Clinical outcomes of the neonates with critical pulmonary stenosis: intrauterine versus postnatal transport.

Background: Pulmonary stenosis (PS) is one rare congenital heart disease (CHD) featuring obstruction of right ventricular outflow tract. Critical pulmonary stenosis (CPS) is neonatal PS having cyanosis and evidence of patent ductus arteriosus (PDA) dependency. There is limited data on the clinical outcomes of CPS with different modes of transportation. This study aimed to investigate clinical features and outcomes of CPS through the intrauterine transport (IT) and postnatal transport (PT). Methods: Single-center retrospective research was performed. Neonates with CPS were grouped into the IT group and PT group. Clinical characteristics and outcomes of the neonates were compared between the two groups. Results: Totally 110 neonates with PS were included in this study, 77 with CPS and 33 with non-CPS. In the infants with CPS, there were 53 and 24 in the IT and PT group respectively. Echocardiography showed that transvalvular pulmonary gradient (TVG) stayed lower in the IT group than that in the PT group {77.0 [interquartile range (IQR), 60.5-91.5] vs. 92.0 (IQR, 73.3-125.0) mmHg, P=0.006}. Levels of serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin I also remained lower in the IT group than those in the PT group [2,256 (IQR, 1,054-4,527) vs. 3,708 (IQR, 2,138-6,789) pg/mL, P=0.02; 0.020 (IQR, 0.011-0.034) vs. 0.042 (IQR, 0.027-0.072) ng/mL, P<0.001, respectively]. All infants with CPS received percutaneous balloon pulmonary valvuloplasty (PBPV) therapy in neonatal period and were discharged from the hospital. Length of hospital stay remained shorter in the IT group than that in the PT group [13.0 (IQR, 11.0-15.0) vs. 15.5 (IQR, 10.8-22.8) days, P=0.03]. Conclusions: IT and early management after birth could effectively reduce the severity of CPS before PBPV treatment and shorten the length of hospital stay among neonates suffering from CPS.

Anaesthesia management of interventional cardiac catheterisation for pulmonary atresia with intact ventricular septum and critical pulmonary stenosis: a retrospective analysis.

OBJECTIVE: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis usually have to undergo treatment in the neonatal period. Compared to traditional surgical intervention, catheter-based cardiac interventions may achieve similar or superior outcomes for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, there is limited literature on anaesthesia techniques, challenges, and risks associated with cardiac catheterisation in this population. METHODS: This article retrospectively analysed the clinical data of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis neonates who were treated with interventional cardiac catheterisation in our hospital from January 2015 to October 2022. Clinical outcomes considered were haemodynamic or pulse oxygen saturation instability, vasoactive requirements, prolonged intubation (>24 h postoperatively), and cardiovascular adverse events. RESULTS: A total of 63 patients met the inclusion criteria. All patients survived the intervention. Among the patients with critical pulmonary stenosis, 40 successfully received percutaneous balloon pulmonary valvuloplasty, while three patients received ductal stenting due to moderate right ventricular dysplasia at the same time. For patients with pulmonary atresia with intact ventricular septum, 17 of the 23 patients successfully underwent percutaneous pulmonary valve perforation and percutaneous balloon pulmonary valvuloplasty. Of these, five patients underwent ductal stenting due to unstable pulmonary blood flow. Three patients only underwent ductal stenting. In addition, three patients received hybrid therapy. CONCLUSIONS: There are various clinical techniques and risk challenges in the interventional cardiac catheterisation of neonatal pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, by mastering the physiological and pathophysiological characteristics of the disease, adequately preparing for the perioperative period, and predicting the procedure process and potential complications, anaesthesia and surgical risks can be effectively managed.

Fetal cardiac interventions: First-year experience of a tertiary referral center in Turkey.

