Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliativ...Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.展开更多
BACKGROUND Double-chambered left ventricle(DCLV)is an extremely rare congenital disease in which the left ventricle(LV)is divided by abnormal muscle tissue.Due to its rarity,there is a lack of data on the disease,incl...BACKGROUND Double-chambered left ventricle(DCLV)is an extremely rare congenital disease in which the left ventricle(LV)is divided by abnormal muscle tissue.Due to its rarity,there is a lack of data on the disease,including its diagnosis,treatment,and prognosis.Accordingly,we report a case in which DCLV was diagnosed and followed up.CASE SUMMARY A 45-year-old man presented to our hospital due to abnormal findings on an electrocardiogram recorded during a health check.He had no specific cardiac symptoms,comorbidities or relevant past medical history.Echocardiography revealed that the LV was divided into two by muscle fibers.There were no findings of ischemia on coronary angiography and coronary computed tomography angiography performed to exclude differential diagnoses.After comprehensive analysis of the images,DCLV was diagnosed.As it seemed to be asymptomatic DCLV,we decided the patient was to be observed without administering any medication.However,follow-up echocardiography revealed a thrombus in the accessory chamber(AC).Anticoagulant medication was initiated,the thrombus resolved,and the patient is currently undergoing follow-up without any specific symptoms.CONCLUSION Asymptomatic,uncomplicated DCLV was diagnosed through multimodal imaging;however,a thrombus in the AC occurred during the follow-up.The findings highlight that multimodal imaging is essential in diagnosing DCLV,and that anticoagulation is important in its management.展开更多
In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old pa...In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians.展开更多
Background: Transradial coronary angiography has established itself as safe alternative to transfemoral approach. Today, the artery of approach lies completely on the operator’s choice. The Right Radial Approach (RRA...Background: Transradial coronary angiography has established itself as safe alternative to transfemoral approach. Today, the artery of approach lies completely on the operator’s choice. The Right Radial Approach (RRA) has been a favorite for most of the interventional cardiologists due to the convenience in operating from the right side. The Left Radial Approach (LRA) has always been a neglected route. LRA does have many advantages over the right, the vascular anatomy being one of them. The aim of our study was to compare the right radial approach of diagnostic coronary angiography with left radial approach. Method: A total of 70 cases of Coronary Angiography (CAG) with normal Allen test and satisfying the inclusion criteria were prospectively observed and studied after randomly assigning them into two equal groups, LRA (Left Radial Approach) n = 35 and RRA (Right Radial Approach) n = 35. Multipurpose TIG (Tiger) catheter was used in both the approaches to catheterize the right as well as left coronary artery. Results: The access time, catheter manipulation time, procedure time, amount of contrast used, hospital stay, intensity of pain experienced, cost of the procedure and quality of coronary angiogram observed were statistically insignificant while the fluoroscopy time was slightly statistically significant which was independent to catheter manipulation time. Conclusions: The neglected Left Radial Approach to coronary angiography is as efficacious, safe and cost effective with reduction in arterial spasm complications when compared to the Right Radial Approach performed by multipurpose Tiger catheter.展开更多
BACKGROUND Despite major advances in pharmacologic treatment,patients with pulmonary arterial hypertension(PAH)still have a considerably reduced life expectancy.In this context,chronic hyperactivity of the neurohormon...BACKGROUND Despite major advances in pharmacologic treatment,patients with pulmonary arterial hypertension(PAH)still have a considerably reduced life expectancy.In this context,chronic hyperactivity of the neurohormonal axis has been shown to be detrimental in PAH,thus providing novel insights on the role of neurohormonal blockade as a potential therapeutic target.AIM To evaluate the application and prognostic effect of neurohormonal inhibitors(NEUi)in a single-center sample of patients with idiopathic PAH and risk factors for left heart disease.METHODS We analyzed data retrospectively collected from our register of right heart catheterizations performed consecutively from January 1,2005 to October 31,2018.Patients on beta-blocker,angiotensin-converting enzyme inhibitor,angiotensin receptor blocker or mineralocorticoid receptor antagonist at the time of right heart catheterization were classified as NEUi users and compared to NEUi nonrecipients.RESULTS Complete data were available for 57 PAH subjects:27 of those(47.4%)were taking at least one NEUi at the time of right heart catheterization and were compared with the remaining 36 NEUi non-recipients.NEUi users were older and had a higher cardiovascular risk profile compared to non-recipients.Additionally,NEUi non-users had a higher probability of dying during the course of follow-up than NEUi recipients(56.7%vs 25.9%,log-rank P=0.020).CONCLUSION The above data highlighted a subgroup of patients with PAH and comorbidities for left heart disease in which NEUi use has shown to be associated with improved survival.Future prospective studies are needed to identify the most appropriate therapeutic strategies in this subset population.展开更多
