Pulmonary Valve Regurgitation Research Articles

Effect of percutaneous pulmonary valve replacement on ventricular electrophysiological remodeling in a porcine model of repaired tetralogy of Fallot

Tetralogy of Fallot is the most common cyanotic congenital heart disease. Late after corrective surgery, patients are at risk of developing right ventricular (RV) failure due to pulmonary valve regurgitation and arrhythmias related to surgical scars and ventricular electrophysiological remodeling. Pulmonary valve replacement has been identified as an interesting strategy to reduce RV dilatation but its impact on the arrhythmic risk remains uncertain. We sought to determine whether percutaneous pulmonary valve implantation (PPVI) at an early pathological stage leads to benefical effects on right and left ventricular electrophysiological remodeling in a porcine model of repaired tetralogy of Fallot (rTOF). Piglets (<10 kg) underwent pulmonary artery banding, pulmonary valve disruption to induce RV hypertrophy and dilatation and a PTFE patch was sewn across the pulmonary annulus. Two-months later, PPVI was performed in a sub-group of rTOF pigs. At 3 months post-surgery, cardiac function was assessed by MRI in rTOF, rTOF-PPVI and Sham-operated pigs. Animals were euthanized and optical mapping of isolated left and right ventricles electrical activity was performed using the voltage-sensitive dye Di-4-ANEPPS. Expression of selected genes was assessed by quantitative RT-PCR. At 3 months post-surgery, pulmonary artery regurgitation, RV end-diastolic and end-systolic volumes normalized to body surface area were significantly increased in rTOF pigs compared to Sham animals. In rTOF-PPVI animals these parameters were partially reversed and not statistically different from Sham pigs. An action potential prolongation was observed in both the left ventricle and the RV epicardium but not in the endocardium of rTOF pigs. Within the RV epicardium, this prolongation was mainly confined to the anterior side. Interestingly, rTOF-PPVI pigs action potential durations were similar to Sham animals. In line with functional investigations, a large increase in BNP expression was found in rTOF RV compared to Sham pigs (fold change = 34) which was not as pronounced in rTOF-PPVi pigs (fold change = 3) while a lower expression of KCND3 and KCNJ2 was found in rTOF pigs. In our rTOF porcine model, PPVI at an early pathological stage reversed RV dilatation. It also successfully reversed action potential prolongation in the left ventricle and the anterior RV epicardium, and may thus have beneficial effects on the arrhythmic risk.

P151 RIGHT VENTRICULAR MECHANICS IN PATIENTS AFFECTED BY PULMONARY VALVE STENOSIS, BEFORE AND AFTER PERCUTANEOUS PULMONARY VALVULOPLASTY

Abstract Aim of the Study To evaluate the mechanical changes of the right ventricle in patients undergoing balloon pulmonary valvuloplasty using transthstandard transthoracic echocardiography (TTE) and speckle–tracking echocardiography (STE) and to investigate the correlation between haemodynamic and echocardiographic parameters before and after treatment. Materials and Methods 43 pediatric patients (19 males), mean age 3,2±4,9 years with severe pulmonary valve stenosis and indication for percutaneous balloon valvuloplasty were recruited. All patients underwent TTE and STE with analysis of right ventricle global longitudinal strain (RVGLS) one day before and after the procedure. For each patient were collected invasive parameters during balloon valvulopasty. Results After the procedure, there was an immediate reduction of both peak–to–peak transpulmonary gradient (Dp post) and ratio between the systolic pressure of right and left ventricle (RV/LV ratio) with a drop of 29,3±14,67mmHg and 0,43±0,03, respectively. Post–procedural echocardiography showed peak and mean transvalvar pressure gradient drop (50±32,23 and 31±17,97, respectively). The degree of pulmonary valve regurgitation was mild in 8% of patients before the procedure, following the intervention it reached 29% with a statistically significant increase (p = 0,007). However, moderate and severe regurgitation remained stable after the procedure. There was a significant improvement of Fractional Area Change (FAC) after the procedure (40,11% vs 44,42%, p = 0,01). TAPSE (p = 0,60) and longitudinal strain (p = 0,31), did not improve significantly after intervention. Finally, pre–procedural invasive RV/LV ratio showed good correlation to echocardiographic transvalvular peak and mean pressure gradient (R = 0,375, p = 0,019 and R = 0,40, p = 0,012, respectively), as well as with FAC (R = 0,31 p = 0,05), TAPSE (R = 0,62 p &amp;lt; 0,001)and RVGLS (R = 0,46 p = 0,01) Conclusions Percutaneous balloon pulmonary valvuloplasty represents an efficient and safe procedure. Right ventricular global systolic function improved following afterload reduction, while longitudinal systolic function did not show improvement immediately after intervention. Finally, invasive preprocedural RV/LV ratio demonstrated better correlation with echocardiographic evaluation of stenosis degree and right ventricular function compared to invasive peak–to–peak pressure gradient. Therefore, RV/LV ratio should be preferred for the assessment of pulmonary valve stenosis.