AIM: To present the first-year experience of fetal cardiac interventions (FCIs) in a tertiary referral hospital and to evaluate the outcomes. METHODS: This retrospective study consisted of four pregnant women who underwent fetal pulmonary or aortic balloon valvuloplasty between November 2020 and June 2021. The procedures were performed with a percutaneous cardiac puncture under the ultrasonography guidance. Gestational age at intervention, procedural success, complications, and perinatal outcomes were evaluated. Procedural complications defined as fetal bradyarrhythmia requiring treatment, pericardial effusion requiring drainage, balloon rupture, and fetal death. The procedure was considered technically successful if the valve was dilated with a balloon catheter. Ultimately successful procedure was defined as the discharge of infants alive with biventricular circulation. RESULTS: A total of 5 FCIs attempted between 26 + 3 and 28 + 2 gestational weeks. While the procedure was technically successful in 2 cases with pulmonary stenosis, both attempts were unsuccessful in the fetus with pulmonary atresia. Although the procedure was technically successful in the patient with critical aortic stenosis, it ultimately failed. No fetal death occurred in our series and there were no procedure-related significant maternal complications. However, three interventions were complicated by fetal bradycardia and pericardial effusion necessitating treatment, and balloon rupture cropped up in one case. CONCLUSION: FCIs may lead to improving the likelihood of a biventricular outcome for selected fetuses. Careful selection of patients and centralization of experience are essential for obtaining favorable outcomes. Operators should be aware of procedural complications. Improved procedural techniques with a lower complication rate will be achieved through advanced medical technology and special balloon catheters.

Prediction of postnatal circulation in pulmonary atresia/critical stenosis with intact ventricular septum: systematic review and external validation of models.

OBJECTIVE: A favorable postnatal prognosis in cases of pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS-IVS) is generally equated with the possibility of achieving biventricular (BV) repair. Identification of fetuses that will have postnatal univentricular (UV) circulation is key for prenatal counseling, optimization of perinatal care and decision-making regarding fetal therapy. We aimed to evaluate the accuracy of published models for predicting postnatal circulation in PA/CS-IVS using a large internationally derived validation cohort. METHODS: This was a systematic review of published uni- and multiparametric models for the prediction of postnatal circulation based on echocardiographic findings at between 20 and 28 weeks of gestation. Models were externally validated using data from the International Fetal Cardiac Intervention Registry. Sensitivity, specificity, predictive values, area under the receiver-operating-characteristics curves (AUCs) and proportion of cases with true vs predicted outcome were calculated. RESULTS: Eleven published studies that reported prognostic parameters of postnatal circulation were identified. Models varied widely in terms of the main outcome (UV (n = 3), non-BV (n = 3), BV (n = 3), right-ventricle-dependent coronary circulation (n = 1) or tricuspid valve size at birth (n = 1)) and in terms of the included predictors (single parameters only (n = 6), multiparametric score (n = 4) or both (n = 1)), and were developed on small sample sizes (range, 15-38). Nine models were validated externally given the availability of the required parameters in the validation cohort. Tricuspid valve diameter Z-score, tricuspid regurgitation, ratios between right and left cardiac structures and the presence of ventriculocoronary connections (VCC) were the most commonly evaluated parameters. Multiparametric models including up to four variables (ratios between right and left structures, right ventricular inflow duration, presence of VCC and tricuspid regurgitation) had the best performance (AUC, 0.80-0.89). Overall, the risk of UV outcome was underestimated and that of BV outcome was overestimated by most models. CONCLUSIONS: Current prenatal models for the prediction of postnatal outcome in PA/CS-IVS are heterogeneous. Multiparametric models for predicting UV and non-BV circulation perform well in identifying BV patients but have low sensitivity, underestimating the rate of fetuses that will ultimately have UV circulation. Until better discrimination can be achieved, fetal interventions may need to be limited to only those cases in which non-BV postnatal circulation is certain. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Outcome Predictors in Catheter Interventions for Severe Right Ventricular Outflow Tract Obstructions.

