Congenital Heart Disease Referred for Surgery: Analysis and Epidemiological Description in the Cardiology Department of CHU Ignace Deen

Introduction: Congenital heart disease includes all cardiac and vascular malformations. It accounts for approximately one third of all congenital malformations and is a public health problem, particularly in developing countries. The aim of this study was to analyze the epidemiological, clinical and paraclinical aspects of congenital heart disease. Methods: This was a retrospective descriptive and analytical study based on the records of 135 patients referred for surgery and followed up in the cardiology department of the Ignace Deen University Hospital, collected in November 2022. Results: Hospital prevalence was 5%. The mean age was 71 months, ranging from 1 month to 19 years. The age group over 24 months was the most represented (62%). The M/F sex ratio was 1.36. Urban origin was predominant (58%). The rate of children not attending school or dropping out was high (16%). Siblings with fewer than 4 children were the most common (88%). A heart murmur was the most frequent sign (78%), followed by cyanosis (36%) and heart failure (29%). The association between heart murmurs and CHD was proven with a p-value Conclusion: CHDs represent the main indication for paediatric cardiac surgery and follow-up (95%). We stress the importance of implementing a screening and management strategy for congenital heart disease.

A New Three-Dimensional(3D)Printing Prepress Algorithm for Simulation of Planned Surgery for Congenital Heart Disease

Background:Three-dimensional printing technology may become a key factor in transforming clinical practice and in significant improvement of treatment outcomes.The introduction of this technique into pediatric cardiac surgery will allow us to study features of the anatomy and spatial relations of a defect and to simulate the optimal surgical repair on a printed model in every individual case.Methods:We performed the prospective cohort study which included 29 children with congenital heart defects.The hearts and the great vessels were modeled and printed out.Measurements of the same cardiac areas were taken in the same planes and points at multislice computed tomography images(group 1)and on printed 3D models of the hearts(group 2).Pre-printing treatment of the multislice computed tomography data and 3D model preparation were performed according to a newly developed algorithm.Results:The measurements taken on the 3D-printed cardiac models and the tomographic images did not differ significantly,which allowed us to conclude that the models were highly accurate and informative.The new algorithm greatly simplifies and speeds up the preparation of a 3D model for printing,while maintaining high accuracy and level of detail.Conclusions:The 3D-printed models provide an accurate preoperative assessment of the anatomy of a defect in each case.The new algorithm has several important advantages over other available programs.They enable the development of customized preliminary plans for surgical repair of each specific complex congenital heart disease,predict possible issues,determine the optimal surgical tactics,and significantly improve surgical outcomes.

Effect of nursing intervention based on Maslow's hierarchy of needs in patients with coronary heart disease interventional surgery

BACKGROUND It is very important to provide effective nursing programs to regulate the physical and mental state of patients and to improve treatment compliance after interventional surgery for coronary heart disease(CHD).AIM To explore the effect of a nursing intervention based on Maslow’s hierarchy of needs theory on patients with CHD undergoing percutaneous coronary intervention.METHODS Ninety-four patients with CHD undergoing interventional surgery in our hospital from January 2020 to February 2021 were randomly divided into a research group(n=47)and a control group(n=47).The control group received routine nursing,and the research group received a nursing intervention based on Maslow’s hierarchy of needs theory.The scores of self-efficacy,negative emotion[depression(SDS),anxiety(SAS)],intervention compliance(standardized medication,moderate exercise,healthy diet,and regular review),and nursing satisfaction were calculated before and after intervention for the two groups.RESULTS Before intervention,there was no significant difference in the scores of disease general management self-efficacy,disease management self-efficacy,and total self-efficacy between the two groups(P=0.795,0.479,and 0.659,respectively).After intervention,these three scores in the research group were higher than those in the control group(P<0.001).Before intervention,there was no significant difference in the scores of SAS and SDS between the two groups(P=0.149 and 0.347,respectively).After intervention,the scores of SAS and SDS in the research group were lower than those in the control group(P<0.001).The standardized drug use rate(97.87%),moderate exercise rate(97.87%),healthy diet rate(95.74%),and regular reexamination rate(97.87%)in the research group were higher than those in the control group(85.11%,82.98%,80.85%,and 87.23%,respectively)(P=0.027,0.014,0.025,and 0.049,respectively).Nursing job satisfaction in the research group(93.62%)was higher than that in the control group(78.72%)(P=0.036).CONCLUSION A nursing program based on Maslow’s hierarchy of needs theory can effectively alleviate negative emotion,enhance self-efficacy and intervention compliance,and ensure that the patients are highly satisfied with the nursing work.

