Background:Women with congenital heart disease(CHD)have increased risk for adverse events during pregnancy and delivery.Prior studies have assessed pregnancy and delivery outcomes at tertiary referral centers(TRC).The...Background:Women with congenital heart disease(CHD)have increased risk for adverse events during pregnancy and delivery.Prior studies have assessed pregnancy and delivery outcomes at tertiary referral centers(TRC).The aim of our study was to assess pregnancy outcomes in women with CHD who deliver in a non-tertiary referral center(non-TRC).Methods:Clinical demographics were collected,including anatomic complexity,physiologic state and pre-pregnancy risk assessment.Patients were stratified by delivery location,either TRC or non-TRC.Maternal and neonatal complications of pregnancy were reported.Results:Women with CHD who delivered in a TRC had a higher pre-pregnancy risk when assessed by the Zahar and CARPREG-II scores,and had more patients fall into a higher WHO classification.There was no difference in rates of maternal cardiac complications between delivery locations(11%)and neonatal complications(20%)between deliveries at TRC and non-TRC.Conclusions:There were not increased maternal cardiac or neonatal complications when delivery occurred at a non-TRC.Neonatal complications remained high regardless of delivery location.This study suggests that proper risk assessment may help identify women who are candidates for safe delivery at non-TRC in women with CHD,and that neonatal resources should be considered when planning delivery location.展开更多
Purpose: The purpose of this review was to identify the short-term and long-term outcomes of pregnancy in women suffering from congenital heart disease (CHD). Methods: An integrative review is used to identify the pre...Purpose: The purpose of this review was to identify the short-term and long-term outcomes of pregnancy in women suffering from congenital heart disease (CHD). Methods: An integrative review is used to identify the pregnancy outcomes in women with CHD. Data search was between 2010 and 2020 using Google Scholar, Scopus, Web of Science, Science Direct, Pub Med, Medline, CINAHL, EBSCO, Cochrane, and EBSCO host. Sixteen articles met the eligibility criteria. Results: The sixteen reviewed articles utilized descriptive retrospective and prospective design. Themes were short-term outcomes that include;cardiac maternal and obstetric outcomes, and long-term outcomes. Conclusion: Previous uncertainty about the ability of CHD patients to successfully become pregnant and deliver safely has replaced by the recognition that a large number can have excellent outcomes. However, these patients do continue to have higher cardiac, obstetric, and fetal risks than the general population. This illuminates the importance of preconception counseling and risk assessment for women suffering from CHD regarding the expected maternal and fetal outcomes. Moreover, there is a need for providing accurate and appropriate education about pregnancy and delivery options.展开更多
Objective:Neonates with congenital heart disease(CHD)and perinatal stroke have high mortality and survivors are at risk for poor long-term neurodevelopmental outcome.The aim of this study was to assess the risk factor...Objective:Neonates with congenital heart disease(CHD)and perinatal stroke have high mortality and survivors are at risk for poor long-term neurodevelopmental outcome.The aim of this study was to assess the risk factors and outcome of neonates with both CHD and MRI-confirmed perinatal stroke(Study Group)and compare those to the risk factors and outcome of infants matched for CHD without stroke(Control-1)and of infants matched for MRI-confirmed stroke without CHD(Control-2).Methods:We conducted a population-based case-control study enrolling 28 term neonates with CHD and MRI-confirmed acute perinatal stroke born between 2007–2017 in the Central-Hungarian Region.Each of the control groups included 56 infants.The Bayley Scales of Infant Development-II,the Brunet-Lézine test and the Binet Intelligence scales-V were used for neurodevelopmental follow-up at a median age of 61 months.Results:Mortality was highest in the Study Group(25%compared to 5%and 2%,respectively,p=0.001).Adverse neurodevelopmental outcome was prevalent in the Study(53%)and Control-2 Groups(52%,p=0.03).Significantly different parameters among the three groups included Apgar scores,mode of delivery,gestational age at birth,cardiac interventions and twin pregnancy.In a multivariable regression analysis adjusted for clinically relevant parameters,patients in the Study Group had significantly higher odds for mortality compared to patients in the Control-1 Group(OR:6.595%CI:1.1–39.4).Conclusions:Neonates with perinatal stroke and CHD are at a higher risk for dying compared to neonates with CHD without stroke.In addition,the stroke-associated direct insult to the brain likely plays an important role in the development of neurodevelopmental morbidity in these patients.展开更多
