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Background: Children with repaired tetralogy of Fallot (rToF) often have pulmonary regurgitation with right ventricular (RV) dilatation and dysfunction, whereas less is known about the effect on the left ventricle (LV). The aim was to investigate LV haemodynamic variables derived from non-invasive pressure-volume loops in children with rToF and how they compare to controls and previous research on adults. Materials and methods: Ten children with rToF and pulmonary regurgitation (12 years [10–13], 6 males) and 10 age- and sex-matched healthy controls (12 years [10–14], 6 males) underwent brachial blood pressure in conjunction with cardiac magnetic resonance imaging. Pressure-volume loops were derived by brachial blood pressure together with LV volumes throughout the cardiac cycle in short-axis cine images yielding several haemodynamic variables, including arterial elastance. The RV endocardial border was delineated in end-diastole and end-systole. Results: Children with rToF and pulmonary regurgitation had larger RV end-diastolic volume (136 [114-156]) than controls (100 [94-112] ml/m2; p = 0.0015) and smaller LV end-diastolic volume (83 [58–91] ml/m2) than controls (101 [92–110] ml/m2; p = 0.002). Arterial elastance was higher in children with rToF (1.5 [1.3-2.7] mmHg/ml) than in controls (1.1 [1.0-1.5] mmHg/ml; p = 0.02). Heart rate was higher in children with rToF (77 [74-81] bpm) than in controls (69 [65–75] bpm; p = 0.027). Conclusion: Children with rToF had higher arterial elastance and heart rate than controls, likely due to increased sympathetic tone to compensate for impaired LV filling following pulmonary regurgitation. If this contributes to increased risk of adverse cardiovascular and cerebrovascular events remains to be studied. [ABSTRACT FROM AUTHOR]

A dilated native right ventricular outflow tract (RVOT) presents unique challenges for transcatheter management using balloon-expandable valves. The Alterra Adaptive Prestent was designed to expand transcatheter therapy to treat patients with dilated RVOTs. The aim of this study was to report 2-year outcomes of the main cohort of the ALTERRA (Multicenter Study of Congenital Pulmonic Valve Dysfunction Studying the SAPIEN 3 THV With the Alterra Adaptive Prestent) pivotal trial using the prestent with transcatheter pulmonary valve replacement. The prestent device used with the 29 mm SAPIEN 3 transcatheter heart valve (THV) was evaluated for the management of patients with moderate or greater pulmonary valve regurgitation (PR). The primary endpoint was THV dysfunction at 6 months, defined as a nonhierarchical composite of RVOT/pulmonary valve reintervention, moderate or greater total PR on transthoracic echocardiography, and mean RVOT/pulmonary valve gradient 35 mm Hg or greater on transthoracic echocardiography. The primary endpoint and outcomes through 2 years are presented in this analysis. Of 97 patients screened, 60 underwent prestent and THV implantation. There was 1 staged procedure. No patients had THV dysfunction at 6 months. At 2 years, the majority of patients (92.5%) had mild or less PR, with no reports of coronary compression, stent fractures warranting reintervention, or endocarditis. Of the 21 patients (34.4%) who experienced early (days 0-1) arrhythmias, 12 had episodes of nonsustained ventricular tachycardia that resolved with medication. One patient underwent reintervention secondary to an iatrogenic RVOT obstruction; there were no deaths or explantations through 2 years. The Alterra prestent in combination with the SAPIEN 3 THV has excellent outcomes at 2 years, with no significant valve dysfunction in the main pivotal cohort. [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract Introduction Pulmonary regurgitation (PR) remains a common sequela in patients following surgically corrected TOF, and may lead to progressive right ventricle dilatation and dysfunction. The conventional approach of redo-sternotomy for pulmonary valve replacement (PVR) is associated with increased operative time as well as risks of bleeding and injury to the heart and great vessels. Thus, left anterior mini-thoracotomy has become an alternative approach in eliminating the risks of redo-sternotomy in these patients. This series aimed to determine the outcomes of minimally invasive pulmonary valve replacement after surgical TOF correction. Methods A retrospective analysis was conducted on 24 patients with severe PR post-surgical TOF correction who underwent left anterior mini-thoracotomy PVR in Penang General Hospital from January 2021 to January 2023. Results The median age was 23.5 years (I.Q.range 17.6-36.3), with a male:female ratio of 1:4. Majority of patients had mild to moderate symptoms prior to surgery and 19 patients (79.1%) were on regular diuretics medication. All patients had severe free-flow PR with evidence of right ventricular dilatation and dysfunction. Magnetic Resonance Imaging and computed tomography of pulmonary artery were performed prior to surgery. Minimally invasive PVR was performed on all patients via left upper anterior mini-thoracotomy and femoral-femoral bypass without cardioplegic arrest. The operative time and cardiopulmonary bypass time were 208 (I.Q.range 172-324) and 98.6 minutes(I.Q.range 87.4-152.4) respectively. The time to wean off inotropes postoperatively was 6.2 hours (I.Q.range1.4-14.8), and no postoperative arrhythmia and chest re-exploration were reported. Most patients stayed in Intensive Care Unit (ICU) for 10.8 hours (I.Q.range 8.4-36.5), and the total hospital stay was 4.2 days (I.Q.range 3.4-7.6). 2 patients (11.1%) required blood transfusion postoperative. There was no paravalvular leak and no mortality during the follow-up period of up to 28 months. Conclusion Minimally invasive PVR after surgical correction of TOF is a safe alternative to the conventional redo-sternotomy approach in patients with favorable anatomy. This approach is able to reduce the risks associated with redo-sternotomy, particularly bleeding and injury to mediastinal structures, with the additional benefit of expedited recovery and hospital discharge. Our series has shown a safe and efficient approach in these patients with favorable outcomes.