Introduction & aim of work: Transcatheter treatment for critical pulmonary stenosis and membranous pulmonary atresia has become the gold standard of care in many centers. We aimed at evaluating the predictors of outcome in interventions for treatment of duct-dependent right ventricular outflow tract obstruction with intact interventricular septum. Subjects & methods: 68 cases with pulmonary atresia with intact interventricular septum (PA/IVS) and 50 cases with critical pulmonary stenosis (CPS), all younger than 3 months of age, were operated during the period of 10 years; excluding patients with tricuspid valve annulus Z-score smaller than -4, evidence of right ventricular-dependent coronary circulation or additional malformations. Results: Age, weight, body surface area as well as tricuspid & pulmonary valve Z-scores were significantly less in PA/IVS; right ventricular pressure was similar in both groups however procedural success and survival to hospital discharge was higher in the CPS group. Lower age, weight and body surface area were associated with procedural failure. Weight was the only predictor of procedural success; while weight and lower post-procedural right ventricular pressure were independent predictors for survival to hospital discharge. Post-procedural right ventricular pressure and length of stay were less in the CPS group. tricuspid and pulmonary valve annulus Z-scores were the only independent predictors of the post-procedural milrinone duration in PA/IVS. Conclusion: We advocate for the use of larger balloon/pulmonary annulus ratio, to achieve a lower right ventricular pressure not fearing excessive pulmonary regurgitation that might be beneficial for right ventricular growth; and for the combination with ductal stenting in borderline or bipartite right ventricles.

Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.

BACKGROUND: Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway. METHODS: PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair. RESULTS: Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5-62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs). CONCLUSIONS: Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.

Early outcomes of emergency operations in neonates with pulmonary atresia and intact ventricle septum / 中国小儿急救医学

Objective:To study the early outcomes of emergency operation employed with one of the prevalent surgical methods in neonates diagnosed as pulmonary atresia with intact ventricle septum(PA/IVS) and critical pulmonary stenosis(CPS), and to analyze the risk factors related to its early results.Methods:A retrospective analysis was conducted to collect the data of neonates suffered from PA/IVS and CPS from January 2016 to January 2020 in cardiothoracic surgery department at Shanghai Children′s Medical Center.According to their Z score, which reflects the development degree of right ventricle, the neonates received one of the relevant popular operations.Early outcomes were summarized and risk factors related to its early stage mortality were analyzed.Results:A total of 65 neonates were enrolled, including 27 CPS cases.They were operated on the basis of their respective Z scores, and also according to these data, the primary surgical procedure was chosen from the following methods: Blalock-Taussig(B-T) shunt, pulmonary valvulotomy and right ventricular outlet enlargement.Totally seven cases died, and mortality was 10.7%.Nine cases received re-operation in several following days because of severe hypoxemia and low cardiac output.All patients had mild improvement in oxygen saturation after operation and relied on large dose of inotropic agent.Multivariate Logistic regression analysis showed that B-T shunt and re-operation in early period were risk factors for death in neonates with PA/IVS and CPS.Conclusion:There is a higher mortality in neonates who received emergency operation for PA/IVS and CPS.Accurate assessment of the right ventricle development degree and selecting the corresponding appropriate surgical method is critical for the optimal result.B-T shunt and early stage re-operation are the risk factors for death in neonates with PA/IVS and CPS who received emergency operation.

Neonatal right mini thoracotomy for repair of critical pulmonary stenosis.

Over the last few decades, due to evolving surgical techniques and medical management, there has been a significant decrease in the rate of mortality and complications for neonates born with critical pulmonary valve stenosis. Median sternotomy is the standard approach; however, this longitudinal midline incision is invasive and leaves a significant scar. A right mini thoracotomy approach to this surgical repair decreases recovery time and the chance of possible future psychological distress from a visible median sternotomy scar. This is the first article to describe a right mini thoracotomy approach for critical pulmonary stenosis during the neonatal period.

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome.

OBJECTIVES: To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). METHODS: We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 (< 24 weeks), 2 (24-30 weeks) and 3 (> 30 weeks). RESULTS: 61/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6% of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44/53 (83.0%) survived. 24/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2 × 1.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (p < 0.001). Tricuspid insufficiency (TI)-Vmax > 2.5 m/s was present in BV outcome group in75.0% (18/24) vs. 14.3% (2/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax < 2.5 m/s, TV/MV ratio < cutoff) to a score, non-BV outcome was correctly predicted when > 1 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4/9 (44.4%) and 5/14 (35.7%) in our interval 2 + 3 with predicted non-BV outcome would have been candidates for intervention. Two (1 × intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention. CONCLUSION: TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.

Serum N-Terminal Pro-B-Type Natriuretic Peptide as a Biomarker of Critical Pulmonary Stenosis in Neonates.