There is a growing population of adults with congenital heart disease(CHD) due to improved survival beyond childhood.It has been suggested that adults with CHD may be at increased risk for mental health problems,parti...There is a growing population of adults with congenital heart disease(CHD) due to improved survival beyond childhood.It has been suggested that adults with CHD may be at increased risk for mental health problems,particularly depression.The reported incidence of depression in CHD varies from 9% to 30%.This review examines the evidence for a higher depression rate in CHD vs general population.Possible explanations are offered from a variety of disease models,ranging from brain injury to the psychoanalytical approach.Risk factors for an abnormal emotional adjustment and depression include early exposure to stress from illness and medical interventions in infancy,separation from the parents during hospitalizations and brain organic syndromes.Later in life,patients often have to cope with physical limitations.Recent improvements in care may be protective.Current patients may benefit from an earlier age at first surgical intervention,fewer reoperations and inclusion to the mainstream schooling,among other factors.At this point,there is little systematic knowledge about evidence-based therapeutic interventions for depression in adults with CHD.Health care providers of patients with CHD should be aware of mental health challenges and may take a more proactive approach to identifying patients at risk for depression.展开更多
Background:Patients with genetic syndromes who undergo surgery to correct congenital heart defects can be at risk for increased morbidity or mortality.Surgical outcomes and postoperative courses following congenital h...Background:Patients with genetic syndromes who undergo surgery to correct congenital heart defects can be at risk for increased morbidity or mortality.Surgical outcomes and postoperative courses following congenital heart surgery in patients with Kabuki-Syndrome(KS)have not been well studied.Objectives:The purpose of this study was to describe the postoperative courses and associated outcomes in the largest set of KS patients undergoing congenital heart surgery to date.Methods:Patients with a confirmed molecular diagnosis of KS and a diagnosis of a CHD admitted to Texas Children’s Hospital between January 1,2000 and January 1,2020 were included(n=20).Demographics and medical histories were collected from the hospitals’electronic health records.Results:Of 20 patients identified with KS and a CHD,15 required surgical correction of their congenital cardiac malformation.Median age and weight at the time of surgery was 2 months and 4.1 kg,respectively.Median duration of hospital stay was 49 days for all surgeries and 151 days for the Norwood procedure.Postoperative infections and pleural effusions were detected and treated in 45.8%and 50%of patients,respectively.There was no in-hospital mortality for any surgery.Median follow up time was 5.6 years;survival at 6 years was 94%.Conclusions:Although KS patients seem to be at increased risk for a more complicated,prolonged postoperative course than that of patients without a genetic syndrome,patients with a diagnosis of a CHD and KS do not appear to be at increased risk of mortality following congenital heart surgery.展开更多
AIM To describe the long-term follow-up of patients with complex congenital heart disease who underwent subcutaneous implantable cardiac defibrillator(S-ICD), focusing on local complications, appropriate and inappropr...AIM To describe the long-term follow-up of patients with complex congenital heart disease who underwent subcutaneous implantable cardiac defibrillator(S-ICD), focusing on local complications, appropriate and inappropriate shocks.METHODS Patients with complex congenital heart disease underwent S-ICD implant in two centers with the conventional technique. Data at follow-up were retrieved from clinical notes and institutional database. RESULTS Eight patients were implanted in two centres between 2010 and 2016. Median age at implant was 37.5 years(range 13-57). All patients who were deemed suitable for S-ICD implant passed the pre-procedural screening. Three patients were previously implanted with a antibradycardia device, one of whom with CRT. In one patient the device was explanted due to local infection. Duringthe total median follow-up of 874 d, one patient had an appropriate and one inappropriate shock triggered by fast atrial tachycardia. None of the patients had inappropriate shocks secondary to T wave oversensing or electrical interference with anti-bradycardia devices.CONCLUSION S-ICD appears to be effective and safe in patients with complex congenital heart disease.展开更多
Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pedia...Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pediatric heart failure is evolving but our ability to prognosticate outcomes in pediatric heart acute heart failure is extremely limited due to lack of data.In adult heart failure patients,the role of cardiac biomarkers has exponentially increased over the last two decades.Current guidelines for management of heart failure emphasize the role of cardiac biomarkers in diagnosis,management and prognostication of heart failure.It is also noteworthy that these biomarkers reflect important biological processes that also open up the possibility of therapeutic targets.There is however,a significant gap present in the pediatric population with regards to biomarkers in pediatric heart failure.Here,we seek to review available data regarding cardiac biomarkers in the pediatric population and also explore some of the emerging biomarkers from adult literature that may be pertinent to pediatric heart failure.展开更多
