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Background: Pulmonary embolism (PE) is one of the most challenging diagnoses in emergency medicine, mainly because symptoms range from asymptomatic disease to sudden death. The role of echocardiography in the workup of suspected PE has been supportive and used primarily to assess the right ventricular (RV) size and function, which is important for risk stratification. Several echocardiographic parameters described in the literature lack the desired accuracy. Recently, a potential value of less well-recognized RV outflow tract (RVOT) Doppler variables has been reported. The early systolic notching (ESN) pattern was observed in 92% of patients with high and intermediate risk PE, making it a promising sign in selected PE patients. Case presentation: In this case series, we demonstrate a typical ESN pattern on RVOT Doppler evaluation in three patients with intermediate-risk PE presenting to our emergency department (ED). None of the patients had been previously diagnosed with pulmonary hypertension or other chronic pulmonary and cardiac disease. The pre-test probability was low. Massive proximal emboli were found on CT angiograms, involving pulmonary truncus or main pulmonary arteries. Previously, the ESN pattern was identified on a focused echocardiogram, which was the only echocardiographic indicator of increased pulmonary vascular resistance. Conclusions: RVOT Doppler flow pattern of ESN has potential clinical utility for the detection of PE in ED patients. ESN could identify patients at higher risk, which are otherwise stratified as low risk according to the latest guidelines. Moreover, this case series illustrates that even in the absence of other echocardiographic findings of RV strain, the presence of ESN should alert to the possibility of acute PE. Further prospective studies are needed to assess its diagnostic value in a selected subgroup of patients, similar to the cases presented, that would have no other obvious reason for the altered RVOT Doppler curve. [ABSTRACT FROM AUTHOR]

Background: Right ventricular (RV) stroke volume (SV) can be calculated via Doppler echocardiography at multiple sites in the right chambers. However, the agreement between the calculated SVs at these sites is unknown. We aimed to assess the repeatability, reliability, and precision of the distal right ventricular outflow tract diameter (RVOTD), the mid-pulmonary artery diameter (MPAD), the right ventricular outflow tract velocity time integral (VTI), and the mid-pulmonary artery velocity time integral (MPAVTI). Additionally, we evaluated the agreement between RVOTSV and MPASV. Methods: Four observers each evaluated approximately 100 patients (n = 406). Basic measurements were made over three cardiac cycles, and the repeatability, reliability, and precision of the measurements were calculated. The agreement between the two methods was presented as intraclass correlation coefficients. Results: The repeatability coefficient ranges for RVOTD, MPAD, RVOTVTI, and MPAVTI were 2–3 mm, 2–4 mm, 2.1–2.8 cm, and 2.4–4.1 cm, respectively. The minimal detectable change ranges for these variables were 2–4 mm, 2–5 mm, 2.2–3.0 cm, and 2.6–4.3 cm, respectively. The respective precision ranges for RVOTD, MPAD, RVOTVTI, and MPAVTI were 2.7–4.7%, 2.4–5.4%, 5.0–7.4%, and 5.4–9.2%. There were significant correlations and agreements between MPASV and RVOTSV, with the Pearson correlation coefficient ranging from 0.63 to 0.89 (P < 0.001). The intraclass correlation coefficient ranged from 0.56 to 0.89 (P < 0.001), although there was a significant bias of 1.9–11.3 mL (P < 0.001). Conclusions: The RVOTD, MPAD, RVOTVTI, and MPAVTI measurements were repeatable, reliable, and precise. The agreement between RVOTSV and MPASV ranged from fair to excellent, although significant bias, along with a wide limit of agreement, was observed. Consequently, these methods cannot be used interchangeably. [ABSTRACT FROM AUTHOR]

The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7–3.8) years; 9.8(8.1–15.1) kgs underwent the procedure at 14(8–44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12–14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9 mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2 mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9 months, IQR 7–22, range 5–73, others are free of re interventions with median RVOT gradient of 42, IQR 27–49, range 21–55 mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results. [ABSTRACT FROM AUTHOR]

Abstract Background Pulmonary embolism (PE) is one of the most challenging diagnoses in emergency medicine, mainly because symptoms range from asymptomatic disease to sudden death. The role of echocardiography in the workup of suspected PE has been supportive and used primarily to assess the right ventricular (RV) size and function, which is important for risk stratification. Several echocardiographic parameters described in the literature lack the desired accuracy. Recently, a potential value of less well-recognized RV outflow tract (RVOT) Doppler variables has been reported. The early systolic notching (ESN) pattern was observed in 92% of patients with high and intermediate risk PE, making it a promising sign in selected PE patients. Case presentation In this case series, we demonstrate a typical ESN pattern on RVOT Doppler evaluation in three patients with intermediate-risk PE presenting to our emergency department (ED). None of the patients had been previously diagnosed with pulmonary hypertension or other chronic pulmonary and cardiac disease. The pre-test probability was low. Massive proximal emboli were found on CT angiograms, involving pulmonary truncus or main pulmonary arteries. Previously, the ESN pattern was identified on a focused echocardiogram, which was the only echocardiographic indicator of increased pulmonary vascular resistance. Conclusions RVOT Doppler flow pattern of ESN has potential clinical utility for the detection of PE in ED patients. ESN could identify patients at higher risk, which are otherwise stratified as low risk according to the latest guidelines. Moreover, this case series illustrates that even in the absence of other echocardiographic findings of RV strain, the presence of ESN should alert to the possibility of acute PE. Further prospective studies are needed to assess its diagnostic value in a selected subgroup of patients, similar to the cases presented, that would have no other obvious reason for the altered RVOT Doppler curve.