Evaluation of Post-Operative Atrial Fibrillation after Cardiac Surgery for Adult Congenital Heart Disease

Background:Post-operative atrial fibrillation(POAF)frequently occurs after cardiac surgery.Although adult congenital heart disease(ACHD)patients have higher rates of arrhythmia than the general population,there is scant literature on POAF in ACHD patients.Objectives:Identify key risk factors associated with post-operative atrial fibrillation and evaluate the short-and mid-term significance of developing POAF.Methods:A retrospective cohort study was conducted of ACHD patients from 2013–2021 at the University of Colorado Hospital and Children’s Hospital of Colorado.The institutional Society of Thoracic Surgeons(STS)surgical registry was used to identify patients≥18-year-old with congenital heart disease who underwent cardiac surgery during the study period.Results:A total of 168 patients(48%female)were included.The median age was 36 years(IQR 28–48).Onehundred and fifty patients(90%)had moderate ACHD anatomical complexity,and 10 patients(6%)had severe ACHD anatomical complexity based on initial ACHD diagnosis.POAF occurred in 40(24%)patients.Older age,history of supraventricular tachycardia,intra-operative arrhythmia,and post-operative hypokalemia independently predicted POAF.POAF was associated with an increased length of stay(8 vs.5 days,p<0.001)and recurrence of atrial fibrillation(46%vs.21%,OR 3.35,p=0.002)but did not predict mortality,stroke,or bleeding event.Conclusion:Atrial fibrillation is a common complication after cardiac surgery in the ACHD population.Older age,history of supraventricular tachycardia,intra-operative arrhythmia,and post-operative hypokalemia independently predicted POAF.Further investigation is needed to understand the long-term impacts of POAF.

Effect of Exercise-Based Cardiac Rehabilitation on Cardiorespiratory Fitness in Adults with Congenital Heart Disease

Background:The purpose of this study was to investigate whether patients with adult congenital heart disease(ACHD)benefit from exercise-based cardiac rehabilitation(CR)short-and long-term with regard to improvement of cardiorespiratory fitness.Methods:Cardiopulmonary exercise tests(CPET)completed by ACHD patients between January 2000 and October 2019 were analysed retrospectively.Linear mixed models were performed for peak oxygen consumption(VO_(2))with patients as random effect and age,sex,disease classification,preceding surgery(≤3 months)and preceding CR(≤4 weeks for short term and>4 weeks for long term)as fixed effects.Results:1056 CPETs of 311 ACHD patients with simple(7),moderate(188)or great(116)complexity heart defects were analysed.The 59 patients who completed a CR(median age 27 yrs,38%females)increased peak VO_(2)from before to after CR by a median of 2.7(IQR–0.6 to 5.5)ml/kg/min.However,in the multivariate mixed model,peak VO_(2)was non-significantly increased short-term after CR(β0.8,95%CI–0.7 to 2.4),not maintained long-term after CR(β0.0,95%CI–1.7 to 1.6)but significantly reduced after surgery(β–5.1,95%CI–7.1 to–3.1).The 20 CR patients after surgery increased their peak VO_(2)by 6.2(IQR 3.6–9.5)ml/kg/min,while the 39 CR patients without preceding surgery increased it by 0.9(IQR–1.5 to 3.1)ml/kg/min.Conclusions:The increase in peak VO_(2)with CR was mainly due to recovery from surgical intervention.The small independent benefit from CR was not maintained long-term,highlighting the potential to improve current CR concepts in ACHD populations.

Management of Specific Complications after Congenital Heart Surgery(I)

In addition to the general consequences of surgery and cardiopulmonary by-pass, lesion-specific complications can occur after surgery for congenital heart disease. It is important for the pediatric intensive care specialist to fully understand the preoperative anatomy and the intraoperative details of these patients. This allows a timely and appropriate treatment of general and lesion-specific complications. In this article we provide a list of commonly-performed surgical procedures and possible associated problems to be anticipated in the early postoperative period. Then it follows a discussion about the diagnosis and management of these complications, based on their pathophysiological features.

