Application of evidence-based nursing intervention in patients with congenital heart diseases treated by transcatheter interventional therapy

Objective To explore the application of evidence-based nursing intervention in patients with congenital heart diseases treated by transcatheter interventional therapy.Methods A total of 64 patients with congenital heart diseases who were administered in our hospital from June 2011 to July 2013 were recruited in our research.

Effect of dobutamine combined with meropenem on serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP in children with congenital heart disease and pneumonia

Objective:To study the effect of dobutamine combined with meropenem on serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP in children with congenital heart disease and pneumonia.Methods:A total of 70 children with congenital heart disease and pneumonia in our hospital from June 2014 to Octomber 2016 were enrolled in this study. The subjects were divided into the control group (n=35) and the treatment group (n=35) randomly. The control group was treated with dobutamine, the treatment group were treated with dobutamine combined with meropenem. The two groups were treated for 10 days. The serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP levels of the two groups before and after treatment were compared.Results: There were no significantly differences of the serum BNP, IGF-1, IGFBP-3, TNF-a, IL-6 and hs-CRP levels of the two groups before treatment. The serum BNP, TNF-a, IL-6 and hs-CRP levels of the two groups after treatment were significantly lower than before treatment, the serum IGF-1 and IGFBP-3 levels of the two groups after treatment were significantly higher than before treatment, and that of the treatment group were significantly better than the control group.Conclusion:Dobutamine combined with meropenem can significantly reduce the serum BNP, TNF-a, IL-6 and hs-CRP levels, improve serum IGF-1 and IGFBP-3 levels of children with congenital heart disease and pneumonia, and it was worthy clinical application.

Having a Partner and Having Children:Comparisons of Adults with Congenital Heart Disease and the General Population:A 15-Year Case-Control Study

Objectives:To examine whether patients with congenital heart disease(CHD)are less likely to have a partner or children than individuals from the general population.Methods:Longitudinal study with two assessments of the same patients(n=244)from a hospital population and controls(n=238)from the German Socio-Economic Panel(GSOEP)using parental education,patients age,and sex as matching criteria.The first patient study was conducted between 5/2003 and 6/2004,the second one between 5/2017 and 4/2019.Controls were drawn from GSOEP-surveys 2004 and 2018.CHD-severity was classified according to type of surgery:curative,reparative,or palliative.Living single was used as outcome measure,for offspring the outcome was having children or not.Results:Among women with CHD the rate of those living single was higher than among controls with the differences depending on disease complexity(curative:OR=5.5;reparative:OR=1.9;palliative:OR=2.7).No statistically significant differences between patients and controls emerged in the male study population.With respect to children a marked difference emerged between women with CHD and controls.Among patients the odds of having children were lower than among controls(curative:OR=0.3;reparative:OR=0.3;palliative:OR=0.2).The rate of patients with children with CHD(women:5.6%;men:4.9%)was higher than expected(1%)if compared with the general population.Conclusions:Using partnership and children as outcome criteria,patients with CHD are disadvantaged if compared to subjects from the general population.In female patients the social consequences of the disease turned out as more pervasive than in women.

Effect of Hemodilution on Fluctuation of Cerebral Oxygen Saturation during Cardiopulmonary Bypass in Children with Cyanotic Congenital Heart Disease

Background:In patients with cyanotic congenital heart disease(CHD),cerebral oxygenation may be maintained by elevations in hematocrit(Hct).Hemodilution accompanying cardiopulmonary bypass(CPB),however,can disrupt cerebral oxygen balance,leading to fluctuations in cerebral oxygen saturation(ScO_(2)).The present study investigated the effects of Hct changes on the fluctuation of ScO_(2)during CPB in cyanotic CHD using performance measurement(PM).Methods:Children with CHD(51 acyanotic and 46 cyanotic)who had undergone cardiac surgery using CPB were enrolled.Median performance error(MDPE),median absolute performance error(MDAPE),and wobble parameters of ScO_(2)were calculated before(reference value),during,and after CPB.Correlations of PM parameters with Hct and reductions in Hct(ΔHct)were also evaluated.Results:Before CPB,patients with cyanotic CHD had lower MDPE and larger wobble than those with acyanotic CHD,although mean ScO_(2)did not differ significantly between the two groups.During CPB,ScO_(2)of acyanotic CHD increased asΔHct increased,but PM variables were not associated withΔHct.In cyanotic CHD,MDPE(r=−0.324,p=0.032)and MDAPE(r=0.339,p=0.024)correlated significantly withΔHct during CPB.After CPB,MDPE(r=0.574,p=0.025)and MDAPE(r=−0.543,p=0.036)were significantly correlated with Hct in children with cyanotic CHD who underwent palliative surgery.Conclusion:Therefore,ScO_(2)fluctuation during CPB in children with cyanotic CHD may be affected by the decrease in Hct,suggesting that excessive hemodilution can negatively influence the maintenance of cerebral oxygenation in these patients.

