目的评价螺旋电极导线行右室流出道(RVOT)间隔部起搏的可行性。方法连续入组195例具有植入起搏器适应证患者,术前随机分为螺旋主动固定电极导线的RVOT间隔起搏组(A组)和翼状被动固定电极导线的右室心尖部(RVA)起搏组(B组),两组中每例入...目的评价螺旋电极导线行右室流出道(RVOT)间隔部起搏的可行性。方法连续入组195例具有植入起搏器适应证患者,术前随机分为螺旋主动固定电极导线的RVOT间隔起搏组(A组)和翼状被动固定电极导线的右室心尖部(RVA)起搏组(B组),两组中每例入选患者均分别行RVA和RVOT两个部位起搏测试,最后固定于相应的位置。比较两组术中手术时间、起搏参数、起搏QRS波宽度、手术成功率及起搏3个月、1年和2年后电极导线参数的变化。结果 A组99例,B组96例。两组起搏后QRS波宽度明显大于起搏前,B组起搏QRS波时限长于A组(176.46±24.54 ms vs 165.45±22.78 ms,P=0.001)。用于固定RVOT间隔部的曝光时间长于RVA。两组术中及术后并发症相似,R波振幅术后2年内及两组间无差别。术中A组起搏阈值高于B组(0.71±0.30 V vs0.56±0.19 V),术后2年内起搏阈值两组内及组间无差异。术后3个月时阻抗下降,A组的阻抗低于B组并持续整个随访期间。术后2年内超声心动图参数组内及组间无差别。结论采用螺旋主动固定电极导线进行RVOT起搏是安全可行的。展开更多
AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies condu...AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.展开更多
BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.MET...BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.METHODS A total of 904 BCS patients from New Delhi,India and 1140 from Mumbai,India were included.The prevalence and incidence of HCC were determined,and among patients with BCS-HCC,the viability and outcomes of interventional therapy were evaluated.RESULTS In the New Delhi cohort of 35 BCS-HCC patients,18 had HCC at index presentation(prevalence 1.99%),and 17 developed HCC over a follow-up of 4601 person-years,[incidence 0.36(0.22-0.57)per 100 person-years].BCS-HCC patients were older when compared to patients with BCS alone(P=0.001)and had a higher proportion of inferior vena cava block,cirrhosis,and long-segment vascular obstruction.The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up(13029 ng/mL vs 500 ng/mL,P=0.01).Of the 35 BCS-HCC,26(74.3%)underwent radiological interventions for BCS,and 22(62.8%)patients underwent treatment for HCC[transarterial chemoembolization in 18(81.8%),oral tyrosine kinase inhibitor in 3(13.6%),and transarterial radioembolization in 1(4.5%)].The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo(P=0.0001).In contrast to the New Delhi cohort,the Mumbai cohort of BCS-HCC patients were predominantly males,presented with a more advanced HCC[Barcelona Clinic Liver Cancer C and D],and 2 patients underwent liver transplantation.CONCLUSION HCC is not uncommon in patients with BCS.Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.展开更多
Background:Parents of children with complex right ventricular outflow tract(RVOT)anomalies are confronted with their child’s need for heart surgery early in life and repeated reoperations later on.Preoperative assess...Background:Parents of children with complex right ventricular outflow tract(RVOT)anomalies are confronted with their child’s need for heart surgery early in life and repeated reoperations later on.Preoperative assessment needs to be performed whenever an indication for reoperation is suspected.The aim was to illuminate the experiences of parents of children diagnosed with RVOT anomalies,in particular,how they experience their child’s heart disease and everyday life during the assessment and after the decision on whether to perform a reoperation.Method:Individual interviews(n=27)were conducted with nine parents on three occasions between 2014 and 2016 and analyzed using reflexive thematic analysis.Results:The analysis resulted in the following five main coexisting themes:The heart surgery keeps my child alive illuminates parents’experiences during and after the assessment and emphasizes that heart surgery,although dreaded,is central for their child’s survival;Everyday struggles illuminates the different struggles parents had to face to ensure that their child would be in the best possible condition;the remaining three themes,Unconditional love,Trust in life,and Togetherness,illuminate the ways in which the parents gained inner strength and confidence in their everyday lives.Conclusion:Although the parents were grateful for the assessment and had learned to navigate among the fears it aroused,they experienced several distressing situations during the assessment process that should be addressed.By inviting both the parents and their child to participate in the child’s care,individualized support can take into account the needs of both parents and child.展开更多
