronary artery bypass grafting were investigated pathologically, and pertinent predictive risk factors and survival were analyzed. Results: Medial degenerative changes with or without intimal proliferation were present in 36.8%, 37.8% and 35.6% of left internal mammary artery (IMA), radial artery and saphenous vein grafts. There were 2 (3.0%) hospital deaths and 9 (14.1%) late deaths. Multinomial logistic regression revealed left IMA pathological changes, dyslipidemia, history of percutaneous transluminal coronary angioplasty/stent deployment and Y-graft were significant predictive risk factors negatively influencing the patients’ long-term survival. Kaplan-Meier survival analysis revealed that the long-term survival of patients with left IMA pathological changes were significantly reduced compared with those without (74.1% vs. 91.4%, P=0.002); whereas no differences were noted in long-term survivals between patients with and without pathological changes of the radial arterial or saphenous vein grafts. Conclusion: Pathological changes may be seen in the bypass graft at the time of harvest. The subtle ultrastructural modifications and the expressions of vascular tone regulators might be responsible for late graft patency. The pathological changes of the left IMA at the time of harvest rather than those of the radial artery or saphenous vein graft affect significantly longterm survival. Non-traumatic maneuver of left IMA harvest, well-controlled dyslipidemia and avoidance of using composite grafts can be helpful in maintaining the architecture of the grafts.

Objective: The present study is to describe the clinical impact of S100 and S100β for the evaluation of cerebral damage in cardiac surgery with or without the use of cardiopulmonary bypass (CPB). Methods: Quantitative results of S100 and S100β reported in the literature of the year range 1990-2014 were collected, screened and analyzed. Results: Cerebrospinal fluid and serum S100 levels showed a same trend reaching a peak at the end of CPB. The cerebrospinal fluid/serum S100 ratio decreased during CPB, reached a nadir at 6 h after CPB and then increased and kept high untill 24 h after CPB. Serum S100 at the end of CPB was much higher in infant than in adults, and in on-pump than in off-pump coronary artery bypass patients. ∆S100 increased with age and CPB time but lack of statistical significances. Patients receiving an aorta replacement had a much higher ∆S100 than those receiving a congenital heart defect repair. Serum S100β reached a peak at the end of CPB, whereas cerebrospinal fluid S100 continued to increase and reached a peak at 6 h after CPB. The cerebrospinal fluid/serum S100β ratio decreased during CPB, increased at the end of CPB, peaked 1 h after CPB, and then decreased abruptly. The increase of serum S100β at the end of CPB was associated with type of operation, younger age, lower core temperature and cerebral damages. ∆S100β displayed a decreasing trend with age, type of operation, shortening of CPB duration, increasing core temperature, lessening severity of cerebral damage and the application of intervenes. Linear correlation analysis revealed that serum S100β concentration at the end of CPB correlated closely with CPB duration. Conclusion: S100 and S100β in cerebrospinal fluid can be more accurate than in the serum for the evaluations of cerebral damage in cardiac surgery. However, cerebrospinal fluid biopsies are limited. But serum S100β and ∆S100β seem to be more sensitive than serum S100 and ∆S100. The cerebral damage in cardiac surgery might be associated with younger age, lower core temperature and longer CPB duration during the operation. Effective intervenes with modified CPB circuit filters or oxygenators and supplemented anesthetic agents or priming components may alleviate the cerebral damage.

The exact mechanisms underlying coronary artery ectasia (CAE) remain uncertain. This study aims to investigate whether and how inflammatory mediators play a role in the pathogenesis of CAE. The data sources of this study were located by literature searches on MEDLINE, Highwire Press and Google search engine for the year range 2000-2013. The most sensitive of the four types of plasma inflammatory mediators were cell adhesion molecules and systemic inflammatory markers followed by cytokines, while proteolytic substances were the least sensitive indicators of CAE. Hypersensitive C-reaction protein, homocysteine, intercellular adhesion molecule 1, vascular cell adhesion molecule 1, matrix metalloproteinase-9, tissue inhibitor of metalloproteinase-2, vascular endothelial growth factor and neopterin levels were significantly higher in CAE and coronary artery disease (CAD) patients than in controls without CAE. The percentage of granulocytes was higher in CAE, in comparison with individuals with normal coronary arteries. Polymerase chain reaction determination of angiotensin converting enzyme genotypes showed that the DD genotype was more prevalent in CAE patients than in CAD patients, while prevalence of the I allele was higher in CAD than in CAE patients. CAE is more a result of inflammatory processes than of extracellular matrix degradation, as demonstrated by investigations of plasma inflammatory mediators, activation markers and angiotensin converting enzyme genotypes. Contemporary theories are unable to explain CAE's predilection for the right coronary artery or the occurrence of multi-vessel and multi-segment involvement.

