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2.
Article in Chinese | WPRIM | ID: wpr-873551

ABSTRACT

@#Objective    To summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum (PA/IVS) and to reflect on the strategies of right ventricular decompression. Methods    The clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively. There were 10 males and 2 females with a median age at the time of surgery was 5 d (range, 1-627 d). Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed. Results    One patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment. There were 2 (16.67%) patients received postoperative intervention. The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg. Mild pulmonary regurgitation was found in 7 patients, moderate in 2 patients, and massive in 1 patient. The median time of mechanical ventilation was 30.50 h (range, 6.00-270.50 h), and the average duration of ICU stay was 164.06±87.74 h. The average postoperative follow-up time was 354.82±331.37 d. At the last follow-up, the average Z score of tricuspid valves was 1.32±0.71, the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg (range, 21-146 mm Hg) and the average percutaneous oxygen saturation was 92.78%±3.73%. Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery, respectively, with the rate of reintervention-free of 81.8%. There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves (r=–0.506, P=0.201). Conclusion    For children with PA/IVS, the simple pursuit of adequate decompression during right ventricular decompression may lead to  severe pulmonary dysfunction, increase the risk of ineffective circular shunt, and induce refractory hypoxemia. The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.

3.
Rev. bras. cir. cardiovasc ; 35(4): 445-451, July-Aug. 2020. tab, graf
Article in English | LILACS, SES-SP | ID: biblio-1137300

ABSTRACT

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Pulmonary Atresia/surgery , Heart Septal Defects/surgery , Cardiac Surgical Procedures , Pulmonary Artery/surgery , Pulmonary Artery/diagnostic imaging , Retrospective Studies , Collateral Circulation
4.
Chinese Journal of Pediatrics ; (12): 96-100, 2020.
Article in Chinese | WPRIM | ID: wpr-799307

ABSTRACT

Objective@#To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS).@*Methods@#The retrospective study included 21 neonates (14 males and 7 females) with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve perforation and balloon dilation in Xinhua Hospital from January 2012 to December 2018. All patients underwent the pulmonary valve perforation by micro-guidewire through the Simmons catheter. During the follow-up period at 1, 3, 6, 12 months postoperatively and annually thereafter, the operative efficacy and the development of the right ventricle (RV) were evaluated by echocardiography. Statistical analyses were performed using t test.@*Results@#A total of 21 neonates with PA-IVS were enrolled, and 13 cases were diagnosed prenatally. The median age of surgery was 6 days, the weight was (3.2±0.5) kg. The balloon/valve ratio was 1.19±0.12, and the RV pressure measured by catheter was (121±33) mmHg (1 mmHg=0.133 kPa) . The immediate postoperative RV pressure was (47±13) mmHg. The median follow-up time was 30 months. All the cases enrolled achieved biventricular circulation without death and serious complications. According to the last follow-up data including 16 cases who were followed up for 1 year or longer, the pulmonary artery transvalvular pressure was (29±15) mmHg. The postoperation ratio of right to left ventricular transverse diameter was significantly higher than that before operation (0.86+0.10 vs. 0.73+0.13, t=-2.96, P=0.006). Compared with preoperative data, the postoperation pulmonary valvular diameter Z-score was significantly higher (-1.41±0.89 vs. -2.83±1.06, t=-3.65, P=0.001) and the tricuspid valvular diameter Z-score was significantly higher (-0.52±0.29 vs. -1.34±0.81, t=-3.55, P=0.001).@*Conclusion@#Transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation is a safe and effective initial therapy for neonatal PA-IVS.

