Right ventricular decompression for pulmonary atresia with intact ventricular septum / 中国胸心血管外科临床杂志

@#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.

Strategy of surgical management for pulmonary atresia with intact ventricular septum and mid-term follow-up / 中国胸心血管外科临床杂志

@#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.

Efficacy of surgical balloon valvuloplasty via right ventricular outflow tract for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum / 中国胸心血管外科临床杂志

@#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.

Effect of off-pump pulmonary valvotomy as the initial surgery for pulmonary atresia with intact ventricular septum: A single-center clinical analysis / 中国胸心血管外科临床杂志

@#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.

Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle

Outcome and follow-up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis / 中华实用儿科临床杂志

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.

Study of prenatal diagnosis and classification and prognostic evaluation in fetuses with pulmonary atresia with intact ventricular septum / 中华超声影像学杂志

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 .

Outcome and follow _up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis / 中华实用儿科临床杂志

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.

Risk factors for prolonged mechanical ventilation in children with transposition of the great arteries and intact ventricular septum that underwent primary arterial switch operation / 中华实用儿科临床杂志

Objective To explore the risk factors for prolonged mechanical ventilation in children with trans-position of great arteries and intact ventricular septum who underwent arterial switch operation. Methods This study was a retrospective,single center study. One hundred and twenty patients with transposition of great arteries and intact ventricular septum who underwent primary arterial switch operation between January 2014 and December 2016 at Fuwai Hospital were eligible for this study. The data of patients from pediatric intensive care unit database and electronic medical records were collected. The data related to postoperative respiratory assist time were collected,including demo-graphic data,preoperative diagnosis,intraoperative data,and postoperative recovery data. The patients were divided into 2 groups according to ventilation time which were prolonged mechanical ventilation group(ventilation time > 72 hours) and non - prolonged mechanical ventilation group(ventilation time ≤72 hours). The data of 2 groups were analyzed by using single factor analysis,and the P≤0. 2 factors were processed into Logistic regression analysis. Results Ninety -six patients were enrolled including 22 patients in prolonged ventilation group and 74 patients in non - prolonged me-chanical ventilation group. No statistical significance was found in 2 groups in gender,age,weight,preoperative lactate, hemoglobin,use of prostaglandin E1,mechanical ventilation,cardiopulmonary time,aortic clamping time,the ratio of left ventricular pressure to right ventricular pressure,immediate postoperative plasma lactate,and vasoactive inotropic score. The weight and postoperative left atrial pressure were significantly different between 2 groups with P < 0. 2. Weight were (3. 5 ± 0. 9)kg in prolonged mechanical ventilation group and (3. 9 ± 1. 0)kg in non - prolonged mechanical ventila-tion group (P = 0. 117). Left atrial pressures were (7. 9 ± 1. 9)mmHg(1 mmHg = 0. 133 kPa)in prolonged mechani-cal ventilation group and (6. 7 ± 2. 0)mmHg in non - prolonged mechanical ventilation group(P = 0. 015). The weight and left atrial pressure were processed into Logistic regression analysis and the results revealed that high left atrial pres-sure was the risk factor for ventilation prolongation(OR = 1. 048,P = 0. 020). Respiratory assist time in prolonged and non - prolonged ventilation group was 112(80,194)h and 26(17,46)h,respectively;ICU time in prolonged and non - prolonged ventilation group was 10(1,14)d and 4(3,6)d,respectively;and all the differences were significant (all P = 0. 000). The number of death in each group was 1 with no significant difference(P = 0. 420). Conclusions High left atrial pressure is the risk factor for prolonged mechanical ventilation in children with transposition of great ar-teries and intact ventricular septum following primary arterial switch operation.

Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review / 华中科技大学学报（医学）（英德文版）

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.