Postperfusion lung syndrome is rare but can be lethal. The underlying mechanism remains uncertain but triggering inflammatory cascades have become an accepted etiology. A better understanding of the pathophysiology and the roles of inflammatory mediators in the development of the syndrome is imperative in the determination of therapeutic options and promotion of patients' prognosis and survival. Postperfusion lung syndrome is similar to adult respiratory distress syndrome in clinical features, diagnostic approaches and management strategies. However, the etiologies and predisposing risk factors may differ between each other. The prognosis of the postperfusion lung syndrome can be poorer in comparison to acute respiratory distress syndrome due to the secondary multiple organ failure and triple acid-base imbalance. Current management strategies are focusing on attenuating inflammatory responses and preventing from pulmonary ischemia-reperfusion injury. Choices of cardiopulmonary bypass circuit and apparatus, innovative cardiopulmonary bypass techniques, modified surgical maneuvers and several pharmaceutical agents can be potential preventive strategies for acute lung injury during cardiopulmonary bypass.

Objective: This article aims to present updated therapeutic options for fetal congenital heart diseases. Methods: Data source for the present study was based on comprehensive literature retrieval on fetal cardiac interventions in terms of indications, technical approaches and clinical outcomes. Results: About 5% of fetal congenital heart diseases are critical and timely intrauterine intervention may alleviate heart function. Candidates for fetal cardiac interventions are limited. These candidates may include critical aortic valve stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum and evolving hypoplastic right heart syndrome, and hypoplastic left heart syndrome with an intact or highly restrictive atrial septum as well as fetal heart block. The advocated option are prenatal aortic valvuloplasty, pulmonary valvuloplasty, creation of atrial communication and fetal cardiac pacing. Conclusion: Fetal cardiac interventions are feasible at midgestation with gradually improved technical success and fetal/postnatal survival due mainly to a well-trained multidisciplinary team, sophisticated equipment and better postnatal care.

Pulmonary artery aneurysms (PAAs) are the most common type of pulmonary involvement in Behçet's disease. However, the relationships between clinical features and prognosis have not been sufficiently evaluated. This article describes the results of a comprehensive review, revealing that PAAs have a predilection for hemoptysis manifestations, increased dimensions, right lower lobar location, multiplicity and concurrent intramural thrombus formation. Surgical intervention was needed in one third of patients. Patients with massive hemoptysis and PAA rupture warranted emergency operations. Conservatively treated patients were prone to PAA progression; interventional embolization was associated with higher risks of recurrence and reintervention for PAAs; and surgically treated patients exhibited the highest mortality rates. In conclusion, PAAs in Behçet's disease are characterized by a predilection for hemoptysis manifestations, right lower lobar location, multiplicity, and concurrent intramural thrombus formation. Both the condition itself and the surgical operations it warrants are linked with high mortality due to PAA hemorrhage.

Postpartum aortic dissection is a rare but lethal event. Until now, only a limited number of cases have been reported, and a comprehensive literature review from 1988 to 2012 yielded 27 cases. Postpartum aortic dissection occurred between Day 1 and Day 42 after delivery, either vaginally or by cesarean section. Pregnancy alone without underlying contributing risk factors and Marfan syndrome were the two main risk factors for postpartum aortic dissection, accounting for 44.4% and 40.7% of cases, respectively. Late presentation and delayed diagnosis may lead to sudden death. Improving prenatal and peripartum care is, therefore, crucial in preventing the development of aortic dissection. Prompt diagnosis and timely treatment of postpartum aortic dissection may prevent patient death.

Mitral valve cleft associated with secundum atrial septal defect (ASD) is uncommon. We report a 39-year-old male patient manifesting symptoms of congestive heart failure 3 months before admission. Echocardiography showed typical mitral valve prolapse and a large ASD of the secundum type. He was diagnosed as severe mitral regurgitation and ASD. At operation, severe mitral valve prolapse with additional degenerative leaflets and a middle-sized cleft in the anterior leaflet were noted. A large ASD of a mixed central and inferior vena cava type was found. Mitral valve repair was impossible. The mitral valve was replaced with an ATS prosthesis. The ASD was repaired with a pericardial patch. Three slow arrhythmias, including nodal rhythm, sinus bradycardia and atrial fibrillation, complicated his early postoperative course. The literature of this entity was reviewed, and the etiology of the postoperative slow arrhythmias was discussed.

Objectives: The aim of this article is to make an evaluation on the clinical features of patients with Kawasaki disease who require a cardiac surgical procedure including coronary artery bypass grafting, coronary arterial aneurysmorrhaphy or heart transplantation. Methods: English literature of Kawasaki disease for cardiac surgery (1990–2011) was retrieved in the Pubmed database. The clinical features of the patient setting from the representative articles were collected and analyzed. Results: Patients with Kawasaki disease were very young, with some requiring a cardiac surgical procedure at a very early age. The interval between the onset and the surgical operation was 9.5±9.4 years. The prevalence of myocardial infarction and re-infarction was high. Giant aneurysm, critical stenosis with calcification and thrombus formation of the coronary arteries often warrant coronary artery bypass, heart transplantation or coronary arterial aneurysm plication. The left internal mammary artery to the left anterior descending coronary artery was the most commonly used graft in coronary artery bypass. Graft patency rate was 82.4% at 21.4±32.3 (range 0.1–252) month follow-up. The early and late mortalities of this patient setting were 0.6 and 3.0%, respectively. Conclusions: Patients with Kawasaki disease may develop coronary artery lesions prone to aneurysmal formation with calcification and thrombus and may require coronary artery bypass at a very early age. With the left internal mammary artery as the first choice of bypass graft, the long-term patency and patient survival was satisfactory.