Objective:The size and morphology of the right ventricular outflow tract(RVOT)in patients suffering from long-term pulmonary regurgitation(PR)after native RVOT(NRVOT)reconstruction are important factors affecting the ...Objective:The size and morphology of the right ventricular outflow tract(RVOT)in patients suffering from long-term pulmonary regurgitation(PR)after native RVOT(NRVOT)reconstruction are important factors affecting the feasibility,safety,and effectiveness of transcatheter pulmonary valve replacement.The purpose of this study was to evaluate the feasibility,safety,and effectiveness of a transthoracic Salus valve(Balance Medical Technology Co.,Ltd,Beijing,China)in patients with moderate-to-severe PR after NRVOT reconstruction.Methods:Patients with moderate-to-severe PR after NRVOT reconstruction were selected between June 2021 and November 2021 at Beijing Anzhen Hospital.Demographic data as well as preoperative,intraoperative,and follow-up data were reviewed.Results:Ten patients with moderate-to-severe PR after NRVOT reconstruction underwent physical examination,transthoracic echocardiography,and cardiovascular magnetic resonance imaging.Seven patients were selected for transthoracic Salus valve replacement.Six patients underwent implantation of the Salus valve successfully.One valve migrated and was embolized during recovery of the delivery device;the Salus valve was surgically explanted and sutured to the inner wall of the main pulmonary artery.At a mean follow-up of(5.5±1.1)months,dysfunction or migration of the Salus valve embolism was not observed.Conclusions:This early feasibility study demonstrates the feasibility,safety,and effectiveness of transthoracic implantation of a Salus valve in patients with moderate-to-severe PR after NRVOT reconstruction.The short-term effectiveness is clear,medium and long-term effectiveness requires longer follow-up.展开更多
The mechanism of idiopathic ventricular tachycardia originating from the right ventricular outflow tract （RVOT） is not clear. Many clinical reports have suggested a mechanism of triggered activity. However, there ar...The mechanism of idiopathic ventricular tachycardia originating from the right ventricular outflow tract （RVOT） is not clear. Many clinical reports have suggested a mechanism of triggered activity. However, there are few studies investigating this be- cause of the technical difficulties associated with examining this theory. The L-type calcium current （/Ca-L）, an important in- ward current of the action potential （AP）, plays an important role in arrhythmogenesis. The aim of this study was to explore differences in the APs of right ventricular （RV） and RVOT cardiomyocytes, and differences in electrophysiological character- istics of the ICa-L in these myocytes. Rabbit RVOT and RV myocytes were isolated and their AP and Ic,-L were investigated us- ing the patch-clamp technique. RVOT cardiomyocytes had a wider range of AP duration （APD） than RV cardiomyocytes, with some markedly prolonged APDs and markedly shortened APDs. The markedly shortened APDs in RVOT myocytes were abolished by treatment with 4-AP, an inhibitor of the transient outward potassium current, but the markedly prolonged APDs remained, with some myocytes with a long AP plateau not repolarizing to resting potential. In addition, early afterdepolariza- tion （EAD） and second plateau responses were seen in RVOT myocytes but not in RV myocytes. RVOT myocytes had a high- er current density for/Ca-L than RV myocytes （RVOT （13.16±0.87） pA pF-1, RV （8.59±1.97） pA pF-1; P〈0.05）. The ICa-L and the prolonged APD were reduced, and the EAD and second plateau response disappeared, after treatment with nifedipine （10 μmol L^-1）, which blocks the Ica-L. In conclusion, there was a wider range of APDs in RVOT myocytes than in RV myocytes, which is one of the basic factors involved in arrhythmogenesis. The higher current density for ICa-L is one of the factors causing prolongation of the APD in RVOT myocytes. The combination of EAD with prolonged APD may be one of the mechanisms of RVOT-VT generation.展开更多