Abstract Background Right ventricular (RV) stroke volume (SV) can be calculated via Doppler echocardiography at multiple sites in the right chambers. However, the agreement between the calculated SVs at these sites is unknown. We aimed to assess the repeatability, reliability, and precision of the distal right ventricular outflow tract diameter (RVOTD), the mid-pulmonary artery diameter (MPAD), the right ventricular outflow tract velocity time integral (VTI), and the mid-pulmonary artery velocity time integral (MPAVTI). Additionally, we evaluated the agreement between RVOTSV and MPASV. Methods Four observers each evaluated approximately 100 patients (n = 406). Basic measurements were made over three cardiac cycles, and the repeatability, reliability, and precision of the measurements were calculated. The agreement between the two methods was presented as intraclass correlation coefficients. Results The repeatability coefficient ranges for RVOTD, MPAD, RVOTVTI, and MPAVTI were 2–3 mm, 2–4 mm, 2.1–2.8 cm, and 2.4–4.1 cm, respectively. The minimal detectable change ranges for these variables were 2–4 mm, 2–5 mm, 2.2–3.0 cm, and 2.6–4.3 cm, respectively. The respective precision ranges for RVOTD, MPAD, RVOTVTI, and MPAVTI were 2.7–4.7%, 2.4–5.4%, 5.0–7.4%, and 5.4–9.2%. There were significant correlations and agreements between MPASV and RVOTSV, with the Pearson correlation coefficient ranging from 0.63 to 0.89 (P

Abstract Morphology of right ventricular outflow tract (RVOT) is potentially related to cardiovascular outcomes. However, this relationship still remains to be verified with direct evidence. We retrospectively reviewed cases from the autopsy specimen library in the Center of Forensic Medicine in Sun Yat-sen University from 2017 to 2023. Six RVOT morphological characteristics were measured and their association with cardiovascular diseases (CVDs), sudden cardiac death (SCD) and age at death was evaluated. Relationship between myocardial fibrosis in RVOT and CVDs was investigated. RVOT characteristics were recruited by machine learning algorithms for diagnosing CVDs. A total of 2370 cases were finally recruited. Perimeter of sub-valve plane (pSBV) in RVOT was positively associated with risk of CVDs and SCD (OR: 1.21, 95%CI: 1.07–1.37, p = 0.003; OR: 1.33, 95%CI: 1.16–1.52, p

Morphology of right ventricular outflow tract (RVOT) is potentially related to cardiovascular outcomes. However, this relationship still remains to be verified with direct evidence. We retrospectively reviewed cases from the autopsy specimen library in the Center of Forensic Medicine in Sun Yat-sen University from 2017 to 2023. Six RVOT morphological characteristics were measured and their association with cardiovascular diseases (CVDs), sudden cardiac death (SCD) and age at death was evaluated. Relationship between myocardial fibrosis in RVOT and CVDs was investigated. RVOT characteristics were recruited by machine learning algorithms for diagnosing CVDs. A total of 2370 cases were finally recruited. Perimeter of sub-valve plane (pSBV) in RVOT was positively associated with risk of CVDs and SCD (OR: 1.21, 95%CI: 1.07–1.37, p = 0.003; OR: 1.33, 95%CI: 1.16–1.52, p < 0.001). Compared with thickness of septum (tS) < 3.0 mm, tS ≥ 3.0 mm was associated with premature death in disease-dominant death (β=-0.16, 95%CI: -0.20 to -0.11, p < 0.001) and SCD (β=-0.15, 95%CI: -0.21 to -0.10, p < 0.001). Degree of myocardial fibrosis in the posterior septum was increased in coronary atherosclerosis (6.86%±2.48% vs. 4.91%±2.14%, p = 0.011) and cardiomyopathies (8.11%±3.24% vs. 4.88%±3.11%, p = 0.005). A logistic regression model, recruiting age, left and right ventricular wall thickness, pSBV, circumference of pulmonary annulus and aortic annulus, was elected as an optimal diagnostic model of CVDs, yielding AUC of 0.734 (95%CI: 0.705–0.763), 0.781 (0.740–0.821) and 0.763 (0.725-0.800) in training, validation and test sets. Increased pSBV significantly correlates with higher risk of CVDs and SCD. And tS ≥ 3.0 mm is an independent risk factor of premature death regardless of diseases. [ABSTRACT FROM AUTHOR]

Brugada syndrome (BrS), a genetic disorder affecting cardiac ion channels, predominantly manifests due to mutations that impair the function of the Nav1.5 sodium channel’s α-subunit. This condition, identified by Josep and Pedro Brugada, is often marked by symptoms such as syncope and episodes of polymorphic ventricular tachycardia (PVT) or ventricular fibrillation (VF). These arrhythmias, if not managed promptly, can escalate to sudden cardiac death (SCD), notably in patients whose cardiac structure appears normal. Given this, the prompt recognition and stratification of individuals at elevated risk are critical. This review elaborates on the current insights into BrS, focusing on recent diagnostic techniques, risk assessment strategies, and therapeutic advancements. It also critically examines ongoing controversies in the field.

