Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliativ...Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.展开更多
Background:Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries(MAPCAs)and outcomes of selective unifocalization of pulmonary atresia with ventricular s...Background:Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries(MAPCAs)and outcomes of selective unifocalization of pulmonary atresia with ventricular septal defects and MAPCAs.Methods:This is a case-series study.Thirteen patients were included.Angiographybased assessment was conducted to determine whether collateral arteries should be unifocalized or treated with intraoperative ligature.Specimens were collected and stained by HE and ET+VG.Results:Twelve patients underwent one-stage unifocalization at a median age of 37 months(range:6–228 months)and a median weight of 14.0 kg(range:5.0–49.0 kg),which produced a favorable right ventricle to aortic systolic pressure ratio of no more than 0.5 except in one patient who died.Patients were divided into three groups:Group 1(n=6),had no native pulmonary arteries,and collateral arteries supplied all pulmonary blood;Group 2(n=6)presented dysplastic native pulmonary arteries on one or both sides,and in some lung lobes or segments,blood was supplied only by collateral arteries;Group 3(n=1)had well-developed left and right pulmonary arteries,and collateral arteries,and pulmonary arteries provided blood flow to the same segments.Pathological reports demonstrated two types of collateral arteries:Elastic arteries presented an arborization distribution similar to native pulmonary artery walls,while muscular arteries showed high resistance and distortion.We selectively unifocalized single-supply collateral arteries with morphologic features based on the arborization distribution.Conclusions:We found that there were two kinds of MAPCAs with different histology,and we performed selective UF for MAPCAs that might belong to the elastic artery.Selective unifocalization achieved a low right ventricle to aortic systolic pressure ratio and favorable surgical effects.展开更多
Introduction:Transposition of the great arteries(TGA)with aortopulmonary window is a rare type of congenital heart disease with limited experience.We reported a neonate aged 25 days receiving the arterial switch opera...Introduction:Transposition of the great arteries(TGA)with aortopulmonary window is a rare type of congenital heart disease with limited experience.We reported a neonate aged 25 days receiving the arterial switch operation and assisted with extracorporeal membrane oxygenation.Conclusion:TGA with aortopulmonary window can be safely correctly with the arterial switch operation.展开更多
Aortopuhnonary window is a rare congenital cardiac malformation, which is previously repaired using cardiopulmonary bypass. However, some cases were reported to repair the aortopulmonary septal defect by transcathetcr...Aortopuhnonary window is a rare congenital cardiac malformation, which is previously repaired using cardiopulmonary bypass. However, some cases were reported to repair the aortopulmonary septal defect by transcathetcr closure. Here we present a 30 year-old woman with a 7-mm aortopulmonary window, who was successfully treated using a modified double-disk PmVSD occluder展开更多
Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels that arise from the aorta or its first-order branches and are distally connected to the pulmonary arterial vasculature, thereby providing pulmon...Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels that arise from the aorta or its first-order branches and are distally connected to the pulmonary arterial vasculature, thereby providing pulmonary blood flow. MAPCAs are often associated with cyanotic congenital heart disease with decreased pulmonary blood flow. Isolated MAPCAs are rare in patients without congenital heart disease with structurally normal hearts. Sometimes, isolated congenital MAPCAs can occur without any lung disease. Isolated MAPCAs represent the occurrence of collaterals in the absence of underlying heart disease, which commonly presents as heart failure, recurrent respiratory tract infection, and pulmonary artery hypertension. We report a rare case of congestive heart failure in a 6-year-old patient with dual arterial supply to an otherwise normal right lung, with a normal bronchial tree, and a structurally normal heart. The patient was successfully managed by the closure of collaterals by Amplatzer vascular plugs II (AVPII) and Amplatzer Piccolo Occluders.展开更多