Background:Fetal balloon atrial septoplasty(BAS)is performed through the restrictive foramen ovale in fetal cases with established hypoplastic left heart syndrome(HLHS)and an intact or highly restrictive atrial septum...Background:Fetal balloon atrial septoplasty(BAS)is performed through the restrictive foramen ovale in fetal cases with established hypoplastic left heart syndrome(HLHS)and an intact or highly restrictive atrial septum(RAS).Methods:In the current report,we present a case of high position BAS in a fetus with HLHS/RAS.Results:Echocardiography confirmed an adequate atrial opening above the foramen ovale and fetal pleural effusion resolved spontaneously 1 day after the procedure.Conclusion:To the best of our knowledge,the creation of a high position hole in the thinner part of the atrial septum,instead of the restrictive tiny hole,has not been reported in fetal cases with HLHS/RAS.展开更多
Objective:To compare the clinical outcomes of minimally invasive right subaxillary vertical thoracotomy and traditional median sternotomy through right atrium in treatment of common congenital heart diseases.Methods:C...Objective:To compare the clinical outcomes of minimally invasive right subaxillary vertical thoracotomy and traditional median sternotomy 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 thoracotomv from May,2011 to February,2013 and 77 cases of same diseases with traditional median sternotomy 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 secondery 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 sternotomy,with statistically significant differences(P<0.05).In six-month lollowup after operation,no complications of residual deformity and pericardial effusion were found in both groups bv doing echocardiography,but mild pectus carinatum was found in X patients in the traditional median sternotomy group(traditional groupi.whereas patients in another group were well recovered.Conclusions:Minimally invasive right subaxillary vertical thoracotomv for common congenital heart diseases is as safe as traditional median sternotomy,without the increasing incidence of postoperative complications.Additionally,compared with traditional median sternotomy,minimally invasive right subaxillary vertical thoracotomv is better in the aspects of hidden incision,appearance,and postoperative recovery.展开更多
Three male patients were diagnosed with new cortical infarctions of the right parietal lobe on the basis of head magnetic resonance imaging; high-intensity signals indicating lesions in the right parietal lobe were no...Three male patients were diagnosed with new cortical infarctions of the right parietal lobe on the basis of head magnetic resonance imaging; high-intensity signals indicating lesions in the right parietal lobe were noted on diffusion-weighted images at admission. Two of them presented with left hand weakness, and one exhibited left upper limb weakness. Treatment for improving blood supply to the brain was administered. One patient died suddenly because of ventricular fibrillation 3 days after admission. The other two patients had increased troponin levels and abnormal electrocardiograms, and were diagnosed with acute myocardial infarction half a month after admission. When lesions exist in field 7 of the parietal cortex (resulting in paralysis of the contralateral hand), the sympathetic center of the posterior lateral nucleus of the hypothalamus demonstrates compensatory excitement, which easily causes tachyarrhythmia and sudden death. Our experimental findings indicate that close electrocardiograph monitoring and cerebral infarction treatment should be standard procedures to predict and help prevent heart disease in patients with cerebral infarction in the right parietal lobe and left upper limb weakness as the main complaint.展开更多