Objectives: To determine the efficacy of serum N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels in predicting critical pulmonary stenosis (CPS) in neonates. Methods: All neonates with pulmonary stenosis (PS) admitted to the neonatal intensive care unit of Xinhua Hospital from October 2014 to December 2020 were retrospectively reviewed. Infants with serum NT-proBNP levels measured within 48 h after birth were enrolled and divided into CPS and non-CPS groups. Serum NT-proBNP levels and cardiac Doppler indices were compared between the two groups. Correlations were determined using the Spearman's rank correlation coefficient. Receiver operator characteristic curve analysis was used to explore the predictive value of NT-proBNP for identifying neonatal CPS. Results: Among 96 infants diagnosed with PS by echocardiography, 46 were enrolled (21 and 25 in the non-CPS and CPS groups, respectively). Serum NT-proBNP levels were significantly higher in the CPS group than in the non-CPS group [3,600 (2,040-8,251) vs. 1,280 (953-2,386) pg/ml, P = 0.003]. Spearman's analysis suggested a positive correlation between Ln(NT-proBNP) levels and the transvalvular pulmonary gradient (r = 0.311, P = 0.038), as well as between Ln(NT-proBNP) levels and pulmonary artery velocity (r = 0.308, P = 0.040). Receiver operating characteristic curve analysis showed that a cutoff serum NT-proBNP level of 2,395 pg/ml yielded a 66.7 and 78.9% sensitivity and specificity for identifying CPS, respectively. The area under the curve was 0.784 (95% CI, 0.637-0.931). A positive correlation was found between Ln(NT-proBNP) and length of hospital stay (r = 0.312, P < 0.05). Conclusion: Serum NT-proBNP level was positively correlated with PS severity and could be used as a biomarker to identify CPS in neonates.

Analysis of interventional treatment for neonatal critical pulmonary stenosis / 中华实用儿科临床杂志

Objective:To evaluate the effectiveness of interventional treatment for neonatal critical pulmonary stenosis(NCPS).Methods:Clinical data of 12 neonates with NCPS who received percutaneous balloon pulmonary valvuloplasty (PBPV) from January 2016 to December 2019 in Department of Cardiology, Shenzhen Children′s Hospital were summarized and analyzed.The collected data included transthoracic echocardiography (TTE), percutaneous oxygen saturation (SPO 2), relevant data on interventional surgery, and follow-up results. Results:All 12 neonates with NCPS received PBPV successfully.The postoperative pressure difference between the right ventricle and the pulmonary artery ranged from 8 to 35 mmHg[(20±7) mmHg, 1 mmHg=0.133 kPa]. The postoperative SPO 2 ranged from 74%-100%[(93.0±5.9)%]. Three neonates with NCPS received Blalock-Taussig (B-T) shunt.One neonate with NCPS developed supraventricular tachycardia during the operation.There was no death for these 12 neonates with NCPS. Conclusions:Interventional treatment of neonates with NCPS could achieve a better effect and be employed as the first treatment option.Some neonates with NCPS would require cardiac B-T shunt or patent ductus arteriosus stent implantation.

Balloon pulmonary valvuloplasty in neonates with critical pulmonary stenosis: Jugular or femoral.

BACKGROUND: Critical pulmonary stenosis (PS) is one of the life-threatening congenital heart diseases which present during the neonatal period with cyanosis. Surgical valvotomy was once the procedure of choice for critical PS; however, balloon pulmonary valvuloplasty (BPV) has now become the standard treatment. Although the procedure is usually simple, crossing the pulmonary valve from the femoral vein can be difficult, especially when severe tricuspid regurgitation and right atrium dilatation are present. In such patients, the maneuver can be simplified by using the right internal jugular vein approach. However, many operators are reluctant to use this approach because of unfamiliarity with the technique, potential complications, and paucity of reports. Until now, there is no literature describing BPV using the transjugular approach in neonates, also none directly comparing the transfemoral and transjugular approaches. OBJECTIVE: We compared transjugular with the transfemoral approach in terms of procedure time and complications. MATERIALS AND METHODS: This was a retrospective cohort study. Participants were neonates with critical PS undergoing BPV in the National Cardiovascular Center Harapan Kita from 2013 to 2018. RESULTS: Of 15 neonates undergoing BPV, eight were done using the transjugular approach and seven using the femoral approach. Mean age and weight in both groups was similar. In all eight patients using transjugular approach, crossing the pulmonary valve was consistently quick and easy. The total procedural time, pulmonary crossing time, and fluoro time was significantly shorter using the transjugular approach (65 ± 8 vs. 108 ± 17.8 min, P < 0.05; 22 ± 3.1 vs. 45 ± 14.8 min, P < 0.01; 29 ± 13 vs. 67 ± 35 min, P < 0.05). There were no complications relating to vascular access. Moreover, the BPV procedure itself demonstrated comparable results in both groups. CONCLUSION: BPV using the transjugular approach is safe and effective to relieve critical PS in neonates compared to the transfemoral approach.