Introduction: Pulmonary regurgitation (PR) remains a common sequela in patients following surgically corrected TOF, and may lead to progressive right ventricle dilatation and dysfunction. The conventional approach of redo-sternotomy for pulmonary valve replacement (PVR) is associated with increased operative time as well as risks of bleeding and injury to the heart and great vessels. Thus, left anterior mini-thoracotomy has become an alternative approach in eliminating the risks of redo-sternotomy in these patients. This series aimed to determine the outcomes of minimally invasive pulmonary valve replacement after surgical TOF correction. Methods: A retrospective analysis was conducted on 24 patients with severe PR post-surgical TOF correction who underwent left anterior mini-thoracotomy PVR in Penang General Hospital from January 2021 to January 2023. Results: The median age was 23.5 years (I.Q.range 17.6-36.3), with a male:female ratio of 1:4. Majority of patients had mild to moderate symptoms prior to surgery and 19 patients (79.1%) were on regular diuretics medication. All patients had severe free-flow PR with evidence of right ventricular dilatation and dysfunction. Magnetic Resonance Imaging and computed tomography of pulmonary artery were performed prior to surgery. Minimally invasive PVR was performed on all patients via left upper anterior mini-thoracotomy and femoral-femoral bypass without cardioplegic arrest. The operative time and cardiopulmonary bypass time were 208 (I.Q.range 172-324) and 98.6 minutes(I.Q.range 87.4-152.4) respectively. The time to wean off inotropes postoperatively was 6.2 hours (I.Q.range1.4-14.8), and no postoperative arrhythmia and chest re-exploration were reported. Most patients stayed in Intensive Care Unit (ICU) for 10.8 hours (I.Q.range 8.4-36.5), and the total hospital stay was 4.2 days (I.Q.range 3.4-7.6). 2 patients (11.1%) required blood transfusion postoperative. There was no paravalvular leak and no mortality during the follow-up period of up to 28 months. Conclusion: Minimally invasive PVR after surgical correction of TOF is a safe alternative to the conventional redo-sternotomy approach in patients with favorable anatomy. This approach is able to reduce the risks associated with redo-sternotomy, particularly bleeding and injury to mediastinal structures, with the additional benefit of expedited recovery and hospital discharge. Our series has shown a safe and efficient approach in these patients with favorable outcomes. [ABSTRACT FROM AUTHOR]