O Contegra, um enxerto de veia jugular bovina, tem sido amplamente utilizado como biomaterial de preferência no tratamento cirúrgico das cardiopatias congênitas, especialmente como um conduto para a reconstrução da via de saída ventricular direita. Este artigo tem como objetivo fazer uma revisão abrangente sobre os desfechos clínicos do Contegra. Foram recuperados, coletados e analisados, relatos de Contegra publicados desde 2002. Havia 1.718 Contegra, aplicados em 1.705 pacientes. Os tamanhos dos condutos foram de 8-22 mm. As idades dos pacientes foram de recém-nascidos até 74,5 anos, com prevalência de pacientes pediátricos. O diagnóstico primário foi cardiopatia congênita em todos os casos, sendo os três diagnósticos principais: tetralogia de Fallot, tronco arterioso e atresia pulmonar, que representaram 25,6%, 16,7% e 13,1%, respectivamente. O Contegra foi utilizado como enxerto tubular na posição pulmonar em 1635 (95,9%) pacientes, como remendo monocúspide em 12 (0,7%), como enxerto na posição da valva pulmonar ou monocúspide em 40 (2,3%), e, como conduto artéria pulmonar-veia cava inferior na operação de Fontan, em 18 (1,1%) pacientes, respectivamente. O reimplante de conduto foi realizado em 141 (8,3%) pacientes, 33,8 ± 37 (8,6-106,8) meses após a inserção do conduto inicial. A plástica do conduto foi necessária em seis (0,4%) e a reintervenção em 83 (4,9%) dos pacientes. As indicações do reimplante do conduto incluíram estenose importante da anastomose distal, pseudoaneurisma da anastomose proximal e regurgitação importante do conduto. Quanto ao bom desempenho, disponibilidade e longevidade, o Contegra é um biomaterial adequado para a reconstrução da via de saída ventricular direita e como remendo para reparo de comunicação interventricular, mas não é apto para a operação de Fontan.Contegra, a bovine jugular vein graft, has been widely used as a preferable biomaterial in the surgical treatment of congenital heart defects, especially as a conduit for the right ventricular outflow tract reconstruction. This article aims to make a comprehensive review on the clinical outcomes of Contegra. Reports of Contegra published since 2002 were comprehensively retrieved, collected and analyzed. There were 1718 Contegra, applied in 1705 patients. The sizes of the conduits were 8-22 mm. The patients aged from newborn to 74.5 years, prevailed by pediatrics. The primary diagnosis was congenital heart defects in all cases, with Tetralogy of Fallot, truncus arteriosus and pulmonary atresia being the first three diagnoses, representing 25.6%, 16.7%, and 13.1%, respectively. Contegra was used as a tube graft in the pulmonary position in 1635 (95.9%) patients, as a monocuspid patch in 12 (0.7%), as a graft in the position of the pulmonary valve or a monocusps in 40 (2.3%), and as an inferior vena cava-pulmonary artery conduit in the Fontan procedure in 18 (1.1%) patients, respectively. Conduit reimplantation was performed in 141 (8.3%) patients 33.8 ± 37 (8.6-106.8) months after the initial conduit insertion. Conduit plasty was necessary in 6 (0.4%), and reintervention in 83 (4.9%) patients. Indications for conduit reimplantation included severe stenosis of the distal anastomosis, pseudoaneurysm of the proximal anastomosis and severe conduit regurgitation. As for the good performance, availability and longevity, Contegra is a biomaterial suitable for the right ventricular outflow tract reconstruction and for patch repair for ventricular septal defect, but not apt for Fontan procedure.