Integrative Perinatal Management Enhanced the Advantage of Prenatal Diagnosis on Critical Pulmonary Valve Stenosis: An Observational Preliminary Study.

Background: Percutaneous balloon valvuloplasty (PBPV) is recommended as a first-choice treatment for critical pulmonary stenosis (CPS). A concept of perinatal integrative management has been developed. Unfortunately, the evidence on the advantage of integrative management for CPS during the perinatal period is absent. Methods: Single-center, observational, preliminary research has been developed, and three groups have been enrolled. There were 42 children with CPS enrolled for this study between January 2014 and December 2017 in our center, and their follow-up duration is at least 1 year. Three groups were set up: the integrative perinatal management group (group I), who received prenatal diagnosis with perinatal management to maintain circulation and an optimized PBPV procedure; the prenatal diagnosis group (group PR), who received a diagnosis of pulmonary stenosis before birth without any monitoring and perinatal management; and the postnatal diagnosis group (group PO), who received the CPS diagnosis after birth. Result: There were 13 patients enrolled in group I, 11 babies enrolled in group PR, and 18 cases included in group PO. Integrative management helped to put the timing of PBPV in advance. The age for PBPV in group I was 9.38 ± 5.58 days, and groups PR and PO were 24.54 ± 4.87 and 49.11 ± 9.50 days, respectively. The average peak transvalvular gradient (PGs) of the perinatal management group (group I) and prenatal diagnosis group (group PR) remained at a stable level. However, the average PGs of group PO were progressively elevated during follow-up. Moreover, the follow-up data from group I revealed an advantage in RV development and functional restoration. There was no difference among the three groups in the ratio of reintervention and postoperative moderate pulmonary regurgitation during 1-year follow-up (p >0.05). Conclusion: Prenatal diagnosis helps to improve the outcomes of PBPV. Moreover, perinatal integrative medical management enhances the advantage of prenatal diagnosis. However, this research is still a small-size cohort study, and the limited population number and follow-up duration were the major limitations to expand the conclusions.

[Percutaneous management of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis]. / Manejo percutáneo de la atresia pulmonar con tabique interventricular íntegro y la estenosis pulmonar crítica.

INTRODUCTION: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae. PATIENTS AND METHODS: We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes. RESULTS: 51 patients were admitted. A total of 9 patients (17.6%) died during the followup. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of follow up in the 42 survivors was 8.9 years (1-16). The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae. CONCLUSIONS: Coronary artery to right ventricle fistulae may not be a contraindication for biventricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class.

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.

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.

Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity-time integral (VTI) and maximal velocity (Vmax) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde Vmax was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.

Intervention in Patients with Critical Pulmonary Stenosis in the Ductal Stenting Era.

We aimed to assess early and midterm outcomes of balloon valvuloplasty (BVP) procedure in patients with critical pulmonary stenosis (CPS) and to describe the predictors of the need for additional pulmonary flow and reintervention in this subgroup of patients. From 2005 to 2014, 56 neonates were diagnosed with CPS and were included in this study. All echocardiographic, catheterization and angiographic data obtained prior to the initial BVP and at follow-up were reviewed. BVP was successful in 55 neonates (98 %). Twenty-one neonates needed pulmonary blood flow augmentation after BVP (38 %). Ductal stenting (DS) was performed in 20. The patients' mean tricuspid valve (TV) annulus diameter was 10.4 ± 2 mm, and the Z score was -1.29 ± 1 (-3.7 to 0.78). The mean pulmonary valve (PV) annulus diameter was 6 ± 0.9 mm, and the Z score was -1.74 ± 1 (-4.34 to 0.05). A transcatheter or surgical reintervention was performed in 11 patients. A TV Z score < -1.93 SD predicted the need for pulmonary blood flow augmentation after a successful BVP, with a sensitivity of 63.2% and a specificity of 84.4%. A PV Z score < -1.69 SD predicted the need for pulmonary flow augmentation, with a sensitivity of 74 %. The presence of bipartite RV was found to be a significant predictor of the need for reintervention (odds ratio 9.6). Our study showed the excellent immediate outcomes of BPV and DS in a pure cohort of patients with CPS. Prophylactic DS in selected cases seems reasonable and safe.