ABSTRACT Introduction and objectives: Surgery for congenital heart defects with right ventricular outflow tract (RVOT) stenosis often results in significant pulmonary regurgitation, requiring pulmonary valve replacement in the long term. Despite the development of balloon-expandable prostheses, the native RVOT frequently dilates beyond the maximum diameters allowed for these valves. To allow percutaneous pulmonary valve implantation (PPVI) in these patients, clinical trials have been initiated with self-expanding prostheses, including the PULSTA valve. The aim of this study was to report the initial experience with this valve at three Spanish hospitals. Methods: Descriptive study presenting the results of PPVI with the PULSTA prosthesis in patients with native RVOT and pulmonary regurgitation. Results: We included 10 patients with a mean age of 15 ± 2.8 years. The implantation was successful in all patients, with no major complications occurring during the procedure. The mean length of follow-up was 18 [range, 2-35] months. In 8 patients, cardiac magnetic resonance was performed at 6 months, revealing a reduction in mean end-diastolic volume (131.7 ± 31.7 mL/m2 vs 100.3 ± 28.9 mL/m2) and end-systolic volume (68 ± 20.8 mL/m2 vs 57 ± 18.5 mL/m2). Conclusions: The PULSTA prosthesis offers a safe, feasible, and effective alternative for PPVI in patients with native...

Right ventricular (RV) diastolic stiffness is an independent predictor of survival and is strongly associated with disease severity in patients with precapillary pulmonary hypertension (PH). Therefore, a fully validated echocardiographic method for assessing RV diastolic stiffness needs to be established. This study aimed to compare echocardiography-derived RV diastolic stiffness and invasively measured pressure-volume loop-derived RV diastolic stiffness in patients with precapillary PH. We studied 50 consecutive patients with suspected or confirmed precapillary PH who underwent cardiac catheterization, magnetic resonance imaging, and echocardiography within a 1-week interval. Single-beat RV pressure-volume analysis was performed to determine the gold standard for RV diastolic stiffness. Elevated RV end-diastolic pressure (RVEDP) was defined as RVEDP ≥ 8 mmHg. Using continuous-wave Doppler and M-mode echocardiography, an echocardiographic index of RV diastolic stiffness was calculated as the ratio of the atrial-systolic descent of the pulmonary artery-RV pressure gradient derived from pulmonary regurgitant velocity (PRPGDAC) to the tricuspid annular plane movement during atrial contraction (TAPMAC). PRPGDAC/TAPMAC showed significant correlation with β (r = 0.54, p < 0.001) and RVEDP (r = 0.61, p < 0.001). A cut-off value of 0.74 mmHg/mm for PRPGDAC/TAPMAC showed 83% sensitivity and 93% specificity for identifying elevated RVEDP. Multivariate analyses indicated that PRPGDAC/TAPMAC was independently associated with disease severity in patients with precapillary PH, including substantial PH symptoms, stroke volume index, right atrial size, and pressure. PRPGDAC/TAPMAC, based on pulmonary regurgitation velocity waveform analysis, is useful for the noninvasive assessment of RV diastolic stiffness and is associated with prognostic risk factors in precapillary PH. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. centers. The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes. [Display omitted] [ABSTRACT FROM AUTHOR]

Absent pulmonary valve syndrome is a rare cardiac malformation, usually associated with tetralogy of Fallot. Its presence with intact interventricular septum is even less common with case reports or very small series in the literature. We describe a case who presented with symptoms of right heart failure due to an absent pulmonary valve with severe pulmonary regurgitation and small atrial septal defect. Diagnostic evaluation revealed grossly dilated main pulmonary artery and rudimentary leaflets of the pulmonary valve with severe regurgitation. Ventricular septum was intact and there was no patent ductus arteriosus. Later, the patient underwent surgical prosthetic pulmonary valve implantation.

