Objective To evaluate effectiveness of urgent surgical correction for infra cardiac total anomalous pulmonary venous drainage (TAPVD) in infants and children. Methods From July 2000 to April 2009,seventeen patients wi...Objective To evaluate effectiveness of urgent surgical correction for infra cardiac total anomalous pulmonary venous drainage (TAPVD) in infants and children. Methods From July 2000 to April 2009,seventeen patients with infra-cardiac type of total anomalous pulmonary venous connection received surgical correction展开更多
To investigate the value of echocardiography in the diagnosis of total anomalous pulmonary venous connection ( TAPVC ), 16 patients in our hospital were diagnosed to have TAPVC by echocardiography from year 1994 to 2...To investigate the value of echocardiography in the diagnosis of total anomalous pulmonary venous connection ( TAPVC ), 16 patients in our hospital were diagnosed to have TAPVC by echocardiography from year 1994 to 2001. In 11 cases the results of echocardiography were compared to those of surgery. Each patient was examined by using a combination of precordial, suprasternal and subcostal windows to visualize all the pulmonary veins and their drainage sites, common pulmonary venous trunk, and other associated abnormalities. Of the 16 cases, the drainage sites were as follow: supracardiac in 10, via vertical vein in 9, directly to superior vena cava in 1; cardiac in 5, via coronary sinus in 2, directly to right atrium in 3. Diagnoses were correctly made in all the 11 cases as confirmed by surgery. Echocardiography can also assess pulmonary arterial pressure and detect other associated abnormalities. It is concluded that echocardiography is the preferred examination method in the diagnosis of TAPVC before surgery. With careful examination using multiple windows and sections, TAPVC can be accurately diagnosed by echocardiography.展开更多
BACKGROUND Alagille syndrome(ALGS)is an autosomal dominant genetic disorder caused by mutations in the JAG1 or NOTCH2 gene.It is characterized by decreased intrahepatic bile ducts associated with a variety of abnormal...BACKGROUND Alagille syndrome(ALGS)is an autosomal dominant genetic disorder caused by mutations in the JAG1 or NOTCH2 gene.It is characterized by decreased intrahepatic bile ducts associated with a variety of abnormalities in many other organ systems,such as the cardiovascular,skeletal,and urinary systems.CASE SUMMARY We report a rare case of ALGS.A 1-month-old male infant presented with sustained jaundice and had a rare congenital heart disease:Total anomalous pulmonary venous connection(TAPVC).Sustained jaundice,particularly with cardiac murmur,caught our attention.Laboratory tests revealed elevated levels of alanine aminotransferase,aspartate aminotransferase,gamma-glutamyl transpeptidase,total bilirubin,and total bile acids,indicating serious intrahepatic cholestasis.Imaging confirmed the presence of butterfly vertebra at the seventh thoracic vertebra.This suggested ALGS,which was confirmed by genetic testing with a c.3197dupC mutation in the JAG1 gene.Ursodiol was administered immediately after confirmation of the diagnosis,and cardiac surgery was performed when the patient was 1.5 month old.He recovered well after treatment and was discharged at the age of 3 mo.At the age of two years,the patient returned to our clinic because multiple cutaneous nodules with xanthomas appeared,and their size and number increased over time.CONCLUSION We report a unique case of ALGS associated with TAPVC and severe xanthomas.This study has enriched the clinical manifestations of ALGS and emphasized the association between JAG1 gene and TAPVC.展开更多
Introduction: To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC). Case Report: A 16-year-old girl was diagnosed with features ...Introduction: To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC). Case Report: A 16-year-old girl was diagnosed with features of an atrial septal defect (ASD) by transthoracic echocardiography and the absence of PLSVC (persistent left superior vena cava) connection to coronary sinus was confirmed by saline contrast echocardiography (“bubble study”). Discussion: The cause of the dilated coronary sinus was due to total anomalous pulmonary venous connection. Conclusion: Coronary sinus atrial septal defect (ASD) is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava (PLSVC) and occurs in less than 1% of cases of ASDs. It is characterized by the absence of at least a portion of the common wall that separates the coronary sinus and left atrium. Isolated defects are associated with low rate of morbidity and mortality.展开更多
Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpat...Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.展开更多
