Critical Pulmonary Valve Stenosis Research Articles

Enormous Enlargement of Right Atrium Occurred Late After Surgical Dilation of Pulmonary Valve Stenosis: Case Report.

Congenital pulmonary valve stenosis is a relatively common congenital defect, often related to abnormal development and fusion of pulmonary valves. Critical pulmonary valve stenosis should be treated, early in life, before dilation of right chambers, and occurrence of arrhythmias. We report one case of enormous enlargement of right atrium revealed by typical atrial flutter which occurred very late, in 48-year-old man, who underwent surgical dilation of pulmonary valve stenosis at eleven years old, but without repairing dilated tricuspid annulus. Despite successfully ablation of atrial flutter in our cathlab, the patient developed atrial fibrillation, because of an enormous enlargement of right atrium and massive tricuspid regurgitation.

Analysis of echocardiographic features and postnatal management of fetal pulmonary valve stenosis

Objective: To investigate the echocardiographic features, consistency of diagnosis between fetal and postnatal periods and postnatal clinical outcomes of fetal pulmonary valve stenosis (PS) with different degrees. Methods: This study was a retrospective cohort study comprising 108 cases of fetal PS diagnosed during the fetal period and followed up postnatally at Xinhua Hospital, Shanghai Jiaotong University School of Medicine from November 2012 to February 2023. Echocardiographic characteristics, including morphological and hemodynamic features were collected for all fetuses who were then were followed up to at least 6 months after birth. One-way analysis of variance and Kruskal-Wallis test were used to compare the differences in the echocardiographic features among fetuses with different degrees of PS. Subsequently, McNemar test was used to assess the consistency of diagnosis between the fetal and postnatal periods. Furthermore, Logistic regression analysis was applied to explore the risk factors for neonatal intervention in fetuses with moderate PS and the receiver operating characteristic (ROC) curve was utilized to ascertain the optimal cut-off value for continuous variables. Results: The age of the mothers of the 108 fetuses at the initial assessment was (30.8±4.0) years, and the gestational age was 26.5 (24.6, 30.0) weeks. The fetuses were categorized into mild (17 cases), moderate (49 cases), and severe groups (42 cases) based on the initial echocardiographic features. Mild PS was characterized by valve thickening and hyperechogenicity combined with systolic flow acceleration or dilation of main pulmonary artery. Moderate PS exhibited both restricted valve motion and a colorful blood flow pattern at the valve orifice. The peak flow velocities of fetuses with moderate and critical PS were notably higher than those in the mild group ((2.66±0.86) and (2.77±1.30) vs. (1.43±0.59)m/s, F=14.52, P<0.001). In critical PS, all cases showed retrograde ductal flow, with a significantly higher proportion of a small right ventricle compared to the mild and moderate PS (42.9% (18/42) vs. 0 and 2.0% (1/49), χ2=31.73, P<0.001). The proportion of severe tricuspid regurgitation was also higher (35.7% (15/42) vs. 0 and 10.2% (5/49), χ2=36.94, P<0.001). Compared to mild and severe PS, the consistency of diagnosis between fetal and postnatal periods in moderate PS was lower (40.8% (20/49) vs.13/17 and 80.3% (35/42), χ2=12.45, P=0.006). The systolic flow velocity was identified as an independent risk factor for neonatal intervention in fetuses with moderate PS (OR=7.21, 95%CI2.11-24.62). A flow velocity of ≥2.18 m/s in second trimester and ≥3.15 m/s in third trimester indicated the necessity of neonatal intervention for fetal moderate PS. Among the 108 fetuses, 68 underwent surgical intervention and all survived. Additionally, 39 fetuses were regularly followed up. A sole non-surgical fatality occurred, leading to a 6-month survival rate of 99.1% (107/108). Conclusions: Various degrees of fetal PS demonstrate distinctive morphological and hemodynamic alterations in echocardiography. The disparity in severity between the postnatal and fetal stages requires ongoing monitoring for fetal PS. The prognosis for fetal PS is generally favorable.