Background: Pulmonary artery dilatation is described mostly in association with pulmonary hypertension. Patients/Methods: Study analysis: 60 patients with pulmonary arterial hypertension in congenital heart disease (PAH-CHD); 64 with repaired tetralogy of Fallot/pulmonary regurgitation (rTOF/PR); and 80 healthy (NORMAL). Measured were: main pulmonary artery (MPA) diameter and MPA/ascending aorta (Ao asc) ratio, by echocardiography (ECHO) and computer tomography or magnetic resonance imaging (CT/MRI). Results: In MPA diameter, significant differences between PAH-CHD, rTOF/PR, and NORMAL were found (median): 37 vs. 27 vs. 21 mm (p < 0.0001). In MPA/Ao asc ratio, there was a difference between PAH-CHD and NORMAL (median): 1.3 vs. 0.8 (p < 0.0001), but not between rTOF/PR and NORMAL: 0.74 vs. 0.8 (p = 0.3). Significant MPA dilatation (>40 mm) was present: in PAH-CHD, 35% (ECHO) and 76.9% (CT/MRI) of patients, while in rTOF/PR, 3.1% (ECHO) and 7.8% (CT/MRI). Severe MPA dilatation (>50 mm) occurred only in PAH-CHD: 16.7% (ECHO) and 31.4% (CT/MRI), while not in rTOF/PR. There was a significant correlation between ECHO and CT/MRI measurements, but ECHO was underestimated in all parameters. Conclusions: MPA dilatation due to pressure overload is more frequent and more severe; volume overload also leads to MPA dilatation but is less severe. The MPA/Ao asc ratio is not reliable for MPA dilatation estimation in rTOF/PR. [ABSTRACT FROM AUTHOR]

In patients with congenital heart disease, transcatheter pulmonary valve implantation is the treatment of choice in dysfunctional right ventricular outflow tracts (RVOT). The self-expandable Venus-P valve was approved recently for use in large native tracs. It is reported the experience of the first case of successful implantation of Venus-P pulmonary valve in Colombia. Ours was a 16-year-old patient with Tetralogy of Fallot, with complete correction at 11 months of life. The patient has severe pulmonary insufficiency and functional class deterioration. Cardiac catheterization was performed to place a 34 x 30 mm Venus-P valve, a procedure carried out without complications, evidencing significant improvement in the diastolic pressure of the pulmonary artery, without final gradient between the right ventricle and pulmonary artery. Reports exist of experiences in Venus-P valve implant globally with favorable results; percutaneous valve placement represents decreased procedure times and hospital stays. We believe percutaneous valve replacement in pulmonary position in native RVOTs is a possible and safe option to improve pulmonary insufficiency and right ventricular dysfunction in these types of patients. [ABSTRACT FROM AUTHOR]