The Prevalence of Congenital Heart Disease among School-Age Children in China:A Meta-Analysis and Systematic Review

Objectives:To estimate the prevalence of Congenital Heart Disease(CHD)in school-age children,to identify the extent to which altitude affects the prevalence of the disease,and to examine trends in prevalence over time in China.Methods:Seven databases were systematically searched and last retrieved on September 10,2021 for all studies reporting the prevalence of CHD in children after 1970 in China,which were then divided into high and low altitude regions based on 2500 meters above sea level.The random-effected model was used to combine prevalence data and subgroups analysis.The baseline data of all cases and individuals were used for comparison to calculate the odds ratio(OR)for overall and different altitude prevalence.Results:A total of 12,926,083 individuals(aged 3-18 years),with 31,835 cases from 86 studies,were included in the analysis.The pooled CHD prevalence of total children was 4.69[95%confidence interval(CI):4.10 to 5.29]per 1000 children.Overall,temporal trends analysis indicated that the prevalence of CHD in children continuously decreased with time,from 6.19(95%CI:4.50 to 7.88)per 1000 children in 1976-1985 to 3.30(95%CI:2.49;4.38)per 1000 children in 2016-2021.The OR for the prevalence of CHD in children from high and low altitudes with baseline data was 2.84(95%CI:2.48 to 3.27)and 1.31(95%CI:1.13 to 1.53)(χ^(2)=53.89,p<0.01),respectively.The OR of the prevalence of CHD in male children compared to females was 0.60(95%CI:0.53 to 0.68)at high altitudes and 0.79(95%CI:0.71 to 0.89)at low altitudes.Among the seven most common subtypes,patent ductus arteriosus was the most common at high altitudes,while atrial septal defects were the most common at low altitudes.Conclusion:This study provides valuable insights for further disease prevention and etiological exploration.The overall decreasing trend in the prevalence of CHD in children over time may indicate a positive effect of perinatal management and treatment during infancy.

Application of voxel-based morphometric method to detect brain changes in children with non-cyanotic congenital heart disease

BACKGROUND Congenital heart disease(CHD)is a cardiovascular malformation caused by abnormal heart and/or vascular development in the fetus.In children with CHD,abnormalities in the development and function of the nervous system are common.At present,there is a lack of research on the preoperative neurological development and injury in young children with non-cyanotic CHD.AIM To determine the changes in white matter,gray matter,and cerebrospinal fluid(CSF)by magnetic resonance imaging(MRI)in children with non-cyanotic CHD as compared with healthy controls.METHODS Children diagnosed with non-cyanotic CHD on ultrasonography(n=54)and healthy control subjects(n=35)were included in the study.All the subjects were aged 1-3 years.Brain MRI was performed prior to surgery for CHD.The SPM v12 software was used to calculate the volumes of the gray matter,white matter,CSF,and the whole brain(sum of the gray matter,white matter,and CSF volumes).Volume differences between the two groups were analyzed.Voxel-based morphometry was used to compare specific brain regions with statistically significant atrophy.RESULTS Compared with the control group,the study group had significantly reduced whole-brain white matter volume(P<0.05),but similar whole-brain gray matter,CSF,and whole-brain volumes(P>0.05).As compared with the healthy controls,children with non-cyanotic CHD had mild underdevelopment in the white matter of the anterior central gyrus,the posterior central gyrus,and the pulvinar.CONCLUSION Children with non-cyanotic CHD show decreased white matter volume before surgery,and this volume reduction is mainly concentrated in the somatosensory and somatic motor nerve regions.

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.