Impact of fetal pulmonary valvuloplasty in in-utero critical pulmonary stenosis: A systematic review and meta-analysis

BackgroundUntreated critical pulmonary stenosis may develop into pulmonary atresia with intact ventricular septum, which is associated with a high risk of morbidity and mortality both in fetuses and neonates. In this meta-analysis, we sought to discover the potential of fetal pulmonary valvuloplasty that might affect patients' survival compared to other available procedures. MethodsThis systematic review and meta-analysis were conducted based on the PRISMA guideline. The authors thoroughly searched the recognized and potential interventions for PA-IVS, including FPV, total ventricular repair, and palliative procedures. The primary outcome was the mortality rate. We used R software (version 4.1.3) to calculate the overall proportion using the random-effects model of proportional meta-analysis. ResultsThe FPV procedure was performed at a mean gestational age of 26.28 weeks (95%CI: 24.83–27.73) and was successful in 87.6 % (95 % CI: 78.3–96.3 %) of patients. A total of 52.9 % patients attained biventricular circulation postnatally (95 % CI: 31.2–74.7 %). Successful FPV was associated with a slightly higher overall mortality rate [periprocedural death 4.7 % (95%CI: 0–10.7 %) and postnatal death 8 % (95%CI: 3–13 %)] compared to the three currently available definitive therapies, namely the Fontan procedure [10 % (95%CI: 4–17 %)], 1.5V repair [11 % (95%CI: 5–17 %), and 2V repair [8 % (95%CI: 1–15 %)]. ConclusionFPV can potentially increase the likelihood of biventricular circulation in fetuses with critical pulmonary valve stenosis.

Development of PR Restenosis after Percutaneous Balloon Pulmonary Valvuloplasty

Background: Pulmonary stenosis is not an uncommon congenital heart disease in our population. Various age groups present with severe pulmonary stenosis require intervention either surgical or percutaneous catheter-based. Often lifesaving surgical intervention is not feasible considering procedural risk, the patient’s physical condition, and young age. Percutaneous balloon pulmonary valvuloplasty is safe, effective, almost painless, economical, and less hospital stay in any age group with immediate and long-term success. This study aimed to analyze the rate of development of (Pulmonary Regurgitation) PR restenosis after Percutaneous Balloon Pulmonary Valvuloplasty (PBPV). Material Methods: This hospital-based prospective observational study was conducted in the Department of Paediatric Cardiology Combined Military Hospital (CMH), Dhaka from January to December 2021. 0-18 years aged 50 population with PS who were referred to the Paediatric Cardiology Department of CMH Dhaka during the study period were selected as study subjects as per inclusion criteria. The purposive Sampling technique was used in this study. Data were collected with a predesigned standard data collection sheet. Statistical significance was set as a 95% confidence level at a 5% acceptable error level. Categorical variables were expressed as frequency and percentages. Mean, standard deviation, and/or medians with interquartile ranges were used to express the continuous variables. Paired t-test was performed in categorical comparison. A p-value of &lt;0.05 was considered statistically significant. Collected data were analyzed using SPSS Version 24. Written informed consent was taken from every parent. Ethical clearance was taken from the Ethics Review Committee of CMH Dhaka for conducting the study. Results: 50(6.39%) had isolated valvular PS. The international standard for the prevalence of isolated PS is 8-10% which is very close to this study’s result. In this study, male predominance was found with the male-female ratio of 3:2 though there was no gender dominance found in other studies. Out of 50 cases, 3(6%) are neonates,7(14%) are infants, 27 (54%) are 1-5 years aged, and 13(26%) are more than 5 years aged. A total of 13(26%) cases developed PR, and all 13 patients developed trivial PR on D1 echo, out of them 1 patient developed mild PR on D30 Echo but in D90 and D180 Echo patients persisted the same mild PR. Out of 50 cases, 46(92%) were successful and 4 cases were procedure failed. None of the successful cases developed restenosis. Out of 4 procedure failed cases 1 patient developed restenosis on D30 follow up and 2 patients develop restenosis on the D90 follow-up. At D180 follow up none of the patients developed restenosis. All the restenosis cases had dysplastic pulmonary valves. One patient’s PS gradient persisted at 30-50 mm of Hg throughout the follow-up period. Overall restenosis in 180-day follow-up is 3(6%) and the remaining 47(94%) cases lead a healthy life in 6 months follow-up. Reballooning was done immediately after diagnosis of restenosis in stenosed cases. Conclusion: Immediate and intermediate-term (6 months) follow-up with Doppler echocardiography showed excellent outcomes in all cases except 3 (6.0%) restenosis cases and 13 (26.0%) insignificant PR cases. The baby who all had restenosis, had the risk factor, like- dysplastic PV. It may be concluded that balloon valvuloplasty is the procedure of choice for moderate, severe, and critical pulmonary valve stenosis.