Decellularized Pulmonary Xenograft Matrix PplusN versus Cryopreserved Homograft for RVOT Reconstruction during Ross Procedure in Adults.

Decellularized pulmonary homografts are being increasingly adopted for right ventricular outflow tract reconstruction in adult patients undergoing the Ross procedure. Few reports presented Matrix PplusN xenograft (Matrix) in a negative light. The objective of this study was to compare our midterm outcomes of Matrix xenograft versus standard cryopreserved pulmonary homograft (CPHG). Eighteen patients received Matrix xenograft between January 2012 and June 2016, whereas 66 patients received CPHG. Using nonparametric statistical tests and survival analysis, we compared midterm echocardiographic and clinical outcomes between the groups. Except for significant age difference (the Matrix group was significantly older with 57 ± 8 years than the CPHG group, 48 ± 9 years, p = 0.02), the groups were similar in all other baseline characteristics. There were no significant differences in cardiopulmonary bypass times (208.3 ± 32.1 vs. 202.8 ± 34.8) or in cross-clamp times (174 ± 33.9 vs. 184.4 ± 31.1) for Matrix and CPHG, respectively. The Matrix group had significantly inferior freedom from reintervention than the CPHG group with 77.8 versus 98.5% (p = 0.02). Freedom from pulmonary valve regurgitation ≥ 2 was not significantly different between the groups with 82.4 versus 90.5% for Matrix versus CPHG, respectively. After median follow-up of 4.9 years, Matrix xenograft developed significantly higher peak pressure gradients compared with CPHG (20.4 ± 15.5 vs. 12.2 ± 9.0 mm Hg; p = 0.04). After 5 years of clinical and echocardiographic follow-up, the decellularized Matrix xenograft had inferior freedom from reintervention compared with the standard CPHG. Closer follow-up is necessary to avoid progression of valve failure into right ventricular deterioration.

336 Right ventricular mechanics in patients affected by pulmonary valve stenosis, before and after percutaneous pulmonary angioplasty

Abstract Aims Pulmonary valve stenosis accounts for 6–9% of all congenital heart diseases. The main effect of this obstructive lesion is a rise in right ventricular pressure; this overload leads to multiple changes in shape, dimensions, and volume of the ventricle. The diagnosis is based on transthoracic echocardiography and invasive heart catheterization. Usually the stenosis is classified into mild, moderate, and severe based on pressure gradient between right ventricle and pulmonary artery and on the ratio between right ventricle and left ventricle systolic pressure. Percutaneous balloon valvuloplasty is the treatment of choice in severe pulmonary valve stenosis in patients of all ages; alternatively surgical valvotomy is an option in selected cases. The aim of this study is to evaluate the mechanical changes of the right ventricle in patients undergoing balloon pulmonary valvuloplasty using transthoracic and speckle-tracking echocardiography (STE). Furthermore, we sought to investigate the correlation between haemodynamic and echocardiographic parameters to better evaluate the degree of pulmonary valve stenosis before and after treatment. Methods and results Forty-three pediatric patients (19 males), mean age 3.2 ± 4.9 years with severe pulmonary valve stenosis and indication for percutaneous balloon valvuloplasty were recruited at the University Hospital of Padua. All patients underwent standard transthoracic echocardiography (TTE), STE with analysis of right ventricle global longitudinal strain (RVGLS) one day before and one day after the procedure. For each patient were collected invasive parameters during the interventional procedure before and after balloon valvuloplasty. After the procedure, there was an immediate statistically significant reduction of both peak-to-peak transpulmonary gradient (Dp post) and ratio between the systolic pressure of right and left ventricle (RV/LV ratio) with a drop of 29.3 ± 14.67 mmHg and 0.43 ± 0.03, respectively. Post-procedural echocardiography showed peak and mean transvalvar pressure gradient drop (50 ± 32.23 and 31 ± 17.97, respectively). The degree of pulmonary valve regurgitation was mild in 8% of patients before the procedure, following the intervention it reached 29% with a statistically significant increase (P = 0.007). However, the incidence of pulmonary valve moderate and severe regurgitation remained stable after the procedure. The analysis of right ventricular function and mechanics showed a significant improvement of Fractional Area Change (FAC) immediately after the procedure (40.11% vs. 44.42%, P = 0,01). On the other hand, right ventricular longitudinal systolic function parameters, TAPSE (P = 0.60) and longitudinal strain (P = 0.31), did not improve significantly after intervention. Finally, pre-procedural invasive RV/LV ratio showed good correlation to echocardiographic transvalvular peak and mean pressure gradient (R = 0.375, P = 0.019 and R = 0.40, P = 0.012, respectively), as well as with FAC (R = 0.31, P = 0.05), TAPSE (R = 0.62, P &amp;lt; 0.001), and RVGLS (R = 0.46, P = 0.01). Conclusions Percutaneous balloon pulmonary valvuloplasty represents an efficient and safe procedure to relieve severe pulmonary valve stenosis. Interestingly, the analysis of right ventricular mechanics on echocardiography demonstrated an immediate global systolic function improvement following afterload reduction. Conversely, longitudinal systolic function did not show improvement immediately after intervention, possibly due to the necessity of longer time to recover. Finally, invasive preprocedural RV/LV ratio demonstrated better correlation with echocardiographic evaluation of stenosis degree and right ventricular function compared to invasive peak-to-peak pressure gradient. Therefore, RV/LV ratio should be preferred for the assessment of pulmonary valve stenosis.

