We performed a retrospective analysis of patients with and without 22q11 deletion undergoing surgery for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals between January 2004 and A...We performed a retrospective analysis of patients with and without 22q11 deletion undergoing surgery for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals between January 2004 and August 2009 at our institutions. Information was collected on collateral origin, arch sidedness, presence of central pulmonary arteries, and presence of an aberrant subclavian vessel. While patients with 22q11 deletion were more likely to have collateral origin from brachiocephalic vessels, patients without 22q11 deletion were more likely to have collateral origin from the descending aorta. There was no significant difference in arch sidedness or the presence of central pulmonary arteries. Patients with 22q11 deletion were more likely to have an aberrant subclavian artery (15/46 vs 5/54, p < 0.05), whether a left or right arch was present. Nine of the fifteen 22q11 deletion patients had a collateral originating from an aberrant subclavian artery. In time, genomic and embryologic research may help determine the exact mechanisms by which 22q11 deletion contributes to the development of congenital heart disease such as pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.展开更多
Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE...Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septaldefect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the sameage (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE.The measurements included: left and right ventricular volume indexed to body surface area (BSA), stroke volume(SV) indexed to BSA, and ejection fraction (EF). Results: The results showed left and right ventricular volumeindexed to BSA and SV indexed to BSA were significantly increased in VSD + PH Group (VSD + PH Groupvs. Control Group), LVEDV/BSA (ml/m2): 48.67 ± 21.46 vs. 25.59 ± 6.96, RVEDV/BSA (ml/m2): 55.98 ±15.35 vs. 27.69 ± 4.37, LVSV/BSA (ml/m2): 24.08 ± 9.30 vs. 15.14 ± 4.29, RVSV/BSA (ml/m2): 26.02 ± 8.87 vs.14.11 ± 2.89, (P < 0.05). While for EF in VSD + PH Group decreased (VSD + PH Group vs. Control Group),LVEF: 50.93 ± 7.50% vs. 59.38 ± 7.24%, RVEF: 45.84 ± 7.71% vs. 51.05 ± 6.90% (P < 0.05). Conclusion: Inchildren with VSD and moderate to severe PH, increased biventricular volume and decreased systolic functionwere observed with RT-3DE, but biventricular systolic function remained within acceptable limits. The childrenin this study recovered well after surgery without serious perioperative complications, suggesting that biventricularsystolic function may help facilitate the surgical decision-making process in children with VSD and moderate-toseverePH.展开更多
Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and...Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and pulmonary arterial hypertension(VSD-PAH).Background:Children with congenital heart disease(CHD)with pulmonary arterial hypertension(PAH)have more complications after surgery than those without PAH.Discharge planning is an effective strategy to help children leave the hospital safely,and receive appropriate care after discharge.Methods:A quasi-experimental design was used.Sixty children and their mothers were recruited and divided into two groups:the control group received conventional care,the intervention group received both conventional care and additional discharge planning care.Results:(1)After admission,maternal caring knowledge between the two groups was similar.(2)At discharge,maternal discharge readiness,maternal caring knowledge and maternal caring behavior in the intervention group was significantly higher compared to the control group(t=3.35,p=0.001;F=84.74,p<0.001;F=23.82,p<0.001).This difference persisted after discharge,and was evident at one month and three months after discharge.(3)However,no significant difference in the readmission rate of children after discharge was evident between the two groups.Conclusions:Discharge planning improves the maternal discharge readiness,maternal caring knowledge and maternal caring behaviors.However,this planning did not reduce the readmission rate of children with CHD-PAH.展开更多