Unmet Needs in Pediatric and Congenital Heart Surgery:A Review

Pediatric and congenital heart disease(PCHD)affects millions of children worldwide,including over one million babies born with congenital heart disease(CHD)each year and 300,000 children dying from rheumatic heart disease(RHD)yearly.Although the vast majority of children born with CHD in high-income countries now reach adulthood and RHD is nearly eradicated in these countries,most of the world cannot access the necessary care to prevent or mitigate PCHD.In low-and middle-income countries,over 90%of children with PCHD cannot receive the care they need,as over 100 countries and territories lack local cardiac surgical capacity.The unmet needs for PCHD are large,albeit still poorly quantified,resulting in a considerable socioeconomic impact at the individual and societal levels.This review highlights the extensive opportunities to improve access to and scale PCHD care by strengthening research,clinical care delivery,capacity-building,advocacy,health policy,and financing.We discuss global disparities in access to congenital heart surgery,the socioeconomic impact of untreated PCHD,and propose strategies for scaling pediatric and congenital cardiac care.Our recommendations focus on enhancing research and data collection,expanding training programs,improving healthcare infrastructure,advocating for policy changes,leveraging technological innovations,fostering international collaborations,and developing comprehensive care models.

Interventional treatment for congenital heart disease and its severe complications

Background To approach the incidence, cause and possible treatment of severe complications induced during or after congenital heart disease interventions. Methods Interventional procedures of congenital heart disease were performed in 654 patients from January 2003 to October 2009, which were divided into four groups, i.e. patent ductus arteriosus （PDA）, atrial septum defect （ASD）, pulmonary stenosis（PS）, ventricular septum defect（VSD）, among them 32 patients with severe complications were retrospectively analyzed. Results A total of 654 cases accepted the treatment of intrusion. There was one death in all the patients, ten patients failed in the operation. The overall severe complication rate was 4.89% （32/654）, in which 2.29%（4/175） in PDA group, 5.26%（10/190） in ASD group, 5.77% （3/52） in PS group, 6.33%（15/237） in VSD group, respectively. Conclusions The severe complication rates of interventional therapy for congenital heart disease are low, it is a relatively safe interventional method, and careful supervision is necessary during or after procedure.

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few centers.This study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary center.Methods:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May 2022.Technical success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new regurgitation.Follow-up clinical records and echocardiography were obtained during the prenatal and postnatal periods.Results:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 years.The median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful procedures.One patient had pulmonary valve perforation without balloon dilation.No procedure-related deaths or significant complications occurred.However,one neonatal death occurred due to heart and renal failure.The median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up visit.Conclusion:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

Comparison of clinical outcomes and postoperative recovery between two open heart surgeries:minimally invasive right subaxillary vertical thoracomy and traditional median sternotomy

Objective: To compare the clinical outcomes of minimally invasive right subaxillary vertical thoracotomy and traditional median stemotomy through right atrium in treatment of common congenital heart diseases. Methods: Clinical data of 59 cases of common congenital heart diseases treated with minimally invasive right axillary vertical thoracotomy from May, 2011 to February, 2013 and 77 cases of same diseases with traditional median stemotomy in the past three years were retrospectively analyzed, including atrial septal defect, membranous ventricular septal defect and partial endocardial cushion defect. The results were compared from the two groups, including the time for operation and cardiopulmonary bypass, amount of blood transfusion, postoperative drainage, ventilation time, hospital stay, and prognosis. Results: No severe complications happened in both groups, like deaths or secondary surgery caused by bleeding. No significant differences were in CPB time and postoperative ventilator time between groups (P>0.05), while for all of the operative time, the length of incision, postoperative drainage and hospital stay, minimally invasive right axillary vertical thoracotomy was superior to median stemotomy, with statistically significant differences (P<0.05). In six month followup after operation, no complications of residual deformity and pericardial effusion were found in both groups by doing echocardiography, but mild pectus carinatum was found in 8 patients in the traditional median sternotomy group (traditional group), whereas patients in another group were well recovered. Conclusions: Minimally invasive right subaxillary vertical thoracotomy for common congenital heart diseases is as safe as traditional median stemotomy, without the increasing incidence of postoperative complications. Additionally, compared with traditional median stemotomy, minimally - invasive right subaxillary vertical thoracotomy is better in the aspects of hidden incision, appearance, and postoperative recovery.