Introduction Obstructed total anomalous pulmonary venous connection(TAPVC)is one of the commonest seen emergencies in pediatric cardiology centers.Case presentation Our case was diagnosed to have this anomaly,showing ...Introduction Obstructed total anomalous pulmonary venous connection(TAPVC)is one of the commonest seen emergencies in pediatric cardiology centers.Case presentation Our case was diagnosed to have this anomaly,showing early respiratory distress resulting from severe pulmonary congestion.Palliative stenting of the obstruction was done,which helped in stabilizing the debilitated hemodynamics of the baby before surgery,thus a good surgical outcome and prognosis are expected.Conclusion This intervention may be listed as a vital measurement in the preoperative cardiac stabilization plan for infants with obstructed TAPVC.展开更多
Objectives We did a retrospective study to summarize the surgical experience of anomalous pulmonary venous drainage (APVD) correction and discuss effective way of improving the surgical outcome. Methods From January 1...Objectives We did a retrospective study to summarize the surgical experience of anomalous pulmonary venous drainage (APVD) correction and discuss effective way of improving the surgical outcome. Methods From January 1985 to May 2008, 127 patients [56 men and 71 women, aged 14-55 years with an average of (26.79±10.62) years] with APVD underwent surgical treatments. Among them, 13 patients had simple partial APVD with intact atrial septum, 104 patients had partial APVD with atrial septal defect and 10 patients had total APVD. Seventy-one patients of them accompanied with other cardiac anomalies which were also corrected in their operations. Results One early operative death due to severe low cardiac output syndrome (LCOS) developed postoperatively, which resulted in a mortality rate of 0.78%. Among other patients, 10 patients complicated with LCOS, 11 patients with arrhythmia, 7 patients with acute renal failure and 4 patients with poor wound healing, all discharged from hospital after treatment. Postoperative echocardiography reexamination revealed 1 case of mild residual shunt in atrial septum but without pulmonary vein stenosis. Conclusion For right atrial and ventricle enlarged patients with or without pulmonary hypertension, surgeons should be vigilance of accompanied APVD whether atrial septal defects exist or not. As long as no contraindications are found, surgical treatment should be performed once accurate diagnosis is obtained.展开更多
Background The surgical outcomes have been greatly improved over the past four decades for functional single ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connection(TA...Background The surgical outcomes have been greatly improved over the past four decades for functional single ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connection(TAPVC),especially obstructed TAPVC,has remained a clinical challenge. Little is known about the results of surgical treatment for patients with FSV-TAPVC in China. Methods Forty consecutive patients with FSV-TAPVC undergoing initial surgical palliation(median age:16.8 months;body weight:8.25 kg)were retrospectively enrolled from 2006 to 2016 in a specialized cardiovascular institute. TAPVC was repaired in 30 patients. The mean follow-up period was 49 months. Results All of the 7 patients with preoperative pulmonary vein obstruction(PVO)underwent repair of TAPVC at the first operation and survived. When a systemic to pulmonary(S-P)shunt or pulmonary artery banding(PAB)was necessary,patients with concomitant TAPVC repair had lower mortality(30.0%)and higher total cavopulmonary connection completion rate(20.0%)than those without TAPVC repair[75.0% and 0%,respectively],although with no statistical significance(Fisher test,P=0.505 and P=0.245). The overall survival rates for the cohort at 1,3 and 5 years after the initial surgical intervention were 80.0%,77.1% and 77.1%,respectively. Multivariate COX regression analysis detected that SP shunt(adjusted odds ratio:6.51;95%CI:1.26-33.71,P=0.030)is the only risk factor for survival. The reintervention rate for postoperative PVO was higher in patients with preoperative PVO than those without(57.1%vs. 4.3%,Log Rank:P=0.006). Conclusion The mid-term results of surgical repair of FSV-TAPVC are still challenging. When PVO exists,surgical repair for TAPVC is suggested as soon as possible. Repair for TAPVC should also be considered when S-P shunt or PAB is necessary. Further study with larger population are warranted to support our finding.展开更多