文摘目的评价螺旋电极导线行右室流出道(RVOT)间隔部起搏的可行性。方法连续入组195例具有植入起搏器适应证患者,术前随机分为螺旋主动固定电极导线的RVOT间隔起搏组(A组)和翼状被动固定电极导线的右室心尖部(RVA)起搏组(B组),两组中每例入选患者均分别行RVA和RVOT两个部位起搏测试,最后固定于相应的位置。比较两组术中手术时间、起搏参数、起搏QRS波宽度、手术成功率及起搏3个月、1年和2年后电极导线参数的变化。结果 A组99例,B组96例。两组起搏后QRS波宽度明显大于起搏前,B组起搏QRS波时限长于A组(176.46±24.54 ms vs 165.45±22.78 ms,P=0.001)。用于固定RVOT间隔部的曝光时间长于RVA。两组术中及术后并发症相似,R波振幅术后2年内及两组间无差别。术中A组起搏阈值高于B组(0.71±0.30 V vs0.56±0.19 V),术后2年内起搏阈值两组内及组间无差异。术后3个月时阻抗下降,A组的阻抗低于B组并持续整个随访期间。术后2年内超声心动图参数组内及组间无差别。结论采用螺旋主动固定电极导线进行RVOT起搏是安全可行的。
文摘AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.
基金This study was reviewed and approved by the Ethics Committee of the All India Institute of Medical Sciences,New Delhi(Approval No.IEC/NP-458/12.12.2014,RP 22-2015).
文摘BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.METHODS A total of 904 BCS patients from New Delhi,India and 1140 from Mumbai,India were included.The prevalence and incidence of HCC were determined,and among patients with BCS-HCC,the viability and outcomes of interventional therapy were evaluated.RESULTS In the New Delhi cohort of 35 BCS-HCC patients,18 had HCC at index presentation(prevalence 1.99%),and 17 developed HCC over a follow-up of 4601 person-years,[incidence 0.36(0.22-0.57)per 100 person-years].BCS-HCC patients were older when compared to patients with BCS alone(P=0.001)and had a higher proportion of inferior vena cava block,cirrhosis,and long-segment vascular obstruction.The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up(13029 ng/mL vs 500 ng/mL,P=0.01).Of the 35 BCS-HCC,26(74.3%)underwent radiological interventions for BCS,and 22(62.8%)patients underwent treatment for HCC[transarterial chemoembolization in 18(81.8%),oral tyrosine kinase inhibitor in 3(13.6%),and transarterial radioembolization in 1(4.5%)].The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo(P=0.0001).In contrast to the New Delhi cohort,the Mumbai cohort of BCS-HCC patients were predominantly males,presented with a more advanced HCC[Barcelona Clinic Liver Cancer C and D],and 2 patients underwent liver transplantation.CONCLUSION HCC is not uncommon in patients with BCS.Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.
基金This study was supported by the Pediatric Heart Center at Skåne University Hospital Lund and Lund University,and by the Swedish Children’s Heart Association.
文摘Background:Parents of children with complex right ventricular outflow tract(RVOT)anomalies are confronted with their child’s need for heart surgery early in life and repeated reoperations later on.Preoperative assessment needs to be performed whenever an indication for reoperation is suspected.The aim was to illuminate the experiences of parents of children diagnosed with RVOT anomalies,in particular,how they experience their child’s heart disease and everyday life during the assessment and after the decision on whether to perform a reoperation.Method:Individual interviews(n=27)were conducted with nine parents on three occasions between 2014 and 2016 and analyzed using reflexive thematic analysis.Results:The analysis resulted in the following five main coexisting themes:The heart surgery keeps my child alive illuminates parents’experiences during and after the assessment and emphasizes that heart surgery,although dreaded,is central for their child’s survival;Everyday struggles illuminates the different struggles parents had to face to ensure that their child would be in the best possible condition;the remaining three themes,Unconditional love,Trust in life,and Togetherness,illuminate the ways in which the parents gained inner strength and confidence in their everyday lives.Conclusion:Although the parents were grateful for the assessment and had learned to navigate among the fears it aroused,they experienced several distressing situations during the assessment process that should be addressed.By inviting both the parents and their child to participate in the child’s care,individualized support can take into account the needs of both parents and child.