5.
Article in Chinese | WPRIM | ID: wpr-799061

ABSTRACT

Objective@#To investigate the clinical outcomes of selective major aortopulmonary collaterals(MAPCAs) unifocalization and report histopathological findings in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals(PA/VSD/ MAPCAs).@*Methods@#The study enrolled 6 MAPCAs/VSD/PA patients with age ranged from 6 to 96 months and body weight ranged from 5.0 to 23.0 kg. These patients underwent selective MAPCAs unifocalization and primary repairs. Preoperative cardiac catheter, selective arteriography, cardiac CTA and intraoperative pathology were performed to identify different function, anatomic distribution and histopathology of MAPCAs.@*Results@#6 MAPCAs/VSD/PA patients underwent selective MAPCAs unifocalization and primary repair. No death occurred after operation and at follow-up which lasted for 1 to 20 months. Preoperative cardiac catheter, selective arteriography and intraoperative histopathology demonstrated distribution of functional MAPCAs similar to native pulmonary artery arborization and participating in arterial gas exchange. Functional MAPCAs were classified into elastic arteries according to histopathology.@*Conclusion@#There are two histological type of MAPCAs which play different roles. Selective unifocalization to functional MAPCAs which are classified into elastic arteries like native pulmonary artery is a safe and effective treatment approach for PA/VSD/MAPCAs.

6.
Article in Chinese | WPRIM | ID: wpr-822561

ABSTRACT

@#Objective    To analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations. Methods    We retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation. Results    The top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation. Conclusion    Series operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.

7.
Article in Chinese | WPRIM | ID: wpr-822484

ABSTRACT

@#To evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center. Methods    From January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed. Results    All patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ. Conclusion    According to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.

8.
Article in Chinese | WPRIM | ID: wpr-822483

ABSTRACT

@#To analyze the mid-long-term outcomes of surgical balloon valvuloplasty (SBV) for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum (PA/IVS). Methods    Clinical data of consecutive 91 patients who were diagnosed with PA/IVS and underwent SBV in our institution from January 2005 to December 2017 were retrospectively analyzed, including 52 (57.1%) males and 39 (42.9%) females. The median age was 3 months (1 d, 24 months) and the median weight was 4.1 (2.5, 12.0) kg. Results    The SBV was performed in all patients, and 62 of whom received other simultaneous surgeries, including ligation of patent ductus arteriosus (PDA, 33 patients), ligation of PDA with modified Blalock-Taussig shunt (23 patients), ligation of PDA with bidirectional Glenn shunt (6 patients). There was no early postoperative death. The median follow-up time was 8.8 (2.5, 13.4) years, 4 patients were lost. There were 7 (8.0%) deaths and 1 (1.1%) patient with a re-SBV for pulmonary stenosis. The one and a half ventricular repair was performed in 5 (5.7%) patients and Fontan procedure in 2 (2.3%) patients. In addition, the mean Z-value of tricuspid valve annulus was −1.7±1.5, which was significant bigger than that before the operation (t=5.587, P<0.001). Conclusion    SBV via right ventricular outflow tract for right ventricular decompression in the treatment of PA/IVS is safe and reliable. The majority of patients can receive biventricular repair instead of single ventricular palliation by SBV with individually customized shunt.

9.
Article in Chinese | WPRIM | ID: wpr-821464

ABSTRACT

@#Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease with high mortality. Due to the complexity and diversity of pathological anatomy, there is no uniform standard for the optimal surgical scheme of PA/IVS. In order to further standardize the surgical treatment strategy of PA/IVS, this expert consensus will focus on the evaluation of right ventricular anatomy and function, operation timing, operation mode and prognosis of PA/IVS. Through the synthesis of many domestic treatment experience, the Chinese expert consensus on PA/IVS surgical treatment has been finally formed.

10.
Article in Chinese | WPRIM | ID: wpr-821150

ABSTRACT

@#Objective    To evaluate the effect of off-pump pulmonary valvulotomy for the patients with pulmonary atresia with intact ventricular septum (PA/IVS). Methods    The clinical data of 61 PA/IVS patients who underwent off-pump right ventricular decompression surgery in our hospital from January 2013 to September 2019 were retrospectively analyzed, including 37 males and 24 females, with an average age of 29.7 (2.0-86.0) d and weight of 4.1 (2.5-6.9) kg. Thirty-nine patients received off-pump pulmonary valvulotomy (an open-view valvulotomy group) and 22 patients received balloon valvuloplasty through the right ventricle pulmonary valve (a hybrid therapy group). The postoperative mortality, early re-intervention, and completion of final operation of the two groups were compared. Results    There were 2 deaths in the study with a mortality rate of 3.3% (2/61), and the mortality rate of the two groups was not significantly different (2.6% vs.4.5%, P=0.68). The rate of early re-intervention in the two groups was 5.3% and 19.0%, respectively (P=0.09). There was no statistical difference in intubation time (56.0±25.9 h vs. 62.0±28.9 h, P=0.41), ICU retention time (4.7±2.9 d vs. 5.5±2.2 d, P=0.23) and postoperative hospital stay time (3.9±0.9 d vs. 4.3±1.1 d, P=0.38) between the two groups. The follow-up time was 45.3 (4.0-84.0) months. There were 5 patients lost to follow-up. During the follow-up period, in the open-view valvulotomy group, 17 patients did not need further operation, 13 patients completed the final operation. In the hybrid therapy group, 7 patients did not need further operation, 8 patients completed the final operation. Heart function classification of all patients was in New York Heart Association class Ⅰ-Ⅱ. Conclusion    Compared with the hybrid therapy, off-pump pulmonary valvulotomy for PA/IVS also has the advantages of simple operation, short operation time and high survival rate, and it may be easier to be promoted in clinical application because of its more economic benefits and relatively lower re-intervention rate.