Introduction: We aimed to investigate the incidence, prenatal factors and outcomes of twin‐to‐twin transfusion (TTTS) with right ventricular outflow tract obstruction (RVOTO). Material and methods: A systematic search was conducted to identify relevant studies published until February 2023 in English using the databases PubMed, Scopus and Web of Science. Studies reporting on pregnancies with TTTS and RVOTO were included. The random‐effect model pooled the mean differences or odds ratios (OR) and the corresponding 95% confidence intervals. Heterogeneity was assessed using the I2 value. Results: A total of 17 studies encompassing 4332 TTTS pregnancies, of which 225 cases had RVOTO, were included. Incidence of RVOTO at time of TTTS diagnosis was 6%. In all, 134/197 (68%) had functional pulmonary stenosis and 62/197 (32%) had functional pulmonary atresia. Of these, 27% resolved following laser and 55% persisted after birth. Of those persisting, 27% required cardiac valve procedures. Prenatal associations were TTTS stage III (53% vs 39% in no‐RVOTO), stage IV TTTS (28% in RVOTO vs 12% in no‐RVOTO) and ductus venosus reversed a‐wave (60% in RVOTO vs 19% in no‐RVOTO). Gestational age at laser and gestational age at delivery were comparable between groups. Survival outcomes were also comparable between groups, including fetal demise of 26%, neonatal death of 12% and 6‐month survival of 82% in RVOTO group. Findings were similar when subgroup analysis was done for studies including head‐to‐head analysis. Conclusions: RVOT occurs in about 6% of the recipient twins with TTTS, especially in stages III and IV and those with reversed ductus venosus a‐wave. The findings from this systematic review support the need for a thorough cardiac assessment of pregnancies complicated by TTTS, both before and after laser, to maximize perinatal outcome, and the importance of early diagnosis of TTTS and timely management. [ABSTRACT FROM AUTHOR]

Purpose: Pre-stenting of the right ventricular outflow tract (RVOT) is commonly performed before percutaneous pulmonary valve implantation (PPVI), to relieve obstruction, prevent valved stent fractures, and provide a landing zone. This study aimed to evaluate the biomechanical characteristics of the stents currently used to perform pre-stenting of the RVOT. Methods: We assessed five commercially available stents: Cheatham-Platinum Stent ("CP Stent"), AndraStent XL, AndraStent XXL, Optimus XL, and Optimus XXL. Following stent deployment at nominal pressure, radial and longitudinal elastic recoils and radial resistance were measured. The bending stiffness of the stents crimped onto the balloons was also evaluated. Results: Three samples were tested for each stent. Our study showed no significant difference between the stent platforms in terms of radial elastic recoil, which was relatively low (< 10%). The longitudinal elastic recoil was also low for all the devices (< 5%). Significant differences were observed in radial resistance (P < 0.001). CP Stent and AndraStent XL exhibited the highest radial resistances. The bending stiffnesses of the stents crimped on their balloons were significantly different (P < 0.00001). Optimus XL and XXL were more flexible than the other stents. Conclusion: This study highlights the significant differences between the stents currently used in RVOT pre-stenting. Stents with good radial resistance are preferred, especially for calcified vessels, and flexibility is crucial for tortuous vessels. We proposed an algorithm for selecting the most suitable stent according to the need for radial force and flexibility, which will help inform clinicians considering RVOT revalvulation. [ABSTRACT FROM AUTHOR]

Objective To investigate the effectiveness and safety of catheter ablation for premature ventricular contraction (PVC) originating from the right ventricular outflow tract ( RVOT) guided by magnetic navigation. Methods We selected 92 patients undergoing catheter ablation for RVOT origin PVC guided by magnetic navigation. They were divided into six groups based on the origin site of PVC: anterior septum group ( n = 35), midseptum group (n = 26), posterior septum group (n = 11), anterior free wall group (n = 4), medium free wall group (n = 10), and posterior free wall group (n = 6). The incidence of PVC originating from different sites of the RVOT was analyzed in the six groups. The acute success rate of ablation, recurrence rate, and safety were compared among the six groups. Results The incidence of PVC was the highest in the anterior septum of the RVOT, with a 100% of acute success rate of ablation. One year after ablation, recurrent PVC was only found in the midseptum of the RVOT, with a recurrence rate of 7. 69%. All patients had no procedure-related complication in intraoperative or postoperative period. Conclusion RVOT origin PVC is mainly originated from the anterior septum and midseptum. Catheter ablation guided by magnetic navigation demonstrates high effectiveness and safety for RVOT originated PVC. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot (TOF) is one of the common cardiac malformation with depletion of the pulmonary circulation. In the long term, preservation of neopulmonary bicuspid valve function in Tetralogy of Fallot has a key value in pediatric cardiac surgery. In 2019, a cardiac surgeon from Iran, Amirghofran A, introduced a completely new method of forming a neopulmonary valve from the appendage of the right atrium. Previously, many attempts have been made to preserve the pulmonary valve using both autologous and foreign tissue, but the results have been disappointing. the usage of the right atrial appendage as a bicuspid valve in the pulmonary position is an alternative method for correction of Tetralogy of Fallot with deformed, hypoplastic and aplasic pulmonary valve. However, more follow-up is required to obtain data that are more reliable.

Abstract Background Myocarditis substantially increases the risk of ventricular arrhythmia. Approximately 30% of all ventricular arrhythmia cases in patients with myocarditis originate from the right ventricular outflow tract (RVOT). However, the role of NLRP3 signaling in RVOT arrhythmogenesis remains unclear. Methods Rats with myosin peptide–induced myocarditis (experimental group) were treated with an NLRP3 inhibitor (MCC950; 10 mg/kg, daily for 14 days) or left untreated. Then, they were subjected to electrocardiography and echocardiography. Ventricular tissue samples were collected from each rat’s RVOT, right ventricular apex (RVA), and left ventricle (LV) and examined through conventional microelectrode and histopathologic analyses. In addition, whole-cell patch-clamp recording, confocal fluorescence microscopy, and Western blotting were performed to evaluate ionic currents, intracellular Ca2+ transients, and Ca2+-modulated protein expression in individual myocytes isolated from the RVOTs. Results The LV ejection fraction was lower and premature ventricular contraction frequency was higher in the experimental group than in the control group (rats not exposed to myosin peptide). Myocarditis increased the infiltration of inflammatory cells into cardiac tissue and upregulated the expression of NLRP3; these observations were more prominent in the RVOT and RVA than in the LV. Furthermore, experimental rats treated with MCC950 (treatment group) improved their LV ejection fraction and reduced the frequency of premature ventricular contraction. Histopathological analysis revealed higher incidence of abnormal automaticity and pacing-induced ventricular tachycardia in the RVOTs of the experimental group than in those of the control and treatment groups. However, the incidences of these conditions in the RVA and LV were similar across the groups. The RVOT myocytes of the experimental group exhibited lower Ca2+ levels in the sarcoplasmic reticulum, smaller intracellular Ca2+ transients, lower L-type Ca2+ currents, larger late Na+ currents, larger Na+–Ca2+ exchanger currents, higher reactive oxygen species levels, and higher Ca2+/calmodulin-dependent protein kinase II levels than did those of the control and treatment groups. Conclusion Myocarditis may increase the rate of RVOT arrhythmogenesis, possibly through electrical and structural remodeling. These changes may be mitigated by inhibiting NLRP3 signaling.