Background:Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome,the right ventricle-pulmonary artery shunt(RVPAS)and the ...Background:Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome,the right ventricle-pulmonary artery shunt(RVPAS)and the modified Blalock-Taussig shunt(mBTS).Methods:The cohort included 27 patients with Hypoplastic left heart syndrome(10 in the mBTS group,17 in the RVPAS group).Longitudinal strain,tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure(30-days,90-days,140-days and 200-days after Norwood).Results:No significant differences were found in all standard echocardiographic functional parameters between the two groups at any times.However,when we compared right ventricle function before and after Norwood procedure,longitudinal strain significantly improved in mBTS group compared to pre-surgical assessment(after 90-days:mBTS+27,35%±43,47 vs.RVPAS-8,20%±25,25,p=0,03;after 200-days:mBTS+10.19%±36.58 vs.RVPAS-21.64%±30.43,p=0.04).Conclusion:The mBTS group,which did not receive any ventriculotomy,showed a significant increase in right ventricle longitudinal strain during convalescence.These data support the use of longitudinal strain in Hypoplastic left heart syndrome evaluation and may suggest potential value in terms of cardiac mechanics in using mBTS,preserving the right ventricle integrity.展开更多
BACKGROUND Balloon aortic valvuloplasty(BAV)is a well-established treatment modality for congenital aortic valve stenosis.AIM To evaluate the role of rapid right ventricular pacing(RRVP)in balloon stabilization during...BACKGROUND Balloon aortic valvuloplasty(BAV)is a well-established treatment modality for congenital aortic valve stenosis.AIM To evaluate the role of rapid right ventricular pacing(RRVP)in balloon stabilization during BAV on aortic regurgitation(AR)in pediatric patients.METHODS A systematic review of the MEDLINE,Cochrane Library,and Scopus databases was conducted according to the PRISMA guidelines(end-of-search date:July 8,2020).The National Heart,Lung,and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment.RESULTS Five studies reporting on 72 patients were included.The studies investigated the use of RRVP-assisted BAV in infants(>1 mo)and older children,but not in neonates.Ten(13.9%)patients had a history of some type of aortic valve surgical or catheterization procedure.Before BAV,58(84.0%),7(10.1%),4(5.9%)patients had AR grade 0(none),1(trivial),2(mild),respectively.After BAV,34(49.3%),6(8.7%),26(37.7%),3(4.3%),patients had AR grade 0,1,2,and 3(moderate),respectively.No patient developed severe AR after RRVP.One(1.4%)developed ventricular fibrillation and was defibrillated successfully.No additional arrhythmias or complications occurred during RRVP.CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV,which could potentially decrease AR rates.展开更多
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...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.展开更多
<div style="text-align:justify;"> A persistent left superior vena cava (PLSVC) is a rare malformation which affects approximately 0.3<span>% </span><span>-</span><span> &l...<div style="text-align:justify;"> A persistent left superior vena cava (PLSVC) is a rare malformation which affects approximately 0.3<span>% </span><span>-</span><span> </span><span>0.5% of the population and it is presented along with a right-sided superior vena cava in 82.2% of the cases reported</span><span minion="" pro="" capt","serif";color:#943634;"="" style=""> </span><span minion="" pro="" capt","serif";color:#943634;background:yellow;"=""></span><span>[<a href="#ref1">1</a>]</span><span color:#943634;background:yellow;"=""></span><span minion="" pro="" capt","serif";color:#943634;background:yellow;"=""></span><span>.</span><span "=""><span> Clinicians diagnose it incidentally by difficulties with pacemaker implantation, central venous catheterization or screening for another etiologies when it is not accompanied by other anomalies it is typically asymptomatic. W. Schummer </span><i><span>et al.</span></i><span> described the embryogenesis and the anatomic variations of persistent LSVC according to the positioning of a central venous catheter on the chest radiograph: type I, normal;type II, only PLSVC;type IIIa, right and left superior vena cava with connection;type IIIb, right and left superior vena cava without connection</span></span><span> </span><span>[<a href="#ref2">2</a>]</span><span>. </span><span "=""><span>In 92% of individuals with PLSVC, the PLSVC drains into a dilated coronary sinus (CS) and rest 8% drain directly into the left atrium. PLSVC is caused by a failure in the closure of the left anterior cardinal vein during embryogenic development</span></span><span minion="" pro="" capt","serif";color:#943634;"=""><span> </span><span style="background:yellow;"></span></span><span color:#943634;"=""><span>[<a href="#ref3">3</a>]</span><span style="background:yellow;"></span></span><span minion="" pro="" capt","serif";color:#943634;background:yellow;"=""></span><span>. </span><span>The coronary sinus (CS) is a vein that transmits venous blood to the right atrium though atrioventricular groove. The CS wall contains atrial myocardium. Thus, it</span><span>s size</span><span> extensively depend</span><span>s</span><span> on variability of blood flow and pressure. We present a variant PLSVC with unknown prevalence and a mild </span><span>platypnea-orthodeoxia</span><span> </span><span>syndrome</span><span> after recovery of COVID-19 related acute respiratory distress syndrome (ARDS).</span> </div> <a href="#ref2"></a>展开更多