A 10-year-old boy who had previously undergone surgical correction for tetralogy of Fallot was referred for cardiac catheterization because of recurrent symptomatic postoperative heart failure owing to major aortopulm...A 10-year-old boy who had previously undergone surgical correction for tetralogy of Fallot was referred for cardiac catheterization because of recurrent symptomatic postoperative heart failure owing to major aortopulmonary collateral arteries （MAPCAs）. A successful occlusion of these MAPCAs was achieved percutaneously using the Amplatzer vascular plug and a detachable coil. Transcatheter occlusion of MAPCAs after surgical correction is an effective method for treatment of post-operative heart failure.展开更多
Background Pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs） remains a challenging complex congenital heart disease nowadays. In the present study, we ...Background Pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs） remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs. Methods Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 （17.9±1.6）. There were a total of 43 MAPCAs in all the patients （3-5 （3.9~0.7） MAPCAs per patient）. The accumulated Nakata index was （222.9＋_29.9） mm2/m2 （ranged from 182 to 272）. All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline sternotomy. Results All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95%-100%, which was significantly higher than pre-operation （P 〈0.01）. During the follow-up period of 3-51 （25.4±15.2） months, there were no late death and no need for reintervention. All the patients enjoyed their lives with good conditions. Conclusions This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative surgical approach in the treatment of PA-VSD and MAPCAs.展开更多
Fetal aortopulmonary septal defect （APSD） is an extremely rare condition, accounting for 0.1%-0.2% of all cardiac defects in live births world wide. Hospital mortality is 13% and 33% for simple and complex APSD,resp...Fetal aortopulmonary septal defect （APSD） is an extremely rare condition, accounting for 0.1%-0.2% of all cardiac defects in live births world wide. Hospital mortality is 13% and 33% for simple and complex APSD,respectively. This rare cardiac defect refers to a congenital malformation in the development of the arteriosus truncus septum, and is usually associated with a wide variety of other structural cardiac anomalies such as ventricular septal defect （VSD）, pulmonary valve stegnosis and so on. Prenatal diagnosis of an APSD is possible by echocardiography. In relevant literature, only two cases, which were diagnosed initially by prenatal echocardiography, have been reported.展开更多
Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)c...Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.展开更多
Background:This study aims to explore the efficacy of selective unifocalization(UF)for major aortopulmonary collateral arteries(MAPCAs)unifocalization in children with pulmonary atresia with ventricular septal defect(...Background:This study aims to explore the efficacy of selective unifocalization(UF)for major aortopulmonary collateral arteries(MAPCAs)unifocalization in children with pulmonary atresia with ventricular septal defect(PA/VSD).Methods:A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December 2019.Sex,age,preoperative cardiovascular angiography test results and McGoon ratio were collected.The properties of the collateral arteries were evaluated by angiography,and selective UF for the“dendritic”MAPCAs and ligation of MAPCAs demonstrating distortion and resistance.Results:A total of 13 severe patients underwent one-stage repair,of which 1 case underwent ventricular septal fenestration and died after 2 weeks of ECMO support.The median age was 37 months,and a median weight was 14.0 kg.A right ventricular to aortic systolic pressure ratio(pRV/pAo)of no more than 0.5 was achieved in 12 living patients.Conclusion:Selective unifocalization based on MAPCAs morphology can achieved a good outcome at the early stage.This surgical concept might be provided a novel insight into treatment for some of the subgroups presenting with this complex form of PA/VSD/MAPCAs.展开更多
Aim: To present a rare occurrence of aortopulmonary fistula due to rupture of proximal aortic dissection in a 48-year-old woman. Introduction: Aortic dissection is defined as disruption of the medial layer provoked by...Aim: To present a rare occurrence of aortopulmonary fistula due to rupture of proximal aortic dissection in a 48-year-old woman. Introduction: Aortic dissection is defined as disruption of the medial layer provoked by intramural bleeding, resulting in separation of aortic wall layers and subsequent formation of a true lumen and a false lumen with or without communication. Case Report: A 48-year-old female presented with vague anterior chest discomfort, high blood pressure, systolic-diastolic murmur in the left sternal border with ECG changes of myocardial ischemia. Echocardiography revealed a dilated aortic root