Speckle tracking imaging （STI） was employed to investigate the effect of right ventricular （RV） volume and pressure overload on left ventricular （LV） rotation and twist in 35 patients with atrial septal defect ...Speckle tracking imaging （STI） was employed to investigate the effect of right ventricular （RV） volume and pressure overload on left ventricular （LV） rotation and twist in 35 patients with atrial septal defect （ASD）, 18 of which with pulmonary hypertension, and 21 healthy subjects serving as controls. The peak rotations of 6 segments at the basal and apical short-axises and the average peak rotation and interval time of the 6 segments in the opposite direction during early systolic phase were measured respectively. LV twist versus time profile was drawn and the peak twist and time to peak twist were calculated. LV ejection fraction （EF） was measured by Biplane Simpson. Compared to ASD patients without pulmonary hypertension and healthy subjects, the peak rotations of posterior, inferior and postsept walls at the basal level were lower （P〈0.05）, and the average counterclockwise peak rotation of 6 segments at the basal level during early systolic phase was higher （P〈0.05）, and the average interval time was delayed （P〈0.05）. LV peak twist was also lower （P〈0.05）, and had a significant negative correlation with pulmonary arterial systolic pressure （r=-0.57, P=0.001）. No significant differences were found in LVEF among the three groups. It was suggested that although RV volume overload due to ASD has no significant effects on LV rotation and twist, LV peak twist is lower in ASD patients with pulmonary hypertension. Thus LV twist may serve as a new indicator of the presence of pulmonary hypertension in ASD patients.展开更多
Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH...Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH). Methods The Streptavidin-peroxidase (SP) immunoassay was used to measure the expression of ET-1 in pulmonary arteriola, pulmonary veinlet, endocardium and endangium of vasa coronary of 20 cases VSD of CHD with PH, and contrast the expression level of these 20 cases VSD of CHD with PH. Results The expression of ET-1 PH patients in pulmonary arteriola, endocardium, and endangium of vasa coronary was much higher than that of the control group (P<0.05), but there was no significance variance in expression level of pulmonary veinlet between two groups (P>0.05). Conclusion If VSD of CHD was accompany with PH, the degree of PH has a positive correlation with the amount of ET-1 in pulmonary arteriola, ET-1 may be the cause of dynamic PH, and also the acceleration factor of the PH. However, the amount of ET-1 in endocardium and endangium of vasa coronary, may have significant connection with the myocardium hypertrophy in dynamic PH.展开更多
Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary ar...Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.展开更多
Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is nee...Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is needed.Therefore,this study aimed to investigate the application of echocardiogram in predicting the severity of pulmonary hypertension and guide subsequent management.Methods This study included VSD patients who underwent right heart catheterization(RHC)examination in Guangdong Provincial People's Hospital from January2011 to December 2022.An estimated pulmonary artery systolic pressure(e PASP)higher than 60 mm Hg was defined as severe PH in this study.Logistic regression analysis and receiver operating characteristic curve(ROC)analysis were used.Results A total of 186 VSD patients with severe PH(e PASP more than 60 mm Hg)were included in this study,with 158 cases in the non-correctable group and 28 cases in the collectable group.In univariable logistic regression,left atrium dimension(LA),left ventricular end-diastolic dimension(LVDd),left ventricular end-systolic dimension(LVDs),peak velocity of the pulmonary valve(PV),peak velocities from the early phase of the mitral inflow(MVE),bidirectional shunting and pericardial effusion were associated with a correctable shunt.When adjusted in multivariable model,only PV and bidirectional shunting remained statistically significant.The ROC curve found that PV exhibits good discriminative ability for correctable shunt(AUC[area under the curve]:0.779,95%CI:0.676-0.871)with a cut-off value of 1.465 m/s.The predictive performance of bidirectional shunting was not satisfactory,with a low AUC of 0.669(95%CI:0.571-0.766).Conclusions PV and bidirectional shunting are simple and clinically available parameters from echocardiogram in predicting PH severity,which not only avoids the repeated unnecessary cardiac catheterization,but also provides a reference basis for follow-up evaluation.展开更多