We report the case of a 16-year-old young girl seen for precordial pain and stage II NYHA dyspnea. Clinical examination found a maximal systolodiastolic murmur in the left subclavicular. Lateral Q waves were noted on ...We report the case of a 16-year-old young girl seen for precordial pain and stage II NYHA dyspnea. Clinical examination found a maximal systolodiastolic murmur in the left subclavicular. Lateral Q waves were noted on the electrocardiogram and echocardiography revealed a right coronary fistula draining in right atrium. CT scan confirmed the diagnosis. Patient was addressed to a specialized center for percutaneous closure of the fistula. In case of signs indicating coronary artery disease, the search for coronary anomalies should be systematic in children by using EKG and transthoracic echocardiography. In case of doubt, a second imaging such as the multislice Ct scan is performed. Coronary angiography has a dual diagnostic and therapeutic interest. The indications for treatment depend on the symptoms and the results of ischemia tests.展开更多
Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospec...Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.展开更多
BACKGROUND Congenitally corrected levo-transposition of the great arteries(L-TGA)is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy.In L-TGA,the double d...BACKGROUND Congenitally corrected levo-transposition of the great arteries(L-TGA)is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy.In L-TGA,the double discordance,atrioventricular and ventriculoarterial,create an acyanotic milieu which allows patients to survive their early decades,however,progressive systemic right ventricle(sRV)dys-function creates complications later in life.sRV dysfunction and remodeling predisposes patients to intracardiac thrombus(ICT)formation.CASE SUMMARY A 40-year-old male with L-TGA presented with symptoms of acute decom-pensated heart failure.In childhood,he had surgical repair of a ventricular septal defect.In adulthood,he developed sRV dysfunction,systemic tricuspid valve(sTV)regurgitation,and left-bundle branch block for which he underwent cardiac resynchronization therapy.Transthoracic echocardiogram showed a sRV ejection fraction of 40%,severe sTV regurgitation,and a newly identified sRV ICT.ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardio-graphy.Our patient was optimized with guideline-directed medical therapy and diuresis.Anticoagulation was achieved with a vitamin K antagonist(VKA)and he was later referred for evaluation by advanced heart failure and heart transplant services.CONCLUSION Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease.This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.展开更多
BACKGROUND Necrotizing enterocolitis(NEC)is a multifactorial disease that predominantly affects premature neonates.Intestinal dysbiosis plays a critical role in NEC pathogenesis in premature neonates.The main risk fac...BACKGROUND Necrotizing enterocolitis(NEC)is a multifactorial disease that predominantly affects premature neonates.Intestinal dysbiosis plays a critical role in NEC pathogenesis in premature neonates.The main risk factor for NEC in term infants is mesenteric hypoperfusion associated with ductaldependent congenital heart disease(CHD)that eventually leads to intestinal ischemia.The incidence of NEC in neonates with critical CHD is 6.8%-13%.However,the role of the intestinal microbiome in NEC pathogenesis in infants with ductal-dependent CHD remains unclear.CASE SUMMARY A male term neonate with right atrial isomerism underwent modified Blalock-Taussig shunt placement on the 14^(th)day of life and had persistent mesenteric hypoperfusion after surgery.The patient had episodes of NEC stageⅡA on the 1^(st)and 28^(th)days after cardiac surgery.Fecal microbial composition was analyzed before and after cardiac surgery by sequencing region V4 of the 16S rRNA gene.Before surgery,species belonging to genera Veillonella and Clostridia and class Gammaproteobacteria were detected,Bifidobacteriaceae showed a low abundance.The first NEC episode was associated with postoperative hemodynamic instability,intestinal ischemiareperfusion injury during cardiopulmonary bypass,and a high abundance of Clostridium paraputrificum(Clostridium sensu stricto I)(56.1%).Antibacterial therapy after the first NEC episode resulted in increased abundance of Gammaproteobacteria,decreased abundance of Firmicutes,and low alpha diversity.These changes in the microbial composition promoted the growth of Clostridium sensu strictoⅠ(72.0%)before the second NEC episode.CONCLUSION A high abundance of Clostridium sensu strictoⅠand mesenteric hypoperfusion may have contributed to NEC in the present case.展开更多
基金supported by Medical Scientific Research Foundation of Guangdong Province,China (No.A2018038)
文摘Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.