English Case report: Succesfully Management of Critical Pulmonary Stenosis in a 12-Month-Old Boy By percutaneous transluminal balloon valvuloplasty

Abstract&#x0D; Critical pulmonary stenosis (PS) is used in infants born with very severe narrowing valves and requires treatment soon after birth. At four months old, an A-12-month-old boy was diagnosed with critical pulmonary stenosis but still successfully managed by percutaneous transluminal balloon valvuloplasty (PTBV) with satisfactory results without serious complications. Since he was born, cyanosis was seen at his lips and fingertip, with oxygen saturation around 60% until 70%. Echocardiography showed critical pulmonary stenosis, atrial septal defect (ASD), and patent ductus arteriosus (PDA). Percutaneous transluminal balloon valvuloplasty (PTBV) is accepted as the treatment of choice for critical pulmonary valve stenosis in many centers worldwide with significant results. After PTBV, he experienced improvement condition as no cyanosis was observed and oxygen saturation was 96%. This case was our first PTBV intervention case in our hospital and gave satisfactory results, although the intervention was delayed due to our limited resources before. &#x0D; Keywords: cyanosis, critical pulmonary stenosis, percutaneous transluminal balloon valvuloplasty

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.

Prenatal Qualification for Postnatal Balloon Valvuloplasty in Isolated Critical Pulmonary Valve Stenosis Based on 10 Cases From a Tertiary Centre of Fetal Cardiology

Prenatal Qualification for Postnatal Balloon Valvuloplasty in Isolated Critical Pulmonary Valve Stenosis Based on 10 Cases From a Tertiary Centre of Fetal Cardiology

Pattern of Intervention in Congenital Heart Disease: Single Centre Analysis of Cases over Five Years in Bangladesh

Introduction: In Bangladesh, 25/1000 cases of neonates are born with mild to severe type of congenital heart disease. Significant numbers of cases from new born to adult age group are reporting to OPD clinics with broad spectrum of congenital and grown up untreated heart diseases. This study will cover pattern of diseases among cases selected for cardiac catheterization and intervention in study group in a cardiac hospital with limited facility for children.&#x0D; Objectives: To analyze all the cases who had cardiac catheterization and intervention in said period and to see the outcome.&#x0D; Materials and Methods: It is a retrospective study conducted in a cardiac hospital from December 2014 to December 2019. All cases of cardiac catheterization and interventions were included in the study. Pattern of diseases, interventions, management and outcomes were analyzed.&#x0D; Results: Out of 1179 cases, 44.87% were male and 55.13% were female. Two hundred fifty four cases(21.54%) were in 0-1 year age group, 44.02% were in &gt;01-05 year age group, 18.66% cases were in &gt; 5 to 10 year , 8.48% cases were in &gt;10 to 20 years, 4.83% cases were in&gt; 20 to 30 years and 2.46% cases were in more than 30 year age group. Among trans-catheter closure patent ductus arteriosus (PDA) was the commonest type of intervention (44.68%), followed by atrial septal defect (ASD) (25.20%), ventricular septal defect (VSD) (11.83%) and balloon valvoplasty (BVP) for pulmonary stenosis (11.63%). Neonatal interventions were minimum as the coronary care unit used as post cathward had limited facility for neonatal care. PDA stenting was performed in 0.81% cases and BAS in 0.91% cases. Many patients underwent more than one intervention at a time; most common was ASD device closure and pulmonary valvoplasty. Cardiac Catheterization studies were minimum in this series and most of the cath study cases were VSD or/with other combinations (4.48%). Among infants (254), PDA device closure was the commonest intervention (64.56%) followed balloon valvoplasty for PS (15.35%) and balloon dilation of Coarctation on aorta (7.87%). Among implants, most commonly used were 8/6 mm (14.59%) Amplatzer Duct occluder (ADO), 6/4 ADOII, MF-Konar (9.79%) device and most common balloon used for valve or vessel dilation was 10x3 mm Tyshak II (3.26%). Only one mortality was noticed in a case of double intervention of critical Aortic and pulmonary valve stenosis.&#x0D; Conclusion: Commonest and established interventions were performed in this study other than few life saving interventions in neonates and outcome was excellent. Skilled team of pediatric cardiologist and technician in catheterization laboratory can bring success at the end of the day in spite of challenges.&#x0D; Journal of Armed Forces Medical College Bangladesh Vol.15 (1) 2019: 3-9