Background The survival rate of preterm infants with critical congenital heart disease (P-CCHD) has been improved by medicine advances. The aims of this study were to investigate the contemporary treatments for shor...Background The survival rate of preterm infants with critical congenital heart disease (P-CCHD) has been improved by medicine advances. The aims of this study were to investigate the contemporary treatments for short-term outcomes of P-CCHD and to evaluate risk factors associated with the outcomes. Methods Sixty-four P-CCHD patients admitted to Guangdong General Hospital between 2011 and 2015 were included in this study. De-mographic characteristics and patient records were reviewed. Logistic regression was used to analyze the risk fac-tors of P-CCHD outcome. Results Thirty-six patients underwent surgical treatments for cardiac anomalies. Moreover, 31.25% of the P-CCHD infants did not receive surgery because these parents refused further treat-ment. The in-hospital mortality rate was 8.3% for the patients who underwent surgeries. During a median follow-up of 1.2 years, the survivors were basically healthy. However, mental and physical growth retardation remained. Conclusions Compared to infants in developed Western countries, the treatments and short-term outcomes of P-CCHD infants were satisfactory. However, the long-term outcomes remain to be determined.展开更多
Objectives: Since Spitz et al. reported the prognostic classification of esophageal atresia (EA) patients in 1994, decades have been past and there have been many advances in surgery and neonatology. Nevertheless, the...Objectives: Since Spitz et al. reported the prognostic classification of esophageal atresia (EA) patients in 1994, decades have been past and there have been many advances in surgery and neonatology. Nevertheless, there have been very few reports according to the recent outcome of the neonates with EA, and otherwise, time has come to re-evaluate the credibility of this classification. The aim of this study was to validate the recent prognosis of the EA. Methods: Patient data were collected from 22 cooperative facilities during the 5 year period from 2005 to 2009 in Kyushu area, Japan. Total of 100 EA patients were retrospectively reviewed according to their characteristics and the outcome. Patient who missed the characteristics and outcome was excluded from the respective data. Results: Only 29.8% (28/94) was prenatally diagnosed and 52.0 (52/100) had associated anomalies including major congenital heart disease (CHD), abnormal chromosome, and others. According to the operation, primary anastomosis was performed 57.0% (57/100) and the staged operation was performed 34.0% (34/100). Survival rate in the neonatal period was 89.0% (89/ 100), and overall survival rate was 78.0% (78/100). According to the Spitz classification, if patients with associated anomalies were excluded, survival rate of Group 1 (>1500 g and no CHD) was 93.8% (61/65), Group 2 (<1500 g or CHD) was 68.4% (13/19), and Group 3 (<1500 g and CHD) was 50% (1/2). Conclusion: EA was proved to be rarely diagnosed prenatally. Primary outcome of the Group 1 and Group 3 in Spitz classification were fairly good, but Group 2 was worse as ever. The comprehensive treatment strategy for EA patients with birth weight under 1500 g or CHD should be reconsidered to improve the overall outcome.展开更多
BACKGROUND Cardiovascular disease is the leading cause of death in patients with Turner syndrome(TS),and cardiovascular surgery is frequently required for management of these patients.TS is associated with medical com...BACKGROUND Cardiovascular disease is the leading cause of death in patients with Turner syndrome(TS),and cardiovascular surgery is frequently required for management of these patients.TS is associated with medical comorbidities than can complicate the care of this patient population.AIM To describe the cardiovascular surgical outcomes of patients with TS.METHODS A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017.The baseline clinical data of these patients were reviewed including demographics,medical comorbidities,congenital heart disease history,and medications.Echocardiographic reports were analyzed in detail.Operative reports and surgical hospital courses were reviewed.Long-term mortality was determined using medical records and the Social Security Death Index.Survival analysis was performed with the Kaplan Meier method.RESULTS The cohort comprised 51 TS patients,average age at the time of surgery at Mayo Clinic was 28(8-41)years,and 23(45%)patients were under the age of 18.At the time of first Mayo Clinic surgery,18(35%)patients had previously undergone cardiac surgery at another institution.The most common procedures were repair of aortic coarctation in 14(28%)patients,aortic valve replacement in 6(12%)patients,and composite aortic root/ascending aorta replacement in 7(14%)patients,with 7 patients undergoing repair of more than one lesion.Aortic dissection required operative intervention in 5 patients.After initial Mayo Clinic surgery,subsequent operations were required in 6(13%)patients.Average hospital length of stay was 6±2 d.There were 4(8%)early surgical deaths.Freedom from death was 97%and 89%at 10 and 20 years,and the freedom from reoperation was 93%and 81%at 10 and 20 years.CONCLUSION Cardiovascular surgery is associated with 8%early mortality given the medical complexity of TS patients.Those who survive to dismissal have good survival.Later cardiovascular reoperations are not rare.展开更多