文摘BACKGROUND Double-chambered left ventricle(DCLV)is an extremely rare congenital disease in which the left ventricle(LV)is divided by abnormal muscle tissue.Due to its rarity,there is a lack of data on the disease,including its diagnosis,treatment,and prognosis.Accordingly,we report a case in which DCLV was diagnosed and followed up.CASE SUMMARY A 45-year-old man presented to our hospital due to abnormal findings on an electrocardiogram recorded during a health check.He had no specific cardiac symptoms,comorbidities or relevant past medical history.Echocardiography revealed that the LV was divided into two by muscle fibers.There were no findings of ischemia on coronary angiography and coronary computed tomography angiography performed to exclude differential diagnoses.After comprehensive analysis of the images,DCLV was diagnosed.As it seemed to be asymptomatic DCLV,we decided the patient was to be observed without administering any medication.However,follow-up echocardiography revealed a thrombus in the accessory chamber(AC).Anticoagulant medication was initiated,the thrombus resolved,and the patient is currently undergoing follow-up without any specific symptoms.CONCLUSION Asymptomatic,uncomplicated DCLV was diagnosed through multimodal imaging;however,a thrombus in the AC occurred during the follow-up.The findings highlight that multimodal imaging is essential in diagnosing DCLV,and that anticoagulation is important in its management.
文摘In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians.
文摘Background: Transradial coronary angiography has established itself as safe alternative to transfemoral approach. Today, the artery of approach lies completely on the operator’s choice. The Right Radial Approach (RRA) has been a favorite for most of the interventional cardiologists due to the convenience in operating from the right side. The Left Radial Approach (LRA) has always been a neglected route. LRA does have many advantages over the right, the vascular anatomy being one of them. The aim of our study was to compare the right radial approach of diagnostic coronary angiography with left radial approach. Method: A total of 70 cases of Coronary Angiography (CAG) with normal Allen test and satisfying the inclusion criteria were prospectively observed and studied after randomly assigning them into two equal groups, LRA (Left Radial Approach) n = 35 and RRA (Right Radial Approach) n = 35. Multipurpose TIG (Tiger) catheter was used in both the approaches to catheterize the right as well as left coronary artery. Results: The access time, catheter manipulation time, procedure time, amount of contrast used, hospital stay, intensity of pain experienced, cost of the procedure and quality of coronary angiogram observed were statistically insignificant while the fluoroscopy time was slightly statistically significant which was independent to catheter manipulation time. Conclusions: The neglected Left Radial Approach to coronary angiography is as efficacious, safe and cost effective with reduction in arterial spasm complications when compared to the Right Radial Approach performed by multipurpose Tiger catheter.
