Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure r...Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.展开更多
BACKGROUND Eisenmenger’s syndrome(ES)is a rare complication of congenital heart disease that includes pulmonary artery hypertension and reversed or bidirectional shunts.The mortality rate of pregnant women with ES is...BACKGROUND Eisenmenger’s syndrome(ES)is a rare complication of congenital heart disease that includes pulmonary artery hypertension and reversed or bidirectional shunts.The mortality rate of pregnant women with ES is 30%-70%due to pathophysiological deterioration.Successful perioperative management of a pregnant patient with ES is a challenge for anesthesiologists.CASE SUMMARY A 38-year-old pregnant woman was admitted to the cardiology department of our hospital at 22 wk of gestation with complaints of chest tightness and shortness of breath for 3 wk.Transthoracic echocardiography revealed a bidirectional shunt between the descending aorta and pulmonary artery after interventional closure of the patent ductus arteriosus and severe pulmonary hypertension.ES in pregnancy was our primary suspicion.The patient elected to terminate the pregnancy under adequate preoperative preparation,rigorous intraoperative monitoring,and perfect epidural anesthesia.She was discharged successfully on postoperative day 16.CONCLUSION Our experience in this case suggests that successful outcomes are possible in pregnant patients with ES for termination of pregnancy under epidural anesthesia and intensive monitoring.展开更多
From September 2006 to January 2007, 2 patients with end-staged heart and lung disease （congenital disease,Eisenmenger＇s syndrome, severe pulmonary artery hypertension and heart failure） underwent heart and lung tr...From September 2006 to January 2007, 2 patients with end-staged heart and lung disease （congenital disease,Eisenmenger＇s syndrome, severe pulmonary artery hypertension and heart failure） underwent heart and lung transplantation （HLT） at the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.展开更多
Background： Eisenmenger syndrome is characterized by the development of pulmonary arterial hypertension with resultant intracardiac right-to-left shunt and hypoxemia in patients with congenital heart defects. In clin...Background： Eisenmenger syndrome is characterized by the development of pulmonary arterial hypertension with resultant intracardiac right-to-left shunt and hypoxemia in patients with congenital heart defects. In clinical practice, we found that these patients might benefit from carvedilol therapy. Thus, we designed this prospective, open label, observational study to evaluate the efficacy of carvedilol for patients with Eisenmenger syndrome. Methods Twenty patients（17.5 ± 4.8 years） with Eisenmenger syndrome were treated with carvedilol for 6 weeks. The efficacy of carvedilol on 6-minute walking distance, World Health Organization（WHO） functional class, arterial oxygen saturation and right ventricle systolic pressure were assessed.Results At the end of observation, change from baseline in 6-minute walking distance increased 36.35 meters（95% confidence intervals [CI] 25.43 to 47.27 m, P 〈 0.01）. WHO functional class was also significantly improved（P 〈 0.05）. Change from baseline in right ventricular systolic pressure assessed by echo was reduced by 8.11 mm Hg（95% confidence intervals [CI],-10.78 to-5.44 mm Hg, P 〈 0.05）. However, arterial oxygen saturation remained unchanged（87.5 ± 3.02 % at baseline versus 87.80 ± 7.29 % at the end of observation）. Conclusions Carvedilol can improve clinical function, symptoms as well as exercise capacity in patients with Eisenmenger syndrome. These findings need to be confirmed in further randomized clinical trials.展开更多
Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estim...Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estimated prevalence of 5– 10% in adult patients,with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension(PAH)-targeted pharmacotherapy.Although limited data exist,targeted PAH pharmacotherapy has proven to be benefi cial in patients with CHD-associated PAH,with observed improvement in functional class,increase in exercise capacity,and improvement in quality of life and cardiopulmonary hemodynamics.Additionally,there has been increasing interest in the“treat-to-close”strategy.PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’operability in repairing the cardiac defect.Although there have been signifi cant advances in the management of this disease state in the past 2 decades,mortality remains high,and ongoing clinical trials are needed to better understand the treat-to-close strategy.展开更多
BACKGROUND Eisenmenger syndrome(ES)is an uncorrected congenital cardiac defect with a left-to-right shunt,leading to pulmonary arterial hypertension.Patients with ES are susceptible to hemodynamic alterations during n...BACKGROUND Eisenmenger syndrome(ES)is an uncorrected congenital cardiac defect with a left-to-right shunt,leading to pulmonary arterial hypertension.Patients with ES are susceptible to hemodynamic alterations during noncardiac surgery with general anesthesia,which increases perioperative morbidity and mortality.Monitored anesthesia care(MAC)is often used during minor procedures in patients with cardiac disease.However,few reports on MAC in patients with ES exist.CASE SUMMARY A 49-year-old man was admitted for a severe headache lasting 30 d.He had been diagnosed with a large perimembranous ventricular septal defect(VSD)with bidirectional shunt flow and pulmonary arterial hypertension 10 years ago.A round mass in the right frontal lobe was revealed by Magnetic resonance imaging.Stereotactic aspiration using a neuronavigation system was performed under MAC.The patient was stayed in the hospital for 5 d,and discharged without complications.CONCLUSION MAC may be effective for craniotomy in patients with ES.展开更多
目的探讨肺移植及心肺联合移植治疗艾森曼格综合征的手术适应证和围手术期处理。方法 2002年9月至2006年3月共收治5例先天性心脏畸形伴艾森曼格综合征(Eisenmengersyndrome,ES)患者,其中先天性心脏畸形房间隔缺损2例,室间隔缺损2例,均...目的探讨肺移植及心肺联合移植治疗艾森曼格综合征的手术适应证和围手术期处理。方法 2002年9月至2006年3月共收治5例先天性心脏畸形伴艾森曼格综合征(Eisenmengersyndrome,ES)患者,其中先天性心脏畸形房间隔缺损2例,室间隔缺损2例,均进行了心脏畸形修补及同期右肺移植;另有1例为先天性心脏病房间隔缺损,采用介入房缺封堵后发生全心功能衰竭,紧急行心肺联合移植。结果 4例心脏畸形修补同期单肺移植中有1例术后20 d 死于原发性移植物失功;1例心肺联合移植术后23 d 因原发性移植物失功死亡。3例患者康复出院,现已分别存活5、8、22个月,生活质量良好。患者术后肺动脉压明显下降、血气分析结果改善明显。结论心脏畸形修补同期单肺移植能有效治疗先天性心脏畸形伴艾森曼格综合征,匹配的供肺、良好的供肺切取和保护、完善的术后处理是患者术后存活的关键。展开更多
文摘Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.
文摘BACKGROUND Eisenmenger’s syndrome(ES)is a rare complication of congenital heart disease that includes pulmonary artery hypertension and reversed or bidirectional shunts.The mortality rate of pregnant women with ES is 30%-70%due to pathophysiological deterioration.Successful perioperative management of a pregnant patient with ES is a challenge for anesthesiologists.CASE SUMMARY A 38-year-old pregnant woman was admitted to the cardiology department of our hospital at 22 wk of gestation with complaints of chest tightness and shortness of breath for 3 wk.Transthoracic echocardiography revealed a bidirectional shunt between the descending aorta and pulmonary artery after interventional closure of the patent ductus arteriosus and severe pulmonary hypertension.ES in pregnancy was our primary suspicion.The patient elected to terminate the pregnancy under adequate preoperative preparation,rigorous intraoperative monitoring,and perfect epidural anesthesia.She was discharged successfully on postoperative day 16.CONCLUSION Our experience in this case suggests that successful outcomes are possible in pregnant patients with ES for termination of pregnancy under epidural anesthesia and intensive monitoring.