One of the most common types of cyanotic congenital heart disease is Tetralogy of Fallot(ToF).Treatment has constantly increased since the first surgical repair in 1954.Excellent treatment having long-term survival(30...One of the most common types of cyanotic congenital heart disease is Tetralogy of Fallot(ToF).Treatment has constantly increased since the first surgical repair in 1954.Excellent treatment having long-term survival(30 years survival ranges from 68.5%to 90.5%)is available for the ToF.However conventional and frequently required re-interventions include residual issues like right ventricular outflow tract obstruction,pulmonary regurgitation and(ventricular)arrhythmia.Right ventricular dysfunction might lead to longstanding pulmonary regurgitation and/or stenosis.It is important to perform pulmonary valve replacement or relief of pulmonary stenosis prior to irreversible right ventricular dysfunction,though determining optimal timing of pulmonary valve replacement is a problematic task due to various reasons.As seen in longstanding pulmonary regurgitation,the biological mechanisms underlying dysfunction of the right ventricle is often unclear.Various techniques of assessing the right ventricle are used to predict imminent dysfunction.The interventricular,ventriculo-arterial and atrioventricular interactions of right ventricle are not completely explained but play significant role in right ventricle performance.This review focuses on providing a brief overview of the history of ToF,describing the current strategies for treatment and describing the long-term survival,residual lesions and re-interventions following repair.Remaining related challenges and present condition of the art regarding these challenges are also illustrated.展开更多
Objective: Long-term results after the Senning operation for transposition of the great arteries are little known. Sinus node dysfunction and systemic ventricular dysfunction are crucial in patient survival. We evalua...Objective: Long-term results after the Senning operation for transposition of the great arteries are little known. Sinus node dysfunction and systemic ventricular dysfunction are crucial in patient survival. We evaluated the results, long term outcome and quality of life in a group of 39 patients. Methods: The study was a retrospective analysis, of 39 (39/40 = 97.5%) surgical surviving patients, submitted to Senning operation, with a mean follow-up time of 14.7 +/– 3.1 years. Electrocardiogram, echocardiogram, and chest radiograph series were performed every 6 months. Thirty six patients of this series underwent Holter study and ergometric test, to evaluate the physical capacity. Three patients living overseas were excluded. Results: There was only one late death (1/39) (late mortality = 2.5%): a 16 year-old patient had a no cardiac death. The actuarial survival was 95.0% (38/40) (simple or with little VSD, TGA). The probability of staying in sinus rhythm, in 39 surviving patients was 77.1% (30) or normal right ventricular function was 76.5% (29), 10 to 20 years after operation. The incidence of sinus node and right ventricular dysfunction increased gradually over time. No re-operations and pacemaker implantation, was performed. Functional class: I = 30 (85.7%) cases and functional class II = 5 (14.3%) cases. Conclusions: Patients with simple TGA submitted to Senning procedure in our experience, presented during late follow-up: 1—Low incidence of right ventricular dysfunction and active arrhythmias;2—Low mortality and no sudden death recorded;3—Good quality of life and 4—Satisfactory surgical results (free of re-operation or definitive pacemaker implantation).展开更多
Background: The vasoactive-ventilation-renal (VVR) score includes pulmonary and renal dysfunctions not previously addressed by the vasoactive inotrope score (VIS) and may be a better predictor of cardiac care unit (CC...Background: The vasoactive-ventilation-renal (VVR) score includes pulmonary and renal dysfunctions not previously addressed by the vasoactive inotrope score (VIS) and may be a better predictor of cardiac care unit (CCU) length of stay (LOS) in patients undergoing re-entry sternotomy (defined as no earlier than 30 days after previous sternotomy) for congenital heart disease (CHD). Methods: Patients undergoing re-entry sternotomy for CHD from August 1, 2009 to June 30, 2016 were studied retrospectively. A total of 96 patients undergoing 133 re-entry procedures were identified. VVR scores were calculated on CCU admission post-procedure (at 0 hour), 24-hour, and 48-hour after admission to the CCU. The response variable was CCU LOS.? Recursive partition analysis identified variables predicting LOS. Results: 133 re-entry sternotomies in 96 patients made up the samples of the database;11 samples were removed due to incomplete data or placement on ECMO. Of the initial 25 features, 5 were removed for near zero variance and 3 categorical features were removed for non-information. Covariance analysis did not demonstrate any significant correlation amongst the remaining features. Initial recursive tree regression using ANOVA, cross validation and conditional predictive p-value (cp) = 0.01 produced 3 trees. The tree with lowest cross validation error was selected. The resulting 2 split trees with ventilator days less than 20 days and VVR score at 48 hours greater than 23 identified three CCU LOS groups with mean CCU LOS of 77.6, 55.1, and 9.5 days. Conclusions: Recursive partition analysis identified ventilator days greater than 20 days and the sub-population VVR at 48 hours as predictive of CCU LOS in patients undergoing re-entry sternotomy for CHD.展开更多
基金supported through Herma Heart Institute and the Medical College ofWisconsin.