In patients with repaired tetralogy of Fallot (rTOF), the regurgitant fraction (RF) in left pulmonary artery (LPA) and right pulmonary artery (RPA) is usually unequal. The morphometrics may play a crucial role in this RF discrepancy. Cardiovascular MR of 79 rTOF patients and 20 healthy controls were retrospectively enrolled. Forty-four from the 79 patients were matched in age, sex and body surface area to the 20 controls and were investigated for: (1) phase-contrast flow of main pulmonary artery (MPA), LPA, and RPA; (2) vascular angles: the angles between the thoracic anterior–posterior line (TAPL) with MPA (θM–AP), MPA with RPA (θM–R), and MPA with LPA (θM–L); (3) cardiac angle, the angle between TAPL and the interventricular septum; (4) area ratio of bilateral lung and hemithorax regions. Compared with the 20 controls, the 44 rTOF patients exhibited wider θM–AP, sharper θM–L angle, and a smaller θM–L/θM–R ratio. In the 79 rTOF patients, LPA showed lower forward, backward, and net flow, and greater RF as compared with RPA. Multivariate analysis showed that the RF of LPA was negatively associated with the θM–L/θM–R ratio and the age at surgery (R2 = 0.255). Conversely, the RF of RPA was negatively associated with the left lung/left hemithorax area ratio and cross-sectional area (CSA) of LPA, and positively associated with CSA of RPA and MPA (R2 = 0.366). In rTOF patients, the RF of LPA is more severe than that of RPA, which may be related to the vascular morphometrics. Different morphometric parameters are independently associated with the RF of LPA or RPA, which may offer potential insights for surgical strategies. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) has expanded and evolved since its initial commercial approval in the United States in 2010. This study sought to characterize real-world practice, including patient selection, procedural outcomes, complications, and off-label usage. Characteristics and outcomes for patients undergoing balloon-expandable TPVR were collected from the American College of Cardiology National Cardiovascular Data Registry IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry. Between April 2016 and March 2021, 4,513 TPVR procedures were performed in patients with a median age of 19 years, 57% with a Melody (Medtronic Inc) and 43% with a SAPIEN (Edwards Lifesciences) valve. Most implanting centers performed <10 cases annually. One-third of transcatheter pulmonary valve implants were into homograft conduits, one-third were into bioprosthetic valves (BPVs), 25% were in native or patched right ventricular outflow tracts (RVOTs), and 6% were into Contegra (Medtronic Inc) conduits. Over the course of the study period, SAPIEN valve use grew from ∼25% to 60%, in large part because of implants in patients with a native/patched RVOT. Acute success was achieved in 95% of patients (95.7% in homografts, 96.2% in BPVs, 94.2% in native RVOTs, and 95.4% in Contegra conduits). Major adverse events occurred in 2.4% of procedures, more commonly in patients with a homograft (2.9%) or native RVOT (3.4%) than a prior BPV (1.4%; P = 0.004). This study describes novel population data on the use and procedural outcomes of TPVR with balloon-expandable valves. Over time, there has been increasing use of TPVR to treat regurgitant native RVOT anatomy, with the SAPIEN valve more commonly used for this application. [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract Background Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. Results When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. Conclusions This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot.

ABSTRACT Introduction and objectives: Percutaneous pulmonary valve implantation is currently a common procedure in patients with congenital heart disease with a dysfunctional right ventricular outflow tract. Until April 2022, there were only balloon-expandable valves available in Europe, which did not cover the needs of the different anatomies of the right ventricular outflow tract. Since that date we have available the self-expandible Venus P-valve (Venus MedTech, China). We present the initial experience with this new percutaneous pulmonary valve in our center. Methods: Description of the valve implants with the new self-expandible valve performed between September and November 2022. Results: Eight valve implants have been performed, all successful and without severe complications during the procedure. All patients had severe pulmonary regurgitation with a dilated right ventricle and severe dilatation of the pulmonary trunk and were not good candidates for percutaneous balloon-expandable valves. Five patients had a tetralogy of Fallot. In 7 patients, the implant was performed through the femoral vein and in one through jugular access. As a safety measure, all valves were implanted through a DrySeal sheath (Gore, W.L. Gore & Associates, Inc., United States). The mean hospital stay was 3-day. Conclusions: Valve implantation with the new self-expandible Venus P-valve was, in our preliminary experience, a safe and feasible procedure, allowing us to treat very dilated right outflow tracts, not suitable for the current balloon-expandable valves.

Background: Transthoracic echocardiography represents the main noninvasive technique for evaluating cardiac morphology and function in dogs. In dogs with particular somatotypes, such as brachymorphic dogs, breed-specific echocardiographic values are needed for a proper echocardiographic interpretation. Nowadays, the Pug represents one of the most popular brachymorphic canine breeds worldwide. However, data on echocardiographic measurements in this breed are currently limited. Aim: We aimed to determine echocardiographic values in a population of apparently healthy Pugs, and to assess the possible effects of body weight, age, and sex on selected echocardiographic variables, with particular emphasis on those related to the left-sided cardiac chambers. Methods: Apparently healthy Pugs underwent a full physical examination, a one-minute six-lead electrocardiogram, and a complete transthoracic echocardiography. Twenty-four echocardiographic variables were measured combining M-mode, two-dimensional and Doppler modalities, and relative values were determined by applying the statistical procedures recommended by the Clinical and Laboratory Standards Institute. Moreover, the effect of selected demographic variables on echocardiographic measurements were tested using a linear mixed model. Results: The investigation included 86 Pugs. Echocardiographic values were provided for each variable and compared with previous veterinary literature. A statistically significant effect of body weight (e.g., end-systolic left ventricular diameter and absolute left atrial diameter), age, and sex was documented for several of the tested variables. Doppler examination demonstrated a trivial pulmonary regurgitation in 24/86 (27.9%) Pugs. Moreover, a persistent left cranial vena cava was suspected in 4/86 (4.7%) dogs. Conclusion: Echocardiographic features of the Pug were addressed and echocardiographic values made available for clinical use. Because our findings were obtained using a standardized echocardiographic analysis in a population of 86 healthy Pugs, they may act as a reliable guide for an accurate echocardiographic interpretation in this breed. [Open Vet J 2023; 13(8.000): 965-976]