文摘BACKGROUND Despite major advances in pharmacologic treatment,patients with pulmonary arterial hypertension(PAH)still have a considerably reduced life expectancy.In this context,chronic hyperactivity of the neurohormonal axis has been shown to be detrimental in PAH,thus providing novel insights on the role of neurohormonal blockade as a potential therapeutic target.AIM To evaluate the application and prognostic effect of neurohormonal inhibitors(NEUi)in a single-center sample of patients with idiopathic PAH and risk factors for left heart disease.METHODS We analyzed data retrospectively collected from our register of right heart catheterizations performed consecutively from January 1,2005 to October 31,2018.Patients on beta-blocker,angiotensin-converting enzyme inhibitor,angiotensin receptor blocker or mineralocorticoid receptor antagonist at the time of right heart catheterization were classified as NEUi users and compared to NEUi nonrecipients.RESULTS Complete data were available for 57 PAH subjects:27 of those(47.4%)were taking at least one NEUi at the time of right heart catheterization and were compared with the remaining 36 NEUi non-recipients.NEUi users were older and had a higher cardiovascular risk profile compared to non-recipients.Additionally,NEUi non-users had a higher probability of dying during the course of follow-up than NEUi recipients(56.7%vs 25.9%,log-rank P=0.020).CONCLUSION The above data highlighted a subgroup of patients with PAH and comorbidities for left heart disease in which NEUi use has shown to be associated with improved survival.Future prospective studies are needed to identify the most appropriate therapeutic strategies in this subset population.
基金supported by grants from National Natural Science Foundation of China(NO 81770316)Mount Tai Scholarship ProjectQingdao Outstanding Health Professional Development Fund(2017)
文摘Background:Fetal balloon atrial septoplasty(BAS)is performed through the restrictive foramen ovale in fetal cases with established hypoplastic left heart syndrome(HLHS)and an intact or highly restrictive atrial septum(RAS).Methods:In the current report,we present a case of high position BAS in a fetus with HLHS/RAS.Results:Echocardiography confirmed an adequate atrial opening above the foramen ovale and fetal pleural effusion resolved spontaneously 1 day after the procedure.Conclusion:To the best of our knowledge,the creation of a high position hole in the thinner part of the atrial septum,instead of the restrictive tiny hole,has not been reported in fetal cases with HLHS/RAS.
基金supported by college medical journal clinicalspecial funds(11321587)
文摘Objective:To compare the clinical outcomes of minimally invasive right subaxillary vertical thoracotomy and traditional median sternotomy 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 thoracotomv from May,2011 to February,2013 and 77 cases of same diseases with traditional median sternotomy 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 secondery 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 sternotomy,with statistically significant differences(P<0.05).In six-month lollowup after operation,no complications of residual deformity and pericardial effusion were found in both groups bv doing echocardiography,but mild pectus carinatum was found in X patients in the traditional median sternotomy group(traditional groupi.whereas patients in another group were well recovered.Conclusions:Minimally invasive right subaxillary vertical thoracotomv for common congenital heart diseases is as safe as traditional median sternotomy,without the increasing incidence of postoperative complications.Additionally,compared with traditional median sternotomy,minimally invasive right subaxillary vertical thoracotomv is better in the aspects of hidden incision,appearance,and postoperative recovery.
文摘Three male patients were diagnosed with new cortical infarctions of the right parietal lobe on the basis of head magnetic resonance imaging; high-intensity signals indicating lesions in the right parietal lobe were noted on diffusion-weighted images at admission. Two of them presented with left hand weakness, and one exhibited left upper limb weakness. Treatment for improving blood supply to the brain was administered. One patient died suddenly because of ventricular fibrillation 3 days after admission. The other two patients had increased troponin levels and abnormal electrocardiograms, and were diagnosed with acute myocardial infarction half a month after admission. When lesions exist in field 7 of the parietal cortex (resulting in paralysis of the contralateral hand), the sympathetic center of the posterior lateral nucleus of the hypothalamus demonstrates compensatory excitement, which easily causes tachyarrhythmia and sudden death. Our experimental findings indicate that close electrocardiograph monitoring and cerebral infarction treatment should be standard procedures to predict and help prevent heart disease in patients with cerebral infarction in the right parietal lobe and left upper limb weakness as the main complaint.