11.
Article in Chinese | WPRIM | ID: wpr-821148

ABSTRACT

@#Pulmonary atresia with ventricular septal defect (PA/VSD) is a complicated congenital heart defect. The consensus of Chinese experts is developed based on the evidence-based data and expert opinions provided by the literature. Tchervenkov classification (A, B and C, three types) is adopted. Echocardiography is preferred to assess the abnormalities of the heart, physiological function and pulmonary artery development, but it is not absolutely accurate for the evaluation of collateral vessels. Furthermore, multi-row CTA can confirm the development of the native pulmonary artery, the number, origin and morphological characteristics of collateral vessels. Additionally, angiography provides more details of the number, origin and distribution of collateral vessels. Genetic testing is important to understand the genetic etiology and to determine the prognosis. After definite diagnosis, treatment plan is made according to the classification and clinical manifestations. Pulmonary artery flow is arterial duct dependent for Type A and surgical treatment is usually performed within 6 months. But patients with severe hypoxia were treated during neonatal period. The strategy for type B/C is more complex. Severe hypoxemia may occur in infants with type B PA/VSD and decreased pulmonary flow, and systemic to pulmonary artery shunt should be performed during neonatal or early infant period, complete repair following after good pulmonary arterial development. On the other hand, for patients with over circulating pulmonary flow, primary repair is perform with 3~6 months for congestive heart failure. For patients with balanced pulmonary flow and repairable condition, one-stage complete repair can be performed in infancy stage. Otherwise, palliative systemic to pulmonary artery shunt or right ventricle to pulmonary artery shunt should be first, and second-staged complete repair or pulmonary arterial development promotion is adopted according to following assessment. The surgical option for type C PA/VSD should be discreet. Pulmonary arterial flow study is demanded after unifocalization to decide to close the VSD completely or not. Close follow-up after operation is imperative and cardiac catheterization is perform to reveal the pulmonary artery and collateral vessels if necessary and dilates the site of stenosis.

13.
Article in Chinese | WPRIM | ID: wpr-800975

ABSTRACT

Objective@#To evaluate the safety and effectiveness of transcatheter or surgical procedure for neonates diagnosed with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (CPS) by retrospectively analyzina the clinical data, thus to discuss the clinical strategy decision.@*Methods@#From November 2006 to August 2018, 105 neonates (PA/IVS 51 cases, CPS 54 cases) went through transcatheter or surgical procedure in Guangdong Provincial Cardiovascular Institute (transcatheter procedure 43 cases, surgery 62 cases). All cases manifested mild to moderate right ventricular dysplasia.Transcatheter procedure included pulmonary radio frequency perforation and percutaneous balloon pulmonary valvuloplasty (PBPV). Surgical procedure included reconstruction of right ventricular outflow tract (transannular patch), pulmonary valvotomy and closed transventricular pulmonary valvuloplasty, while Aortic-Pulmonary shunt was performed in certain cases.Average follow-up period was (40.1±36.9) months.Hospitalization and follow-up data were analyzed to evaluate the safety and efficacy of transcatheter and surgical procedure.@*Results@#Operative time, hospital stay, complication rate were lower in transcatheter procedure group than those of surgery group[(107.8±61.6) min vs.(183.3±36.8) min, (18.6±7.9) d vs.(31.1±13.4) d, 30.2% vs.80.6%], and the differences were significant(all P=0.000). In transcatheter procedure group, 2 cases (4.7%) died and biventricular circulation is obtained in all survival cases.In surgery group, 5 cases (8.1%) died.Of the survival cases from surgery group, 39 cases (69.6%) obtained biventricular circulation, 3 cases (5.4%) obtained one and a half ventricular circulation while other 14 cases (25.0%) were candidates for biventricular repair.@*Conclusions@#Biventricular circulation can be achieved in most PA/IVS or CPS neonates with mild-moderate right ventricular dysplasia.PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.