OBJECTIVE: D-dimer and C-reactive protein are of diagnostic and predictive values in patients have thrombotic tendency, such as vascular thrombosis, coronary artery disease and aortic dissection. However, the comparative study in these biomarkers between the patients with acute aortic dissection and coronary artery disease has not been sufficiently elucidated. METHODS: Consecutive surgical patients for acute type A aortic dissection (20 patients), aortic aneurysm (nine patients) or coronary artery disease (20 patients) were selected into this study. Plasma from preoperative blood samples and supernatant of aortic homogenate of the surgical specimens were detected for D-dimer and hypersensitive C-reactive protein (hs-CRP). RESULTS: Plasma D-dimer and hs-CRP values in type A aortic dissection or aortic aneurysm were much higher than in coronary artery disease patients or the healthy control (for D-dimer, aortic dissection: coronary artery disease, 0.4344 ± 0.2958 µg/ml vs. 0.0512 ± 0.0845 µg/ml, P < 0.0001; aortic dissection: healthy control, 0.4344 ± 0.2958 µg/ml vs. 0.1250 ± 0.1295 µg/ml, P = 0.0005; aortic aneurysm: coronary artery disease, 0.4200 ± 0.4039 µg/ml vs. 0.0512 ± 0.0845 µg/ml, P = 0.0013; and aortic aneurysm: healthy control, 0.4200 ± 0.4039 µg/ml vs. 0.1250 ± 0.1295 µg/ml, P = 0.0068; and for hs-CRP, aortic dissection: coronary artery disease, 4.400± 3.004 mg/L vs. 1.232±0.601 mg/L, P < 0.0001; aortic dissection:healthy control, 4.400 ± 3.004 mg/L vs. 0.790 ± 0.423 mg/L, P < 0.0001; aortic aneurysm: coronary artery disease, 2.314 ± 1.399 mg/L vs. 1.232 ± 0.601 mg/L, P = 0.0084; aortic aneurysm: healthy control, 2.314 ± 1.399 mg/L vs. 0.790 ± 0.423 mg/L, P = 0.0002; and coronary artery disease: healthy control, 1.232 ± 0.601 mg/L vs. 0.790 ± 0.423 mg/L, P = 0.0113). Besides, there were close correlations between plasma D-dimer and hs-CRP in overall (Y = 4.8798X + 0.8138, r² = 0.4497, r = 0.671, P < 0.001), aortic dissection (Y = 2.6298X + 1.2098, r² = 0.5762, r = 0.759, P < 0.001), and aortic aneurysm (Y = 7.1341X + 1.3006, r² = 0.4935, r = 0.7025, P = 0.048) groups rather than in the coronary artery disease or healthy control subjects. In addition, there were no significant differences between D-dimer and hs-CRP values of the aortic supernatant among groups except for undetectable D-dimer in the aortic supernatant of the coronary artery disease group. CONCLUSIONS: The patients with acute aortic dissection and aortic aneurysm may reflect the extensive inflammatory reaction and severe coagulopathies in the patients with acute type A aortic dissection, and thoracic aortic aneurysm in comparison to the coronary patients and healthy control individuals. The detections after onset in the patients with acute chest pain may help making a differential diagnosis between the aortopathies and ischemic heart disease. The scanty significance of the tissue biomarkers may preclude their diagnostic value in clinical practice.OBJETIVO: D-dímero e proteína C reativa são de valores de diagnóstico e preditivo em pacientes com tendência trombótica, como a trombose vascular, doença arterial coronária e dissecção aórtica. No entanto, o estudo comparativo desses biomarcadores entre os pacientes com dissecção aguda da aorta e doença arterial coronariana não foi suficientemente esclarecido. MÉTODOS: Pacientes cirúrgicos consecutivos foram selecionados para este estudo por tipo de dissecção aguda aórtica (20 pacientes), aneurisma da aorta (9 pacientes) ou doença arterial coronária (20 pacientes). O plasma a partir de amostras de sangue no pré-operatório e sobrenadante de homogenato de aorta dos espécimes cirúrgicos foi detectado para o D-dímero e proteína C reativa hipersensível. RESULTADOS: Os valores do plasma de D-dímero e proteína-C reativa em dissecção aórtica tipo A ou aneurisma da aorta foram muito superiores em pacientes com doença arterial coronariana ou de controles saudáveis (pelo D-dímero, dissecção aórtica: doença arterial coronariana, 0,4344 ± 0,2958 µg/ml vs 0,0512 ± 0,0845 µg/ml, P

The aim of article is to give a brief description to the surgical strategies for patients with lone atrial fibrillation without associated cardiac operations, and present the possible indications of on-pump Maze procedures through a mid-sternotomy approach.O objetivo do artigo é fazer uma breve descrição das estratégias cirúrgicas para pacientes com fibrilação atrial isolada sem operações cardíacas associadas, e apresentar possíveis indicações de procedimentos Maze com circulação extracorpórea por meio de esternotomia mediana