There are no investigations about the outcomes of idiopathic PVC catheter ablation (CA) in athletes compared to the sedentary population. We conducted a prospective single-centre observational study. The primary and secondary procedural outcomes were the post-ablation reduction of premature ventricular contractions (PVCs) in an athletes vs. non-athletes group and in agonist vs. leisure-time athletes. The third was the evaluation of the resumption of physical activity and the improvement of symptoms in agonist and leisure-time athletes. From January 2020 to October 2022 we enrolled 79 patients with RVOT/LVOT/fascicular PVC presumed origin. The median percentage of decrease between the pre-procedure and post-procedure Holter monitoring in the non-athletes group was 96 (IQR 68–98) and 98 in the athletes group (IQR 92–99) (p = 0.08). Considering the athletes, the median percentage of decrease in the number of PVCs was 98 (IQR 93–99) and 98 (IQR 87–99), respectively, in leisure-time and agonistic athletes (p = 0.42). Sixteen (70%) leisure time and seventeen (90%) agonist athletes (p = 0.24) have resumed physical activity 3 months after PVC CA; among agonistic athletes, 59% have resumed competitive physical activity. Many leisure-time (88%) and agonist (70%) athletes experienced an improvement in symptoms after ablation. PVC CA was effective and safe in both groups, reducing symptoms and allowing a quick and safe return to sports activities in athletes. [ABSTRACT FROM AUTHOR]

Background: Metabolic stress predisposes to ventricular arrhythmias and sudden cardiac death. Right ventricular outflow tract (RVOT) is the common origin of ventricular arrhythmias. Adenosine monophosphate‐regulated protein kinase (AMPK) activation is an important compensatory mechanism for cardiac remodeling during metabolic stress. Objectives: The purpose of this study was to access whether AMPK inhibition would modulate RVOT electrophysiology, calcium (Ca2+) regulation, and RVOT arrhythmogenesis or not. Methods: Conventional microelectrodes were used to record electrical activity before and after compound C (10 µM, an AMPK inhibitor) in isoproterenol (1 µM)‐treated rabbit RVOT tissue preparations under electrical pacing. Whole‐cell patch‐clamp and confocal microscopic examinations were performed in baseline and compound C‐treated rabbit RVOT cardiomyocytes to investigate ionic currents and intracellular Ca2+ transients in isolated rabbit RVOT cardiomyocytes. Results: Compound C decreased RVOT contractility, and reversed isoproterenol increased RVOT contractility. Compound C decreased the incidence, rate, and duration of isoproterenol‐induced RVOT burst firing under rapid pacing. Compared to baseline, compound C‐treated RVOT cardiomyocytes had a longer action potential duration, smaller intracellular Ca2+ transients, late sodium (Na+), peak L‐type Ca2+ current density, Na+‐Ca2+ exchanger, transient outward potassium (K+) current, and rapid and slow delayed rectifier K+ currents. Conclusion: AMPK inhibition modulates RVOT electrophysiological characteristics and Ca2+ homeostasis, contributing to lower RVOT arrhythmogenic activity. Accordingly, AMPK inhibition might potentially reduce ventricular tachyarrhythmias. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The Ross procedure represents an excellent treatment option in younger patients with aortic stenosis but is limited by poor availability of homografts. In this study, we investigated the hydrodynamic performance of 3 different types of right ventricular outflow tract replacement with pericardium or synthetic material. METHODS Three different types of valved conduits were constructed using pericardium and/or synthetic material (Group PEPE: pericardial cusps and pericardial conduit, Group PEPR: pericardial cusps and Dacron conduit, Group PRPR: expanded polytetrafluoroethylene cusps and Dacron conduit). The conduits were designed according to the Ozaki method. Their hydrodynamic performance (effective orifice area, mean pressure gradient and leakage volume) were evaluated in a mock circulation loop at different hydrodynamic conditions. RESULTS Hydrodynamic assessment showed significantly larger effective orifice area of PEPE and PEPR compared to PRPR under all conditions and there were no significant differences between PEPE and PEPR [for condition 2: PEPE 2.43 (2.35–2.54) cm2, PEPR: 2.42 (2.4–2.5) cm2, PRPR: 2.08 (1.97–2.21) cm2, adjusted pairwise comparisons: PEPE versus PEPR: P  = 0.80, PEPE versus PRPR: P  < 0.001, PEPR versus PRPR: P  < 0.001]. Mean pressure gradient was significantly lower for PEPE and PEPR compared with PRPR, whereas no significant differences were seen between PEPE and PEPR. Leakage volume was significantly lower for PEPE and PEPR compared with PRPR under all conditions while leakage was similar between PEPE and PEPR. CONCLUSIONS Pulmonary graft reconstruction with pericardium cusps showed superior hydrodynamic performance compared with polytetrafluoroethylene cusps. Our results suggest that it could be considered as an alternative substitute for right ventricular outflow tract replacement during the Ross procedure. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes. METHODS We performed a retrospective single-centre study of patients who underwent aortic root translocation between 2006 and 2019. RESULTS Aortic root translocation was performed in 23 patients at a median age of 1.6 years [interquartile range (IQR) 0.9–2.5]. Concomitant repairs were done in 52.2% of patients (12/23) including the Senning atrial switch in 34.8% (8/23). The right ventricular outflow tract was reconstructed with valved conduits in 39.1% (9/23), direct anastomoses in 4.35% (1/23) and pulmonary autografts in 56.5% of patients (13/23). Aortic cross-clamp time was significantly longer in patients with double root translocation [308 min (IQR 270–259) vs 209 min (IQR 179–281), P  =   0.02]; 2 patients in this group required temporary mechanical circulatory support. There were no early deaths. Median follow-up time was 7.5 years (IQR 3.3–10.5). The estimated 10-year survival was 90% [95% confidence interval (CI): 47.3%, 98.5%]. There was no recurrent left ventricular outflow tract obstruction. Freedom from any reoperation was 64.2% (95% CI: 40.8%, 80.3%) at 3 years and 44.5% (95% CI: 21.2%, 65.5%) at 6 years. The main indication for late reoperation was conduit degeneration. Freedom from a right ventricular outflow tract reoperation was significantly higher, and the number of reoperations per patient was lower when a double root translocation had been performed (P  =   0.03). CONCLUSIONS The Bex-Nikaidoh operation effectively relieved left ventricular outflow tract obstruction. A double root translocation further increased procedural complexity but was associated with better mid-term freedom from a right ventricular outflow tract reoperation. It should be considered in suitable patients. [ABSTRACT FROM AUTHOR]