Balloon valvuloplasty for critical pulmonary valve stenosis in a newborn

Abstract Critical pulmonary valvar stenosis causes cyanosis and can lead to potentially lethal in newborn infants. Following initiation of Prostaglandin infusion, percutaneous balloon pulmonary valvuloplasty (BPV) is considered to be the treatment of choice. The percutaneous femoral venous approach is the most preferred route for BPV in most cardiac centers. We present the case of a 5-day-old female infant with critical pulmonary valvar stenosis who successfully underwent BPV through the femoral venous access. The narrowed pulmonary valve was significantly improved after intervention. This was the first case at our center that was undertaken BPV at the earliest postnatal day.

Right Ventricular Growth and Function After Balloon Valvuloplasty for Critical Pulmonary Valve Stenosis in Infants and Neonates.

Little data are published about right ventricular (RV) growth and function in infants and neonates after balloon pulmonary valvuloplasty (BPV) for critical valvular pulmonary stenosis (PS). We aimed to assess the RV growth and function during 1year after BPV for critical valvular PS in neonates and infants. A total of 41 infants and neonates with isolatedcriticalvalvular PSwho underwent BPV from August 2016 to Sep 2018 were enrolled in the study. Complete Echocardiographic examination was performed to all infants before, 1week, 1month, 3months, 6months, and 1year after BPV. The RV systolic pressure and transvalvular pulmonary pressure gradient significantly decreased 1week after BPV and continue to decrease more slowly during the period of follow-up. During follow-up, RV end-diastolic dimension increased significantly, whereas RV anterior wall thickness significantly decreased. The mean diameters of pulmonary valve annulus and tricuspid valve annulus significantly increased after BPV over the period of follow-up. RV systolic and diastolic dysfunction improved significantly as reported by reduced RV Tei index and tricuspid annular systolic velocity and increased RV E/A ratio. RV growth and function were significantly improved after BPV for critical PS in infants and neonates and catch-up growth of right ventricular structures occurred after 6months of BPV.

Long-term results of percutaneous balloon valvuloplasty in neonatal critical pulmonary valve stenosis: a 20-year, single-centre experience.

We carried out a retrospective study of all consecutive newborns with critical pulmonary valve stenosis subjected to percutaneous balloon valvuloplasty at a single centre, between 1994 and 2014, to assess its immediate and long-term safety and efficacy. A total of 24 neonates presented with critical pulmonary valve stenosis. The mean diameter of the pulmonary annulus was 7 mm (±1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment. Percutaneous balloon valvuloplasty was performed at a mean age of 4.0±4.3 days using, on average, a balloon-to-pulmonary annulus ratio of 1.18 mm (with a range from 0.9 to 1.43). Immediate success was achieved in 22/24 patients (92%) with a reduction in the pulmonary transvalvular peak gradient (p<0.05) and in the right ventricle/systemic pressure ratio (p<0.05). There was one death (4%) 6 days after the procedure, and 29.2% of them had transient rhythm complications. For a mean follow-up time of 8.4 years, the re-intervention rate was 42.9%. In total, 14 re-interventions were performed in nine neonates, including surgery in six. Freedom from re-intervention was 50% at 8 years and 43% at 10 and 15 years. This series, to the best of our knowledge, has had the longest follow-up of neonates with critical pulmonary valve stenosis. Percutaneous balloon valvuloplasty is a safe and effective treatment, and in our study 75% of the patients were exclusively treated using this technique.