OBJECTIVES: Transforming growth factor (TGF)-β/Smad signaling pathway in aortic dissection patients and normal subjects has not been previously described. The present study was designed to evaluate the TGF-β/Smad signaling expressions in the patients with acute type A aortic dissection in comparison with those in the patients with thoracic aortic aneurysm and with coronary artery disease, and (or) the healthy subjects. METHODS: Consecutive surgical patients for acute type A aortic dissection (20 patients), aortic aneurysm (nine patients) or coronary artery disease (20 patients) were selected into this study. Blood samples (4 ml) were obtained from the right radial arterial indwelling catheter after systemic heparinization prior to the start of cardiopulmonary bypass in the operating room. Twenty-one young healthy volunteers without underlying health issues who donated forearm venous blood samples (4 ml) were taken as control. The surgical specimens of the aortic tissues were obtained immediately after they were severed during the operations of the replacement of the aorta in the patients with aortic dissection or aortic aneurysm. In patients receiving coronary artery bypass grafting, the tiny aortic tissues were taken when the punch holes of the proximal anastomosis on the anterior wall of the ascending aorta were made. The aortic tissues were for RNA, protein, or supernatant preparations until detection of TGF-β1 mRNA by quantitative real-time reverse transcription polymerase chain reaction, of TGF-β1, TGF-β receptor I, Smad2/3, Smad4 and Smad7 by Western blot, and of TGF-β1 by enzyme-linked immunosorbent assay, respectively. In particular, the linear correlations of the relative grayscales between different proteins of each group, and those correlations between the quantitative TGF-β1 by enzyme-linked immunosorbent assay and the time interval from the onset to surgery or the maximal dimensions of the aorta of the aortic dissection group were assessed. RESULTS: Quantitative real-time reverse transcription polymerase chain reaction showed that TGF-β1 mRNA were upregulated in all surgical groups (1.59 ± 0.33 vs. 1.45 ± 0.34 vs. 1.48 ± 0.48, P > 0.05). Western blot revealed that the expressions of TGF-β1, TGF-β receptor I, Smad2/3, Smad4 and Smad7 were positive in the aortic tissues of all three investigated groups. Of the quantitative relative grayscales, a significant reverse correlation was noted between TGF-β1 and Smad2/3 (Y = -0.8552X + 1.6417, r = -0.759, P < 0.0001), and a close direct correlation between Smad4 and Smad7 (Y = 0.5905X + 0.2805, r = 0.781, P < 0.0001) in the Aortic Dissection Group. In the Aortic Aneurysm Group, Smad4 and Smad7 were also closely correlated (Y = 0.5228X + 0.1642, r = 0.727, P = 0.026), and in the Coronary Artery Disease Group, TGF-β1 and Smad7 were much significantly correlated (Y = 0.5301X + 0.5758, r = 0.917, P = 0.004). By enzyme-linked immunosorbent assay, TGF-β1 level of the aortic tissue was lower in the aortic dissection than in the aortic aneurysm and coronary artery disease groups with no statistical significance (319.52 ± 129.21 pg/mg protein vs. 324.09 ± 49.70 pg/mg protein vs. 304.15 ± 29.39 pg/mg protein, P > 0.05). The plasma TGF-β1 levels were 1158.30 ± 11.54 pg/ ml, 1170.27 ± 8.26 pg/ml, 1225.00 ± 174.42 pg/mL and 1160.25 ± 13.01 pg/mL in the four groups, respectively, showing significant intergroup differences (P < 0.05). No significant correlation was found between the aortic or plasma TGF-β1 levels and the time interval from the onset to surgery or the maximal dimensions of the aorta in the patients of the aortic dissection group. CONCLUSIONS: Aortic dissection, aortic aneurysm and atheroslerosis might be associated with an enhanced TGF β/Smad signaling function, with aortic dissection exhibiting a less prominent upregulation. It might have implications for downstream signal activation presumably translating into matrix degradation in the condition of aortic dissection in comparison to matrix deposition in aortic aneurysm and coronary artery diseaseOBJETIVOS: Fator transformador de crescimento (TGF) -β/ Smad como via de sinalização em casos de dissecção aórtica e indivíduos normais não foi descrito anteriormente. O presente estudo foi elaborado para avaliar as expressões TGF-β/Smad como via de sinalização nos pacientes com dissecção aguda da aorta, em comparação com que nos pacientes com aneurisma da aorta torácica e com doença arterial coronariana, e (ou) com indivíduos saudáveis. MÉTODOS: Pacientes cirúrgicos consecutivos para o tipo A de dissecção aguda da aorta (20 pacientes), aneurisma da aorta (nove pacientes) ou doença arterial coronária (20 pacientes) foram selecionados para este estudo. Amostras de sangue (4 ml) foram obtidas a partir do cateter arterial radial direito após heparinização sistêmica antes do início da circulação extracorpórea na sala de cirurgia. Vinte e um voluntários jovens e saudáveis, sem problemas de saúde subjacentes que doaram amostras de sangue venoso do antebraço (4 ml) foram tomados como controle. Os espécimes cirúrgicos de tecidos aórtico foram obtidos imediatamente após terem sido cortados durante as operações da substituição da aorta nos pacientes com dissecção aórtica ou aneurisma da aorta. Em pacientes que foram submetidos à cirurgia de revascularização miocárdica, os tecidos da aorta minúsculos foram obtidos quando os orifícios da anastomose proximal na parede anterior da aorta ascendente foram feitos. Os tecidos da aorta foram para a RNA, proteínas ou preparações sobrenadantes até a detecção de TGF-β1 mRNA pela reação de transcrição reversa quantitativa em tempo real em cadeia da polimerase, de TGF-β1, receptor I de TGF-β, Smad2/3, Smad4 e Smad7 por Western Blot, e de TGF-β1 pelo teste de ELISA, respectivamente. Em particular, as correlações lineares dos tons de cinza relativo entre diferentes proteínas de cada grupo, e aquelas correlações entre os quantitativos TGF-β1 pelo teste de ELISA e o intervalo de tempo desde o início da cirurgia ou as dimensões máximas da aorta do grupo de dissecção da aorta foram avaliados. RESULTADOS: Reação de transcrição reversa quantitativa em tempo real em cadeia da polimerase mostrou que o mRNA TGF-β1 foi supra-regulado em todos os grupos cirúrgicos (1,59 ± 0,33 vs 1,45 ± 0,34 vs 1,48 ± 0,48, P> 0,05). Western blot revelou que as expressões de TGF-β1, receptor I de TGF-β, Smad 2/3, Smad4 e Smad7 foram positivos nos tecidos da aorta de todos os três grupos investigados. Dos tons de cinza quantitativa relativa, uma correlação inversa significativa foi observada entre TGF-β1 e Smad 2/3 (Y = 1,6417 +-0.8552X, r = -0,759, P 0,05). Os níveis de plasma TGF-β1 foram 1.158,30 ± 11,54 pg/ml, 1.170,27 ± 8,26 pg/ml, 1225,00 ± 174,42 pg/mL e 1.160,25 ± 13,01 pg/mL nos quatro grupos, respectivamente, mostrando diferenças significativas entre grupos (P