Background Pulmonary atresia with ventricular septal defect(PA/VSD)is a rare and complex congenital heart disease(CHD).The optimal palliative surgical strategy for pulmonary atresia with ventricular septal defect(PA/V...Background Pulmonary atresia with ventricular septal defect(PA/VSD)is a rare and complex congenital heart disease(CHD).The optimal palliative surgical strategy for pulmonary atresia with ventricular septal defect(PA/VSD)in neonates and young infants is controversial.Surgery mainly includes the following two options,right ventricle to pulmonary artery connection(RV-PA)and systemic-to-pulmonary artery shunt surgery(SPS).Objectives:To determine the impact of the right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy on promoting the development of pulmonary vasculature in patients with pulmonary atresia and ventricular septal defect(PA/VSD).Methods:From January 2010 to December 2019,104 patients with PA/VSD in Guangdong Cardiovascular Institute who underwent right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy to promoting the development of pulmonary vasculature were identified and enrolled in this retrospective study.The cohort was divided into two groups:group-Right Ventricle to Pulmonary Artery Connection(RV-PA),who underwent initial palliation with staged repair(n=51),and group-Systemic-to-Pulmonary Artery Shunt(SPS)(n=53).Preoperative and postoperative the development of pulmonary vasculature data have been collected and compared.Results:Before the surgery,the Mc Goon Ratio of the RV-PA group significantly high than and the SPS group(P<0.05).After the surgery,in the RV-PA group,the Nakata index,Mc Goon Ratio score significantly increased during the interstage period(P<0.01).At the meantime,the HCT,RBC and HB significantly reduced in the RV-PA group compared with the SPS group(P<0.05).The shunt group performed better in these areas:length of hospital stay,ACC time and CPB time(P<0.05).Conclusion:Compared with systemic-to-pulmonary artery shunt,right ventricle to pulmonary artery connection is more effective to promote the development of the pulmonary vasculature for pulmonary atresia with ventricular septal defect.展开更多
Background Congenital heart disease with severe pulmonary arterial hypertension （SPAH）,previously thought to have irreversible pulmonary vascular disease （PVD）,has been recently successfully corrected using diagno...Background Congenital heart disease with severe pulmonary arterial hypertension （SPAH）,previously thought to have irreversible pulmonary vascular disease （PVD）,has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team.This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect （VSD） and SPAH using diagnostic treatment and repair strategy.Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed.All patients received advanced pulmonary arterial hypertension （PAH） therapy and radical repairs were performed when transcutaneous oxygen saturation （SPO2） increased up to 93％.Results There were no operative deaths.SPO2 and baseline six-minute walk test （SMWT） distance of all selected patients increased significantly and mean pulmonary artery pressure （MPAP） regressed significantly after diagnostic treatment and at late follow-up （P ＜0.01）.The incidence of late postoperative PAH was seen in six （10.7％） patients and by Logistic regression analysis,early postoperative PAH was an independent risk factor related to late postoperative PAH.Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent.Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.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 Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.Howev...Background Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.However,in China,patients with IAA associated with ventricular septal defect （VSD） and patent ductus arteriosus （PDA） over one year of age are common.So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.Methods From January 2009 to December 2012,19 patients with IAA have undergone complete single-stage repair.The patients＇ mean age was 4.4 years,ranging 1 to 15 years; and their mean weight was 12.8 kg,ranging 4.2 to 36.0 kg.Fifteen IAA were type A,four were type B.Preoperative cardiac catheterization data were available from all patients.Mean pulmonary arterial pressure （mPAP） and pulmonary vascular resistance （PVR） were measured.The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case.All patients underwent echocardiographic examinations before discharged from the hospital.In addition,cardiac catheterization and echocardiographic examinations were performed during follow-up.Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients.Mean follow-up was （1.6±0.8） years.Results There were two hospital deaths （2/19,11％）.One patient died of pulmonary hypertension crisis,and another died of postoperative low cardiac output.Five cases had other main postoperative complications but no postoperative neurologic complications.Seventeen survivors were followed up,and there were no late deaths or reoperation.Mean cross-clamp duration was （85±22） minutes and selective brain perfusion duration was （34±11） minutes.Two patients required delayed sternal closure at two days postoperatively.Intensive care unit and hospital stays were （9±8） days and （47±24） days,respectively.Pressure gradients across the anastomosis at most recent follow up were less than 22 mmHg.mPAP regressed significantly from preoperative （62.1±8.1） mmHg to postoperative （37.3±11.3） mmHg （P ＜0.001） and （24.2±6.0） mmHg at six months after discharged from the hospital （P ＜0.001）.The pulmonary vascular resistance also regressed significantly from preoperative （1 501.4±335.7） dyn·s·cm-5 to （485.0±215.1） dyn·s·cm-5 at six months after discharged from the hospital （P ＜0.001）.The majority of the seventeen patients （89％） were in New York Heart Association （NYHA） class Ⅰ,and 11％ remained in NYHA class Ⅱ.Conclusions Single-stage repair of patients with IAA,VSD and PDA over one year of age can have good surgical results and functional outcomes.Assessment and treatment of pulmonary artery pressure pre-operatively and postoperatively was crucial.mPAP and pulmonary vascular resistance may have regress significantly compared to preoperative values.展开更多
目的:探讨密闭式吸痰对室间隔缺损(VSD)伴重度肺动脉高压(PAH)患儿的应用效果及对氧代谢动力学参数的影响。方法:选取2019年5月—2021年6月收治的VSD伴重度PAH患儿107例,依据吸痰方式不同分为常规组(53例)和密闭组(54例)。常规组给予开...目的:探讨密闭式吸痰对室间隔缺损(VSD)伴重度肺动脉高压(PAH)患儿的应用效果及对氧代谢动力学参数的影响。方法:选取2019年5月—2021年6月收治的VSD伴重度PAH患儿107例,依据吸痰方式不同分为常规组(53例)和密闭组(54例)。常规组给予开放式吸痰,密闭组给予密闭式吸痰,对比两组患儿心率(HR)、动脉血压(ABP)、氧代谢动力学参数以及右心导管检查和预后情况。结果:两组患儿吸痰后2 min HR、收缩压、舒张压水平较吸痰前2 min均升高,但密闭组升高幅度低于常规组(P<0.05)。吸痰前后各时间段两组患儿动脉血氧分压(PaO_(2))、动脉血二氧化碳分压(PaCO_(2))、动脉血氧饱和度(SaO_(2))水平比较,差异无统计学意义(P>0.05)。常规组吸痰前2 min至吸痰后15 min PaO_(2)和SaO_(2)水平呈先降低后升高趋势,PaCO_(2)水平呈先升高后降低趋势,吸痰即刻、吸痰后2 min PaO_(2),PaCO_(2),SaO_(2)水平与吸痰前2 min比较,差异有统计学意义(P<0.05);吸痰前2 min与吸痰后15 min PaO_(2),PaCO_(2),SaO_(2)水平比较差异无统计学意义(P>0.05)。密闭组吸痰前2 min至吸痰后15 min PaO_(2)水平呈逐渐升高趋势,PaCO_(2)水平呈先升高后降低趋势,SaO_(2)水平呈先降低后升高趋势,吸痰即刻、吸痰后2 min、吸痰后15 min PaO_(2),PaCO_(2),SaO_(2)水平与吸痰前2 min比较差异无统计学意义(P>0.05)。两组患儿干预1个月末肺动脉收缩压(PASP)、肺动脉收缩压/体循环动脉收缩压(Pp/Ps)水平较吸痰前2 min降低,且密闭组低于常规组(P<0.05)。密闭组呼吸机相关性肺炎发生率1.85%(1/54),低于常规组的9.43%(5/53);密闭组低氧血症发生率1.85%(1/54),低于常规组的5.66%(3/53),但差异无统计学意义(P>0.05)。两组患儿痰液喷出发生率比较,差异有统计学意义(P<0.05)。结论:密闭式吸痰可调节VSD伴重度PAH患儿HR和ABP水平,维持氧代谢动力学参数稳定,调节右心导管检查水平,安全性较高,可避免痰液喷出,提高临床疗效。展开更多
文摘We performed a retrospective analysis of patients with and without 22q11 deletion undergoing surgery for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals between January 2004 and August 2009 at our institutions. Information was collected on collateral origin, arch sidedness, presence of central pulmonary arteries, and presence of an aberrant subclavian vessel. While patients with 22q11 deletion were more likely to have collateral origin from brachiocephalic vessels, patients without 22q11 deletion were more likely to have collateral origin from the descending aorta. There was no significant difference in arch sidedness or the presence of central pulmonary arteries. Patients with 22q11 deletion were more likely to have an aberrant subclavian artery (15/46 vs 5/54, p < 0.05), whether a left or right arch was present. Nine of the fifteen 22q11 deletion patients had a collateral originating from an aberrant subclavian artery. In time, genomic and embryologic research may help determine the exact mechanisms by which 22q11 deletion contributes to the development of congenital heart disease such as pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
基金Wuhan Health and Family Planning Commission Grant/Award(WX16D18).