299 - Critical pulmonary valve stenosis of the newborn: early and long term outcome

299 - Critical pulmonary valve stenosis of the newborn: early and long term outcome

Long-Term Outcomes of Balloon Valvuloplasty for Isolated Pulmonary Valve Stenosis.

To evaluate the long-term cumulative incidence of ≥ moderate pulmonary regurgitation (PR) and re-intervention following balloon pulmonary valvuloplasty (BPV). While BPV for pulmonary valve stenosis (PS) relieves obstruction acutely, long-term outcomes are not well documented. Between 1982 and 2002, 211 patients had BPV for isolated PS. Follow-up data were available for 103 patients. Cumulative incidence of ≥ moderate PR and re-intervention was evaluated and risk factors for ≥ moderate PR assessed in univariate and multivariable analyses. Median age at BPV was 0.7years (range 1day-42.2years); peak catheter gradient was 65mmHg (range 31-169mmHg); 23% had critical PS. Sixty-two patients had a recent echocardiogram with median follow-up 15.1years (range 10.1-26.3years); 60% had ≥ moderate PR. Three patients had pulmonary valve replacement following BPV due to symptomatic severe PR. In univariate analysis, critical PS, younger age, smaller BSA, and smaller pulmonary annulus at the time of BPV, as well as greater baseline PS gradient by catheterization, were associated with ≥ moderate PR (all p<0.05). In multivariable analysis, only BSA<0.3m2 was independently associated with ≥ moderate PR (adjusted odds ratio 6.4, 95% confidence interval 1.2-33.6). In the largest study to date of > 10-year outcomes following BPV, 60% of patients with available follow-up data developed ≥ moderate PR. Few patients had pulmonary valve replacement. Patients with lower BSA at the time of BPV were more likely to have greater PR at late follow-up.

English

English

EP08.08: Critical pulmonary valve stenosis in dichorionic and diamniotic twins: prenatal diagnosis, pregnancy outcomes and postnatal development.

EP08.08: Critical pulmonary valve stenosis in dichorionic and diamniotic twins: prenatal diagnosis, pregnancy outcomes and postnatal development.

Balloon valvuloplasty for critical pulmonary valve stenosis in newborn: A single center ten-year experience

Critical pulmonary stenosis (CPS) represents an emergency in neonates. Percutanous balloon pulmonary valvuloplasty (BPV) is now the first therapeutic option. The study present our experience between April 2003 and June 2013. We analyzed 72 patients with CPS and they were followed up from 18months to 9years. BPV was accomplished in 68 neonates. Arterial oxygen saturation significantly elevated after the procedure. There were significant reductions in peak-to-peak pressure gradient across the pulmonary valve. 2 patients had hemopericardium. PGE1 was continued because the right ventricle showed a dynamic RV outflow tract obstruction (RVOTO) in 7 patients. Two patients had PDA stent placement. Follow-up revealed a mean transpulmonary systolic gradient of <35mmHg. 7 patients required a second balloon dilatation with good results. These findings suggest that BPV is relatively safe and effective in neonates with CPS.