文摘Background:Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome,the right ventricle-pulmonary artery shunt(RVPAS)and the modified Blalock-Taussig shunt(mBTS).Methods:The cohort included 27 patients with Hypoplastic left heart syndrome(10 in the mBTS group,17 in the RVPAS group).Longitudinal strain,tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure(30-days,90-days,140-days and 200-days after Norwood).Results:No significant differences were found in all standard echocardiographic functional parameters between the two groups at any times.However,when we compared right ventricle function before and after Norwood procedure,longitudinal strain significantly improved in mBTS group compared to pre-surgical assessment(after 90-days:mBTS+27,35%±43,47 vs.RVPAS-8,20%±25,25,p=0,03;after 200-days:mBTS+10.19%±36.58 vs.RVPAS-21.64%±30.43,p=0.04).Conclusion:The mBTS group,which did not receive any ventriculotomy,showed a significant increase in right ventricle longitudinal strain during convalescence.These data support the use of longitudinal strain in Hypoplastic left heart syndrome evaluation and may suggest potential value in terms of cardiac mechanics in using mBTS,preserving the right ventricle integrity.
文摘BACKGROUND Balloon aortic valvuloplasty(BAV)is a well-established treatment modality for congenital aortic valve stenosis.AIM To evaluate the role of rapid right ventricular pacing(RRVP)in balloon stabilization during BAV on aortic regurgitation(AR)in pediatric patients.METHODS A systematic review of the MEDLINE,Cochrane Library,and Scopus databases was conducted according to the PRISMA guidelines(end-of-search date:July 8,2020).The National Heart,Lung,and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment.RESULTS Five studies reporting on 72 patients were included.The studies investigated the use of RRVP-assisted BAV in infants(>1 mo)and older children,but not in neonates.Ten(13.9%)patients had a history of some type of aortic valve surgical or catheterization procedure.Before BAV,58(84.0%),7(10.1%),4(5.9%)patients had AR grade 0(none),1(trivial),2(mild),respectively.After BAV,34(49.3%),6(8.7%),26(37.7%),3(4.3%),patients had AR grade 0,1,2,and 3(moderate),respectively.No patient developed severe AR after RRVP.One(1.4%)developed ventricular fibrillation and was defibrillated successfully.No additional arrhythmias or complications occurred during RRVP.CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV,which could potentially decrease AR rates.
文摘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.
文摘<div style="text-align:justify;"> A persistent left superior vena cava (PLSVC) is a rare malformation which affects approximately 0.3<span>% </span><span>-</span><span> </span><span>0.5% of the population and it is presented along with a right-sided superior vena cava in 82.2% of the cases reported</span><span minion="" pro="" capt","serif";color:#943634;"="" style=""> </span><span minion="" pro="" capt","serif";color:#943634;background:yellow;"=""></span><span>[<a href="#ref1">1</a>]</span><span color:#943634;background:yellow;"=""></span><span minion="" pro="" capt","serif";color:#943634;background:yellow;"=""></span><span>.</span><span "=""><span> Clinicians diagnose it incidentally by difficulties with pacemaker implantation, central venous catheterization or screening for another etiologies when it is not accompanied by other anomalies it is typically asymptomatic. W. Schummer </span><i><span>et al.</span></i><span> described the embryogenesis and the anatomic variations of persistent LSVC according to the positioning of a central venous catheter on the chest radiograph: type I, normal;type II, only PLSVC;type IIIa, right and left superior vena cava with connection;type IIIb, right and left superior vena cava without connection</span></span><span> </span><span>[<a href="#ref2">2</a>]</span><span>. </span><span "=""><span>In 92% of individuals with PLSVC, the PLSVC drains into a dilated coronary sinus (CS) and rest 8% drain directly into the left atrium. PLSVC is caused by a failure in the closure of the left anterior cardinal vein during embryogenic development</span></span><span minion="" pro="" capt","serif";color:#943634;"=""><span> </span><span style="background:yellow;"></span></span><span color:#943634;"=""><span>[<a href="#ref3">3</a>]</span><span style="background:yellow;"></span></span><span minion="" pro="" capt","serif";color:#943634;background:yellow;"=""></span><span>. </span><span>The coronary sinus (CS) is a vein that transmits venous blood to the right atrium though atrioventricular groove. The CS wall contains atrial myocardium. Thus, it</span><span>s size</span><span> extensively depend</span><span>s</span><span> on variability of blood flow and pressure. We present a variant PLSVC with unknown prevalence and a mild </span><span>platypnea-orthodeoxia</span><span> </span><span>syndrome</span><span> after recovery of COVID-19 related acute respiratory distress syndrome (ARDS).</span> </div> <a href="#ref2"></a>
文摘We report the case of a 16-year-old young girl seen for precordial pain and stage II NYHA dyspnea. Clinical examination found a maximal systolodiastolic murmur in the left subclavicular. Lateral Q waves were noted on the electrocardiogram and echocardiography revealed a right coronary fistula draining in right atrium. CT scan confirmed the diagnosis. Patient was addressed to a specialized center for percutaneous closure of the fistula. In case of signs indicating coronary artery disease, the search for coronary anomalies should be systematic in children by using EKG and transthoracic echocardiography. In case of doubt, a second imaging such as the multislice Ct scan is performed. Coronary angiography has a dual diagnostic and therapeutic interest. The indications for treatment depend on the symptoms and the results of ischemia tests.