14.
Article in Chinese | WPRIM | ID: wpr-754855

ABSTRACT

To explore the prenatal diagnosis classification and prognostic evaluation of fetal pulmonary atresia with intact ventricular septum ( PA/IVS) . Methods Thirty‐nine fetal PA/IVS were classified by the developmental condition of the right ventricle and ventriculo‐coronary artery communication ( VCAC) ,and tricuspid Z score was calculated . The associated abnormality ,chromosome abnormality were follow‐up analyzed . Results Fifteen fetuses were diagnosed with type Ⅰ PA/IVS ,14 fetuses with type ⅡPA/IVS ,and 10 with type Ⅲ PA/IVS . One case with type Ⅰ was associated with right aortic arch ,and other 38 fetuses were not associated with other cardiac abnormalities . T hirty‐nine fetuses were normal karyotype .Fetuses with type Ⅰ PA/IVS manifested right ventricular inlet portion ,well‐developed trabecular and infundibulum portions ,and no VCAC . T he tricuspid Z score of type Ⅰ PA/IVS was from -0 .07 to -2 .82 ,and 9 of the fetuses had biventricular repair and 6 had termination . Type Ⅱ PA/IVS manifested right ventricular trabecular portion absence ,small inlet and infundibulum portions ,and no VCAC . T he tricuspid Z score of type Ⅱ PA/IVS was from -3 .35 to -5 .21 ,and 7 of the fetuses had single ventricle palliation ,2 underwent fetal interventional procedures ,and 5 had termination . Type Ⅲ PA/IVS manifested absence of right ventricular trabecular and infundibulum portions ,small inlet portion ,and VCAC . T he tricuspid Z score of type Ⅲ PA/IVS was from -4 .33 to -6 .01 ,and 4 of the fetuses had single ventricle palliation and 6 had termination . The area under the ROC curve of tricuspid Z score in diagnosing PA/IVS postnatal biventricular repair was 1 .000 ( P <0 .01 ,95% CI :1 .00-1 .00) ,the cutoff value was -3 .08 ,the sensitivity was 100% ,and the specificity was 100% . Conclusions Echocardiography can perform diagnostic classification of fetal PA/IVS and obtain fetal tricuspid valve Z score of > -3 .08 and predict the postnatal outcome in PA/IVS . T he findings may have important implication for prenatal diagnosis and prognosis evaluation for PA/IVS .

15.
Article in Chinese | WPRIM | ID: wpr-754832

ABSTRACT

To explore the prenatal diagnosis classification and prognostic evaluation of fetal pulmonary atresia with ventricular septal defect ( PA/VSD ) . Methods T hirty‐one fetal pulmonary atresia with ventricular septal defect were classified Ⅰ - Ⅳ type by Boston classification ,and the McGoon indexes were calculated ,w hether associated with malformation and chromosomal abnormalities ,and follow‐up . Results T hirteen fetuses were diagnosed type Ⅰ PA/VSD , 6 fetuses were associated with malformation ,2 fetuses were chromosomal abnormalities , 7 fetuses′ McGoon index > 1 .20 ,6 fetuses′McGoon index<1 .20 ,8 cases had operation ( 6 cases had radical operation and had a good follow up ,2 cases had palliative operation and were waiting for radical operation) , 5 cases received termination of pregnancy . Six fetuses were diagnosed as type Ⅱ PA/VSD ,5 fetuses were associated with malformation ,1 fetus was chromosomal abnormalities ,1 fetus′s McGoon index> 1 .20 ,5 fetuses′ McGoon index< 1 .20 ,2 cases had operation ( 1 case had radical operation and had a good follow up ,1 case had palliative operation and was waiting for radical operation) ,4 fetuses received termination of pregnancy . Four fetuses were diagnosed as type Ⅲ PA/VSD ,3 fetuses were associated with malformation ,no fetus was chromosomal abnormalities ,4 fetuses′McGoon index<1 .20 ,1 case had palliative operation and was waiting for radical operation , 3 cases received termination of pregnancy . Eight fetuses were diagnosed as type Ⅳ PA/VSD ,3 fetuses were associated with malformation , 3 fetuses were chromosomal abnormalities , 1 case had unifocalization operation ,but died after operation in one day ,7 cases received termination of pregnancy . T he area under the ROC curve of McGoon index in hinting PA/VSD postnatal radical operation was 1 .000 ( P = 0 .002 ,95%CI :1 .0000 - 1 .000 ) , the border value was 1 .255 , the sensitivity and specificity were 100% ,85 .7% , respectively . Conclusions Echocardiography can diagnose the classification of fetal PA/VSD . The radical operation for cases of McGoon index >1 .255 is feasible ,the cases of type Ⅳ PA/VSD and PA/VSD with associated malformation and chromosomal abnormalities have a poor follow up .