Abstract 14229: Carcinoid Heart Disease: What Do We Know About Characteristics and Outcomes of Tricuspid Valve Replacement? A Nationwide Analysis

Introduction: In patients with severe tricuspid and pulmonary valve regurgitation and symptoms of heart failure secondary to carcinoid heart disease, valve replacement is the only effective treatment and can improve survival. Previous data on outcomes of valve replacement in carcinoid heart disease have been limited to case reports or single-centre study. Therefore we aimed to perform a nationwide database to ascertain the outcomes of tricuspid valve replacement (TVR) in carcinoid heart disease. Methodology: We utilized nationwide readmission database (NRD) to identify hospitalizations for tricuspid valve replacement in 2018. Hospitalizations with valve replacement for infective endocarditis were excluded. Characteristics and outcomes were compared between patients undergoing TVR for carcinoid versus other reasons. Multivariable logistic regression was performed to examine the predictors of in-hospital mortality after TVR. Results: A total of 1192 hospitalizations for TVR were included in the analysis of which 57 had carcinoid heart disease. There were no significant differences in short-term outcomes including in-hospital mortality (12.6% vs 11.5%, p=0.839), acute renal failure, blood transfusion, cardiogenic shock, new pacemaker insertion, length of stay and 30-day readmissions between TVR for carcinoid vs other etiologies (Table). The independent predictors of mortality in patients undergoing TVR were advanced liver disease (4.67 (2.59-8.43), p&lt;0.001), end stage renal disease (5.09 (2.10-12.01), p&lt;0.001), acute heart failure (2.00 (1.10-3.67), p=0.022), malnutrition (2.24 (1.13-4.31), p=0.017), and age (1.02 (1.01-1.04), p=0.020), whereas carcinoid heart disease was not an independent predictor of mortality (aOR: 1.52 (0.39-4.65), p=0.497). Conclusion: Tricuspid valve replacement in patients with carcinoid heart disease is associated with comparable short-term outcomes as for other etiologies.

Midterm Outcomes for Polytetrafluoroethylene Valved Conduits

BackgroundVarious conduits for right ventricular outflow tract reconstruction have been reported, but most of them are not available in China. The study investigators developed a simple handsewn valved conduit using expanded polytetrafluoroethylene (ePTFE). This study evaluated the midterm outcomes for this conduit. MethodsThis retrospective study included a total of 72 patients who underwent right ventricular outflow tract reconstruction with ePTFE valved conduits between January 2014 and June 2020. During follow-up, echocardiograms were performed for all patients, and magnetic resonance imaging was performed for patients with repaired tetralogy of Fallot. ResultsPatients had a median age of 69 months (interquartile range, 28-127 months) and a median follow-up period of 33 months (interquartile range, 9-51 months). There was no early death, but 2 late deaths (2.78%) occurred. The median conduit size was 18 mm (interquartile range, 18-20 mm) and the z score was +1.3 (interquartile range, +0.6-+2). Peak velocity across the ePTFE valve was 2.38 m/s (95% CI, 2.11-2.63 m/s). Pulmonary valve regurgitation was none or trivial in 27 (38.5%) patients, mild in 42 (60.0%) patients, and moderate in 1 (1.4%) patient. Conduit dysfunction occurred in 5 patients: 4 had moderate conduit stenosis, and 1 had moderate regurgitation. The right ventricular end-diastolic volume index in repaired tetralogy of Fallot was significantly decreased after surgery (171 mL/m2 vs 130 mL/m2; P < .001). No reintervention or conduit replacement was needed. ConclusionsThis handsewn ePTFE valved conduit was associated with appreciable hemodynamic outcomes during the midterm follow-up period. Long-term follow-up studies are needed to corroborate these findings.

Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure.

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14–64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.

Valvulopatía tricúspide y valvulopatía pulmonar

Right-sided valve diseases have traditionally taken a secondary place in the management of valve disease, as they have a longer asymptomatic latency time and are considered “benign”. Recent advances, especially in tricuspid valve regurgitation, have increased the interest in earlier diagnosis and effective treatment to change the natural history of the disease. The aetiology can be congenital or acquired. The most common cause of pulmonary and tricuspid valve regurgitation is functional, secondary to dilatation of the right ventricle or pulmonary artery, respectively. The most prevalent aetiology is rheumatic in tricuspid valve stenosis, and congenital in pulmonary valve stenosis. Retrograde right heart failure symptoms predominate, characterised by systemic venous congestion (oedema in the lower limbs, ascites, hepatosplenomegaly) and anterograde congestion in the form of low output (fundamentally dyspnoea). Pulmonary congestion is rare and appears in cases of concomitant left-sided involvement. Diagnosis is based on echocardiography, which enables study of the valve anatomy and degree of severity. Surgical treatment is reserved for cases that are resistant to medical treatment (diuretics), and percutaneous treatment is an alternative in selected cases.

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China.

Objectives: The management of atrial isomerism with complex congenital heart disease remains challenging. Experience has been largely obtained in advanced countries. The clinical diversity is greater in China. We evaluated the early- and medium-term outcomes of surgical treatment of these patients.Methods: We reviewed 86 patients of atrial isomerism with complex congenital heart disease undergoing varied surgeries in our center in 2008–2020. Cox regression models were used to analyze the risk factors for mortality.Results: There were 75 cases of right and 11 of left atrial isomerism. Eighty-three (96.5%) patients underwent single-ventricle staged palliation approach, with 10 early and 7 late deaths. The overall 1-, 5-, and 10-year survival rates were 84.7, 79.3, and 79.3%, respectively. Thirty-six (43.4%) patients completed the Fontan procedure with median age of 48 months and freedom from death or Fontan failure at 1-, 5-, and 8-years were 94.4, 87.4, and 80.7%, respectively. Concomitant total anomalous pulmonary venous connection [hazard ratio (HR): 5.15 (1.95–12.94), p = 0.008], more than moderate atrioventricular valve regurgitation [HR: 4.82 (2.42–6.79), p = 0.003], and the need for first-stage palliative surgery [HR: 4.58 (1.64–10.76), p = 0.015] were independent risk factors for mortality.Conclusions: Despite even greater clinical diversity, the surgical outcomes of atrial isomerism with complex congenital heart disease are improving in China. The early and intermediate outcomes are comparable to many previous reports. Concomitant total anomalous pulmonary venous connection, moderate or severe atrioventricular valve regurgitation, and the need for a first-stage palliative surgery are still independent risk factors for mortality.

Outcomes of pulmonary valve replacement using a bioprosthetic valve and polytetrafluoroethylene bicuspid valve for pulmonary valve regurgitation after intracardiac repair of tetralogy of Fallot.

Excellent results have been reported regarding pulmonary valve replacement (PVR) for pulmonary valve regurgitation (PR) after intracardiac repair (ICR) in patients with tetralogy of Fallot (TOF). However, there are apparently no reports comparing the following procedures: PVR using a bioprosthetic valve and PVR using a polytetrafluoroethylene (PTFE) bicuspid valve. Herein, we retrospectively analyzed the outcomes of PVR for PR after ICR of TOF in our institution and assessed which of the two PVR procedures was better. From June 2008 to December 2017, we performed PVR for PR after ICR of TOF in 34 patients. Patients with the right ventricle to the pulmonary artery conduits were excluded. Preoperative and postoperative cardiac magnetic resonance imagings (cMRIs) were performed in all patients. The patients were divided into the bioprosthetic valve group (BV group, n = 17) and the PTFE bicuspid valve group (PTFE group, n = 17). There were no significant differences in the preoperative cMRI data and perioperative factors between the two groups. There were no deaths in either group. Postoperative cMRI showed that the PR fraction and the right ventricular end-diastolic volume index (RVEDVI)/left ventricular end-diastolic volume index ratio were significantly improved in both groups. However, RVEDVI was significantly improved only in the BV group. Re-PVR was required in four patients in the PTFE group. PVR using a bioprosthetic valve was more effective for PR treatment after ICR of TOF than PVR using a PTFE bicuspid valve.