Two Infant Cases with Pulmonary Atresia, Intact Ventricular Septum, Right Ventricle-Dependent Coronary Circulation, and Myocardial Ischemic Symptoms / 日本心臓血管外科学会雑誌

We describe two cases with pulmonary atresia, intact ventricular septum (PA/IVS), and right ventricle-dependent coronary circulation, who suffered from myocardial ischemic symptoms. Case 1 : A female infant diagnosed with PA/IVS during the fetal period was born after 40 weeks of gestation, weighing 3,078 g. Aortography demonstrated that the right coronary artery (RCA) was interrupted at the proximal segment, and the left coronary artery (LCA) did not show any stenosis. Right ventriculogram revealed sinusoidal connections filling the distal part of the right coronary artery and fistulous communications to the left anterior descending artery. Temporary ST segmental depression in ECG during milk feeding or crying improved spontaneously with time. Bidirectional Glenn shunt (BDG) was performed at 3 months old, and she underwent Fontan repair at 4 years old. She is doing well with no evidence of myocardial ischemic change. Case 2 : A female infant was born after 39 weeks of gestation, weighing 3,062 g. Aortography demonstrated that the RCA was interrupted at the middle segment, and the distal area depended on the right ventricle. LCA ostium at the ascending aorta was absent. Multiple sinusoidal connections were seen in the right ventricular myocardium with filling of the LCAs but no reflux into the aortic sinuses. After ventricular tachycardia occurred at 2 months old, left ventricular function deteriorated. BDG with ligation of the arterial duct was performed at age 3 months. The arterial blood pressure was 76/53 (62) mmHg and pressure in SVC was 21 mmHg immediately after the operation. However, arterial oxygen saturation, right atrium venous oxygen saturation and left ventricular ejection fraction were 50%, 16%, and 10%, respectively. Although extracorporeal membrane oxygenation was established for circulatory and myocardial oxygenation support on the postoperative day 2, her left ventricular function did not recover and she died on postoperative day 5. It is difficult to rescue such a case in which almost all coronary circulation depends on the right ventricle filled with desaturated blood.

Clinical research of individual operation scheme in pulmonary atresia with intact ventricular septum / 中华胸心血管外科杂志

Objective Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare complicated congenital heart disease.The intracardiac structure is also complicated.We sum up the corporal surgical procedure of PA/IVS.Methods Between May 2004 and May 2010,we performed emergency or sub-emergency operation in 72 newborns,infants and children [age,1 day to 5.2 years; mean age,(1.3 ±0.6) years] with favorable anatomy.The surgical procedures were divided into three kinds:1.16 cases were performed with Blalock-Tassuing (B-T) shunt operation for A group; 2.33 cases were performed with transannular patch from RVOT-MPA with or without B-T shunt operation for B group.3.22 cases were performed a hybrid procedure with or without B-T shunt operation for C group.From all the patients,15 cases had been finished the two staged operation.Results 10 patients has been died in which 6 patients were in B group with B-T shunt.Only 2 patients were for the hybrid procedure.The ECHO showed the gradient across the pulmonary annular was 15-39 mm Hg [(23 ± 5) mm Hg (1 mm Hg =0.133 kPa)] after operation.The tricuspid insufficient had been siginificantly improved.There were 58 cases were preoperatively serious tricuspid insufficient.There were 11 cases and 13 cases were respectively for middle and mild to middle tricuspid insufficient postoperatively.Follow-up with 2-5 years,there were radical operation for 8 cases,one and half ventrical operation for 5 cases and BDG and Fontan operation for 2 cases.Conclusions.Conclusion BT shunt in common procedure and using a hybrid approach is more safe and feasible than traditional procedure for the patients with PA/IVS.Z vale and right ventricular growth were importment for procedure in the future including radical 、one and half ventrical and Fon tan operation.

Hybrid treatment of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in neonates / 中华实用儿科临床杂志

Objective To investigate the outcomes of hybrid treatment of pulmonary valve's balloon valvuloplasty via right ventricle without cardiopulmonary bypass for pulmonary atresia with intact ventricular septum(PA/IVS) and critical pulmonary stenosis(PS) in neonates.Methods The clinical features and data of 3 children(1 case of PA/ IVS and 2 cases of critical PS),who underwent hybrid treatment of balloon valvuloplasty,were recorded and retrospectively analyzed.All patients' right ventricle grow well,tricuspid valve Z score ＞-2,and underwent pulmonary valve balloon valvuloplasty via fight ventricle without cardiopulmonary bypass.Results All the 3 children underwent pulmonary valve balloon valvuloplasty via fight ventricle without cardiopulmonary bypass,and there was no death.The mean arterial blood oxygen saturation after operation went up,and there was no complication related to balloon valvuloplasty.The children were followed up for 3 to 6 months,and all the patients had mild pulmonary stenosis,but they did not need any treatment.Conclusions Pulmonary valvuloplasty with hybrid technique is effective in treatment of selective PA/IVS children(fused leaflets of pulmonary valve) and critical PS in neonates.Hybrid therapy may provide adequate pulmonary blood flow and promote further growth of tricuspid valve and right ventricular cavity with less trauma and more safety.