Reconstruction of right ventricular outflow tract in patients with congenital heart disease in various age groups remains a controversial issue. Currently, a little is known about the fate of secondary and subsequent conduit. The aim of the study was to determine risk factors of conduit failure, evaluate long-term conduit survival, find out which type of conduit should be preferred in case of reoperations. We performed a retrospective analysis of a total of 249 records of valved conduit secondary and subsequent replacement in right ventricular outflow tract in 197 patients. Median follow-up was 5.7 years. The study endpoints were defined as conduit explants; balloon dilatation of the graft (excluding balloon dilatation of left/right pulmonary artery), transcatheter pulmonary valve implantation; heart transplantation or death of the patient. There were total of 21 deaths (11% mortality) among 197 patients during the follow-up, 2 patients underwent heart transplant, in 23 implanted conduits pulmonary angioplasty or/including transcatheter pulmonary valve implantation was afterwards performed due to graft failure, conduit had to be explanted in 46 cases. After 28 years follow-up, freedom from graft failure after 5 years was 77%, 48% after 10 years and 21% after 15 years. Reoperative right ventricular outflow tract reconstruction demonstrates good mid-term and acceptable long-term outcomes regardless of the type of conduit implanted. Worse long-term graft survival of secondary and further conduits is associated with younger age of the recipient at implantation, small size of the conduit, younger age of donor and male donor in case of allograft implantation. [ABSTRACT FROM AUTHOR]

Assessment of the right ventricular outflow tract and pulmonary arteries (RVOT) for percutaneous pulmonary valve implantation (PPVI) uses discrete measurements (diameters and lengths) from medical images. This multi-centre study identified the 3D RVOT shape features prevalent in patients late after surgical repair of congenital heart disease (CHD). A 3D RVOT statistical shape model (SSM) was computed from 81 retrospectively selected CHD patients (14.7 ± 6.8 years) who required pulmonary valve replacement late after surgical repair. A principal component analysis identified prevalent shape features (modes) within the population which were compared with standard geometric measurements (diameter, length and surface area) and between sub-groups of diagnosis, RVOT type and dysfunction. Shape mode 1 and 2 represented RVOT size and curvature and tapering and length, respectively. Shape modes 3–5 related to branch pulmonary artery calibre, conical vs. bulbous RVOTs and RVOT curvature, respectively. Tetralogy of Fallot, transannular patch type and regurgitant RVOTs were larger and straighter while conduit and stenotic types were longer and more cylindrical than other subgroups. This SSM analysed the main 3D shape features present in a population of RVOTs, exploiting the wide 3D anatomical information provided by routine imaging. This morphological information may have implications for PPVI patient selection and device design.

Background This study aimed to investigate the predictive value of parameters of every precordial lead and their combinations in differentiating between idiopathic ventricular arrhythmias (IVAs) from the right ventricular outflow tract and aortic sinus of Valsalva (ASV). Methods and Results Between March 1, 2018, and December 1, 2021, consecutive patients receiving successful ablation of right ventricular outflow tract or ASV IVAs were enrolled. The amplitude and duration of the R wave and S wave were measured in every precordial lead during IVAs. These parameters were either summed, subtracted, multiplied, or divided to create different indexes. The index with the highest area under the curve to predict ASV IVAs was developed, compared with established indexes, and validated in an independent prospective multicenter cohort. A total of 150 patients (60 men; mean age, 45.3±16.4 years) were included in the derivation cohort. The RV1+RV3 index (summed R‐wave amplitude in leads V1 and V3) had the highest area under the curve (0.942) among the established indexes. An RV1+RV3 index >1.3 mV could predict ASV IVAs with a sensitivity of 95% and a specificity of 83%. Its predictive performance was maintained in the validation cohort (N=109). In patients with V3 R/S transition, an RV1+RV3 index >1.3 mV could predict ASV IVAs, with an area under the curve of 0.892, 93% sensitivity, and 75% specificity. Conclusions The RV1+RV3 index is a simple and novel criterion that accurately differentiates between right ventricular outflow tract and ASV IVAs. Its performance outperformed established indexes, making it a valuable tool in clinical practice.