16.
Article in Chinese | WPRIM | ID: wpr-754801

ABSTRACT

Objective To analyze the echocardiographic findings , associated anomalies and chromosomal characteristics in fetuses with pulmonary atresia with ventricular septal defect ( PA/VSD ) . Methods T he echocardiographic data and follow‐up materials were retrospectively reviewed in 30 256 fetuses from December 2012 to M arch 2018 in the consultation center of fetal heart disease in maternal‐fetal medicine in Anzhen hospital . Of all the fetuses ,59 cases ( 0 .19% ) had PA/VSD . T he echocardiographic findings ,associated anomalies and chromosomal characteristics were retrospectively analyzed in all the 59 fetuses with PA/VSD . Based on w hether the presence of the native pulmonary arteries and the major aortopulmonary collateral arteries ( M APCAs) or not ,the PA‐VSD was classified into type A ,type B ,and type C . Results A large ventricular defect was demonstrated in five‐chamber view with 61 .7% of the mean ratio of the aortic overriding . O ther fetal echocardiographic features of all the 59 fetuses with PA/VSD included :the right aortic arch ( n =19 ) ,reversal flow in the ductus arteriosus ( n =40 ) ,M APCAs ( n =24) . T he classification of the PA/VSD included :type A ( n =35) ,type B ( n =5) and type C ( n =19) . Associated anomalies :persistent left superior vena cava ( n = 13 ) ,anomalous pulmonary vein connection ( n=5 ) ,complete atrioventricular septal defect ( n = 5 ) ; single umbilical artery ( n = 3 ) ,right atrial isomerism ( n =3) . Of all the 30 cases performed chromosomal test ,3 cases had aneuploidy and 7 cases had microdeletion of chromosome . Conclusions The fetal echocardiographic findings of the PA/VSD are characteristic . For prenatal diagnosis of PA/VSD ,the type of PA/VSD should be defined and chromosomal test should be performed ,w hich can be helpful for prenatal consulting .

17.
Article in Chinese | WPRIM | ID: wpr-752309

ABSTRACT

Objective To evaluate the safety and effectiveness of transcatheter or surgical procedure for neo_nates diagnosed with pulmonary atresia with intact ventricular septum(PA/IVS)or critical pulmonary stenosis(CPS) by retrospectively analyzina the clinical data,thus to discuss the clinical strategy decision. Methods From November 2006 to August 2018,105 neonates(PA/IVS 51 cases,CPS 54 cases)went through transcatheter or surgical procedure in xuangdong Provincial Cardiovascular Institute(transcatheter procedure 43 cases,surgery 62 cases). All cases mani_fested mild to moderate right ventricular dysplasia. Transcatheter procedure included pulmonary radio frequency perfora_tion and percutaneous balloon pulmonary valvuloplasty( PBPV). Surgical procedure included reconstruction of right ventricular outflow tract(transannular patch),pulmonary valvotomy and closed transventricular pulmonary valvuloplas_ty,while Aortic _Pulmonary shunt was performed in certain cases. Average follow _up period was(40. 1 ± 36. 9) months. Hospitalization and follow_up data were analyzed to evaluate the safety and efficacy of transcatheter and surgi_cal procedure. Results Operative time,hospital stay,complication rate were lower in transcatheter procedure group than those of surgery group[(107. 8 ± 61. 6)min υs.(183. 3 ± 36. 8)min,(18. 6 ± 7. 9)d υs.(31. 1 ± 13. 4)d, 30. 2% υs. 80. 6%],and the differences were significant(all P=0. 000). In transcatheter procedure group,2 cases (4. 7%)died and biventricular circulation is obtained in all survival cases. In surgery group,5 cases(8. 1%)died. Of the survival cases from surgery group,39 cases(69. 6%)obtained biventricular circulation,3 cases(5. 4%)obtained one and a half ventricular circulation while other 14 cases( 25. 0%) were candidates for biventricular repair. Conclusions Biventricular circulation can be achieved in most PA/IVS or CPS neonates with mild_moderate right ventricular dysplasia. PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.