with intimal flaps, a leak into the pulmonary artery and regional hypokinesis with contractile dysfunction suggesting a proximal aortic dissection with rupture into the main pulmonary artery. Discussion: The etiology of aortic dissection was mostly hypertension in 80% of cases and aortopathies such as connective tissue disorders, inflammatory and idiopathic. Aortic wall stress is a major trigger of intimal tear and two-dimensional transthoracic echocardiography is an excellent, initial diagnostic gold standard to detect the dissecting flaps, especially in proximal aortic dissection. Conclusion: Blood pressure control is the mainstay of treatment and urgent surgery is indicated in proximal aortic dissection since there is higher chance of rupture with an increase in mortality.展开更多
Background： Outflow tract （OFT） septation defects are a common cause of congenital heart disease. Numerous studies have focused on the septation mechanism of the OFT, but have reported inconsistent conclusions. Thi...Background： Outflow tract （OFT） septation defects are a common cause of congenital heart disease. Numerous studies have focused on the septation mechanism of the OFT, but have reported inconsistent conclusions. This study, therefore, aimed to investigate the septation of the aortic sac and the OFT in the early embryonic human heart. Methods： Serial sections of 27 human embryonic hearts from Carnegie stage （CS） 10 to CS19 were immunohistochemically stained with antibodies against α-smooth muscle actin （α-SMA） and myosin heavy chain. Results： At CS10-CS11, the OFT wail was an exclusively myocardial structure that was continuous with the aortic sac at the margin of the pericardial cavity. From CS13 onward, the OFT was divided into nonmyocardial and myocardial portions. The cushion formed gradually, and its distal border with the OFT myocardium was consistently maintained. The aortic sac between the fourth and sixth aortic arch arteries was degenerated. At CS16, the α-SMA-positive aortopulmonary septum formed and fused with the two OFT cushions, thus septating the nonmyocardial portion of the OFT into two arteries. At this stage, the cushions were not fused. At CS19, the bilateral cushions were fused to septate the myocardial portion of the OFT. Conclusions： Data suggest that the OFT cushion is formed before the aortopulmonary septum is formed. Thus, the OFT cushion is not derived from the aortopuhnonary septum. In addition, the nonmyocardial part of the OFT is septated into the aorta and pulmonary trunk by the aortopulmonary septum, while the main part of the cushion fuses and septates the myocardial portion of the OFT.展开更多
基金supported by Medical Scientific Research Foundation of Guangdong Province,China (No.A2018038)
文摘Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.
基金supported by the Central Public-interest Scientific Institution Basal Research Fund(2019XK320050)Central University Basic Research Fund(APL20100410010302004).
文摘Background:Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries(MAPCAs)and outcomes of selective unifocalization of pulmonary atresia with ventricular septal defects and MAPCAs.Methods:This is a case-series study.Thirteen patients were included.Angiographybased assessment was conducted to determine whether collateral arteries should be unifocalized or treated with intraoperative ligature.Specimens were collected and stained by HE and ET+VG.Results:Twelve patients underwent one-stage unifocalization at a median age of 37 months(range:6–228 months)and a median weight of 14.0 kg(range:5.0–49.0 kg),which produced a favorable right ventricle to aortic systolic pressure ratio of no more than 0.5 except in one patient who died.Patients were divided into three groups:Group 1(n=6),had no native pulmonary arteries,and collateral arteries supplied all pulmonary blood;Group 2(n=6)presented dysplastic native pulmonary arteries on one or both sides,and in some lung lobes or segments,blood was supplied only by collateral arteries;Group 3(n=1)had well-developed left and right pulmonary arteries,and collateral arteries,and pulmonary arteries provided blood flow to the same segments.Pathological reports demonstrated two types of collateral arteries:Elastic arteries presented an arborization distribution similar to native pulmonary artery walls,while muscular arteries showed high resistance and distortion.We selectively unifocalized single-supply collateral arteries with morphologic features based on the arborization distribution.Conclusions:We found that there were two kinds of MAPCAs with different histology,and we performed selective UF for MAPCAs that might belong to the elastic artery.Selective unifocalization achieved a low right ventricle to aortic systolic pressure ratio and favorable surgical effects.