Congenital Heart Disease in Rural Senegal: A Retrospective Study of 79 Patients

Introduction: Congenital heart disease (CHD) is a malformation of the heart present at birth and resulting from a developmental defect during embryonic life. The aim was to assess CHD in rural areas. Methodology: This is a retrospective study conducted over a period of 2 years in rural Senegal. Results: During the study period, we collected 79 patients with CHD, representing a hospital prevalence of 2.6%. The mean age at diagnosis was 17.05 months and the sex ratio was 1.19. The mean birth weight was 2826 g. The main comorbidities were anaemia (54.43%) and respiratory infections 38 cases (48.10%). Respiratory distress was the most common sign with 98.73%. Radiological cardiomegaly was noted in 86.7%. The most frequent CHD were interventricular septal defect (IVD) 21.51% and atrial septal defect (ASD) 8.86%. No patient was able to receive surgical treatment. Seven children died (8.86%) and 21 children were lost to follow-up (26.58%). On Doppler echocardiography, 16.45% of the patients had pulmonary arterial hypertension (PAH). Conclusion: In light of this work, emphasis should be placed on the quality of antenatal consultations, the quality of management and the regular availability of echocardiography and a cardio-paediatrician in order to reduce morbidity and mortality.

Effects of Palivizumab Guideline Changes on RSV Admissions in Patients with Congenital Heart Disease and Prematurity

Background: Respiratory syncytial virus (RSV) causes significant morbidity and mortality in patients with a history of prematurity and congenital heart disease (CHD). In 2014, the guidelines for Palivizumab became more restrictive for this population. We hypothesized the percentage of RSV+ admissions would increase overall and in this target group (TG) specifically. Methods: We conducted a retrospective review of patients under age 2 years admitted with bronchiolitis two seasons prior to the change (Pre) and two seasons after (Post). Our TG included patients who were eligible prior to the 2014 changes but currently no longer eligible. We used chi-square analysis to answer the two main hypotheses: 1) Percent RSV+/total bronchiolitis Pre vs Post and 2) Percent of TG/RSV+ Pre vs Post. Results: 1283 patients (546 pre, 737 post) were admitted with the diagnosis of RSV between 2012-2016, 866 actually tested positive for RSV (367 Pre, 499 Post). There was no significant difference in the number of total patients admitted with RSV (Pre = 67.2%, Post = 67.7%) or in our TG (Pre 7.1% vs Post 8.2%). TG overall had a more complicated course: longer length of stay, median 5 days, IQR 2 - 12 vs 3 days, IQR 1 - 5, (p < 0.001), intensive care unit admissions (36% vs 22.8%, p = 0.02), positive pressure ventilation (25.4% vs 15.4%, p = 0.03) and intubation (16.4% vs 6.8%, p = 0.004), but there was no difference Pre vs Post. Conclusion: The TG had an overall higher acuity, but there was no increase in the number of patients hospitalized with RSV or severity as a result of the Palivizumab guideline changes.

Effects of Ticagrelor on oxidative stress, coagulation function, platelet function and related factors in patients with coronary artery disease undergoing interventional therapy

Objective: To explore the effects of Ticagrelor on oxidative stress, coagulation function, platelet function and related factors in patients with coronary artery disease undergoing interventional therapy. Methods: A total of 140 patients with coronary artery disease who underwent percutaneous coronary intervention in our hospital from October 2016 to March 2018 were selected as the study subjects and were divided into control group (70 cases) and observation group (70 cases) by drawing lots. Both groups were treated with symptomatic routine therapy before operation. On this basis, the control group was treated with clopidogrel before operation, and the observation group was treated with Ticagrelor before operation on the basis of routine treatment. The changes of oxidative stress, coagulation index, platelet function and related factors were compared between the two groups before and after treatment. Results: Before treatment, there were no significant differences in oxidative stress factors, coagulation parameters, platelet parameters and related factors factors between the two groups. After treatment, the levels of MDA, MPAR, VEGF and MMP-9 in the two groups were lower than those before treatment, while the levels of SOD, APTT, TT and PT were higher than those before treatment;and the levels of MDA, MPAR, VEGF, MMP-9, APTT and PT in the observation group were significantly lower than those in the control group after treatment, while the levels fo SOD, TT and IPA in the observation group were significantly higher than those in the control group. Conclusions: Ticagrelor can better reduce oxidative stress injury, improve coagulation function and coronary stenosis, and inhibit platelet aggregation in patients with coronary heart disease undergoing PCI than clopidogrel. It has clinical popularization significance.