Open in new tab Download slide OBJECTIVES Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS Single-centre, retrospective analysis of patients who underwent surgical repair with the diagnosis of ToF-CAVC from 1979 to 2022, divided into 2 different periods and compared. RESULTS A total of 116 patients were included: 1979–2007 (n  = 61) and 2008–2021 (n  = 55). Balanced CAVC (80%) and Rastelli type C CAVC (81%) were most common. Patients in the later era were younger (4 vs 14 months, P  < 0.001), fewer had trisomy 21 (60% vs 80%, P  = 0.019) and fewer had prior palliative prior procedures (31% vs 43%, P  < 0.001). In the earlier era, single-patch technique was more common (62% vs 16%, P  < 0.001), and in recent era, double-patch technique was more common (84% vs 33%, P  < 0.001). In the earlier era, right ventricular outflow tract was most commonly reconstructed with transannular patch (51%), while in more recent era, valve-sparing repairs were more common (69%) (P  < 0.001). In-hospital mortality was 4.3%. The median follow-up was 217 and 74 months for the first and second eras. Survival for earlier and later eras at 2-, 5- and 10-year follow-up was (85.1%, 81.5%, 79.6% vs 94.2%, 94.2%, 94.2% respectively, log-rank test P  = 0.03). CONCLUSIONS The surgical approach to ToF-CAVC has evolved over time. More recently, patients tended to receive primary repair at younger ages and had fewer palliative procedures. Improved surgical techniques allowing for earlier and complete repair have shown a decrease in mortality, more valve-sparing procedures without an increase in total reoperations. Presented at the 37th EACTS Annual Meeting, Vienna, Austria. [ABSTRACT FROM AUTHOR]

Background: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF). Methods: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length. Results: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p

Ventricular hemangiomas are rare benign tumors, pose diagnostic and therapeutic complexities. We report a case of a 52-year-old female with essential hypertension who developed a systolic ejection murmur during a hypertension clinic visit. The echocardiogram revealed a hyperechoic mass obstructing the right ventricular outflow tract, causing enlargement of the right atrium and ventricle, with a reduction in the right ventricular ejection fraction. Due to the risk of death, the patient underwent an emergency surgical resection along with tricuspid valve replacement. Postoperative recovery was uneventful, and subsequent cardiac magnetic resonance imaging showed an improvement in ejection fraction without residual tumor. This case highlights the diagnosis and therapeutic complexities of ventricular hemangiomas. With this report, we aim to provide a comprehensive review of ventricular hemangiomas and to enhance understanding of this condition for improved patient care. [ABSTRACT FROM AUTHOR]

A 72-year-old man presented with breathlessness and a systolic murmur. Extensive diagnostic work-up revealed a rare pulmonary artery intimal sarcoma mimicking a right ventricular outflow tract thrombus and impacting a cardiac pacemaker lead. Surgical resection, pathology confirmation, and management strategies are discussed, highlighting the challenges of treating this rare malignancy.

The most performed percutaneous valve replacement procedure is for the aortic valve. In recent years, there have been developments in percutaneous valve replacement methods for other valves, including the pulmonary valve. Computed tomography plays a crucial role in various stages of procedure planning and provides essential information regarding potential complications after the procedure. We present images documenting step by step how to evaluate cardiac computed tomography in the qualification procedure for transcatheter pulmonary valve implantation in patients with dysfunctional native right ventricular outflow tract. [ABSTRACT FROM AUTHOR]

Right ventricular (RV) function is a determining factor for clinical outcomes in patients undergoing tricuspid valve surgery (TVS). Our aim was to investigate the importance of the function of the right ventricular outflow tract (RVOT), which is an important anatomical region of the RV, in patients underwent TVS. 104 patients who underwent TVS were analyzed retrospectively. Patients with previous cardiac surgery, congenital heart disease, or heart failure were excluded. The parasternal short-axis view at the level of the aortic root was used to measure RVOT dimensions and RVOT fractional shortening (RVOT-FS). The effect of RVOT diameter and function on major adverse cardiac events (MACE) after TVS was investigated. In our study, MACE, consisting of pacemaker implantation, acute kidney injury, postoperative atrial fibrillation and mortality, was developed at 44 (42.3%) patients.We compared the predictive performances of RVOT end-systolic (RVOTs) diameter, RVOT end-diastolic (RVOTd) diameter, RVOT-FS and RV diameters in prediction of MACE. The model including the RVOTs had higher AUC, R2 and likelihood ratio X2 values (0.775, 0.287 and 25.0, respectively) than RVOTd (0.770, 0.279 and 24.2, respectively) and RVOT-FS (0.750, 0.215 and 18.1, respectively). RVOT diameters showed better performance in predicting MACE than RV diameters. Moreover, there was statistically significant association between RVOTs, RVOTd and MACE (p value were 0.014 and 0.027, respectively), while no association between RVOT-FS and MACE (p value was 0.177). In summary, we determined that the RVOT diameters are important predictors for the in-hospital clinical outcomes of patients who underwent TVS. [ABSTRACT FROM AUTHOR]

Ventricular premature beats originating from the right ventricular outflow tract can have myocardial extensions to the pulmonary valve and pulmonary artery. Treatment may consist of catheter ablation combined with the use of three-dimensional mapping to determine the exact location of ectopy. The location of ectopy relative to the pulmonary valve may be hard to ascertain. Intracardiac echocardiography (ICE) is a noninvasive approach by which one can determine the relationship of the pulmonary valve relative to the ablation catheter prior to ablation. ICE has achieved increasing popularity during the ablation of other arrhythmias such as tricuspid valve arrhythmias and has been shown to be helpful in guiding catheter placement prior to ablation. The additional information gained from deploying ICE may ensure more precise ablation, prevent theoretical damage to the pulmonary valve, and alleviate the need for a repeat procedure. Here, we present a case involving the use of ICE during a pediatric patients second ablation procedure to precisely determine the location of ectopy of nonsustained ventricular tachycardia originating from the distal pulmonary valve.