It has been demonstrated that pulmonary veins (PV)play an important role in the initiation and perpetuation of paroxysmal atrial fibrillation (PAF).1-5 Therefore, PV isolation has become the cornerstone for cathet...It has been demonstrated that pulmonary veins (PV)play an important role in the initiation and perpetuation of paroxysmal atrial fibrillation (PAF).1-5 Therefore, PV isolation has become the cornerstone for catheter ablation in the majority of these AF patients.3-5 In addition, ectopic foci in the non-PVs areas, such as superior vena cava(SVC),展开更多
文摘Objective To evaluate effectiveness of urgent surgical correction for infra cardiac total anomalous pulmonary venous drainage (TAPVD) in infants and children. Methods From July 2000 to April 2009,seventeen patients with infra-cardiac type of total anomalous pulmonary venous connection received surgical correction
文摘To investigate the value of echocardiography in the diagnosis of total anomalous pulmonary venous connection ( TAPVC ), 16 patients in our hospital were diagnosed to have TAPVC by echocardiography from year 1994 to 2001. In 11 cases the results of echocardiography were compared to those of surgery. Each patient was examined by using a combination of precordial, suprasternal and subcostal windows to visualize all the pulmonary veins and their drainage sites, common pulmonary venous trunk, and other associated abnormalities. Of the 16 cases, the drainage sites were as follow: supracardiac in 10, via vertical vein in 9, directly to superior vena cava in 1; cardiac in 5, via coronary sinus in 2, directly to right atrium in 3. Diagnoses were correctly made in all the 11 cases as confirmed by surgery. Echocardiography can also assess pulmonary arterial pressure and detect other associated abnormalities. It is concluded that echocardiography is the preferred examination method in the diagnosis of TAPVC before surgery. With careful examination using multiple windows and sections, TAPVC can be accurately diagnosed by echocardiography.
文摘BACKGROUND Alagille syndrome(ALGS)is an autosomal dominant genetic disorder caused by mutations in the JAG1 or NOTCH2 gene.It is characterized by decreased intrahepatic bile ducts associated with a variety of abnormalities in many other organ systems,such as the cardiovascular,skeletal,and urinary systems.CASE SUMMARY We report a rare case of ALGS.A 1-month-old male infant presented with sustained jaundice and had a rare congenital heart disease:Total anomalous pulmonary venous connection(TAPVC).Sustained jaundice,particularly with cardiac murmur,caught our attention.Laboratory tests revealed elevated levels of alanine aminotransferase,aspartate aminotransferase,gamma-glutamyl transpeptidase,total bilirubin,and total bile acids,indicating serious intrahepatic cholestasis.Imaging confirmed the presence of butterfly vertebra at the seventh thoracic vertebra.This suggested ALGS,which was confirmed by genetic testing with a c.3197dupC mutation in the JAG1 gene.Ursodiol was administered immediately after confirmation of the diagnosis,and cardiac surgery was performed when the patient was 1.5 month old.He recovered well after treatment and was discharged at the age of 3 mo.At the age of two years,the patient returned to our clinic because multiple cutaneous nodules with xanthomas appeared,and their size and number increased over time.CONCLUSION We report a unique case of ALGS associated with TAPVC and severe xanthomas.This study has enriched the clinical manifestations of ALGS and emphasized the association between JAG1 gene and TAPVC.
文摘Introduction: To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC). Case Report: A 16-year-old girl was diagnosed with features of an atrial septal defect (ASD) by transthoracic echocardiography and the absence of PLSVC (persistent left superior vena cava) connection to coronary sinus was confirmed by saline contrast echocardiography (“bubble study”). Discussion: The cause of the dilated coronary sinus was due to total anomalous pulmonary venous connection. Conclusion: Coronary sinus atrial septal defect (ASD) is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava (PLSVC) and occurs in less than 1% of cases of ASDs. It is characterized by the absence of at least a portion of the common wall that separates the coronary sinus and left atrium. Isolated defects are associated with low rate of morbidity and mortality.
文摘Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.
文摘Introduction Obstructed total anomalous pulmonary venous connection(TAPVC)is one of the commonest seen emergencies in pediatric cardiology centers.Case presentation Our case was diagnosed to have this anomaly,showing early respiratory distress resulting from severe pulmonary congestion.Palliative stenting of the obstruction was done,which helped in stabilizing the debilitated hemodynamics of the baby before surgery,thus a good surgical outcome and prognosis are expected.Conclusion This intervention may be listed as a vital measurement in the preoperative cardiac stabilization plan for infants with obstructed TAPVC.