Effects and side effects of maternal administration of indomethacin for fetal tricuspid valve dysplasia

We report on the course of two fetuses with tricuspid valve dysplasia (TVD) who were treated with indomethacin. Case 1 presented at 24 weeks' gestation with severe tricuspid regurgitation (TR), retrograde flow in the arterial duct, no antegrade flow across the pulmonary valve and free pulmonary regurgitation (PR). There was cardiomegaly and fetal hydrops with ascites, skin edema and pleural effusion. The Simpson–Andrews–Sharland (SAS) prenatal severity score for TVD was 7, which predicted fetal demise. The SAS score ranges from 0 to 10, with no predicted survivors scoring ≥ 51. Case 2 presented at 16 weeks' gestation with moderate TR, antegrade flow across the pulmonary valve, progressing to functional pulmonary atresia by 21 weeks' gestation, and a SAS score of 4. At 33 + 5 weeks, there was evidence of further progression with increasing cardiomegaly (cardiothoracic ratio (CTR): 0.80), severe TR and PR and a SAS score of 8. Estimated fetal weight (EFW) dropped from 17th to 3rd centile, umbilical artery (UA) pulsatility index was > 95th centile and amniotic fluid index (AFI) was normal (8.9 cm). Maternal indomethacin (100 mg three times a day (TDS)) was commenced in both cases (Figure 1), leading to constriction/closure of the arterial duct, reduction in PR and antegrade flow across the pulmonary valve. In Case 1, by 26 weeks' gestation, fetal hydrops had completely resolved, indomethacin was replaced by ibuprofen (200 mg TDS) due to a more favorable side-effect profile. However, the arterial duct reopened and indomethacin was reintroduced. Following this, the arterial duct constricted, but oligohydramnios developed (AFI: 4.4 cm). At 30 weeks' gestation, there was faltering growth, EFW of 1.2 kg and anhydramnios. Indomethacin was stopped and the AFI normalized (17.1 cm), but cardiomegaly persisted with severe TR. Intrauterine death occurred at 31 + 6 weeks' gestation. In Case 2, following commencement of indomethacin, CTR reduced to 0.65 but oligohydramnios worsened (AFI: 4.2 cm). Indomethacin was stopped and AFI normalized. Indomethacin was restarted at a lower dose (50 mg TDS), but despite failure to constrict the arterial duct, oligohydramnios recurred (AFI: 1.5 cm) and therapy was ceased. The baby was delivered at 36 weeks' gestation due to absent end-diastolic UA flow, weighing 1.4 kg, and was stable without intervention at 1 year of age. Prenatally detected cases of TVD are often those at the most severe end of the spectrum, presenting with cardiomegaly secondary to severe TR1. Cardiomegaly leads to compression of the developing lung tissue, pulmonary hypoplasia and increased risk of postnatal demise. The fetal environment is disadvantageous for the fetus with TVD as the right ventricle (RV) faces systemic pressures and high pulmonary vascular resistance, worsening TR and subsequent right atrial dilatation, which in itself will exacerbate TR. Severe TR causes reduced antegrade flow across the pulmonary valve and reversal of flow in the arterial duct. This ‘steal’ from the systemic arterial circulation inhibits antegrade pulmonary blood flow and causes PR, which results in further volume loading of the RV. Furthermore, RV dilatation has an adverse effect on left ventricular geometry and function, causing additional fetal hemodynamic compromise2, explaining the high perinatal mortality of 45–71%3. Whilst there have been apparently favorable reports of indomethacin for a circular shunt in TVD2, 4, this treatment led to anhydramnios/oligohydramnios in both our cases. This may be due to pre-existing renal hypoperfusion secondary to poor ventricular-ventricular interaction and ductal steal. Furthermore, through its action on prostaglandins, indomethacin can lead to renal vasoconstriction and may cause oligohydramnios5. The potential benefits of ductal constriction for circular shunts need to be balanced against the risk of renal perfusion, necessitating close cardiac and fetal medicine supervision for affected fetuses. A prospective trial is needed to evaluate the risk-benefit ratio of this new treatment for fetal TVD. We acknowledge the contribution of Dr Wayne Tworetzky from the Children's Hospital in Boston, USA, whose group published the rationale for indomethacin therapy in this group of fetuses. Dr Tworetzky advised on the indomethacin and ibuprofen treatment used in the first case. Data available on request due to privacy/ethical restrictions

A Case of Pulmonary Artery Aneurysm Related to Pulmonary Valve Regurgitation

A Case of Pulmonary Artery Aneurysm Related to Pulmonary Valve Regurgitation

Percutaneous Balloon Pulmonary Valvuloplasty of Critical Pulmonary Stenosis and severe pulmonary stenosis in Neonates and Early Infancy: A Challenge in the Cyanotic.