Background: Transthoracic echocardiography represents the main noninvasive technique for evaluating cardiac morphology and function in dogs. In dogs with particular somatotypes, such as brachymorphic dogs, breed-specific echocardiographic values are needed for a proper echocardiographic interpretation. Nowadays, the Pug represents one of the most popular brachymorphic canine breeds worldwide. However, data on echocardiographic measurements in this breed are currently limited. Aim: We aimed to determine echocardiographic values in a population of apparently healthy Pugs, and to assess the possible effects of body weight (BW), age, and sex on selected echocardiographic variables, with particular emphasis on those related to the left-sided cardiac chambers. Methods: Apparently healthy Pugs underwent a full physical examination, a 1-minute six-lead electrocardiogram, and a complete transthoracic echocardiography. Twenty-four echocardiographic variables were measured by combining M-mode, two-dimensional and Doppler modalities, and relative values were determined by applying the statistical procedures recommended by the Clinical and Laboratory Standards Institute. Moreover, the effect of selected demographic variables on echocardiographic measurements was tested using a linear mixed model. Results: The investigation included 86 Pugs. Echocardiographic values were provided for each variable and compared with previous veterinary literature. A statistically significant effect of BW, age, and sex was documented for several of the tested variables. Doppler examination demonstrated a trivial pulmonary regurgitation in 24/86 (27.9%) Pugs. Moreover, a persistent left cranial vena cava was suspected in 4/86 (4.7%) dogs. Conclusion: Echocardiographic features of the Pug were addressed and echocardiographic values were made available for clinical use. Because our findings were obtained using a standardized echocardiographic analysis in a population of 86 healthy Pugs, they may act as a reliable guide for an accurate echocardiographic interpretation in this breed. [ABSTRACT FROM AUTHOR]

Background: Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. Results: When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. Conclusions: This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot. [ABSTRACT FROM AUTHOR]

Background: Severe pulmonary regurgitation (PR) often occurs following a transannular patch repair of tetralogy of Fallot, resulting in an enlarged native right ventricular outflow tract (nRVOT) with varying shapes. Methods: We conducted a single-center study with eight patients having severe PR and enlarged nRVOT (diameters ≥ 29 mm). Transcatheter pulmonary valve replacement (TPVR) was performed using the self-expanding PT-Valve. Preoperative evaluation included echocardiography, computed tomography, and magnetic resonance imaging. A 3D-printed model of the nRVOT was used for preoperative assessment. Follow-up data were collected in 1-year follow-up. Results: PT-Valve was successfully implanted in all patients, resulting in immediate improvement of severe PR. Pulmonary artery diastolic pressure increased significantly (p < 0.001). No deaths or coronary compression occurred during the procedure. Over a 1-year follow-up, no stent displacement or fracture occurred. Only two patients had trace paravalvular leaks. Magnetic resonance imaging revealed a reversal of right ventricular remodeling, with a significant reduction in right ventricular end-diastolic volume index (p < 0.001) and improved right ventricular ejection fraction (p < 0.001). All patients achieved primary endpoints. Conclusion: 3D printing-guided PT-Valve implantation in enlarged nRVOT for severe PR is safe and effective, expanding TPVR indications and offering potential treatment for a broader patient population. [ABSTRACT FROM AUTHOR]