基金supported by the National Key Research and Development Program of China(2020YFC1107903).
文摘Objective:The size and morphology of the right ventricular outflow tract(RVOT)in patients suffering from long-term pulmonary regurgitation(PR)after native RVOT(NRVOT)reconstruction are important factors affecting the feasibility,safety,and effectiveness of transcatheter pulmonary valve replacement.The purpose of this study was to evaluate the feasibility,safety,and effectiveness of a transthoracic Salus valve(Balance Medical Technology Co.,Ltd,Beijing,China)in patients with moderate-to-severe PR after NRVOT reconstruction.Methods:Patients with moderate-to-severe PR after NRVOT reconstruction were selected between June 2021 and November 2021 at Beijing Anzhen Hospital.Demographic data as well as preoperative,intraoperative,and follow-up data were reviewed.Results:Ten patients with moderate-to-severe PR after NRVOT reconstruction underwent physical examination,transthoracic echocardiography,and cardiovascular magnetic resonance imaging.Seven patients were selected for transthoracic Salus valve replacement.Six patients underwent implantation of the Salus valve successfully.One valve migrated and was embolized during recovery of the delivery device;the Salus valve was surgically explanted and sutured to the inner wall of the main pulmonary artery.At a mean follow-up of(5.5±1.1)months,dysfunction or migration of the Salus valve embolism was not observed.Conclusions:This early feasibility study demonstrates the feasibility,safety,and effectiveness of transthoracic implantation of a Salus valve in patients with moderate-to-severe PR after NRVOT reconstruction.The short-term effectiveness is clear,medium and long-term effectiveness requires longer follow-up.
文摘The mechanism of idiopathic ventricular tachycardia originating from the right ventricular outflow tract （RVOT） is not clear. Many clinical reports have suggested a mechanism of triggered activity. However, there are few studies investigating this be- cause of the technical difficulties associated with examining this theory. The L-type calcium current （/Ca-L）, an important in- ward current of the action potential （AP）, plays an important role in arrhythmogenesis. The aim of this study was to explore differences in the APs of right ventricular （RV） and RVOT cardiomyocytes, and differences in electrophysiological character- istics of the ICa-L in these myocytes. Rabbit RVOT and RV myocytes were isolated and their AP and Ic,-L were investigated us- ing the patch-clamp technique. RVOT cardiomyocytes had a wider range of AP duration （APD） than RV cardiomyocytes, with some markedly prolonged APDs and markedly shortened APDs. The markedly shortened APDs in RVOT myocytes were abolished by treatment with 4-AP, an inhibitor of the transient outward potassium current, but the markedly prolonged APDs remained, with some myocytes with a long AP plateau not repolarizing to resting potential. In addition, early afterdepolariza- tion （EAD） and second plateau responses were seen in RVOT myocytes but not in RV myocytes. RVOT myocytes had a high- er current density for/Ca-L than RV myocytes （RVOT （13.16±0.87） pA pF-1, RV （8.59±1.97） pA pF-1; P〈0.05）. The ICa-L and the prolonged APD were reduced, and the EAD and second plateau response disappeared, after treatment with nifedipine （10 μmol L^-1）, which blocks the Ica-L. In conclusion, there was a wider range of APDs in RVOT myocytes than in RV myocytes, which is one of the basic factors involved in arrhythmogenesis. The higher current density for ICa-L is one of the factors causing prolongation of the APD in RVOT myocytes. The combination of EAD with prolonged APD may be one of the mechanisms of RVOT-VT generation.