Introduction: Pulmonary stenosis with an intact ventricular septum (PS-IVS) is one of the common causes of cyanotic heart disease in neonates with diverse morphologies as well as management and treatment protocols. The aim of this study was to evaluate short and midterm results of balloon pulmonary valvuloplasty (BPV) for this disorder. Methods: Between 2012 and 2016, Totally 45 neonates and infants under 6 months old were evaluated.The patients had a minimum right-to-left ventricular pressure ratio of 1, right-to-left shunting at the patent foramen ovale or atrial septal defect level, and tricuspid valve Z-scores higher than -4. Results: Immediately after the procedure, the right ventricular pressure dropped to the normal values in 8 (20%) patients. The immediate procedural success rate was seen in 42 (93.3%) cases: the right-to-left ventricular pressure ratio dropped to below 50% or the level of O2 saturation rose above 75%. Of three cases unresponsive to BPV, two of them underwent patent ductus arteriosus (PDA) stenting and one procedural death occurred. At 6 months’ follow-up, of 42 patients, this pressure was still with in the normal range in 36 (80%) infants, while it had returned to high values in 9 (20%) patients and necessitated repeat valvuloplasty. After BPV, severe pulmonary valve regurgitation was observed in14.2% patients; the condition was more common when high-profile noncompliant balloons were used. Conclusion: Balloon pulmonary valvuloplasty in infants with PS-IVS confers acceptable results insofar as it improves echocardiographic parameters and hemodynamic changes at short- and midterm followups.Balloon selection with sizes more than 1.2 of the diameter of the pulmonary valve annulus and the use of noncompliant high-pressure balloons results in higher degrees of pulmonary regurgitation.

P24: MANAGEMENT OF RHEUMATIC VALVE DISEASE OF THE AORTIC VALVE USING THE OZAKI PROCEDURE WITH AUTOLOGOUS PERICARDIUM

Abstract Case-Study A 15-year-old boy was referred to our tertiary centre from his local paediatric services with a background of rheumatic fever, severe aortic regurgitation (AR) and mild to moderate mitral regurgitation. He had a history of angina and dyspnoea on exertion, a 2/6 ejection systolic murmur and 2/4 end diastolic murmur. Transthoracic echocardiography showed severe aortic valve insufficiency (with flow reversal seen in the descending aorta and an LV end diastolic volume of 173 ml/m2) and trivial pulmonary valve regurgitation. Autograft failure following the favoured Ross procedure deemed the patient as a candidate for an Ozaki procedure. Autologous pericardium was used to replace the diseased aortic valve. Intraoperative transoesophageal echocardiography showed a deficient left coronary cusp leaflet and a retracted right coronary cusp leaflet. The patient was under cardiopulmonary bypass for 124 minutes and on cross-clamping for 99 minutes with no intraoperative complications. Histological examination of the aortic valve leaflets showed neovascularisation, myxoid changes and disarray of the fibrous stroma. Postoperative recovery was uneventful. The postoperative echocardiogram showed trivial AR, end diastolic volume 217ml, end systolic volume 12 ml and 40% ejection fraction. There was full resolution of the dyspnoea, angina and diastolic murmur on follow-up 4-months postoperatively as supported by healthy valve function on echocardiography. This case highlights that in those of risk of multiple valve pathology, such as in rheumatic valve disease, an Ozaki procedure using autologous pericardium is a viable surgical option for paediatric aortic valve repair with good outcomes. Take-home message In cases of systemic conditions affecting the heart valves where there is multiple valve pathology and risk of autograft failure, such as rheumatic valve disease, the use of autologous pericardium to replace these valves has shown to be a viable option in this paediatric case.

Right ventricular remodelling after endo-exclusion during pulmonary valve replacement: evaluation by cardiac magnetic resonance.