文摘Introduction:Transposition of the great arteries(TGA)with aortopulmonary window is a rare type of congenital heart disease with limited experience.We reported a neonate aged 25 days receiving the arterial switch operation and assisted with extracorporeal membrane oxygenation.Conclusion:TGA with aortopulmonary window can be safely correctly with the arterial switch operation.
文摘Aortopuhnonary window is a rare congenital cardiac malformation, which is previously repaired using cardiopulmonary bypass. However, some cases were reported to repair the aortopulmonary septal defect by transcathetcr closure. Here we present a 30 year-old woman with a 7-mm aortopulmonary window, who was successfully treated using a modified double-disk PmVSD occluder
文摘Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels that arise from the aorta or its first-order branches and are distally connected to the pulmonary arterial vasculature, thereby providing pulmonary blood flow. MAPCAs are often associated with cyanotic congenital heart disease with decreased pulmonary blood flow. Isolated MAPCAs are rare in patients without congenital heart disease with structurally normal hearts. Sometimes, isolated congenital MAPCAs can occur without any lung disease. Isolated MAPCAs represent the occurrence of collaterals in the absence of underlying heart disease, which commonly presents as heart failure, recurrent respiratory tract infection, and pulmonary artery hypertension. We report a rare case of congestive heart failure in a 6-year-old patient with dual arterial supply to an otherwise normal right lung, with a normal bronchial tree, and a structurally normal heart. The patient was successfully managed by the closure of collaterals by Amplatzer vascular plugs II (AVPII) and Amplatzer Piccolo Occluders.
文摘A 10-year-old boy who had previously undergone surgical correction for tetralogy of Fallot was referred for cardiac catheterization because of recurrent symptomatic postoperative heart failure owing to major aortopulmonary collateral arteries （MAPCAs）. A successful occlusion of these MAPCAs was achieved percutaneously using the Amplatzer vascular plug and a detachable coil. Transcatheter occlusion of MAPCAs after surgical correction is an effective method for treatment of post-operative heart failure.
文摘Background Pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs） remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs. Methods Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 （17.9±1.6）. There were a total of 43 MAPCAs in all the patients （3-5 （3.9~0.7） MAPCAs per patient）. The accumulated Nakata index was （222.9＋_29.9） mm2/m2 （ranged from 182 to 272）. All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline sternotomy. Results All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95%-100%, which was significantly higher than pre-operation （P 〈0.01）. During the follow-up period of 3-51 （25.4±15.2） months, there were no late death and no need for reintervention. All the patients enjoyed their lives with good conditions. Conclusions This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative surgical approach in the treatment of PA-VSD and MAPCAs.
文摘Fetal aortopulmonary septal defect （APSD） is an extremely rare condition, accounting for 0.1%-0.2% of all cardiac defects in live births world wide. Hospital mortality is 13% and 33% for simple and complex APSD,respectively. This rare cardiac defect refers to a congenital malformation in the development of the arteriosus truncus septum, and is usually associated with a wide variety of other structural cardiac anomalies such as ventricular septal defect （VSD）, pulmonary valve stegnosis and so on. Prenatal diagnosis of an APSD is possible by echocardiography. In relevant literature, only two cases, which were diagnosed initially by prenatal echocardiography, have been reported.
基金The report was conducted in accordance with the Nuremberg Code and Declaration of Helsinki,and the protocol was approved by the Institutional Review Board of National Cardiovascular Center Harapan Kita(No.LB.02.01/VII/037/KEP037/2022).
文摘Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.