Patient-specific three-dimensional printed heart models benefit preoperative planning for complex congenital heart disease

Background Preoperative planning for children with congenital heart diseases remains crucial and challenging.This study aimed to investigate the roles of three-dimensional printed patient-specific heart models in the presurgical planning for complex congenital heart disease.Methods From May 2017 to January 2018,15 children diagnosed with complex congenital heart disease were included in this study.Heart models were printed based on computed tomography (CT) imaging reconstruction by a 3D printer with photosensitive resin using the stereolithography apparatus technology.Surgery options were first evaluated by a sophisticated cardiac surgery group using CT images only,and then surgical plans were also set up based on heart models.Results Fifteen 3D printed heart models were successfully generated.According to the decisions based on CT,13 cases were consistent with real options,while the other 2 were not.According to 3D printed heart models,all the 15 cases were consistent with real options.Unfortunately,one child diagnosed with complete transposition of great arteries combined with interruption of aortic arch (type A) died 5 days after operation due to postoperative low cardiac output syndrome.The cardiologists,especially the younger ones,considered that these 3D printed heart models with tangible,physical and comprehensive illustrations were beneficial for preoperative planning of complex congenital heart diseases.Conclusion 3D printed heart models are beneficial and promising in preoperative planning for complex congenital heart diseases,and are able to help conform or even improve the surgery options.

Congenital coronary artery anomalies silent until geriatric age： non-invasive assessment, angiography tips, and treatment

Coronary artery anomalies （CAAs） may be discovered more often as incidental findings during the normal diagnostic process for other cardiac diseases or less frequently on the basis of manifestations of myocardial ischemia. The cardiovascular professional may be involved in their angiographic diagnosis, fimctional assessment and eventual endovascular treatment. A complete angiographic definition is mandatory in order to understand the functional effects and plan any intervention in CAAs： computed tomography and magnetic resonance imaging are useful non-invasive tools to detect three-dimensional morphology of the anomalies and its relationships with contiguous cardiac structures, whereas coronary arteriography remains the gold standard for a definitive anatomic picture. A practical idea of the possible functional sig- nificance is mandatory for deciding how to manage CAAs： non-invasive stress tests and in particular the invasive pharmacological stress tests with or without intravascular ultrasound monitoring can assess correctly the functional significance of the most CAAs. Finally, the knowledge of the particular endovascular techniques and material is of paramount importance for achieving technical and clinical success. CAAs represent a complex issue, which rarely involve the cardiovascular professional at different levels. A timely practical knowledge of the main issues regarding CAAs is important in the management of such entities.

Valvuloplasty of fetal pulmonary atresia with intact ventricular septum and hypoplastic right heart: Mid-term follow-up results

Objective:This study aimed to analyze and evaluate the results of mid-term follow-up after fetal pulmonary valvuloplasty(FPV)in fetuses with pulmonary atresia with intact ventricular septum(PA/IVS).Methods:From August 31,2018,to May 31,2019,seven fetuses with PA/IVS and hypoplastic right heart were included in this study.All underwent echocardiography by the same specialist and were operated on by the same team.Intervention and echocardiography data were collected,and changes in the associated indices noted during follow-up were analyzed.Results:All seven fetuses successfully underwent FPV.The median gestational age at FPV was 27.54 weeks.The average FPV procedural time was 6 min.Persistent bradycardia requiring treatment occurred in 4/7 procedures.Finally,five pregnancies were successfully delivered,and the other two were aborted.Compared to data before fetal cardiac interventions(FCI),tricuspid valve annulus diameter/mitral valve annulus diameter(TV/MV)and right ventricle diameter/left ventricle diameter(RV/LV)of all fetuses had progressively improved.The maximum tricuspid regurgitation velocity decreased from 4.60 m/s to 3.64 m/s.The average follow-up time was 30.40±2.05 months.During the follow-up period,the diameter of the tricuspid valve ring in five children continued to improve,and the development rate of the tricuspid valve was relatively obvious from 6 months to 1 year after birth.However,the development of the right ventricle after birth was relatively slow.It was discovered that there were individual variations in the development of the right ventricle during follow-up.Conclusion:The findings support the potential for the development of the right ventricle and tricuspid valve in fetuses with PA/IVS who underwent FCI.Development of the right ventricle and tricuspid valve does not occur synchronously during pregnancy.The right ventricle develops rapidly in utero,but the development of tricuspid valve is more apparent after birth than in utero.