文摘Background:Women with congenital heart disease(CHD)have increased risk for adverse events during pregnancy and delivery.Prior studies have assessed pregnancy and delivery outcomes at tertiary referral centers(TRC).The aim of our study was to assess pregnancy outcomes in women with CHD who deliver in a non-tertiary referral center(non-TRC).Methods:Clinical demographics were collected,including anatomic complexity,physiologic state and pre-pregnancy risk assessment.Patients were stratified by delivery location,either TRC or non-TRC.Maternal and neonatal complications of pregnancy were reported.Results:Women with CHD who delivered in a TRC had a higher pre-pregnancy risk when assessed by the Zahar and CARPREG-II scores,and had more patients fall into a higher WHO classification.There was no difference in rates of maternal cardiac complications between delivery locations(11%)and neonatal complications(20%)between deliveries at TRC and non-TRC.Conclusions:There were not increased maternal cardiac or neonatal complications when delivery occurred at a non-TRC.Neonatal complications remained high regardless of delivery location.This study suggests that proper risk assessment may help identify women who are candidates for safe delivery at non-TRC in women with CHD,and that neonatal resources should be considered when planning delivery location.
文摘Purpose: The purpose of this review was to identify the short-term and long-term outcomes of pregnancy in women suffering from congenital heart disease (CHD). Methods: An integrative review is used to identify the pregnancy outcomes in women with CHD. Data search was between 2010 and 2020 using Google Scholar, Scopus, Web of Science, Science Direct, Pub Med, Medline, CINAHL, EBSCO, Cochrane, and EBSCO host. Sixteen articles met the eligibility criteria. Results: The sixteen reviewed articles utilized descriptive retrospective and prospective design. Themes were short-term outcomes that include;cardiac maternal and obstetric outcomes, and long-term outcomes. Conclusion: Previous uncertainty about the ability of CHD patients to successfully become pregnant and deliver safely has replaced by the recognition that a large number can have excellent outcomes. However, these patients do continue to have higher cardiac, obstetric, and fetal risks than the general population. This illuminates the importance of preconception counseling and risk assessment for women suffering from CHD regarding the expected maternal and fetal outcomes. Moreover, there is a need for providing accurate and appropriate education about pregnancy and delivery options.
基金Financial support for this work(E.V.)was provided by the New National Excellence Program of the Ministry for Innovation and Technology from the Source of the National Research,Development and Innovation Fund(ÚNKP-21–3-II-SE-5)(www.unkp.hu)by the Semmelweis University(EFOP-3.6.3.-VEKOP-16–2017–00009)(https://semmelweis.hu/phd/2021/11/09/).
文摘Objective:Neonates with congenital heart disease(CHD)and perinatal stroke have high mortality and survivors are at risk for poor long-term neurodevelopmental outcome.The aim of this study was to assess the risk factors and outcome of neonates with both CHD and MRI-confirmed perinatal stroke(Study Group)and compare those to the risk factors and outcome of infants matched for CHD without stroke(Control-1)and of infants matched for MRI-confirmed stroke without CHD(Control-2).Methods:We conducted a population-based case-control study enrolling 28 term neonates with CHD and MRI-confirmed acute perinatal stroke born between 2007–2017 in the Central-Hungarian Region.Each of the control groups included 56 infants.The Bayley Scales of Infant Development-II,the Brunet-Lézine test and the Binet Intelligence scales-V were used for neurodevelopmental follow-up at a median age of 61 months.Results:Mortality was highest in the Study Group(25%compared to 5%and 2%,respectively,p=0.001).Adverse neurodevelopmental outcome was prevalent in the Study(53%)and Control-2 Groups(52%,p=0.03).Significantly different parameters among the three groups included Apgar scores,mode of delivery,gestational age at birth,cardiac interventions and twin pregnancy.In a multivariable regression analysis adjusted for clinically relevant parameters,patients in the Study Group had significantly higher odds for mortality compared to patients in the Control-1 Group(OR:6.595%CI:1.1–39.4).Conclusions:Neonates with perinatal stroke and CHD are at a higher risk for dying compared to neonates with CHD without stroke.In addition,the stroke-associated direct insult to the brain likely plays an important role in the development of neurodevelopmental morbidity in these patients.