基金This study was supported by the Clinical and Translational Medicine Research Fund(2019XK320050)of the Basic Research Service Fund of the Central Public Welfare Research Institute of the Chinese Academy of Medical Sciences.
文摘Background:This study aims to explore the efficacy of selective unifocalization(UF)for major aortopulmonary collateral arteries(MAPCAs)unifocalization in children with pulmonary atresia with ventricular septal defect(PA/VSD).Methods:A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December 2019.Sex,age,preoperative cardiovascular angiography test results and McGoon ratio were collected.The properties of the collateral arteries were evaluated by angiography,and selective UF for the“dendritic”MAPCAs and ligation of MAPCAs demonstrating distortion and resistance.Results:A total of 13 severe patients underwent one-stage repair,of which 1 case underwent ventricular septal fenestration and died after 2 weeks of ECMO support.The median age was 37 months,and a median weight was 14.0 kg.A right ventricular to aortic systolic pressure ratio(pRV/pAo)of no more than 0.5 was achieved in 12 living patients.Conclusion:Selective unifocalization based on MAPCAs morphology can achieved a good outcome at the early stage.This surgical concept might be provided a novel insight into treatment for some of the subgroups presenting with this complex form of PA/VSD/MAPCAs.
文摘Aim: To present a rare occurrence of aortopulmonary fistula due to rupture of proximal aortic dissection in a 48-year-old woman. Introduction: Aortic dissection is defined as disruption of the medial layer provoked by intramural bleeding, resulting in separation of aortic wall layers and subsequent formation of a true lumen and a false lumen with or without communication. Case Report: A 48-year-old female presented with vague anterior chest discomfort, high blood pressure, systolic-diastolic murmur in the left sternal border with ECG changes of myocardial ischemia. Echocardiography revealed a dilated aortic root with intimal flaps, a leak into the pulmonary artery and regional hypokinesis with contractile dysfunction suggesting a proximal aortic dissection with rupture into the main pulmonary artery. Discussion: The etiology of aortic dissection was mostly hypertension in 80% of cases and aortopathies such as connective tissue disorders, inflammatory and idiopathic. Aortic wall stress is a major trigger of intimal tear and two-dimensional transthoracic echocardiography is an excellent, initial diagnostic gold standard to detect the dissecting flaps, especially in proximal aortic dissection. Conclusion: Blood pressure control is the mainstay of treatment and urgent surgery is indicated in proximal aortic dissection since there is higher chance of rupture with an increase in mortality.
文摘Background： Outflow tract （OFT） septation defects are a common cause of congenital heart disease. Numerous studies have focused on the septation mechanism of the OFT, but have reported inconsistent conclusions. This study, therefore, aimed to investigate the septation of the aortic sac and the OFT in the early embryonic human heart. Methods： Serial sections of 27 human embryonic hearts from Carnegie stage （CS） 10 to CS19 were immunohistochemically stained with antibodies against α-smooth muscle actin （α-SMA） and myosin heavy chain. Results： At CS10-CS11, the OFT wail was an exclusively myocardial structure that was continuous with the aortic sac at the margin of the pericardial cavity. From CS13 onward, the OFT was divided into nonmyocardial and myocardial portions. The cushion formed gradually, and its distal border with the OFT myocardium was consistently maintained. The aortic sac between the fourth and sixth aortic arch arteries was degenerated. At CS16, the α-SMA-positive aortopulmonary septum formed and fused with the two OFT cushions, thus septating the nonmyocardial portion of the OFT into two arteries. At this stage, the cushions were not fused. At CS19, the bilateral cushions were fused to septate the myocardial portion of the OFT. Conclusions： Data suggest that the OFT cushion is formed before the aortopulmonary septum is formed. Thus, the OFT cushion is not derived from the aortopuhnonary septum. In addition, the nonmyocardial part of the OFT is septated into the aorta and pulmonary trunk by the aortopulmonary septum, while the main part of the cushion fuses and septates the myocardial portion of the OFT.