Pulmonary valve replacement (PVR) performed for pulmonary valve regurgitation is the most common indication for reoperation during mid-to-long-term follow-up after tetralogy of Fallot repair. An aneurysmal dilation of the infundibulum is often associated secondary to the infundibulotomy performed in the first operation. The right ventricular outflow tract reconstruction with endo-exclusion aims to exclude the non-contractile segments of the dilated right ventricular. This study intends to assess the safety and efficiency of the endo-exclusion technique. Between January 2010 and December 2018, 86 patients underwent a PVR with (n = 46) or without (n = 40) endo-exclusion. The current study compares the outcomes in terms of survival, reintervention, structural valve deterioration, right ventricular function (volume and right ventricular ejection fraction) and pulmonary valve gradient. The median follow-up time was 4.45 years (1.9 months to 9.87 years). There was no 30-day mortality. There was no difference in the freedom from reintervention at 7 years (without endo-exclusion, 97%, versus with endo-exclusion, 94%, log-rank = 0.68) or in the freedom from structural pulmonary valve deterioration at 7 years (without endo-exclusion, 94%, versus with endo-exclusion, 89%, log-rank = 0.94). No significant difference was observed in the indexed right ventricular end-diastolic volume (102.2 ± 34 ml/m2 in the PVR without endo-exclusion group and 93.3 ± 22 ml/m2 in the PVR with endo-exclusion group, P = 0.61). No significant difference was observed in the right ventricular function (right ventricular ejection fraction: 46 ± 11% in the PVR without endo-exclusion group and 46 ± 9% in the PVR with endo-exclusion group, P = 0.88). PVR with or without endo-exclusion is a safe and effective procedure. PVR with endo-exclusion allows implantation without structural deformation of the valve and therefore excellent short- and medium-term results.

Acute Idiopathic Pulmonic and Tricuspid Regurgitation after Heart Transplant

Introduction Right sided valvular regurgitation is a rare complication after heart transplantation, and can occur with hyperacute rejection or later from repeated endomyocardial biopsy. Early surgical intervention has been recommended when managing a tricuspid regurgitant valve post transplantation. We describe a case of severe tricuspid and pulmonic valve regurgitation after heart transplantation. Case Report A 39 yo male with D-TGA, post Mustard repair that progressed into end stage HF. He underwent uncomplicated orthotopic heart transplantation with bicaval anastomosis from a young donor with normal cardiac structures and function on TEE and visual survey. Intraoperative TEE showed moderate tricuspid and moderate pulmonic valve regurgitation. A PA catheter was thought to be contributing to the central regurgitant jet; the RV size and function were normal, the PA pressures were also normal. Post operatively patient developed progressive right heart failure syndrome with worsening regurgitation despite diuresis and removal of the PA catheter. Endomyocardial biopsy did not show any signs of rejection. Repeated TEE showed normal biventricular function, torrential TR with pan systolic flow reversal in the hepatic vein due to non coaptation of the leaflets. The pulmonic valve was also severely insufficient with a central regurgitant jet and holodiastolic flow reversal in the pulmonary arteries. The patient underwent a tricuspid repair with a 30mm tricuspid ring and pulmonic valve replacement with a 25 mm bovine valve on POD 10. Surgical endomyocardial biopsy had no evidence of rejection. His heart failure symptoms resolved and he was discharged 4 weeks post op. Summary We describe a novel case of a 39 yo orthotopic heart transplant recipient with acute post operative concomitant TR and PI with right heart failure requiring a tricuspid ring annuloplasty and pulmonic valve replacement. To date, there are no such reported complications after heart transplant. The incidence of isolated tricuspid valve regurgitation after heart transplant have decreased with the bicaval transplantation technique however not eliminated. Literature suggests that isolated moderate to severe tricuspid valve regurgitation should under a repair or replacement early post transplantation. Pulmonic valve insufficiency is extremely rare after heart transplant and management strategies are not well defined.

Echocardiographic comparison between pulmonary valve preservation and transannular patch techniques in children with repaired Tetralogy of Fallot