It is well-known that hypertension is a very common disease, and severe cerebrovascular accidents might occur if the blood pressure is not properly controlled. However, conditions associated with uncontrolled hypertension may be overlooked, and may become critical and eventually require a surgical intervention on an urgent basis. Coronary artery disease, acute aortic syndrome, congenital and valvular heart disease, and arrhythmias are under this topic of discussion. Of them, coronary artery disease including myocardial infarction and especially postinfarction myocardial rupture, and aortic dissection are major critical situations that physicians may encounter in clinical practice. The role that hypertension plays in these conditions can be complex, including hemodynamic, electrophysiological and biomolecular factors, where the latter may prevail in the current era. Coronary artery disease may be associated with a reduced nitric oxide synthesis. Transforming growth factor and matrix metalloproteinases have been observed in relation to aortic syndrome. Wnt, p38 and JNK signaling pathway may be involved in the development of ventricular hypertrophy responsible for cardiac arrythmias. Various gene phynotypes may present in different congenital heart defects. This article is to present these conditions, and to further discuss the possible etiologies and the potential treatment strategies so as to highlight the relevance at a prognostic level.É sabido que a hipertensão é uma doença muito comum, e que os acidentes cerebrovasculares graves podem ocorrer se a pressão sanguínea não for apropriadamente controlada. Contudo, as condições associadas à hipertensão não controlada podem ser negligenciadas, e tornarem-se críticas, necessitando, eventualmente, uma intervenção cirúrgica urgente. Doença coronariana, síndrome aórtica aguda, cardiopatias congênitas, valvopatias e arritmias são sob este tópico de discussão. Dentre eles, a doença corornariana, inclusive o infarto do miocárdio e especialmente a ruptura cardíaca pós-infarto e a dissecção aórtica, são as situações críticas principais que os médicos podem encontrar na prática clínica. O papel que a hipertensão desempenha nessas condições pode ser complexo, incluindo fatores hemodinâmicos, eletrofisiológicos e biomoleculares, nos quais o último pode prevalecer atualmente. A doença coronariana pode associar-se com uma redução na síntese de óxido nítrico. Fator de crescimento transformador e nas metaloproteinases da matriz têm sido observados em relação à síndrome aórtica. O Wnt, p38 e a via de sinalização JNK caminho podem estar implicado no desenvolvimento da hipertrofia ventricular responsável por arritmias cardíacas. Vários fenótipos dos genes podem apresentar defeitos cardíacos congênitos diferentes. Este artigo apresenta essas condições, e discute, além disso, possíveis etiologias e as estratégias de tratamento potenciais bem destacar sua importância quanto a prognóstico.