18.
Rev. méd. Hosp. José Carrasco Arteaga ; 10(3): 250-255, nov. 2018. Imagenes
Article in Spanish | LILACS | ID: biblio-999908

ABSTRACT

INTRODUCCIÓN: La tetralogía de Fallot representa la cardiopatía congénita cianógena más frecuente después del primer año de vida, ocurre aproximadamente en 1 de cada 3 500 nacimientos y representa el 7 al 10% de todas las Enfermedades Cardiacas Congénitas. Su variante extrema que se acompaña de atresia pulmonar representa el 1 a 2% de las cardiopatías congénitas. Presentamos una Tetralogía de Fallot Extrema Ductus Dependiente de manejo complejo. CASO CLÍNICO: Se trata de un neonato a término de 12 días de vida, adecuado para la edad gestacional, sin factores de riesgo prenatales, ni antecedentes patológicos familiares, el cual es diagnosticado de Tetralogía de Fallot Extrema por ecocardiograma. EVOLUCIÓN: Paciente inicialmente tratado con infusión de prostaglandina E1. Posteriormente mediante fístula Blalock-Taussig izquierda. CONCLUSIONES: Para mejorar el pronóstico y sobrevida de un paciente con Tetralogía de Fallot Extrema el diagnóstico prenatal oportuno y el tratamiento apropiado es de vital importancia, sin embargo el tratamiento definitivo con derivaciones pulmonares sistémicas representan complicaciones con riesgo de mortalidad. El presente caso tuvo un desenlace desfavorable.


BACKGROUND: The Tetralogy of Fallot represents the most common cyanogenic heart disease after the first year of life. It occurs in 1 of 3 500 live births and accounts as 7 to 10% of all Congenital Heart Diseases. Its extreme variant is accompanied by pulmonary atresia and accounts 1 to 2% of the Congenital Heart Diseases. We present an Extreme Tetralogy of Fallot­ Ductus Dependent of complex management. CLINICAL CASE: A full term 12 day neonate, adequate for gestational age, without prenatal risk factors or pathologic family antecedents, is diagnosed with Extreme Tetralogy of Fallot by echocardiogram. EVOLUTION: The patient was treated initially with an infusion of prostaglandin E1. Then, a left Blalock-Taussig shunt was performed. CONCLUSIONS: To improve the prognosis and survival rates of patients with Extreme Tetralogy of Fallot , a timely diagnosis and treatment are of vital importance. Definitive treatment with systemic pulmonary shunts carries high risk complications. The present case had an unfavorable evolution.


Subject(s)
Humans , Infant, Newborn , Tetralogy of Fallot , Case Management , Pulmonary Atresia , Blalock-Taussig Procedure/methods , Heart Septal Defects, Ventricular
19.
Chinese Journal of Surgery ; (12): 427-431, 2018.
Article in Chinese | WPRIM | ID: wpr-809996