28. Critical pulmonary valve stenosis: Medical management beyond balloon dilation

Background Neonates with critical pulmonary valve stenosis (PVS) tend to remain oxygen dependentpost successful balloon valvuloplasty. Phentolamine and captopril were observed by us to improve oxygen saturation. Objective Describe the role of phentolamine and captopril in the treatment of these category of patients. Methods In a retrospective cohort study, 28 neonates with critical PVS underwent balloon valvuloplasty. Twenty remained oxygen or/prostaglandin (PGE)-dependent post-interventionand were treated with phentolamine or captopril accordingly. Oxygen saturation was monitored pre and post intervention and following treatment. Results Mean age and weight was 25.2 days and 3.1 kg. Before balloon dilation 18/20 (90%) neonates were on prostaglandin while after the procedure only 6/18 patients required it. A total of 20 patients required oxygen after intervention. Nine patients (45%) werestarted on phentolamine. One with severe infundibular stenosis did not respondfavorably. Eleven patients (55%) were started directly on captopril. After introducing phentolamine or captopril, PGE could be discontinued after a mean time of 15.9 h and mean oxygen saturation increased from 76.7% to 93.0%. Conclusion Phentolamine and/or Captopril have a therapeutic role in neonates with critical PVS who remain oxygen dependent after balloon dilation. Both medicationslead to vasodilatation of pulmonary and systemic vascularity. They facilitate inflowto the right ventricle. Right to left shunt across a PFO or/ ASD minimizesand saturation improves leading to a significantreduction in length of hospitalization.

Restrictive right ventricular performance assessed by cardiac magnetic resonance after balloon valvuloplasty of critical pulmonary valve stenosis.

Little data are published about right ventricular diastolic performance in patients with critical pulmonary valve stenosis after balloon pulmonary valvuloplasty thus far. A total of 44 patients with isolated critical pulmonary valve stenosis who had undergone balloon valvuloplasty with haemodynamic recordings were enrolled to the study; 33 patients who came for follow-up underwent further imaging by echocardiography after 6 months and their right ventricular functional parameters were compared with 33 control patients of the same age and sex. Out of 33 patients, 21 underwent cardiac MRI with late gadolinium enhancement to assess the presence of right ventricular fibrosis. The right ventricular systolic pressure (p<0.0001) and right ventricular outflow tract gradient (p<0.0001) decreased acutely (p<0.0001) after balloon valvuloplasty. During follow-up, M-mode left ventricular end diastolic dimension (p<0.001) and end systolic dimension increased (p<0.001), whereas right ventricular end diastolic dimension decreased (p<0.001). Compared with controls, patients (n=33) had significantly reduced tricuspid annular Ea and higher E/Ea (p<0.001). Right ventricular systolic dysfunction was also suggested by reduced tricuspid annular systolic velocity (p<0.001). Late gadolinium enhancement was demonstrated in 13 out of 21 patients with restrictive physiology, which involves the anterior right ventricular outflow tract, anterior wall, and inferior wall. The right ventricular late gadolinium enhancement score correlated positively with age (r=0.7, p<0.001) and right ventricular mass index (r=0.52, p<0.001). The persistence of right ventricular diastolic dysfunction after relief of chronic pressure overload of critical pulmonary valve stenosis suggests that a factor - other than increase in afterload - is involved in this physiology. Fibrosis is the most likely factor responsible for persistence of restrictive physiology as documented by late gadolinium enhancement.