BACKGROUND: Osteopontin (OPN) has been verified to be closely associated with oncogenesis and remodeling processes. But this cytokine was rarely assessed in the presence of aortopathies, especially acute aortic dissection. The aim of the present study was to evaluate the expressions of OPN by way of molecular biological approaches so as to offer a better understanding of the possible mechanisms of the aortopathies. METHODS: Consecutive patients with type A acute aortic dissection (20 patients), aortic aneurysm (nine patients) or coronary artery disease (21 patients) referred to this hospital for surgical operations were enrolled into this study. Blood samples of the surgical patients after systematic heparinization, and control fast morning blood samples drawn from 21 young healthy volunteers who had no evidence of any healthy problems were investigated for enzyme linked immunosorbent assay (ELISA). The surgical specimens of the aortic tissues collected from the surgical patients during the operations were obtained for quantitative realtime reverse transcription polymerase chain reaction (RT-PCR) for OPN mRNA, western blot assay for OPN protein, and for immunohistochemical staining of OPN. Ascending aortic tissues from the autopsies of the healthy individuals dying of accident were obtained as controls of immunohistochemistry. RESULTS: By quantitative RT-PCR, the expressions of OPN mRNA were all upregulated in all three surgical groups. The quantitative results did not reveal any intergroup differences. Western blot assay revealed that OPN was positive with similar intensities of expressions in all three surgical groups. Quantitative western blot analyses of OPN expressions did not show any significance between groups. The OPN expressions by ELISA in the aortic tissue were 3.09311 ± 1.65737, 3.40414 ± 1.15095, and 1.68243 ± 0.31119 pg/mg protein in the aortic dissection, aortic aneurysm, and coronary artery disease groups, respectively. The OPN level of the patients with coronary artery disease was much lower than those with aortic dissection (P = 0.033) or with aortic aneurysm (P = 0.019). By unparametric tests, there were significant differences in the aortic OPN contents among aortic dissection, aortic aneurysm and coronary artery disease groups (P < 0.01). A significant direct correlation was present between plasma OPN concentration and the time interval from the onset to surgery of aortic dissection (Y = 0.1420X + 2.4838, r² = 0.5623, r = 0.750, P = 0.032). By immunohistochemistry, OPN was expressed in the aortic cells: in the intima, it was weaker in all three surgical groups in comparison with the healthy control; in the media, it was weak in the aortic dissection, intense positive in aortic aneurysm, focal positive in the coronary artery disease, but evenly positive in the healthy control groups; and in the adventitia, it was positive in the aortic dissection, coronary artery disease and healthy control groups, but weak positive in the aortic aneurysm group. CONCLUSION: These data may provide evidences that OPN may play a role in the pathogenesis of aortopathies including aortic dissection, aortic aneurysm, and coronary artery disease. OPN might be of potential perspective as a clinically diagnostic tool in the evaluations of the complex remodeling process incorporating vascular injury and repair.OBJETIVOS: A osteopontina (OPN) está estreitamente associada com os processos de oncogênese e remodelação. Entretanto, essa citocina era raramente avaliada na presença de aortopatias, especialmente na dissecção aórtica aguda. O objetivo do presente estudo foi avaliar a expressão de OPN por meio de abordagens moleculares biológicas, de modo a oferecer uma melhor compreensão dos possíveis mecanismos das aortopatias. MÉTODOS: Pacientes consecutivos com um tipo de dissecção aguda da aorta (20 pacientes), aneurisma da aorta (nove pacientes) ou doença arterial coronária (21 pacientes) foram incluídos neste estudo. As amostras de sangue depois da heparinização sistemática e de 21 voluntários jovens e saudáveis não apontaram nenhuma evidência de qualquer problema ao serem investigados por ensaio imunoenzimático (ELISA). Os espécimes cirúrgicos dos tecidos aórtica coletados dos pacientes durante as operações foram obtidos para a reação de transcrição reversa quantitativa em tempo real em cadeia da polimerase (RT-PCR) para OPN mRNA, técnica de Western blot para a proteína OPN, e imunohistoquímica de OPN. Amostras da aorta de indivíduos saudáveis que morreram de acidente foram obtidos para controle imunohistoquímico. RESULTADOS: Com uso do RT-PCR quantitativo, as expressões de OPN mRNA foram suprarreguladas em todos os três grupos cirúrgicos. Os resultados quantitativos não revelaram quaisquer diferenças intergrupais. Western blot revelou que OPN foi positiva com intensidade semelhante de expressões em todos os três grupos. As análises quantitativas Western blot de expressões OPN não apresentaram significâncias entre os grupos. As expressões OPN medidas pelo teste ELISA no tecido aórtico foram 3,09311 ± 1,65737, 3,40414 ± 1,15095 e 1,68243 ± 0,31119 pg/mg de proteína na dissecção de aorta, aneurisma da aorta, e grupos de doença arterial coronariana, respectivamente. O nível de OPN dos pacientes com doença arterial coronariana foi muito menor do que aqueles com dissecção aórtica (P = 0,033) ou com aneurisma da aorta (P = 0,019). Testes não-paramétricos apontaram diferenças significativas nos teores de aorta OPN entre dissecção aórtica, aneurisma da aorta e grupos com doença arterial coronariana (P