文摘Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septaldefect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the sameage (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE.The measurements included: left and right ventricular volume indexed to body surface area (BSA), stroke volume(SV) indexed to BSA, and ejection fraction (EF). Results: The results showed left and right ventricular volumeindexed to BSA and SV indexed to BSA were significantly increased in VSD + PH Group (VSD + PH Groupvs. Control Group), LVEDV/BSA (ml/m2): 48.67 ± 21.46 vs. 25.59 ± 6.96, RVEDV/BSA (ml/m2): 55.98 ±15.35 vs. 27.69 ± 4.37, LVSV/BSA (ml/m2): 24.08 ± 9.30 vs. 15.14 ± 4.29, RVSV/BSA (ml/m2): 26.02 ± 8.87 vs.14.11 ± 2.89, (P < 0.05). While for EF in VSD + PH Group decreased (VSD + PH Group vs. Control Group),LVEF: 50.93 ± 7.50% vs. 59.38 ± 7.24%, RVEF: 45.84 ± 7.71% vs. 51.05 ± 6.90% (P < 0.05). Conclusion: Inchildren with VSD and moderate to severe PH, increased biventricular volume and decreased systolic functionwere observed with RT-3DE, but biventricular systolic function remained within acceptable limits. The childrenin this study recovered well after surgery without serious perioperative complications, suggesting that biventricularsystolic function may help facilitate the surgical decision-making process in children with VSD and moderate-toseverePH.
文摘Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and pulmonary arterial hypertension(VSD-PAH).Background:Children with congenital heart disease(CHD)with pulmonary arterial hypertension(PAH)have more complications after surgery than those without PAH.Discharge planning is an effective strategy to help children leave the hospital safely,and receive appropriate care after discharge.Methods:A quasi-experimental design was used.Sixty children and their mothers were recruited and divided into two groups:the control group received conventional care,the intervention group received both conventional care and additional discharge planning care.Results:(1)After admission,maternal caring knowledge between the two groups was similar.(2)At discharge,maternal discharge readiness,maternal caring knowledge and maternal caring behavior in the intervention group was significantly higher compared to the control group(t=3.35,p=0.001;F=84.74,p<0.001;F=23.82,p<0.001).This difference persisted after discharge,and was evident at one month and three months after discharge.(3)However,no significant difference in the readmission rate of children after discharge was evident between the two groups.Conclusions:Discharge planning improves the maternal discharge readiness,maternal caring knowledge and maternal caring behaviors.However,this planning did not reduce the readmission rate of children with CHD-PAH.
