AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS betwe...AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS between June 1988 and May 2008.The diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography,computed tomography,magnetic resonance imaging,or venography.The median follow-up period was 103 ± 156 [interquartile range(IQR)] mo.RESULTS:The median age of the patients was 47 ± 16(IQR) years.At diagnosis,54 patients had cirrhosis,25(37.3%) Child-Pugh class A,23(34.3%) Child-Pugh class B,and six(9.0%) patients Child-Pugh class C.During the follow-up period,HCC was developed in 17 patients,and the annual incidence of HCC in patients with BCS was 2.8%.Patients in HCC group(n = 17) had higher hepatic venous pressure gradient(HVPG) than those in non-HCC group(n = 50)(21 ± 12 mmHg vs 14 ± 7 mmHg,P = 0.019).The survival rate of BCS patients was 86.2% for 5 years,73.8% for 10 years,and 61.2% for 15 years.In patients with BCS and HCC,survival was 79% for 5 years,43.1% for 10 years,and 21.5% for 15 years.CONCLUSION:The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South Korea.The HVPG is expected to provide additional information for predicting HCC development in BCS patients.展开更多
Objective To explore the outcome of patients with Brugada syndrome by remedial or prophylactic implantable cardioverter defibrillator （ ICD ） for ventricular tachyarrhythmia. Methods Sixteen single chamber ICDs and ...Objective To explore the outcome of patients with Brugada syndrome by remedial or prophylactic implantable cardioverter defibrillator （ ICD ） for ventricular tachyarrhythmia. Methods Sixteen single chamber ICDs and 5 double chamber ICDs in 21 consecutive patients [ 17 males, 4 females; mean age, （ 36 ± 11） years ] with Brugada syndrome were involved. Fifteen patients with spontaneous or induced ventricular tachycardia/ventricular fibrillation （VT/VF） received the remedial and prophylactic ICD implantation. Six patients only with positive family history or syncope accepted the ICD implantation for prophylaxis. Results After a median follow-up of （22.3 ± 13. 4） months, all patients were alive. One patient was suspected with febrile syndrome due to endocarditis on the auricular face of the lead of defibrillator 18 months after 1CD implantation and disappearance of vegetation was found after receiving one-month antibiotic treatment. Nine appropriate ICD interventions took place in 3 individuals with documented spontaneous ventricular arrhythmias. Multivariable analysis suggested that occurrence of appropriate interventions was related to the spontaneous ventricular arrhythmias （ P 〈 0. 0001 ）. Eleven inappropriate ICD interventions occurred in 3 patients for atrial fibrillation or atrial flutter with rapid ventricular rate. Multivariable analysis suggested that occurrence of inappropriate interventions was related to documented spontaneous supraventricular arrhythmias （P 〈 O. 0062 ）. All appropriate interventions and inappropriate interventions occurred within six months after ICD implantation. Conclusion Appropriate interventions may associate to spontaneous ventricular arrhythmias before 1CD implantation in patients with Brugada syndrome. Patients may suffer from inappropriate ICD intervention for supraventricular arrhythmias with rapid ventricular rate when they had a history of supraventricular arrhythmias.展开更多
基金Supported by A grant of the South Korea Healthcare Technology R and D Project,Ministry of Health and Welfare,South Korea,No.A102065
文摘AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS between June 1988 and May 2008.The diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography,computed tomography,magnetic resonance imaging,or venography.The median follow-up period was 103 ± 156 [interquartile range(IQR)] mo.RESULTS:The median age of the patients was 47 ± 16(IQR) years.At diagnosis,54 patients had cirrhosis,25(37.3%) Child-Pugh class A,23(34.3%) Child-Pugh class B,and six(9.0%) patients Child-Pugh class C.During the follow-up period,HCC was developed in 17 patients,and the annual incidence of HCC in patients with BCS was 2.8%.Patients in HCC group(n = 17) had higher hepatic venous pressure gradient(HVPG) than those in non-HCC group(n = 50)(21 ± 12 mmHg vs 14 ± 7 mmHg,P = 0.019).The survival rate of BCS patients was 86.2% for 5 years,73.8% for 10 years,and 61.2% for 15 years.In patients with BCS and HCC,survival was 79% for 5 years,43.1% for 10 years,and 21.5% for 15 years.CONCLUSION:The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South Korea.The HVPG is expected to provide additional information for predicting HCC development in BCS patients.
文摘Objective To explore the outcome of patients with Brugada syndrome by remedial or prophylactic implantable cardioverter defibrillator （ ICD ） for ventricular tachyarrhythmia. Methods Sixteen single chamber ICDs and 5 double chamber ICDs in 21 consecutive patients [ 17 males, 4 females; mean age, （ 36 ± 11） years ] with Brugada syndrome were involved. Fifteen patients with spontaneous or induced ventricular tachycardia/ventricular fibrillation （VT/VF） received the remedial and prophylactic ICD implantation. Six patients only with positive family history or syncope accepted the ICD implantation for prophylaxis. Results After a median follow-up of （22.3 ± 13. 4） months, all patients were alive. One patient was suspected with febrile syndrome due to endocarditis on the auricular face of the lead of defibrillator 18 months after 1CD implantation and disappearance of vegetation was found after receiving one-month antibiotic treatment. Nine appropriate ICD interventions took place in 3 individuals with documented spontaneous ventricular arrhythmias. Multivariable analysis suggested that occurrence of appropriate interventions was related to the spontaneous ventricular arrhythmias （ P 〈 0. 0001 ）. Eleven inappropriate ICD interventions occurred in 3 patients for atrial fibrillation or atrial flutter with rapid ventricular rate. Multivariable analysis suggested that occurrence of inappropriate interventions was related to documented spontaneous supraventricular arrhythmias （P 〈 O. 0062 ）. All appropriate interventions and inappropriate interventions occurred within six months after ICD implantation. Conclusion Appropriate interventions may associate to spontaneous ventricular arrhythmias before 1CD implantation in patients with Brugada syndrome. Patients may suffer from inappropriate ICD intervention for supraventricular arrhythmias with rapid ventricular rate when they had a history of supraventricular arrhythmias.