The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration–approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT). One-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients. Eligible patients had severe PR by echocardiography or PR fraction ≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n = 42) or TPV25 (n = 45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately. In the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 group and 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient >40 mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately. The Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability. [Display omitted] [ABSTRACT FROM AUTHOR]

This study retrospectively evaluated long-term post-operative aortic and pulmonary valve functions in outlet ventricular septal defects. We evaluated aortic regurgitation and pulmonary regurgitation using pre- and post-operative echocardiograms. Overall, 158 patients who underwent intracardiac repair due to outlet ventricular septal defects with aortic valve deformity or congestive heart failure were included. The median follow-up period was 7 years (interquartile range: 0–17 years), without deaths or pacemaker implantations. The age, weight, ventricular septal defect size, and mild aortic regurgitation at surgery were factors associated with post-operative residual aortic regurgitation. Mild pulmonary regurgitation was observed in 12%, 30%, and 40% of patients 5, 10, and 15 years after surgery, respectively. There were no significant differences in age and weight at which surgery was performed between patients with mild pulmonary regurgitation and those with less than mild pulmonary regurgitation. However, the number of sutures across the pulmonary valve was associated with post-operative pulmonary regurgitation (P < 0.01). As some patients with mild pre-operative aortic regurgitation may not improve even after surgery, early surgical intervention is necessary when aortic regurgitation appears. Some patients may develop post-operative pulmonary regurgitation in the long term, suggesting the need for careful follow-up. [ABSTRACT FROM AUTHOR]

Background: Accurate detection of significant pulmonary regurgitation (PR) is critical in management of patients after right ventricular (RV) outflow reconstruction in Tetralogy of Fallot (TOF) patients, because of its influence on adverse outcomes. Although pressure half time (PHT) of PR velocity is one of the widely used echocardiographic markers of the severity, shortened PHT is suggested to be seen in conditions with increased RV stiffness with mild PR. However, little has been reported about the exact characteristics of patients showing discrepancy between PHT and PR volume in this population. Methods: Echocardiography and cardiac magnetic resonance imaging (MRI) were performed in 74 TOF patients after right ventricular outflow tract (RVOT) reconstruction [32 ± 10 years old]. PHT was measured from the continuous Doppler PR flow velocity profile and PHT < 100 ms was used as a sign of significant PR. Presence of end-diastolic RVOT forward flow was defined as RV restrictive physiology. By using phase-contrast MRI, forward and regurgitant volumes through the RVOT were measured and regurgitation fraction was calculated. Significant PR was defined as regurgitant fraction ≥ 25%. Results: Significant PR was observed in 54 of 74 patients. While PHT < 100 ms well predicted significant PR with sensitivity of 96%, specificity of 52%, and c-index of 0.72, 10 patients showed shortened PHT despite regurgitant fraction < 25% (discordant group). Tricuspid annular plane systolic excursion and left ventricular (LV) ejection fraction were comparable between discordant group and patients showing PHT < 100 ms and regurgitant fraction ≥ 25% (concordant group). However, discordant group showed significantly smaller mid RV diameter (30.7 ± 4.5 vs. 39.2 ± 7.3 mm, P < 0.001) and higher prevalence of restrictive physiology (100% vs. 42%, P < 0.01) than concordant group. When mid RV diameter ≥ 32 mm and presence of restrictive physiology were added to PHT, the predictive value was significantly improved (sensitivity: 81%, specificity: 90%, and c-index: 0.89, P < 0.001 vs. PHT alone by multivariable logistic regression model). Conclusion: Patients with increased RV stiffness and non-enlarged right ventricle showed short PHT despite mild PR. Although it has been expected, this was the first study to demonstrate the exact characteristics of patients showing discrepancy between PHT and PR volume in TOF patients after RVOT reconstruction. [ABSTRACT FROM AUTHOR]