SURGICAL EXPERIENCE OF COMPLEX CONGENITAIL HEART DEFECTS IN CHILDREN AND INFANTS

From January 1982 to June 1990, 2730 patients with congenital heart defects (CHDS) were treated at Xinhua Hospital there were 537 cases of complex lesions. Fifty of 537 patients died, the hospital mortality rate was 9.31%. On the basis of our clinical experience, it is important that the accurate diagnosis was made promptly in neonate with complex CHDs. The surgical results can be improved by the use of PGEI and balloon atrial septostomy in the cyanotic neonate. For the congestive CHDs, the operation must be per formed in the early life to prevent pulmonary hypertension. Improved methods of preoperative and postoperative care have contributed to these results.

Prediction of Pulmonary Arterial Pressure Level after Repair of Congenital Cardiac Communications and Discharge from the Hospital: Role of Down Syndrome and Early Postoperative Hemodynamics

Background:Postoperative pulmonary hypertension limits the success of surgical treatment in some patients with unrestrictive congenital cardiac communications.Identifying patients at risk of developing postoperative pulmonary hypertension is important to individualize follow-up strategies.Methods:We analyzed a prospective cohort of 52 pediatric patients(age 3 to 35 months)looking for perioperative predictors of mildly elevated pulmonary arterial pressure 6 months after surgery,defined as a systolic pressure greater than 30 mmHg by transthoracic echocardiography.This corresponds to a mean pulmonary arterial pressure of>20 mmHg.Clinical,echocardiographic and hemodynamic parameters were investigated.Perioperative hemodynamics was assessed by directly measuring pulmonary and systemic arterial pressures using indwelling catheters.Early postoperative pulmonary hemodynamics was defined as the mean pulmonary/systemic mean arterial pressure ratio(PAP/SAP)obtained per patient during the first 6 h of postoperative care.Results:Among the factors that were investigated as possible predictors,perioperative hemodynamics and the presence of Down syndrome were initially selected using univariate analysis(p<0.030).Early postoperative PAP/SAP was correlated with PAP/SAP obtained in the operating room just after cardiopulmonary bypass(r=0.70,p<0.001),and it was higher in subjects with Down syndrome than in nonsyndromic individuals(p=0.003).Early postoperative PAP/SAP was the only predictor selected using multivariate analysis.It was characterized as an independent predictor after adjustments for possible confounders.An early postoperative PAP/SAP of>0.35 was 76%sensitive and 74%specific at predicting a systolic pulmonary arterial pressure of>30 mmHg 6 months after surgery(hazard ratio with 95%CI 8.972[2.428–33.158],p=0.002).Conclusion:The hypertensive early postoperative behavior of the pulmonary circulation was strongly but not exclusively associated with Down syndrome,and it was characterized as an independent predictor of altered pulmonary arterial pressure after discharge from the hospital.

Blood conservation pediatric cardiac surgery in all ages and complexity levels

AIM To demonstrate the feasibility of blood conservation methods and practice across all ages and risk categories in congenital cardiac surgery.METHODS We retrospectively analyzed a collected database of 356 patients who underwent cardiac surgery using cardiopulmonary bypass(CPB) from 2010-2015. The patients were grouped into blood conservation(n = 138) and nonconservation(n = 218) groups and sub-grouped based on their ages and procedural complexity scores. RESULTS There were no statistical differences in gender,weight,pre-operative and pre-CPB hematocrit levels in both groups. Despite equivalent hematocrit levels during and after CPB for both groups,there was significantly less operative homologous blood utilized in blood conservation group across all ages and complexity levels. CONCLUSION Blood conservation surgery can be performed in con-genital patients needing cardiac surgery in all age groups and complexity categories. The above findings in addition to attendant risks and side effects of blood transfusion and the rising cost of safer blood products justify blood conservation in congenital cardiac surgery.