文摘Speckle tracking imaging （STI） was employed to investigate the effect of right ventricular （RV） volume and pressure overload on left ventricular （LV） rotation and twist in 35 patients with atrial septal defect （ASD）, 18 of which with pulmonary hypertension, and 21 healthy subjects serving as controls. The peak rotations of 6 segments at the basal and apical short-axises and the average peak rotation and interval time of the 6 segments in the opposite direction during early systolic phase were measured respectively. LV twist versus time profile was drawn and the peak twist and time to peak twist were calculated. LV ejection fraction （EF） was measured by Biplane Simpson. Compared to ASD patients without pulmonary hypertension and healthy subjects, the peak rotations of posterior, inferior and postsept walls at the basal level were lower （P〈0.05）, and the average counterclockwise peak rotation of 6 segments at the basal level during early systolic phase was higher （P〈0.05）, and the average interval time was delayed （P〈0.05）. LV peak twist was also lower （P〈0.05）, and had a significant negative correlation with pulmonary arterial systolic pressure （r=-0.57, P=0.001）. No significant differences were found in LVEF among the three groups. It was suggested that although RV volume overload due to ASD has no significant effects on LV rotation and twist, LV peak twist is lower in ASD patients with pulmonary hypertension. Thus LV twist may serve as a new indicator of the presence of pulmonary hypertension in ASD patients.
文摘Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH). Methods The Streptavidin-peroxidase (SP) immunoassay was used to measure the expression of ET-1 in pulmonary arteriola, pulmonary veinlet, endocardium and endangium of vasa coronary of 20 cases VSD of CHD with PH, and contrast the expression level of these 20 cases VSD of CHD with PH. Results The expression of ET-1 PH patients in pulmonary arteriola, endocardium, and endangium of vasa coronary was much higher than that of the control group (P<0.05), but there was no significance variance in expression level of pulmonary veinlet between two groups (P>0.05). Conclusion If VSD of CHD was accompany with PH, the degree of PH has a positive correlation with the amount of ET-1 in pulmonary arteriola, ET-1 may be the cause of dynamic PH, and also the acceleration factor of the PH. However, the amount of ET-1 in endocardium and endangium of vasa coronary, may have significant connection with the myocardium hypertrophy in dynamic PH.
文摘Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.
基金supported by the Guangdong Peak Project(No.DFJH201802)the National Natural Science Foundation of China(No.82371963)+1 种基金Guangdong Basic and Applied Basic Research Foundation(No.2021A1515012232/No.2023A1515011366)the Natural Science Foundation lauching fund of the Guangdong Provincial People's Hospital(No.8197070830/No.8207070477/No.8227070211)。
文摘Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is needed.Therefore,this study aimed to investigate the application of echocardiogram in predicting the severity of pulmonary hypertension and guide subsequent management.Methods This study included VSD patients who underwent right heart catheterization(RHC)examination in Guangdong Provincial People's Hospital from January2011 to December 2022.An estimated pulmonary artery systolic pressure(e PASP)higher than 60 mm Hg was defined as severe PH in this study.Logistic regression analysis and receiver operating characteristic curve(ROC)analysis were used.Results A total of 186 VSD patients with severe PH(e PASP more than 60 mm Hg)were included in this study,with 158 cases in the non-correctable group and 28 cases in the collectable group.In univariable logistic regression,left atrium dimension(LA),left ventricular end-diastolic dimension(LVDd),left ventricular end-systolic dimension(LVDs),peak velocity of the pulmonary valve(PV),peak velocities from the early phase of the mitral inflow(MVE),bidirectional shunting and pericardial effusion were associated with a correctable shunt.When adjusted in multivariable model,only PV and bidirectional shunting remained statistically significant.The ROC curve found that PV exhibits good discriminative ability for correctable shunt(AUC[area under the curve]:0.779,95%CI:0.676-0.871)with a cut-off value of 1.465 m/s.The predictive performance of bidirectional shunting was not satisfactory,with a low AUC of 0.669(95%CI:0.571-0.766).Conclusions PV and bidirectional shunting are simple and clinically available parameters from echocardiogram in predicting PH severity,which not only avoids the repeated unnecessary cardiac catheterization,but also provides a reference basis for follow-up evaluation.