文摘There is a growing population of adults with congenital heart disease(CHD) due to improved survival beyond childhood.It has been suggested that adults with CHD may be at increased risk for mental health problems,particularly depression.The reported incidence of depression in CHD varies from 9% to 30%.This review examines the evidence for a higher depression rate in CHD vs general population.Possible explanations are offered from a variety of disease models,ranging from brain injury to the psychoanalytical approach.Risk factors for an abnormal emotional adjustment and depression include early exposure to stress from illness and medical interventions in infancy,separation from the parents during hospitalizations and brain organic syndromes.Later in life,patients often have to cope with physical limitations.Recent improvements in care may be protective.Current patients may benefit from an earlier age at first surgical intervention,fewer reoperations and inclusion to the mainstream schooling,among other factors.At this point,there is little systematic knowledge about evidence-based therapeutic interventions for depression in adults with CHD.Health care providers of patients with CHD should be aware of mental health challenges and may take a more proactive approach to identifying patients at risk for depression.
文摘Background:Patients with genetic syndromes who undergo surgery to correct congenital heart defects can be at risk for increased morbidity or mortality.Surgical outcomes and postoperative courses following congenital heart surgery in patients with Kabuki-Syndrome(KS)have not been well studied.Objectives:The purpose of this study was to describe the postoperative courses and associated outcomes in the largest set of KS patients undergoing congenital heart surgery to date.Methods:Patients with a confirmed molecular diagnosis of KS and a diagnosis of a CHD admitted to Texas Children’s Hospital between January 1,2000 and January 1,2020 were included(n=20).Demographics and medical histories were collected from the hospitals’electronic health records.Results:Of 20 patients identified with KS and a CHD,15 required surgical correction of their congenital cardiac malformation.Median age and weight at the time of surgery was 2 months and 4.1 kg,respectively.Median duration of hospital stay was 49 days for all surgeries and 151 days for the Norwood procedure.Postoperative infections and pleural effusions were detected and treated in 45.8%and 50%of patients,respectively.There was no in-hospital mortality for any surgery.Median follow up time was 5.6 years;survival at 6 years was 94%.Conclusions:Although KS patients seem to be at increased risk for a more complicated,prolonged postoperative course than that of patients without a genetic syndrome,patients with a diagnosis of a CHD and KS do not appear to be at increased risk of mortality following congenital heart surgery.
文摘AIM To describe the long-term follow-up of patients with complex congenital heart disease who underwent subcutaneous implantable cardiac defibrillator(S-ICD), focusing on local complications, appropriate and inappropriate shocks.METHODS Patients with complex congenital heart disease underwent S-ICD implant in two centers with the conventional technique. Data at follow-up were retrieved from clinical notes and institutional database. RESULTS Eight patients were implanted in two centres between 2010 and 2016. Median age at implant was 37.5 years(range 13-57). All patients who were deemed suitable for S-ICD implant passed the pre-procedural screening. Three patients were previously implanted with a antibradycardia device, one of whom with CRT. In one patient the device was explanted due to local infection. Duringthe total median follow-up of 874 d, one patient had an appropriate and one inappropriate shock triggered by fast atrial tachycardia. None of the patients had inappropriate shocks secondary to T wave oversensing or electrical interference with anti-bradycardia devices.CONCLUSION S-ICD appears to be effective and safe in patients with complex congenital heart disease.
文摘Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pediatric heart failure is evolving but our ability to prognosticate outcomes in pediatric heart acute heart failure is extremely limited due to lack of data.In adult heart failure patients,the role of cardiac biomarkers has exponentially increased over the last two decades.Current guidelines for management of heart failure emphasize the role of cardiac biomarkers in diagnosis,management and prognostication of heart failure.It is also noteworthy that these biomarkers reflect important biological processes that also open up the possibility of therapeutic targets.There is however,a significant gap present in the pediatric population with regards to biomarkers in pediatric heart failure.Here,we seek to review available data regarding cardiac biomarkers in the pediatric population and also explore some of the emerging biomarkers from adult literature that may be pertinent to pediatric heart failure.