文摘From September 2006 to January 2007, 2 patients with end-staged heart and lung disease （congenital disease,Eisenmenger＇s syndrome, severe pulmonary artery hypertension and heart failure） underwent heart and lung transplantation （HLT） at the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
文摘Background： Eisenmenger syndrome is characterized by the development of pulmonary arterial hypertension with resultant intracardiac right-to-left shunt and hypoxemia in patients with congenital heart defects. In clinical practice, we found that these patients might benefit from carvedilol therapy. Thus, we designed this prospective, open label, observational study to evaluate the efficacy of carvedilol for patients with Eisenmenger syndrome. Methods Twenty patients（17.5 ± 4.8 years） with Eisenmenger syndrome were treated with carvedilol for 6 weeks. The efficacy of carvedilol on 6-minute walking distance, World Health Organization（WHO） functional class, arterial oxygen saturation and right ventricle systolic pressure were assessed.Results At the end of observation, change from baseline in 6-minute walking distance increased 36.35 meters（95% confidence intervals [CI] 25.43 to 47.27 m, P 〈 0.01）. WHO functional class was also significantly improved（P 〈 0.05）. Change from baseline in right ventricular systolic pressure assessed by echo was reduced by 8.11 mm Hg（95% confidence intervals [CI],-10.78 to-5.44 mm Hg, P 〈 0.05）. However, arterial oxygen saturation remained unchanged（87.5 ± 3.02 % at baseline versus 87.80 ± 7.29 % at the end of observation）. Conclusions Carvedilol can improve clinical function, symptoms as well as exercise capacity in patients with Eisenmenger syndrome. These findings need to be confirmed in further randomized clinical trials.
文摘Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estimated prevalence of 5– 10% in adult patients,with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension(PAH)-targeted pharmacotherapy.Although limited data exist,targeted PAH pharmacotherapy has proven to be benefi cial in patients with CHD-associated PAH,with observed improvement in functional class,increase in exercise capacity,and improvement in quality of life and cardiopulmonary hemodynamics.Additionally,there has been increasing interest in the“treat-to-close”strategy.PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’operability in repairing the cardiac defect.Although there have been signifi cant advances in the management of this disease state in the past 2 decades,mortality remains high,and ongoing clinical trials are needed to better understand the treat-to-close strategy.
文摘BACKGROUND Eisenmenger syndrome(ES)is an uncorrected congenital cardiac defect with a left-to-right shunt,leading to pulmonary arterial hypertension.Patients with ES are susceptible to hemodynamic alterations during noncardiac surgery with general anesthesia,which increases perioperative morbidity and mortality.Monitored anesthesia care(MAC)is often used during minor procedures in patients with cardiac disease.However,few reports on MAC in patients with ES exist.CASE SUMMARY A 49-year-old man was admitted for a severe headache lasting 30 d.He had been diagnosed with a large perimembranous ventricular septal defect(VSD)with bidirectional shunt flow and pulmonary arterial hypertension 10 years ago.A round mass in the right frontal lobe was revealed by Magnetic resonance imaging.Stereotactic aspiration using a neuronavigation system was performed under MAC.The patient was stayed in the hospital for 5 d,and discharged without complications.CONCLUSION MAC may be effective for craniotomy in patients with ES.
文摘目的探讨肺移植及心肺联合移植治疗艾森曼格综合征的手术适应证和围手术期处理。方法 2002年9月至2006年3月共收治5例先天性心脏畸形伴艾森曼格综合征(Eisenmengersyndrome,ES)患者,其中先天性心脏畸形房间隔缺损2例,室间隔缺损2例,均进行了心脏畸形修补及同期右肺移植;另有1例为先天性心脏病房间隔缺损,采用介入房缺封堵后发生全心功能衰竭,紧急行心肺联合移植。结果 4例心脏畸形修补同期单肺移植中有1例术后20 d 死于原发性移植物失功;1例心肺联合移植术后23 d 因原发性移植物失功死亡。3例患者康复出院,现已分别存活5、8、22个月,生活质量良好。患者术后肺动脉压明显下降、血气分析结果改善明显。结论心脏畸形修补同期单肺移植能有效治疗先天性心脏畸形伴艾森曼格综合征,匹配的供肺、良好的供肺切取和保护、完善的术后处理是患者术后存活的关键。