Abstract Funding Acknowledgements Type of funding sources: None. INTRODUCTION Many centers have recently adopted pulmonary valve (PV) preservation procedures to prevent the detrimental long-term effects of chronic pulmonary regurgitation after tetralogy of Fallot (ToF) repair. PURPOSE The aim of our study was to investigate pulmonary valve (PV) and right ventricular function by echocardiography in paediatric patients with repaired Tetralogy of Fallot (ToF), comparing PV preservation surgical strategies to standard transannular patch (TAP) repair. METHODS All patients undergoing transatrial-transpulmonary repair for ToF at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 main groups, according to the different techniques used: patients undergoing a PV preservation strategy and patients undergoing TAP repair. All patients underwent standard echo-Doppler study including RV end-diastolic area (RVEDA), end-systolic area (RVESA), fractional area change (FAC) and TAPSE; PR was assessed by Color Doppler, continuous-wave (CW) Doppler and derived parameters such as pressure half time (PHT) and pulmonary regurgitation (PR) index. By speckle tracking we measured also, in a subgroup of patients, right atrial strain (RAS), RV and left ventricle (LV) global longitudinal strain (RVGLS, LVGLS) and their time to peak (TTP) values. RESULTS Overall, 82 patients underwent a successful PV preservation strategy while 34 underwent a standard TAP repair. At index surgery, BSA (0.31 ± 0.1 m2, P = 0.3), age (4.8 ± 0.3 months, P = 0.5) and preoperative PV Z-score (-3.20 ± 0.1, P = 0.1) did not different between groups. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p = 0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at follow up (HR: 1.85, 95% CI: 1.09, 3.15; p = 0.02). At a mean follow-up of 6.9 ± 0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9 ± 0.8% vs 42.5 ± 1.7%, P &amp;lt; 0.001) and tricuspid annular plane systolic excursion (TAPSE) z-score (-3.36 ± 0.3% vs -4.7 ± 0.4%, P = 0.005), while maintaining better PV competence in terms of pulmonary regurgitation index (87.9 ± 1.2% vs 82.7 ± 2.4%, P = 0.02). At speckle tracking subanalysis, patients undergoing PV preservation (n= 23), compared to the TAP group (n = 13) showed also higher values of RAS (37.5 ± 6.0% vs 29.3 ± 8.2% , P &amp;lt; 0.006), shorter right TTP (319 ± 39ms vs 357.5 ± 45.2 ms, P &amp;lt; 0.01) and higher values of LVGLS (-20,6 ± 4,2% vs -17.5 ± 3.0, P &amp;lt; 0.03). CONCLUSIONS Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible. Abstract Figure. Degree of pulmonary regurgitation

Tetralogy of Fallot and Pulmonary Valve Replacement: Can We Break the Vicious Cycle?

Since the introduction of surgical management for patients with tetralogy of Fallot (TOF) in 1945, early and late survival have dramatically improved, with current mortality rates being less than 3%. However, surgical repair is not the cure yet, and TOF remains a never-ending clinical story. Indeed, the majority of these patients continue to have residual right ventricular outflow tract pathology, most commonly pulmonary valve regurgitation, which can result in exercise intolerance, heart failure, arrhythmias, and sudden death.

Bovine monocusp in surgery for tetralogy of Fallot

Aim. To evaluate the functionality of a bovine monocusp in surgery for tetralogy of Fallot.Methods. We analyzed the results of surgical treatment in the early postoperative period in eight infants and one year after surgery in seven infants (age range, 0–12 months; mean weight = 7.5 ± 2.3 kg). We used echocardiography to evaluate the residual pressure gradient between the right ventricle and the pulmonary artery, the degree of regurgitation on the pulmonary valve after surgery and to calculate Z-score and the fibrous ring diameter of the pulmonary valve in the late follow-up period. The degree of insufficiency was assessed by the width and depth of the regurgitation flow using the color Doppler mode.Results. The pressure gradient on the eve of discharge did not exceed 25 mmHg for any patient. Pulmonary valve regurgitation was mild in four patients and moderate in the remaining patients. Seven children passed the observation one year after surgery. In one case, we discovered a right ventricle outflow tract obstruction. In other cases, the systolic pressure gradient did not exceed 25 mmHg. Four patients in the late follow-up period had severe valve regurgitation. Despite such valve insufficiency, the leaflet mobility in the bovine jugular vein patch was normal.Conclusion. Early postoperative results for reconstructive surgery to repair tetralogy of Fallot with a bovine monocusp were promising. However, long-term results are comparable with those using other materials. The mobility of the native leaflet remains full even a year after surgery. Consequently, we have positive prospects in material improvement for making patches with native monocusps. Received 14 May 2020. Revised 8 June 2020. Accepted 10 June 2020. Funding: The study did not have sponsorship. Conflict of interest: Authors declare no conflict of interest. Author contributionsConception and study design: A.A. Svobodov, E.G. Levchenko, V.T. KostavaData collection and analysis: G.S. Netalieva, M.V. ZelivyanskayaStatistical analysis: E.G. Levchenko, M.V. ZelivyanskayaDrafting the article: A.A. Svobodov, V.S. RasumovskyCritical revision of the article: V.S. RasumovskyFinal approval of the version to be published: A.A. Svobodov, E.G. Levchenko, G.S. Netalieva, V.T. Kostava, M.V. Zelivyanskaya, V.S. Rasumovsky

Minimally invasive pulmonary valve replacement via left anterior minithoracotomy.

ABSTRACT Pulmonary valve replacement is one of the most common indications for reoperation in adults with congenital heart disease. Finding an alternative to repeat median sternotomy may facilitate recovery and shorten hospital stay. We present our technique of left anterior minithoracotomy for pulmonary valve replacement.