Abstract Pulmonary artery sarcoma (PAS) is a sporadic malignant tumor that mainly originates from the pulmonary arteries. However, PAS may also involve the right ventricular outflow tract (RVOT) and lead to obstruction, syncope, or sudden death. Early diagnosis and complete surgical resection are essential to prolong survival and improve the quality of life of patients with PAS. Herein, we report a case of a young female patient admitted for pulmonary malignancy and acute pulmonary embolism. The patient had a mass in the RVOT, which was detected by transthoracic echocardiography. Computed tomography and magnetic resonance imaging revealed the invasion depth and extent of the lesions. Surgical resection improved hemodynamics, while pathological and immunohistochemical tests confirmed the diagnosis of a pulmonary artery sarcoma. Local recurrence was detected in the adjacent tissues about two months after the surgery. Given the potential risk of reoperation, the patient was suggested to undergo conservative treatment.

Abstract Background Radiofrequency catheter ablation is the preferred treatment choice for ventricular arrhythmias (VAs) originating from right ventricular outflow tract (RVOT) in symptomatic patients and is usually performed under fluoroscopy guidance. Zero‐fluoroscopy (ZF) ablations using 3D mapping system applied for treatment of various types of arrhythmias are trending and practiced in many centers around the world, but rarely done in Vietnam. The objective of this study was to evaluate the efficacy and safety of zero‐fluoroscopy ablation of RVOT VAs, compared with fluoroscopy‐guided ablation without a 3D electroanatomic mapping (EAM) system. Methods and Results We conducted a nonrandomized, prospective single‐center study including 114 patients with RVOT VAs that had electrocardiographic features of typical left bundle branch block, inferior axis QRS morphology, and a precordial transition ≥ V3, from May 2020 to July 2022. The patients were assigned (without randomization) to two different approaches of either zero‐fluoroscopy ablation under the guidance of the Ensite system (ZF group) or fluoroscopy‐guided ablation without a 3D EAM (fluoroscopy group) in a 1:1 ratio. After a follow‐up time of 5.0 ± 4.9 months and 6.9 ± 9.3 months in the ZF and fluoroscopy groups, respectively, the results showed a higher success rate in the fluoroscopy group than in the complete ZF group (87.3% vs 86.8%), although the difference was not statistically significant. No major complication was noted in both the groups. Conclusion ZF ablation for RVOT VAs can be done safely and effectively using the 3D electroanatomic mapping system. The results of ZF approach are comparable to that of the fluoroscopy‐guided approach without a 3D EAM system.

Abstract Background Reinterventions after tetralogy of Fallot repair (TOF) remains a common clinical problem. The objective of this study was to evaluate types of reintervention after TOF repair and identify the risk factors for reinterventions. Methods This retrospective study was conducted from 2010 to 2022 and included 171 patients with complete TOF repair. Patients were grouped according to the occurrence of reintervention into two groups: patients who did not have reintervention (n = 138) and those who required reintervention (n = 33). Results Median follow-up was 36 (13–67) months. The first reintervention was required in 33 patients. Freedom from the first reintervention at 1, 3, 5, and 7 years was 91%, 85%, 81%, and 76%, respectively. Surgical reintervention was required in 12 patients and transcatheter intervention in 21 patients. Second reinterventions were required in 11 patients; 4 had surgery, and 7 had a transcatheter intervention. Third reinterventions were performed on two patients; one had surgery, and one had a transcatheter intervention. The most common interventions were performed at the level of pulmonary arteries (n = 17), followed by the pulmonary valve and the right ventricular outflow tract (n = 15). The risk of reintervention was associated with the low weight (HR: 0.65 (95% CI: 0.48–0.88); P = 0.005) and small LPA diameter (HR: 0.36 (95% CI: 0.21–0.60); P < 0.001) at the time of the primary intervention and the nonuse of the transannular patch (HR: 0.27 (95% CI: 0.08–0.85); P = 0.026). Conclusions The risk of reintervention is high after tetralogy of Fallot repair. In our experience, the smaller the left pulmonary artery and weight at the repair time increased the risk of reintervention. Using a transannular patch in our series was associated with a lower risk of reintervention.

Background: Surgical repair of tetralogy of Fallot (TOF) consists of adequate ventricular septal defect closure and relief of right ventricular outflow tract (RVOT) obstruction to the greatest extent possible. The residual RVOT gradient can be due to dynamic and fixed obstruction, and high gradient is sometimes seen even after a satisfactory RVOT resection often confirmed with intraoperative TransEsophageal Echocardiogram (TEE). Aim: The present study was conducted to study the changes in RVOT residual gradient in the early postoperative period. We analyzed the change in residual gradient by invasive monitoring intraoperatively after separating from cardiopulmonary bypass (CPB) in a case of intracardiac repair (ICR) for TOF and compared the readings 24 h after extubating the patients in intensive care unit (ICU). Materials and Methods: This was an observation study done in the Department of CTVS, Advanced Cardiac Centre, PGIMER, and Chandigarh from February 2018 to March 2019. A total of thirty patients with preoperative diagnosis of TOF were included in the study. After ICR for TOF, postseparation from CPB, RVOT gradient was measured using 23G needle connected to pressure transducer and compared with RVOT gradient measured 24 h postextubating using invasive line kept intraoperatively in pulmonary artery and RVOT. Results: There was a significant decrease in residual RVOT gradient postoperatively in ICU after 24 h of extubating, in comparison to intraoperative postbypass residual RVOT gradient. Postbypass residual RVOT gradient was 11.33 ± 1.39 that decreased to 7.81 ± 1.29 24 h after extubating (P < 0.05). Patients in whom pulmonary valve was preserved had greater postbypass residual RVOT gradient (12.44 ± 1.13) than patients with transannular patch (10.5 ± 0.90). However, both decreased after 24 h of extubating (9 ± 0.7 and 6.9 ± 0.8, respectively). Conclusion: Once satisfactory RVOT resection for fixed obstruction is done and is confirmed using TEE, the residual gradient, if marginally high, can be ignored as residual gradient significantly decreases after extubation and hemodynamic improvement is seen in postoperative period.