基金supported by the National Natural Science Foundation of China(No.8167060825)
文摘Background The survival rate of preterm infants with critical congenital heart disease (P-CCHD) has been improved by medicine advances. The aims of this study were to investigate the contemporary treatments for short-term outcomes of P-CCHD and to evaluate risk factors associated with the outcomes. Methods Sixty-four P-CCHD patients admitted to Guangdong General Hospital between 2011 and 2015 were included in this study. De-mographic characteristics and patient records were reviewed. Logistic regression was used to analyze the risk fac-tors of P-CCHD outcome. Results Thirty-six patients underwent surgical treatments for cardiac anomalies. Moreover, 31.25% of the P-CCHD infants did not receive surgery because these parents refused further treat-ment. The in-hospital mortality rate was 8.3% for the patients who underwent surgeries. During a median follow-up of 1.2 years, the survivors were basically healthy. However, mental and physical growth retardation remained. Conclusions Compared to infants in developed Western countries, the treatments and short-term outcomes of P-CCHD infants were satisfactory. However, the long-term outcomes remain to be determined.
文摘Objectives: Since Spitz et al. reported the prognostic classification of esophageal atresia (EA) patients in 1994, decades have been past and there have been many advances in surgery and neonatology. Nevertheless, there have been very few reports according to the recent outcome of the neonates with EA, and otherwise, time has come to re-evaluate the credibility of this classification. The aim of this study was to validate the recent prognosis of the EA. Methods: Patient data were collected from 22 cooperative facilities during the 5 year period from 2005 to 2009 in Kyushu area, Japan. Total of 100 EA patients were retrospectively reviewed according to their characteristics and the outcome. Patient who missed the characteristics and outcome was excluded from the respective data. Results: Only 29.8% (28/94) was prenatally diagnosed and 52.0 (52/100) had associated anomalies including major congenital heart disease (CHD), abnormal chromosome, and others. According to the operation, primary anastomosis was performed 57.0% (57/100) and the staged operation was performed 34.0% (34/100). Survival rate in the neonatal period was 89.0% (89/ 100), and overall survival rate was 78.0% (78/100). According to the Spitz classification, if patients with associated anomalies were excluded, survival rate of Group 1 (>1500 g and no CHD) was 93.8% (61/65), Group 2 (<1500 g or CHD) was 68.4% (13/19), and Group 3 (<1500 g and CHD) was 50% (1/2). Conclusion: EA was proved to be rarely diagnosed prenatally. Primary outcome of the Group 1 and Group 3 in Spitz classification were fairly good, but Group 2 was worse as ever. The comprehensive treatment strategy for EA patients with birth weight under 1500 g or CHD should be reconsidered to improve the overall outcome.
文摘BACKGROUND Cardiovascular disease is the leading cause of death in patients with Turner syndrome(TS),and cardiovascular surgery is frequently required for management of these patients.TS is associated with medical comorbidities than can complicate the care of this patient population.AIM To describe the cardiovascular surgical outcomes of patients with TS.METHODS A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017.The baseline clinical data of these patients were reviewed including demographics,medical comorbidities,congenital heart disease history,and medications.Echocardiographic reports were analyzed in detail.Operative reports and surgical hospital courses were reviewed.Long-term mortality was determined using medical records and the Social Security Death Index.Survival analysis was performed with the Kaplan Meier method.RESULTS The cohort comprised 51 TS patients,average age at the time of surgery at Mayo Clinic was 28(8-41)years,and 23(45%)patients were under the age of 18.At the time of first Mayo Clinic surgery,18(35%)patients had previously undergone cardiac surgery at another institution.The most common procedures were repair of aortic coarctation in 14(28%)patients,aortic valve replacement in 6(12%)patients,and composite aortic root/ascending aorta replacement in 7(14%)patients,with 7 patients undergoing repair of more than one lesion.Aortic dissection required operative intervention in 5 patients.After initial Mayo Clinic surgery,subsequent operations were required in 6(13%)patients.Average hospital length of stay was 6±2 d.There were 4(8%)early surgical deaths.Freedom from death was 97%and 89%at 10 and 20 years,and the freedom from reoperation was 93%and 81%at 10 and 20 years.CONCLUSION Cardiovascular surgery is associated with 8%early mortality given the medical complexity of TS patients.Those who survive to dismissal have good survival.Later cardiovascular reoperations are not rare.