Prostaglandin E1 used as a pre-operative treatment for the neonates with transposition of great vessels with intact ventricular septum / 中华急诊医学杂志

Objective To study the therapeutic effects of prostaglandin E1 on the neonates with transposition of the great vessels with intact ventricular septum (TGV/IVS) retrospectively. Method From January 2004 to June 2009, 34 neonates with TGV/IVS were enrolled in this study. The pulse rate and oxygen saturation (SpO2) of patients were measured percutaneouly at admission. Lipo-prostaglandin E1 (Lipo-PGE1) was administered via peripheral vein with pumping infusion continuously after diagnosis by echocardiography in order to keep the ductus arteriosus (DA) patent. The dose and the time required for the Lipo-PGEl to produce effect were recorded. The changes of SpO2 before and after administration of Lipo-PGE1 were observed. The changes of DA's diameter detected by using echocardiography before and during the operation. Results In all patients the initial dose of Lipo-PGEl was 5 ng/( kg·min) except 3 patients whom larger dosed were required to give guided by the change of SpO2 with 10 ng/(kg·min) in two patients and 15 ng/(kg·min) in one patient. The time required for Lipo-PGE to produce the effect was 5-15 minutes in most infants with mean of (12 ± 3) minutes. The mean SpO2 of the patients measured at admission was (80.05±7.64)%, and it was (86.41±4.83)% two hours before operation (P < 0.05). The average diameter of DA was (0.37±0.08) cm at the time diagnosis and it was (0.51 ±0.15) cm during the operation. The adverse effects occurred in two patients and one of them had apnea and was treated mechanical ventilation. Conclusions Lipo-PGE1 given by continuous pumping infusion via peripheral vein in dose of 5 ng per kilogram per minute can maintainthe DA patency and promote the systemic oxygenation and perfusion, improving the circulation and oxygenation and correcting the acidosis until the plastic surgery performed. Most of the adverse effects of PGE1 are dose related.

Outcomes in Neonates with Pulmonary Atresia and Intact Ventricular Septum Underwent Pulmonary Valvulotomy and Valvuloplasty Using a Flexible 2-French Radiofrequency Catheter

PURPOSE: Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify the factors favorable for RPVV as the treatment of choice. MATERIALS AND METHODS: From May 2000 to January 2008, 6 patients with PAIVS were included in this retrospective study. They were aged 1 day to 90 days old. Study modalities included review of recordings of presentations and profiles of chest radiography, electrocardiography, echocardiography, and cardiac catheterization with angiography. Hemodynamic profiles from the echocardiography and the cardiac catheterization were analyzed. RESULTS: Echocardiography showed severe tricuspid regurgitation, membranous atresia of the pulmonary valve, intact ventricular septum, patent ductus arteriosus, and hypoplastic right ventricle in 6 patients. The pulmonary valve annulus were 4.2 to 6.9 mm in diameters, and those of the tricuspid valve were 7.1 to 10.1 mm. Elevated serum level of cardiac enzymes were found in 1 patient with ventriculocoronary communication (VCC). At cardiac catheterization, the ratio of systolic pressure of the right ventricle to that of the left ventricle ranged from 1.43 to 2.33 before RPVV, and from 0.54 to 1.15 after RPVV (p=0.027). The pressure gradients ranged from 76 to 136 mmHg before RPVV, and from 15 to 39 mmHg after RPVV (p=0.028). The echocardiographic gradients ranged from 16 to 32 mmHg within 24 hours after RPVV, and from 15 to 50 mmHg at the follow-ups. CONCLUSION: RPVV can be a treatment of choice for neonates with PAIVS, if there is patent infundibulum, no right-ventricular dependent coronary circulation, and adequate tricuspid valve and pulmonary valve.

Percutaneous Pulmonary Balloon Valvotomy and PDA Stent Insertion for Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum has continued to have a high mortality and morbidity. The initial palliation is commonly a pulmonary valvotomy to achieve right ventricular decompression with or without a systemic to pulmonary shunt. There are only a few publications reporting practical experience with ductal stenting in neonates with pulmonary atresia. We report a case of pulmonary valvotomy followed of PDA stent insertion for the treatment of pulmonary atresia with intact ventricular septum.