Perinatal integrative intervention for critical pulmonary artery valve stenosis

To investigate the effect of different operation time to percutaneous balloon pulmonic valvuloplasty (PBPV) to critical pulmonary valve stenosis (CPS). Twenty-one infants (age ≤ 60 days at operating day) suffered from CPS, diagnosed by fetal echocardiogram and confirmed by echocardiography after birth, were enrolled in this case-control-study with written informed consent during April 2007 to December 2011. Of the 21 cases, 7 had prenatal diagnosis in our prenatal diagnosis center (prenatal group, Pre) and 14 were referred from other hospitals, who were divided into postpartum group A (Post A, referred within 28 days after birth) and postpartum group B (Post B, referred 29 to 60 days after birth). To Pre-group, the integrative interventional protocol was cautiously made by the consultative specialists, including intrauterine diagnosis, perinatal care and urgent PBPV soon after birth. To Post-group, emergency PBPV was preformed after the referral. Tei index of right ventricular and pressure-gradient (PG) between right ventricular and pulmonary artery were measured before and at different time points one year after PBPV. The values of SpO2 in Pre-group ranged from 82%-92% (86.57% ± 5.34%) under the state of continuous intravenous infusion of alprostadil. PBPV was successfully preformed within 3-6 days after birth. The values of SpO2 increased to 97.33% ± 1.15% post procedure. The values of PG pre- and post- procedure were (86.34 ± 11.77) mm Hg and (31.43 ± 8.46) mm Hg respectively. Preoperative RV Tei-index was 0.68 ± 0.05, it decreased rapidly after procedure, and recovered to normal one month after procedure. Only one case showed restenosis seven months after procedure and repeated PBPV. Fourteen referral cases (6 cases in Post A group and 8 cases in Post B group, accompanied in 1 and 3 cases with heart failure), the values of SpO2 ranged from 83%-91% under state of continuous intravenous infusion of alprostadil. And the operating time was 10-57 days after birth. The values of SpO2 recovered to normal post procedure, and heart failure alleviated. Increased preoperative RV pressure obviously decreased significantly post-procedure. And increased Tei-index declined gradually, at one-year follow-up, the value of Tei-index in Post A group recovered to normal, whereas that of Post B was (0.51 ± 0.06), compared to Pre and Post A groups, the difference was significant (P < 0.05) . One case showed restenosis nine months after procedure and repeated PBPV was performed. The hypoxic exposure durations were (4.43 ± 0.68) , (16.33 ± 4.46) , (41.25 ± 9.19) , respectively, and the difference among the three groups was significant (P < 0.05). To the fetuses with definite prenatal diagnosis of critical pulmonary valve stenosis, preoperative general condition can be adjusted to more suitable for emergency operation. Early PBPV can achieve shorter hypoxic exposure and better recovery of right ventricular function post procedure. Perinatal integrated intervention for CPS can significantly improve the prognosis and quality of life in this patient population.

ASSA13-10-8 Clinical Study of Perinatal Integrative Intervention For Critical Pulmonary Stenosis

<h3>Objective</h3> Investigate the role of “Perinatal integrated intervention model” in Three Level Prevention of critical pulmonary valve stenosis. <h3>Methods</h3> Twenty-one infants (age ≤ 60 days at operating day) suffered from critical pulmonary valve stenosis (CPS) were enrolled in this case-control-study with written informed consent. In which there were 7 cases of prenatal diagnosis in our prenatal diagnosis centre (prenatal group, Pre) and 14 cases of referral (Postpartum group, according to the time of operation less or more than 28 days, postpartum group was divided into two groups, postpartum group A and postpartum group B, which were named Post A and Post B). To Pre-group, the integrative interventional protocol was cautiously made by the consultative specialists, including intrauterine diagnosis, perinatal care and urgent percutaneous balloon pulmonary valvuloplasty (PBPV) soon after birth. To Post-group, emergency PBPV was preformed after the referral. Tei index of right ventricular and Pressure-gradient (PG) between right ventricular and pulmonary artery were measured before and at different time points with one year after PBPV. <h3>Results</h3> ➀ Seven cases of critical and extremely critical PS were diagnosed by foetal echocardiogram and confirmed by echocardiography after birth during April 2007 to December 2011, and the values of SpO₂ ranged from 82%-92%, which was named Pre group. PBPV was successfully preformed within 3–6 days after birth. The values of pre- and post- procedure PG were 86.34 ± 8.77 mmHg and 31.43 ± 8.46 mmHg respectively. Preoperative RV Tei-index was 0.68 ± 0.05, decreased rapidly after procedure, and recovered to normal one month after procedure. Only one case revealed restenosis seven months after procedure and repeated PBPV. ➁ 14 cases of referral (6 cases in Post A group and 8 cases in Post B group), the values of SpO₂ ranged from 83%-91%, and the operating time was 10 –57 days after birth. Increased preoperative RV pressure and Tei-index were obviously decreased significantly post-procedure. Only one case revealed restenosis nine months after procedure and repeated PBPV. <h3>Conclusions</h3> Compared with the referral cases, preoperative condition and postoperative right ventricular function were better, as well as the time of hypoxic exposure was shorter in the infants of Pre group. “Perinatal integrated intervention model” with multidisciplinary cooperation is an efficient strategy in Three Level Prevention to infants suffered from CPS, and can significantly improve the prognosis and quality of life in this patient population.