Epidemiology of Cardiovascular Diseases in Children at the Teaching Hospital of Brazzaville, Congo

Context: Several studies were conducted throughout the world on heart diseases in children;no data is available in Congolese child. Objective: To evaluate epidemiological profile of Congolese children and teenager carrying cardiovascular diseases. Methods: A descriptive and prospective study was carried out during 4 years in the pediatric department of teaching hospital of Brazzaville, near the children received in consultation of pediatric cardiology. Results: On 41,472 patients admitted in pediatric service, 526 patients were received in consultation for suspicion of heart diseases. Among them, 444 had cardiopathy (incidence of 10.7‰). It was about a congenital heart disease to 316 (60%) incidence of 7.6‰;Acquired heart disease to 128 (24.4%) incidence of 3.1‰. Among congenital heart defects observed frequency of patients with ASD was 20.3%, isolated in 10.1% of cases, and associated with ECD (11.8%). The VSD was observed in 30.1% of cases, and the Tetralogy of Fallot in 10.1% of cases. Among the acquired heart diseases, severe hypo kinetic dilated cardiomyopathy (DCM) was noted in 24.4% of cases. The rheumatic heart diseases accounted for 41.4% of cases. It was mitral regurgitation (33.6%), a mitral stenosis (1.6%). Pericarditis was objectified at 10.1% of the patients. The evolution was favorable for 43.3% of patients. An aggravation of symptoms was observed to 2.7% of patients. Mortality was 11.9% and 71.9% of deaths were observed to not operate carriers of congenital heart disease. 69.9% of dead patients were carrying a cyanogen heart disease. Left to right shunt represented 21.7% of the deaths. Conclusion: Heart diseases are real problem of public health for Congolese children.

Heart Failure in Children: Epidemiological, Etiological and Diagnostic Aspects in Diamniadio Children’s Hospital (Senegal)

Introduction: Heart failure is a major cause of morbidity and mortality in children. The authors aimed to report the epidemiological and etiological characteristics of heart failure (HF) in children in Senegal. Patients and Methods: This was a retrospective study conducted at the Children’s Hospital of Diamniadio (CHD) from 01-01-2016 to 31-12-2017. Children aged 0 to 15 years with heart failure confirmed by echocardiography were included. Results: Sixty-six children were admitted for heart failure. The prevalence of HF was 5.3‰. The mean age of the children was 41.59 months ± 53.35 months (range: 0 to 168 months). The 0 - 5 age group was the most represented (n = 48;72.72%). The HF concerned 38 boys and 28 girls, a sex ratio (M/F) of 1.36. The majority of our patients came from the semi-urban area (42.4%). The etiology of HF was represented in order of frequency by congenital heart disease, rheumatic heart disease and cardiomyopathy with 59.09% of cases, 22.73% of cases and 7.58% of cases respectively. It was unknown in 4.54% of the cases. At echocardiography, the left ventricular ejection fraction (LVEF) was impaired (less than 50%) in 19 patients, in 29.2% of cases with an average of 64% +/- 15.16% (extremes: 24% and 86%). Anemia was the comorbidity most commonly associated with their condition (n = 33%;50%). Conclusion: Heart failure in children is uncommon. The main etiology is represented by congenital heart disease.

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.

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.

Clinical cardiac regenerative studies in children

Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients. We review here the available literature data, searching the MEDLINE, Google Scholar and EMBASE database for completed, and www.clinicaltrials.gov homepage for ongoing studies involving pediatric cardiac regeneration reports. Because of difficulties conducting randomized blinded clinical trials in pediatric patients, mostly case reports or cohort studies with a limited number of individuals have been published in the field of pediatric regenerative cardiology. The majority of pediatric autologous cell transplantations into the cardiac tissue have been performed in critically ill children with severe or terminal heart failure. Congenital heart disease, myocarditis, and idiopathic hypertrophic or dilated cardiomyopathy leading to congestive heart failure are some possible areas of interest for pediatric cardiac regeneration therapy. Autologous bone marrow mononuclear cells, progenitor cells, or cardiospheres have been applied either intracoronary or percutaneously intramyocardially in severely ill children, leading to a reported clinical benefit of cell-based cardiac therapies. In conclusion, compassionate use of autologous stem cell administration has led to at least short-term improvement in heart function and clinical stability in the majority of the critically ill pediatric patients.

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.

Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease

Background:Pulmonary artery hypertension associated withh congenital heart disease(PAH-CHD)occurs predominantly among patients with uncorrected CHD.Treatment of severe pediatric PAH-CHD remains a major intractability.This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction.Methods:The data for 59 children with severe PAH-CHD who underwent surgical correction,with or without postoperative medication,between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively.A regression analysis,receiver-operating characteristic(ROC)curves,and Kaplan-Meier curves were used for survival analysis.Results:Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction,with or without specific anti-PAH drugs postoperatively,were included in this study.The pulmonary pressure,heart function,and ending events were observed and median observation period was 49±20 months.Twenty-eight patients(50%)received at least one additional anti-PAH drug after correction.The survival rate after 2 years was 91.5%(54/59);two patients were in a critical condition,and three were lost to follow-up.Twelve patients(29%)still received over one additional PAH-specific therapy at follow-up,whereas 42(75%)had successfully stopped drug treatment.Two patients(3.5%)died and one underwent a second thoracotomy to remove the ventricular septal defect patch.Acute vasoreactivity test(AVT)criteria had limited efficacy in predicting pediatric PAH-CHD,whereas pulmonary vascular resistance(PVR)<6.65 Wood units(WU)/m^2 or PVR/systemic vascular resistance(SVR)<0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3%(95%confidence interval[CI]:96.0-100%),98.4%(95%CI:96.0-100%)sensitivity of 100%,100%and specificity of 82.1%,92.9%,respectively.Conclusions:Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD,PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD.Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR<6.65 WU/m2 and PVR/SVR<0.39 after iloprost aerosol inhalation.

Optical coherence tomography of the pulmonary arteries in children with congenital heart diseases:A systematic review

Importance:Optical coherence tomography(OCT)is a high-resolution intravascular imaging tool and has shown promise for providing real-time quantitative and qualitative descriptions of pulmonary vascular structures in vivo in adult pulmonary hypertension(PH),while not popular in pediatric patients with congenital heart diseases(CHD).Objective:The aim of this review is to summarize all the available evidence on the use of OCT for imaging pulmonary vascular remodeling in pediatric patients.Methods:We conducted the systematic literature resources(Cochran Library database,Medline via PubMed,EMBASE,and Web of Knowledge)from January 2010 to December 2021 and the search terms were“PH”,“child”,“children”,“pediatric”,“OCT”,“CHD”,“pulmonary vessels”,“pulmonary artery wall”.Studies in which OCT was used to image the pulmonary vessels in pediatric patients with CHD were considered for inclusion.Results:Five studies met the inclusion criteria.These five papers discussed the study of OCT in the pulmonary vasculature of different types of CHD,including common simple CHD,complex cyanotic CHD,and Williams-Beuren syndrome.In biventricular anatomy,pulmonary vascular remodeling was primarily reflected by pulmonary intima thickening from two-dimensional OCT.In single-ventricle anatomy,due to the state of hypoxia,the morphology of pulmonary vessels was indirectly reflected by the number and shape of nourishing vessels from three-dimensional OCT.Interpretation:OCT may be an adequate imaging procedure for the demonstration of pulmonary vascular structures and provide additional information in pediatric patients.

Cheatham-Platinum支架置入治疗儿童主动脉缩窄的疗效评价

目的：评价Cheatham-Platinum （CP）支架治疗儿童主动脉缩窄的早中期疗效.方法：对14例主动脉缩窄（coarctation of the aorta,CoA）儿童行CP支架置入术,其中男性8例,女性6例;年龄4～14岁（中位年龄11岁）;体质量19.9～60kg（中位体质量38.2kg）.6例（43％）为未经治疗CoA;8例（57％）为再狭窄CoA.收集和分析CP支架置入前后的数据和随访资料.结果：14例患儿均成功置入CP支架,其中6例裸支架,8例覆膜支架.术后即刻CoA最窄处直径由（6.45±1.39）mm增加至（11.79±1.59）mm,P＜0.001;CoA/Dao比由0.41±0.12增加至0.74 ±0.10,P＜0.001;导管测得跨狭窄压差由[（34.86±17.48） mmHg（1 mmHg=0.133kPa）,下降至（1.64±1.64） mmHg,P＜0.001];心脏超声测得跨狭窄压差由[（59.76±15.92） mmHg,下降至（23.89±7.30） mmHg,P＜0.001];上肢收缩压由[（142.07±28.95） mmHg降为（124.79±25.92） mmHg,P＜0.001];下肢收缩压由[（105.21±21.35）mmHg升为（122.29±25.29）mmHg,P＜0.05].未见主动脉瘤和主动脉夹层的发生.1例患儿术中发生髂动脉内膜撕脱导致术后死亡.随访结果未见再狭窄,未见支架的移位断裂.结论：CP支架治疗儿童CoA早中期疗效好,但远期效果尚需进一步的随访和更多病例的研究.