A Case of the Senning Procedure in a Patient with Transposition of the Great Arteries with Intact Ventricular Septum and Bicuspid Pulmonary Valvular Stenosis Associated with Pulmonary Hypertension / 日本心臓血管外科学会雑誌

We performed the Senning operation and pulmonary valvotomy in an 11-month-old baby with transposition of the great arteries (TGA) with an intact ventricular septum (IVS), and bicuspid pulmonary valvular stenosis associated with pulmonary hypertension (PH). Preoperative catheterization showed a pressure gradient (PG) between the left ventricle (LV) and main pulmonary artery (MPA) of 35mmHg, mean pulmonary artery pressure (MPAP) of 56mmHg, and pulmonary vascular resistance (PVR) of 11.2unit·m². The pure oxygen inhalation test showed a decrease in MPAP from 56 to 38mmHg, and a decrease in PVR from 11.2 to 5.5 unit·m². We could not perform lung biopsy to determine the surgical indications in terms of PH due to preoperative progressive congestive heart failure in this patient. Postoperative catheterization (28 days after the Senning operation) showed a decrease in PG between the LV and MPA to 8mmHg, and MPAP also decreased to 17mmHg. Two radical operations were possible in this patient. One was the arterial switch operation (ASO), and the other was the atrial switch operation, i. e. the Senning or the Mustard operation. We selected the Senning operation because there was the possibility that the new aortic valve might develop persistent stenosis and regurgitation after ASO and pulmonary valvotomy. The Senning operation may be an alternative in selected patients with TGA with IVS and pulmonary valvular stenosis.

Echocardiographic Parameters of Pulmonary Atresia with Intact Ventricular Septum(PA/IVS)

PURPOSE: To understand morphologic and hemodynamic variations in patients with pulmonary atresia with intact ventricular septum(PA/IVS), and to decide the best treatment modalities, we measured right ventricular volume, inflow, and outflow valvular annulus size in these patients and compared them with those of normal newborns. METHODS: Eight patients with PA/IVS diagnosed by echocardiography from January to December 2001 were enrolled in this study. Among the total eight patients, five were male and three were female. The mean age of patients was 6.9 days(1-34 days), and the mean body weight was 3,343 gm (2,970-4,000 gm). Ten fullterm newborn infants with sepsis or hyperbilirubinemia without heart disease were enrolled as a control group. Echocardiographic and Doppler studies using Acuson Aspen (7Mh probe) were recorded on super-VHS videotape and later on, with review mode. We measured volumes of right and left ventricles, aortic, pulmonic, mitral and tricuspid valvular annulus sizes using an installed program, and then these parameters were compared with those of the control group. RESULTS: Mean Z-value of tricuspid valvular annulus in PA/IVS was -3.69+/-2.80(-8.4--0.45), and tricuspid/mitral valvular annulus size ratio 0.68+/-0.15(0.43--0.84). The more the tricuspid/mitral valvular annulus size ratio, the more Z-value of tricuspid valvular annulus(P=0.003, r=0.885). Those patients who underwent pulmonary valvuloplasty(balloon or surgical) had a tendency toward larger volume of the right ventricle, more Z-value of pulmonic and tricuspid valvular annulus, and more tricuspid/mitral valvular annulus size ratio than those patients who underwent a shunt operation. CONCLUSION: Compared to a measurement of the volume of the right ventricle, measurements of tricuspid/mitral valvular annulus size ratio and Z-value of tricuspid valvular annulus may be easier and better parameters to decide the treatment method and to predict prognosis in PA/IVS patients.

Two Cases of Transposition of the Great Arteries with Intact Ventricular Septum Prenatally Diagnosed by Fetal Eechocardiography / 대한산부인과학회잡지

Transposition of the great arteries with intact ventricular septum (TGA IVS) is a cyanotic congenital heart disease with high neonatal mortality without early diagnosis. But TGA IVS is known to have good prognosis if treated by arterial switch operation (ASO) within 2 weeks after birth with early diagnosis. We diagnosed two cases of TGA IVS prenatally by fetal echocardiography. A case was diagnosed at 26th weeks of gestation, and the mother was transferred to a cardiac center for planned delivery. The baby had received ASO on postpartum 7th day, but he died of right ventricular failure on postoperative 8th day. The second case was diagnosed at 37th gestational weeks and was transferred to Asan medical center immediately after birth. And he is doing well without any treatment after ASO.