ABSTRACT

Objective@#To assess the feasibility and efficacy of a staged invasive treatment strategy for the treatment of pulmonary atresia with intact ventricular septum, in the first stage using a catheter-based stent placement, second-stage surgery, and three-stage atrial septal occlusion.@*Methods@#Totally 19 children with pulmonary atresia with intact ventricular septum were enrolled at Department of Cardiovascular Surgery, the Children′s Hospital of Zhejiang University School of Medicine from January 2009 to December 2017, including 11 male and 8 female patients. The age was (13.8±7.7) days (ranging from 3 to 35 days). The weight was (3.4±0.5) kg (ranging from 2.8 to 4.1 kg). Among them, there were 13 cases of type Ⅱ and 6 cases of type Ⅲ. Regular follow-up visits for patients with stage Ⅰ arterial duct stenting after 1, 3, and 6 months; and routine follow-ups for 1, 3, and 6 months after stage Ⅱ surgery.The t test was used to compare the oxygen saturation between pre-operation and post-operation.@*Results@#All 19 patients underwent arterial catheterization. The preoperative peripheral oxygen saturation was (64.4±5.3)%, while increased to (86.0±3.0)% after operation (t=16.353, P=0.000). One patient died during follow-up and 2 patients lost follow-up. One patient received stent balloon dilatation due to oxygen saturation decrease.Twelve patients completed the second-stage surgery and 1 case died after surgery. The rest were alive, including 2 cases of double-ventricular correction and 9 cases of semi-ventricular treatment in one room; 2 cases had completed three-stage atrial septal occlusion.@*Conclusions@#Stage Ⅰ arterial duct stent, stage Ⅱ surgery, and stage Ⅲ atrial septal occlusion to treat pulmonary atresia with intact ventricular septum in children is feasible and effective. The method can be used as the important treatment direction for pulmonary atresia with intact ventricular septum.

20.
Chinese Journal of Pediatrics ; (12): 445-450, 2018.
Article in Chinese | WPRIM | ID: wpr-809984

ABSTRACT

Objectives@#Two cases who underwent fetal pulmonary valvuloplasty (FPV) for pulmonary atresia with intact ventricular septum (PA-IVS) or critical pulmonary stenosis with intact ventricular septum (CPS-IVS) successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus.@*Methods@#One case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Parameters of right ventricle development and hemodynamics from echocardiography included tricuspid/mitral annulus (TV/MV), right ventricle/left ventricle long-axis (RV/LV), pulmonary/aortic annulus (PV/AV), tricuspid inflow duration/cardiac cycle, degree of tricuspid regurgitation (TR), blood flow direction of arterial duct and ductus venosus. Multidisciplinary team including the maternal-fetal cardiology, pediatric cardiology, cardiac surgery, obstetrics, neonatology and anesthesiology was summoned to discuss the indications and timing of PFV. Two cases underwent ultrasound-guiding trans-abdominal PFV at the 28 weeks of gestational age. Echocardiography was performed to observe the opening and closing of the pulmonary valve, and to evaluate the development of right ventricle and improvement in hemodynamics every 2-4 weeks until delivery.@*Results@#From the technical perspective, pulmonary balloon valvuloplasty was successfully performed in these two cases. The opening of pulmonary valve improved in these two cases at 2-4 weeks after FPV. However, an obvious restenosis was detected in the first case at 5-8 weeks after FPV. In the first case, the echocardiography parameters including TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle increased from 0.56, 0.42, 0.85,0.26 to 0.59, 0.51, 0.87, 0.32 at 5-8 weeks after FPV, respectively. However, the direction of blood flow through the arterial duct was still reverse. In the second case, TV/MV, RV/LV, PV/AV and tricuspid inflow duration/cardiac cycle ratio increased from 0.70, 0.63, 0.91,0.35 to 0.80, 0.80, 0.97, 0.42 at 5-8 weeks after FPV, respectively. The direction of blood flow through the arterial duct changed to bidirectional. Both fetuses were born alive. The first case underwent pulmonary valve commissurotomy and modified Blalock-Taussig shunt on the 8th day after delivery and received follow-up for 6 months. The strategy for the next-step therapy was still pending. The second case underwent transcutaneous pulmonary balloon valvuloplasty on the 19th day after delivery and received follow-up for 3 months. The opening of pulmonary valve improved obviously and the cardiac function was normal in the second case.@*Conclusions@#FPV is safe and effective for fetus during the second and third trimester of pregnancy, and FPV is beneficial for the development of fetal ventricle, valve and large artery. In addition, FPV may help to avoid the postnatal surgery for isolated single ventricle, improve fetal heart failure and prevent fetal death.

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