Evaluating the risk factors of reintervention of neonates with PA/IVS and CPS/IVS after PBPV as initial intervention method.

OBJECTIVES: To analyze the clinical features, immediate and short-term outcome of percutaneous balloon pulmonary valvuloplasty (PBPV) of neonates with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis with intact ventricular septum (CPS/IVS) who underwent PBPV as initial intervention methods to evaluate the risk factors of reintervention. METHOD: Thirty-eight neonates with PA/IVS or CPS/IVS admitted to Shanghai Xinhua Hospital between June 2009 and November 2014 who underwent PBPV as initial procedures were respectively studied. We analyzed their clinical features, the immediate and short-term outcome of PBPV, and evaluated the risk factors for reintervention. RESULTS: Thirty-eight neonates with PA/IVS and CPS/IVS who underwent PBPV as initial procedures were included in our study with a mean age of 13±8 days. Among the 12 patients who underwent reintervention, 7 patients had PA/IVS (64%) and 5 had CPS/IVS (36%). Patients with PA/IVS (p=0.005), small pulmonary valve diameter (p=0.035), and bad pulmonary valve development were important risk factors. The immediate and short-term outcome of PBPV was better in patients who did not need reintervention, and the peak-to-peak pulmonary transvalvular gradient in the 1st month after PBPV had the best capability in predicting reintervention with help of receiver operating characteristic curve and the logistic regression analysis. Days of prostaglandin E1 (PGE1) infusion after PBPV >5 days was also found to be a risk factor for reintervention. CONCLUSION: The short- and mid-term outcome of pulmonary valve perforation and PBPV in neonates with PA/IVS and CPS/IVS was favorable. Risk factors include PA/IVS, small pulmonary valve, bad short-term pulmonary valve development, the peak-to-peak pulmonary transvalvular gradient in the 1st month after procedure >42mmHg, and PGE1 infusion >5 days.

The impact of prenatal diagnosis on treatment and prognosis of neonatal pulmonary atresia with intact ventricular ;septum and critical pulmonary stenosis with intact ventricular septum / 临床儿科杂志

Objective To explore the impact of prenatal diagnosis on the early treatment and short and medium term outcome of neonatal pulmonary atresia with intact ventricle septum (PA/IVS) or critical pulmonary stenosis with intact ventricle septum (CPS/IVS). Methods According to the case-control method, twenty-eight neonates with (PA/IVS) or (CPS/IVS) who had percutaneous pulmonary balloon valvuloplasty (PBPV) surgery indications, were divided into the prenatal diagnosis group (n?=?15) and the postnatal diagnosis group (n?=?13). The prenatal diagnosis group was diagnosed in fetal period and the intervention program was since developed . The postnatal diagnosis group was referred from other hospitals, and the intervention program was developed after conifrmation of the diagnosis. All the neonates accepted the PBPV surgery after the hemodynamic parameters stable. All neonates were followed-up at 1 month, 3 months, 6 months, 1 year, and 2 years after surgery. Clinical situations, echocardiography results, and interventional cardiology measurements were compared between two groups. Result The average age and weigh was 7.53?±?3.18 days and 3102.32?±?708.40 g respectively at the time of PBPV surgery in 28 neonates. Among them, 9 neonates were PA/IVS and 19 neonates were CPS/IVS. The mean follow-up time was 18.8?±?5.22 months and there were no death. The ages at admission and at the ifrst treatment were signiifcantly younger in the prenatal diagnosis group than those in the postnatal diagnosis group (P??0.05). Conclusion Prenatal diagnosis is helpful for the early intervention in neonates with PA/IVS and CPS/IVS, and can reduce the complications after surgery.