基金funding by Science and Technology Projects in Guangzhou,China(No.202206010049)Guangdong Provincial Clinical Research Center for Cardiovascular disease(No.2020B1111170011)。
文摘Background Pulmonary atresia with ventricular septal defect(PA/VSD)is a rare and complex congenital heart disease(CHD).The optimal palliative surgical strategy for pulmonary atresia with ventricular septal defect(PA/VSD)in neonates and young infants is controversial.Surgery mainly includes the following two options,right ventricle to pulmonary artery connection(RV-PA)and systemic-to-pulmonary artery shunt surgery(SPS).Objectives:To determine the impact of the right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy on promoting the development of pulmonary vasculature in patients with pulmonary atresia and ventricular septal defect(PA/VSD).Methods:From January 2010 to December 2019,104 patients with PA/VSD in Guangdong Cardiovascular Institute who underwent right ventricle to pulmonary artery connection or systemic-to-pulmonary artery shunt surgery as the initially palliated surgical strategy to promoting the development of pulmonary vasculature were identified and enrolled in this retrospective study.The cohort was divided into two groups:group-Right Ventricle to Pulmonary Artery Connection(RV-PA),who underwent initial palliation with staged repair(n=51),and group-Systemic-to-Pulmonary Artery Shunt(SPS)(n=53).Preoperative and postoperative the development of pulmonary vasculature data have been collected and compared.Results:Before the surgery,the Mc Goon Ratio of the RV-PA group significantly high than and the SPS group(P<0.05).After the surgery,in the RV-PA group,the Nakata index,Mc Goon Ratio score significantly increased during the interstage period(P<0.01).At the meantime,the HCT,RBC and HB significantly reduced in the RV-PA group compared with the SPS group(P<0.05).The shunt group performed better in these areas:length of hospital stay,ACC time and CPB time(P<0.05).Conclusion:Compared with systemic-to-pulmonary artery shunt,right ventricle to pulmonary artery connection is more effective to promote the development of the pulmonary vasculature for pulmonary atresia with ventricular septal defect.
文摘Background Congenital heart disease with severe pulmonary arterial hypertension （SPAH）,previously thought to have irreversible pulmonary vascular disease （PVD）,has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team.This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect （VSD） and SPAH using diagnostic treatment and repair strategy.Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed.All patients received advanced pulmonary arterial hypertension （PAH） therapy and radical repairs were performed when transcutaneous oxygen saturation （SPO2） increased up to 93％.Results There were no operative deaths.SPO2 and baseline six-minute walk test （SMWT） distance of all selected patients increased significantly and mean pulmonary artery pressure （MPAP） regressed significantly after diagnostic treatment and at late follow-up （P ＜0.01）.The incidence of late postoperative PAH was seen in six （10.7％） patients and by Logistic regression analysis,early postoperative PAH was an independent risk factor related to late postoperative PAH.Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent.Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.
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This study was supported by Capital Citizens＇ Health Project Cultivation of Beijing Municipal Science and Technology Commissions （No. Z 11110007491100） and Beijing Municipal Natural Science Foundation （No. 7112046）.