Large vessel stenosis is currently successfully treated with percutaneous stent implantation. Zephyr stent is a cobalt-chromium peripheral stent specifically designed for large-vessel stenting. Its specific “S-” and “C-” shaped flexible polylinks prevent stent foreshortening and recoil. We report our experience of using Zephyr among the pediatric population and their medium-term outcome. The study included a total of 19 patients treated with 21 Zephyr stents. The indications for stenting included coarctation of the aorta (four patients), stenosed pulmonary artery (eight patients), stenosed right ventricular outflow tract (five patients) or conduit (one patient), and percutaneous Fontan (one patient). All the procedures were successful. None of the patients showed any stent-related complications. Stent fractures, foreshortening, or recoil were not seen during follow-up. Our medium-term (1–20 months) follow-up showed a favorable outcome. One patient developed restenosis. Zephyr stent is a good therapeutic option for large-vessel stenting. However, it needs large-scale studies for further confirmation.

The right ventricular outflow tract (RVOT) is the major origin of ventricular arrhythmias, including premature ventricular contractions, idiopathic ventricular arrhythmias, Brugada syndrome, torsade de pointes, long QT syndrome, and arrhythmogenic right ventricular cardiomyopathy. The RVOT has distinct developmental origins and cellular characteristics and a complex myocardial architecture with high shear wall stress, which may lead to its high vulnerability to arrhythmogenesis. RVOT myocytes are vulnerable to intracellular sodium and calcium overload due to calcium handling protein modulation, enhanced CaMKII activity, ryanodine receptor phosphorylation, and a higher cAMP level activated by predisposing factors or pathological conditions. A reduction in Cx43 and Scn5a expression may lead to electrical uncoupling in RVOT. The purpose of this review is to update the current understanding of the cellular and molecular mechanisms of RVOT arrhythmogenesis. [ABSTRACT FROM AUTHOR]

Large vessel stenosis is currently successfully treated with percutaneous stent implantation. Zephyr stent is a cobalt-chromium peripheral stent specifically designed for large-vessel stenting. Its specific "S-" and "C-" shaped flexible polylinks prevent stent foreshortening and recoil. We report our experience of using Zephyr among the pediatric population and their medium-term outcome. The study included a total of 19 patients treated with 21 Zephyr stents. The indications for stenting included coarctation of the aorta (four patients), stenosed pulmonary artery (eight patients), stenosed right ventricular outflow tract (five patients) or conduit (one patient), and percutaneous Fontan (one patient). All the procedures were successful. None of the patients showed any stent-related complications. Stent fractures, foreshortening, or recoil were not seen during follow-up. Our medium-term (1-20 months) follow-up showed a favorable outcome. One patient developed restenosis. Zephyr stent is a good therapeutic option for large-vessel stenting. However, it needs large-scale studies for further confirmation. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot (ToF) is characterized by stenosis causing partial obstruction of the right ventricular outflow tract, typically alleviated through the surgical application of a vessel patch made from a biocompatible material. In this study, we use computational simulations to compare the mechanical performance of four patch materials for various stenosis locations. Nine idealized pre-operative ToF geometries were created by imposing symmetrical stenoses on each of three anatomical sub-regions of the pulmonary arteries of three patients with previously repaired ToF. A virtual surgery methodology was implemented to replicate the steps of vessel de-pressurization, surgical patching, and subsequent vessel expansion after reperfusion. Significant differences in patch average stress (p < 0.001) were found between patch materials. Biological patch materials (porcine xenopericardium, human pericardium) exhibited higher patch stresses in comparison to synthetic patch materials (Dacron and PTFE). Observed differences were consistent across the various stenosis locations and were insensitive to patient anatomy. [ABSTRACT FROM AUTHOR]

Patch augmentation of the right ventricular outflow tract (RVOT) and pulmonary artery (PA) arterioplasty are relatively common procedures in the surgical treatment of patients with congenital heart disease. To date, several patch materials have been applied with no agreed upon clinical standard. Each patch type has unique performance characteristics, cost, and availability. There are limited data describing the various advantages and disadvantages of different patch materials. We performed a review of studies describing the clinical performance of various RVOT and PA patch materials and found a limited but growing body of literature. Short-term clinical performance has been reported for a multitude of patch types, but comparisons are limited by inconsistent study design and scarce histologic data. Standard clinical criteria for assessment of patch efficacy and criteria for intervention need to be applied across patch types. The field is progressing with improvements in outcomes due to newer patch technologies focused on reducing antigenicity and promoting neotissue formation which may have the ability to grow, remodel, and repair. [ABSTRACT FROM AUTHOR]

An aclinical Havanese dog was diagnosed with a membranous restrictive ventricular septal defect. The patient was represented later in their natural history due to the development of syncope. At that time the patient was diagnosed with acquired pulmonary infundibular stenosis. Balloon dilation of the stenosis was performed successfully twice over the patient's lifetime. The patient died suddenly approximately 14 months after the second balloon dilation. A discussion regarding primary infundibular pulmonary stenosis versus causes of acquired infundibular pulmonary stenosis including anomalous muscle bundles (double chamber right ventricle), tetralogy of Fallot, and infundibular stenosis is presented. [ABSTRACT FROM AUTHOR]