文摘One of the most common types of cyanotic congenital heart disease is Tetralogy of Fallot(ToF).Treatment has constantly increased since the first surgical repair in 1954.Excellent treatment having long-term survival(30 years survival ranges from 68.5%to 90.5%)is available for the ToF.However conventional and frequently required re-interventions include residual issues like right ventricular outflow tract obstruction,pulmonary regurgitation and(ventricular)arrhythmia.Right ventricular dysfunction might lead to longstanding pulmonary regurgitation and/or stenosis.It is important to perform pulmonary valve replacement or relief of pulmonary stenosis prior to irreversible right ventricular dysfunction,though determining optimal timing of pulmonary valve replacement is a problematic task due to various reasons.As seen in longstanding pulmonary regurgitation,the biological mechanisms underlying dysfunction of the right ventricle is often unclear.Various techniques of assessing the right ventricle are used to predict imminent dysfunction.The interventricular,ventriculo-arterial and atrioventricular interactions of right ventricle are not completely explained but play significant role in right ventricle performance.This review focuses on providing a brief overview of the history of ToF,describing the current strategies for treatment and describing the long-term survival,residual lesions and re-interventions following repair.Remaining related challenges and present condition of the art regarding these challenges are also illustrated.
文摘Objective: Long-term results after the Senning operation for transposition of the great arteries are little known. Sinus node dysfunction and systemic ventricular dysfunction are crucial in patient survival. We evaluated the results, long term outcome and quality of life in a group of 39 patients. Methods: The study was a retrospective analysis, of 39 (39/40 = 97.5%) surgical surviving patients, submitted to Senning operation, with a mean follow-up time of 14.7 +/– 3.1 years. Electrocardiogram, echocardiogram, and chest radiograph series were performed every 6 months. Thirty six patients of this series underwent Holter study and ergometric test, to evaluate the physical capacity. Three patients living overseas were excluded. Results: There was only one late death (1/39) (late mortality = 2.5%): a 16 year-old patient had a no cardiac death. The actuarial survival was 95.0% (38/40) (simple or with little VSD, TGA). The probability of staying in sinus rhythm, in 39 surviving patients was 77.1% (30) or normal right ventricular function was 76.5% (29), 10 to 20 years after operation. The incidence of sinus node and right ventricular dysfunction increased gradually over time. No re-operations and pacemaker implantation, was performed. Functional class: I = 30 (85.7%) cases and functional class II = 5 (14.3%) cases. Conclusions: Patients with simple TGA submitted to Senning procedure in our experience, presented during late follow-up: 1—Low incidence of right ventricular dysfunction and active arrhythmias;2—Low mortality and no sudden death recorded;3—Good quality of life and 4—Satisfactory surgical results (free of re-operation or definitive pacemaker implantation).
文摘Background: The vasoactive-ventilation-renal (VVR) score includes pulmonary and renal dysfunctions not previously addressed by the vasoactive inotrope score (VIS) and may be a better predictor of cardiac care unit (CCU) length of stay (LOS) in patients undergoing re-entry sternotomy (defined as no earlier than 30 days after previous sternotomy) for congenital heart disease (CHD). Methods: Patients undergoing re-entry sternotomy for CHD from August 1, 2009 to June 30, 2016 were studied retrospectively. A total of 96 patients undergoing 133 re-entry procedures were identified. VVR scores were calculated on CCU admission post-procedure (at 0 hour), 24-hour, and 48-hour after admission to the CCU. The response variable was CCU LOS.? Recursive partition analysis identified variables predicting LOS. Results: 133 re-entry sternotomies in 96 patients made up the samples of the database;11 samples were removed due to incomplete data or placement on ECMO. Of the initial 25 features, 5 were removed for near zero variance and 3 categorical features were removed for non-information. Covariance analysis did not demonstrate any significant correlation amongst the remaining features. Initial recursive tree regression using ANOVA, cross validation and conditional predictive p-value (cp) = 0.01 produced 3 trees. The tree with lowest cross validation error was selected. The resulting 2 split trees with ventilator days less than 20 days and VVR score at 48 hours greater than 23 identified three CCU LOS groups with mean CCU LOS of 77.6, 55.1, and 9.5 days. Conclusions: Recursive partition analysis identified ventilator days greater than 20 days and the sub-population VVR at 48 hours as predictive of CCU LOS in patients undergoing re-entry sternotomy for CHD.