文摘Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.
文摘BACKGROUND Congenitally corrected levo-transposition of the great arteries(L-TGA)is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy.In L-TGA,the double discordance,atrioventricular and ventriculoarterial,create an acyanotic milieu which allows patients to survive their early decades,however,progressive systemic right ventricle(sRV)dys-function creates complications later in life.sRV dysfunction and remodeling predisposes patients to intracardiac thrombus(ICT)formation.CASE SUMMARY A 40-year-old male with L-TGA presented with symptoms of acute decom-pensated heart failure.In childhood,he had surgical repair of a ventricular septal defect.In adulthood,he developed sRV dysfunction,systemic tricuspid valve(sTV)regurgitation,and left-bundle branch block for which he underwent cardiac resynchronization therapy.Transthoracic echocardiogram showed a sRV ejection fraction of 40%,severe sTV regurgitation,and a newly identified sRV ICT.ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardio-graphy.Our patient was optimized with guideline-directed medical therapy and diuresis.Anticoagulation was achieved with a vitamin K antagonist(VKA)and he was later referred for evaluation by advanced heart failure and heart transplant services.CONCLUSION Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease.This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.
基金Supported by the Russian Science Foundation,No.22-25-00484。
文摘BACKGROUND Necrotizing enterocolitis(NEC)is a multifactorial disease that predominantly affects premature neonates.Intestinal dysbiosis plays a critical role in NEC pathogenesis in premature neonates.The main risk factor for NEC in term infants is mesenteric hypoperfusion associated with ductaldependent congenital heart disease(CHD)that eventually leads to intestinal ischemia.The incidence of NEC in neonates with critical CHD is 6.8%-13%.However,the role of the intestinal microbiome in NEC pathogenesis in infants with ductal-dependent CHD remains unclear.CASE SUMMARY A male term neonate with right atrial isomerism underwent modified Blalock-Taussig shunt placement on the 14^(th)day of life and had persistent mesenteric hypoperfusion after surgery.The patient had episodes of NEC stageⅡA on the 1^(st)and 28^(th)days after cardiac surgery.Fecal microbial composition was analyzed before and after cardiac surgery by sequencing region V4 of the 16S rRNA gene.Before surgery,species belonging to genera Veillonella and Clostridia and class Gammaproteobacteria were detected,Bifidobacteriaceae showed a low abundance.The first NEC episode was associated with postoperative hemodynamic instability,intestinal ischemiareperfusion injury during cardiopulmonary bypass,and a high abundance of Clostridium paraputrificum(Clostridium sensu stricto I)(56.1%).Antibacterial therapy after the first NEC episode resulted in increased abundance of Gammaproteobacteria,decreased abundance of Firmicutes,and low alpha diversity.These changes in the microbial composition promoted the growth of Clostridium sensu strictoⅠ(72.0%)before the second NEC episode.CONCLUSION A high abundance of Clostridium sensu strictoⅠand mesenteric hypoperfusion may have contributed to NEC in the present case.