Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient w...Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.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
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 Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patient...BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patients with seronegative RA(SNRA).It is unclear whether there is an overlap between ASS and RA,or if ASS sometimes mimics RA.Pulmonary hypertension(PAH)is common in connective tissue diseases(CTDs).However,published reports on CTD-PAH do not include overlapping CTDs,and its incidence and impact on patient prognosis are unclear.CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years.The patient was diagnosed with RA and interstitial lung disease.The patient repeatedly presented to the hospital’s respiratory and rhe-umatology departments with arthralgia,plus shortness of breath after activity.Relevant tests indicated that anti-CCP and RF remained negative,while anti-J0-1 and anti-Ro-52 were strongly positive.It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS.Joint pain was relieved after regular anti-rheumatic treatment.Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years;however,they showed gradual widening of the pulmonary artery,and cardiac ultrasound indicated elevated pulmonary artery systolic pressure.The prescribed treatment of PAH was not effective in improving shortness of breath.CONCLUSION Overlap of RA and ASS may be missed.Further research is necessary to facilitate early diagnosis,effective evaluation,and prognosis.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
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
文摘Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.
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.
基金Supported by the Natural Science Foundation of China,No.82060010。
文摘BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patients with seronegative RA(SNRA).It is unclear whether there is an overlap between ASS and RA,or if ASS sometimes mimics RA.Pulmonary hypertension(PAH)is common in connective tissue diseases(CTDs).However,published reports on CTD-PAH do not include overlapping CTDs,and its incidence and impact on patient prognosis are unclear.CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years.The patient was diagnosed with RA and interstitial lung disease.The patient repeatedly presented to the hospital’s respiratory and rhe-umatology departments with arthralgia,plus shortness of breath after activity.Relevant tests indicated that anti-CCP and RF remained negative,while anti-J0-1 and anti-Ro-52 were strongly positive.It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS.Joint pain was relieved after regular anti-rheumatic treatment.Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years;however,they showed gradual widening of the pulmonary artery,and cardiac ultrasound indicated elevated pulmonary artery systolic pressure.The prescribed treatment of PAH was not effective in improving shortness of breath.CONCLUSION Overlap of RA and ASS may be missed.Further research is necessary to facilitate early diagnosis,effective evaluation,and prognosis.
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.