基金supported by Natural Science Foundation of Guangdong Province ( No.5001161)Medical Science Foundation of Guangdong Province (No.A2005049)
文摘Objectives We did a retrospective study to summarize the surgical experience of anomalous pulmonary venous drainage (APVD) correction and discuss effective way of improving the surgical outcome. Methods From January 1985 to May 2008, 127 patients [56 men and 71 women, aged 14-55 years with an average of (26.79±10.62) years] with APVD underwent surgical treatments. Among them, 13 patients had simple partial APVD with intact atrial septum, 104 patients had partial APVD with atrial septal defect and 10 patients had total APVD. Seventy-one patients of them accompanied with other cardiac anomalies which were also corrected in their operations. Results One early operative death due to severe low cardiac output syndrome (LCOS) developed postoperatively, which resulted in a mortality rate of 0.78%. Among other patients, 10 patients complicated with LCOS, 11 patients with arrhythmia, 7 patients with acute renal failure and 4 patients with poor wound healing, all discharged from hospital after treatment. Postoperative echocardiography reexamination revealed 1 case of mild residual shunt in atrial septum but without pulmonary vein stenosis. Conclusion For right atrial and ventricle enlarged patients with or without pulmonary hypertension, surgeons should be vigilance of accompanied APVD whether atrial septal defects exist or not. As long as no contraindications are found, surgical treatment should be performed once accurate diagnosis is obtained.
基金the National Key R&D Program of China(No.2018YFC1002600)National Natural Science Foundation of China(No.81903287)+4 种基金Natural Science Foundation of Guangdong Province(No.2018A030313329)the Science and Technology Planning Project of Guangdong Province(No.2017A070701013)the Science and Technological Program of Guangzhou,China(No.201704020126)Guangdong Medical Research Foundation(No.A2017328)Guangdong Provincial Administration of Traditional Chinese Medicine(No.20181004).
文摘Background The surgical outcomes have been greatly improved over the past four decades for functional single ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connection(TAPVC),especially obstructed TAPVC,has remained a clinical challenge. Little is known about the results of surgical treatment for patients with FSV-TAPVC in China. Methods Forty consecutive patients with FSV-TAPVC undergoing initial surgical palliation(median age:16.8 months;body weight:8.25 kg)were retrospectively enrolled from 2006 to 2016 in a specialized cardiovascular institute. TAPVC was repaired in 30 patients. The mean follow-up period was 49 months. Results All of the 7 patients with preoperative pulmonary vein obstruction(PVO)underwent repair of TAPVC at the first operation and survived. When a systemic to pulmonary(S-P)shunt or pulmonary artery banding(PAB)was necessary,patients with concomitant TAPVC repair had lower mortality(30.0%)and higher total cavopulmonary connection completion rate(20.0%)than those without TAPVC repair[75.0% and 0%,respectively],although with no statistical significance(Fisher test,P=0.505 and P=0.245). The overall survival rates for the cohort at 1,3 and 5 years after the initial surgical intervention were 80.0%,77.1% and 77.1%,respectively. Multivariate COX regression analysis detected that SP shunt(adjusted odds ratio:6.51;95%CI:1.26-33.71,P=0.030)is the only risk factor for survival. The reintervention rate for postoperative PVO was higher in patients with preoperative PVO than those without(57.1%vs. 4.3%,Log Rank:P=0.006). Conclusion The mid-term results of surgical repair of FSV-TAPVC are still challenging. When PVO exists,surgical repair for TAPVC is suggested as soon as possible. Repair for TAPVC should also be considered when S-P shunt or PAB is necessary. Further study with larger population are warranted to support our finding.
基金This study was supported by the Natural Science Foundation of Hubei Province, China (No.2008CDB 187) the Project Sponsored by the Scientific Research Foundation for the Returned Oerseas Chinese Scholars, State Education Ministry, China (No.2008890)Wuhan Twiling Program, China (No.200850731368).
文摘It has been demonstrated that pulmonary veins (PV)play an important role in the initiation and perpetuation of paroxysmal atrial fibrillation (PAF).1-5 Therefore, PV isolation has become the cornerstone for catheter ablation in the majority of these AF patients.3-5 In addition, ectopic foci in the non-PVs areas, such as superior vena cava(SVC),