OBJECTIVE: Associations between cardiovascular diseases and serum enzymes or coagulation activities have been sufficiently documented in patients with myocardial infarction. However, the alterations of these biomarkers in patients with postinfarction myocardial rupture have rarely been reported. The aim of this study is to present the profiles of the markers in patients with postinfarction myocardial rupture. METHODS: From 2004 to 2008, 19 consecutive patients were referred to this hospital for surgical repair of postinfarction myocardial rupture. Eight (42.1%) patients had free wall rupture, 5 (26.3%) had papillary muscle rupture, 5 (26.3%) had ventricular septal rupture, and 1 (5.3%) had double structure (ventricular septum + free wall) rupture. Thirteen patients survived the operation, and 6 died. Laboratory findings including serum enzymes and coagulation activities were collected and analyzed. RESULTS: The coagulation markers and serum enzymes except for fibrinogen increased significantly after the development of myocardial rupture. Statistical differences in D-dimer, partial thromboplastin time, peak lactate dehydrogenase, peak creatine kinase and creatine kinase fraction MB were found between non-survivors and survivors. Troponin I values were elevated significantly during the early days after the onset or surgical repair of myocardial rupture. Multivariant regression analysis did not show any significant relationship between creatine phosphokinase fraction MB (Y) and D-dimer (X1) or fibrinogen (X2). CONCLUSION: Myocardial rupture leads to extremely high serum enzyme and coagulation activities except for fibrinogen after the onset. The evaluation of these biomarkers may help in making diagnostic and treatment decisions and in judging the clinical prognosis of such patients.OBJETIVO: As associações entre doenças cardiovasculares e enzimas sorológicas ou atividades de coagulação foram amplamente documentadas em pacientes com infarto do miocárdio. No entanto, as alterações destes biomarcadores em pacientes com ruptura cardíaca após infarto do miocárdio foram raramente relatadas. O objetivo deste estudo é apresentar o perfil dos biomarcadores em pacientes com ruptura cardíaca após infarto do miocárdio. MÉTODOS: De 2004 a 2008, 19 pacientes consecutivos foram referidos a este hospital para correção cirúrgica de ruptura cardíaca após infarto do miocárdio. Oito (42,1%) pacientes tiveram ruptura livre de parede, cinco (26,3%) ruptura de músculo papilar, cinco (26,3%) ruptura do septo interventricular e um (5,3%) ruptura dupla de estruturas, envolvendo tanto septo ventricular como parede livre. Treze pacientes sobreviveram à operação e seis faleceram. Amostras sanguíneas foram coletadas e analisadas para mensuração de enzimas sorológicas e atividade de coagulação. RESULTADOS: Os marcadores de coagulação e enzimas com exceção de fibrinogênio aumentaram significativamente depois do desenvolvimento da ruptura do miocárdio. Diferenças estatísticas foram achadas entre não-sobreviventes e sobreviventes em relação a concentração de dímeros-D, tempo de trombina, pico de lactato desidrogenase, creatinoquinase máximo e fração MB da creatinoquinase. Os valores de troponina I foram elevados significativamente durante os primeiros dias depois do infarto ou do reparo cirúrgico da ruptura do miocárdio. A análise de regressão multivariada não mostrou qualquer relação significativa entre fração MB da creatinoquinase e dímeros-D nem fibrinogênio. CONCLUSÕES: A ruptura do miocárdio induz importante elevação de marcadores enzimáticos e de atividade de coagulação, exceto fibrinogênio. As diferenças nestes biomarcadores entre não-sobreviventes e sobreviventes podem ser de grande ajuda no diagnóstico e nas decisões de tratamento, assim como na avaliação do prognóstico clínico de tais pacientes.

Cystic medial necrosis (CMN) is a disorder of large arteries, in particular the aorta, characterized by an accumulation of basophilic ground substance in the media with cyst-like lesions. CMN is known to occur in certain connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, and annuloaortic ectasia, which usually result from degenerative changes in the aortic wall. The relationships between CMN and congenital heart defects as well as other disorders have been evidenced. The mechanisms are still controversial, even though many molecular studies have been conducted. The aim of the present article is to provide a comprehensive overview of the CMN lesion in terms of pathologic features, clinical implications and etiologies based on molecular research results.A necrose cística da média (NCM) é uma desordem das grandes artérias, em particular a aorta, caracterizada por acúmulo de substância basofílica na camada média com lesões císticas-símile. É sabido que a NCM ocorre em certas doenças do tecido conjuntivo tal como síndrome de Marfan, síndrome de Ehlers-Danlos, e ectasia ânulo-aórtica, que normalmente resulta de mudanças degenerativas na parede aórtica. A relação entre NCM e defeitos congênitos do coração, assim como outras desordens, tem sido evidenciada. Os mecanismos são ainda controversos, embora muitos estudos moleculares tenham sido conduzidos. O objetivo do presente artigo é fornecer uma visão geral da NCM em termos de características patológicas, implicações clínicas e etiologia baseada em resultados de pesquisa molecular.

OBJECTIVES: The biological functions of transforming growth factor-β signaling that involves Smad proteins have not been previously investigated with respect to coronary artery bypass grafts. The aim of the present study was to observe the immunostaining of proteins that are related to this signaling pathway. METHODS: Fifteen remnants of coronary artery bypass grafts, including nine saphenous veins, three radial arteries and three mammary arteries, were collected from 12 patients who were undergoing coronary artery bypass. Hematoxylin and eosin, Masson's trichrome, and immunohistochemical staining of transforming growth factor-β1, type I receptor of transforming growth factor-β, Smad2/3, Smad4, and Smad7 were performed. RESULTS: The saphenous veins showed more severe intimal degeneration, more severe smooth muscle cell proliferation and more collagen deposition than the arterial grafts, as evidenced by hematoxylin and eosin and Masson's trichrome stainings. Immunohistochemical assays demonstrated that the majority of the transforming growth factor-β1 signaling cytokines were primarily localized in the cytoplasm in the medial layers of all three types of grafts, whereas ectopic transforming growth factor-β1, type I receptor of transforming growth factor-β, and Smad7 overexpressions in the interstices were observed particularly in the saphenous vein and radial arterial grafts. CONCLUSION: Enhanced transforming growth factor-β1 signal transduction with medial smooth muscle cell proliferation and ectopic transforming growth factor-β1, the presence of the type I receptor of transforming growth factor-β, and Smad7 overexpressions in the extracellular matrix may provide primary evidence for early or late graft failure.