Coronary Artery Complications after Right Ventricular Outflow Tract Reconstruction Surgery

Background:Mechanisms and clinical manifestations of coronary artery complications after right ventricular outflow tract reconstruction surgery are not well known.Methods:Patients who had coronary artery complications after pulmonary valve replacement or the Rastelli procedure at a single tertiary centre were retrospectively analysed.Results:Coronary artery complications were identified in 20 patients who underwent right ventricular outflow tract reconstruction surgery.The median age at diagnosis of coronary artery complication was 21 years(interquartile range:13–25 years).Mechanisms of coronary artery complications were compression by adjacent materials in 12 patients,dynamic compression of intramural course of coronary artery in two patients,and intraoperative injury in six patients.Congenital coronary artery anomalies were identified in 50%(10/20)of patients.Four patients presented with early postoperative haemodynamic instability.Fourteen patients showed late onset symptoms or signs of coronary insufficiency,including chest pain,ventricular dysfunction,or ventricular arrhythmias.Coronary artery stenosis was incidentally found on cardiac computed tomography angiography in two asymptomatic patients.Four patients underwent surgical interventions,and one patient underwent percutaneous coronary intervention for coronary stenosis.One patient with recurrent ventricular tachycardia required an implantable cardioverter-defibrillator.There were two deaths in patients with intraoperative coronary injury.Conclusion:Preoperative coronary evaluation and long-term follow-up for the development of coronary artery complications are required in patients undergoing right ventricular outflow tract reconstruction surgery to prevent ventricular dysfunction,arrhythmias,and death,especially among those with congenital coronary anomalies.

Interventional closure operation increases angiotensin-(1-7) level in pulmonary arterial hypertension due to congenital heart disease

Background Recent studies have demonstrated that angiotensin(Ang)-(1-7) plays an important role in the development of pulmonary arterial hypertension(PAH). Our previously study showed that serum Ang-(1-7) level was decreased in patients with PAH due to congenital heart disease(CHD). The present study aimed to investigate the changes of serum Ang-(1-7) before and after intervention closure. Methods fifty-nine patients with CHD were included. The patients were divided into non-PAH(group A), mild PAH(group B) and moderate PAH(group C). The serum Ang-(1-7) was detected by enzyme-linked immunosorbent assay(ELISA) at 1 day before operation and 2 days after operation. Results Before intervention closure, serum angiotensin-(1-7) level was significantly lower in group C than that in group A and group B. After intervention closure operation, serum Ang-(1-7) level was increased in group B and group C. Conclusion Serum Ang-(1-7) level is increased in PAH patients after interventional closure operation of CHD, which might be used as potential clinical biomarkers to evaluate the postoperative prognosis for these patients.[S Chin J Cardiol 2019;20(4):264-268]

Cardiovascular surgery in Turner syndrome-early outcome and long-term follow-up

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.

Population health and sociodemographic variables as predictors of access to cardiac medicine and surgery in Haiti

Background In Haiti,cardiovascular disease is a leading cause of morbidity and mortality,with congenital and rheumatic heart disease comprising a large portion of disease burden.However,domestic disparities in cardiac care access and their impact on clinical outcomes remain poorly understood.We analyzed population-level sociodemographic variables to predict cardiac care outcomes across the 10 Haitian administrative departments.Methods This cross-sectional study combined data from a 2016-17 Haitian national survey with aggregate outcomes from the Haiti Cardiac Alliance(HCA)database(n=1817 patients).Using univariate and multivariable regression analyses,the proportion of HCA patients belonging to each of three clinical categories(active treatment,lost to follow-up,deceased preoperatively)was modeled in relation to six population-level variables selected from national survey data at the level of the administrative department.Results In univariate analysis,higher department rates of childhood growth retardation were associated with a lower proportion of patients in active care(OR=0.979[0.969,0.989],p=0.002)and a higher proportion of patients lost to follow-up(OR=1.016[1.006,1.026],p=0.009).In multivariable analysis,the proportion of department patients in active care was inversely associated with qualified prenatal care(OR=0.980[0.971,0.989],p=0.005),and child growth retardation(OR=0.977[0.972,0.983]),p=0.00019).Similar multivariable results were obtained for department rates of loss to follow-up(child growth retardation:OR=1.018[1.011,1.025],p=0.002;time to nearest healthcare facility in an emergency:OR=1.004[1.000,1.008,p=0.065)and for preoperative mortality(prenatal care:OR=0.989[0.981,0.997],p=0.037;economic index:OR=0.996[0.995,0.998],p=0.007;time to nearest healthcare facility in an emergency:OR=0.992[0.988,0.996],p=0.0046).Conclusions Population-level survey data on multiple variables predicted domestic disparities in HCA clinical outcomes by region.These findings may help to identify underserved areas in Haiti,where increased cardiac care resources are required to improve health equity.This approach to analyzing clinical outcomes through the lens of population-level survey data may inform future health policies and interventions designed to increase cardiac care access in Haiti and other low-income countries.