文摘Background Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.However,in China,patients with IAA associated with ventricular septal defect （VSD） and patent ductus arteriosus （PDA） over one year of age are common.So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.Methods From January 2009 to December 2012,19 patients with IAA have undergone complete single-stage repair.The patients＇ mean age was 4.4 years,ranging 1 to 15 years; and their mean weight was 12.8 kg,ranging 4.2 to 36.0 kg.Fifteen IAA were type A,four were type B.Preoperative cardiac catheterization data were available from all patients.Mean pulmonary arterial pressure （mPAP） and pulmonary vascular resistance （PVR） were measured.The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case.All patients underwent echocardiographic examinations before discharged from the hospital.In addition,cardiac catheterization and echocardiographic examinations were performed during follow-up.Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients.Mean follow-up was （1.6±0.8） years.Results There were two hospital deaths （2/19,11％）.One patient died of pulmonary hypertension crisis,and another died of postoperative low cardiac output.Five cases had other main postoperative complications but no postoperative neurologic complications.Seventeen survivors were followed up,and there were no late deaths or reoperation.Mean cross-clamp duration was （85±22） minutes and selective brain perfusion duration was （34±11） minutes.Two patients required delayed sternal closure at two days postoperatively.Intensive care unit and hospital stays were （9±8） days and （47±24） days,respectively.Pressure gradients across the anastomosis at most recent follow up were less than 22 mmHg.mPAP regressed significantly from preoperative （62.1±8.1） mmHg to postoperative （37.3±11.3） mmHg （P ＜0.001） and （24.2±6.0） mmHg at six months after discharged from the hospital （P ＜0.001）.The pulmonary vascular resistance also regressed significantly from preoperative （1 501.4±335.7） dyn·s·cm-5 to （485.0±215.1） dyn·s·cm-5 at six months after discharged from the hospital （P ＜0.001）.The majority of the seventeen patients （89％） were in New York Heart Association （NYHA） class Ⅰ,and 11％ remained in NYHA class Ⅱ.Conclusions Single-stage repair of patients with IAA,VSD and PDA over one year of age can have good surgical results and functional outcomes.Assessment and treatment of pulmonary artery pressure pre-operatively and postoperatively was crucial.mPAP and pulmonary vascular resistance may have regress significantly compared to preoperative values.
文摘目的:探讨密闭式吸痰对室间隔缺损(VSD)伴重度肺动脉高压(PAH)患儿的应用效果及对氧代谢动力学参数的影响。方法:选取2019年5月—2021年6月收治的VSD伴重度PAH患儿107例,依据吸痰方式不同分为常规组(53例)和密闭组(54例)。常规组给予开放式吸痰,密闭组给予密闭式吸痰,对比两组患儿心率(HR)、动脉血压(ABP)、氧代谢动力学参数以及右心导管检查和预后情况。结果:两组患儿吸痰后2 min HR、收缩压、舒张压水平较吸痰前2 min均升高,但密闭组升高幅度低于常规组(P<0.05)。吸痰前后各时间段两组患儿动脉血氧分压(PaO_(2))、动脉血二氧化碳分压(PaCO_(2))、动脉血氧饱和度(SaO_(2))水平比较,差异无统计学意义(P>0.05)。常规组吸痰前2 min至吸痰后15 min PaO_(2)和SaO_(2)水平呈先降低后升高趋势,PaCO_(2)水平呈先升高后降低趋势,吸痰即刻、吸痰后2 min PaO_(2),PaCO_(2),SaO_(2)水平与吸痰前2 min比较,差异有统计学意义(P<0.05);吸痰前2 min与吸痰后15 min PaO_(2),PaCO_(2),SaO_(2)水平比较差异无统计学意义(P>0.05)。密闭组吸痰前2 min至吸痰后15 min PaO_(2)水平呈逐渐升高趋势,PaCO_(2)水平呈先升高后降低趋势,SaO_(2)水平呈先降低后升高趋势,吸痰即刻、吸痰后2 min、吸痰后15 min PaO_(2),PaCO_(2),SaO_(2)水平与吸痰前2 min比较差异无统计学意义(P>0.05)。两组患儿干预1个月末肺动脉收缩压(PASP)、肺动脉收缩压/体循环动脉收缩压(Pp/Ps)水平较吸痰前2 min降低,且密闭组低于常规组(P<0.05)。密闭组呼吸机相关性肺炎发生率1.85%(1/54),低于常规组的9.43%(5/53);密闭组低氧血症发生率1.85%(1/54),低于常规组的5.66%(3/53),但差异无统计学意义(P>0.05)。两组患儿痰液喷出发生率比较,差异有统计学意义(P<0.05)。结论:密闭式吸痰可调节VSD伴重度PAH患儿HR和ABP水平,维持氧代谢动力学参数稳定,调节右心导管检查水平,安全性较高,可避免痰液喷出,提高临床疗效。