Performance of cardiovascular magnetic resonance (CMR) in the planning phase of percutaneous pulmonary valve implantation (PPVI) is needed for the accurate delineation of the right ventricular outflow tract (RVOT), coronary anatomy and the quantification of right ventricular (RV) volume overload in patients with significant pulmonary regurgitation (PR). This helps to find the correct timings for the intervention and prevention of PPVI-related complications such as coronary artery compression, device embolization and stent fractures. A defined CMR study protocol should be set for all PPVI candidates to reduce acquisition times and acquire essential sequences that are determinants for PPVI success. For correct RVOT sizing, contrast-free whole-heart sequences, preferably at end-systole, should be adopted in the pediatric population thanks to their high reproducibility and concordance with invasive angiographic data. When CMR is not feasible or contraindicated, cardiac computed tomography (CCT) may be performed for high-resolution cardiac imaging and eventually the acquisition of complementary functional data. The aim of this review is to underline the role of CMR and advanced multimodality imaging in the context of pre-procedural planning of PPVI concerning its current and potential future applications. [ABSTRACT FROM AUTHOR]

Background: The myocardial kinetic energy (KE) and its association with pulmonary regurgitation (PR) have yet to be investigated in repaired tetralogy of Fallot (rTOF) patients. Purpose: To evaluate the adaptation of myocardial KE in rTOF patients by tissue phase mapping (TPM). Study Type: Prospective. Population: A total of 49 rTOF patients (23 ± 5 years old; male = 32), 47 normal controls (22 ± 1 year old; male = 29). Field Strength/Sequence: 3‐T/2D dark‐blood three‐directional velocity‐encoded gradient‐echo sequence. Assessment: Left and right ventricle (LV, RV) myocardial KE in radial (KEr), circumferential (KEø), longitudinal (KEz) directions. The proportions of KE in each direction to the sum of all KE (KErøz): %KEr, %KEø, %KEz. PR fraction. Statistical Test: Student's t test, multivariable regression. Statistical significance: P < 0.05. Results: In rTOF group, LV KEz remained normal in systole (P = 0.565) and diastole (P = 0.210), whereas diastolic LV %KEz (62% ± 14% vs. 72% ± 7%) and systolic LV %KEø (9% ± 6% vs. 20% ± 7%) were significantly decreased. The KEr and %KEr of both ventricles significantly increased in the rTOF group (RV in diastole: 6 ± 3 vs. 3 ± 1 μJ and 54% ± 13% vs. 27% ± 7%). The rTOF group exhibited significantly higher RV/LV ratios of %KEr (systole: 1.3 ± 0.3 vs. 1.0 ± 0.3) and %KEø (systole: 1.6 ± 0.8 vs. 1.0 ± 0.3) and significantly lower ratios of %KEz in systole (0.7 ± 0.2 vs. 1.0 ± 0.1) and diastole (0.5 ± 0.2 vs. 0.9 ± 0.1). In multivariable regression analysis, the RV peak systolic KErøz, RV systolic KEz, and LV diastolic %KEø were independently associated with PR fraction in the rTOF group (adjusted R2 = 0.479). Data Conclusion: In rTOF patients, the adaptation of the KE proportion occurred earlier than that of the KE amplitude, and the biventricular balance of %KE was disrupted. PR may cause differential KE adaptation in RV and LV. TPM‐derived KE may be useful in investigation of myocardial adaptation in rTOF patients. Evidence Level: 2 Technical Efficacy: Stage 3 [ABSTRACT FROM AUTHOR]

A 68-year-old man with a history of valve-sparing aortic root replacement and endoscopic aortic valve replacement was admitted to our hospital with dyspnea. Transthoracic echocardiography revealed severe pulmonary valve regurgitation. The patient had undergone cardiac surgery twice, through median sternotomy and right thoracotomy; therefore, we planned endoscopic pulmonary valve replacement via the left thoracic approach. The patient was placed in a modified right lateral decubitus position and underwent mild hypothermic cardiopulmonary bypass. An on-pump beating-heart technique was used during surgery. The 3D endoscopic system and trocars for surgical instruments were inserted through the left 3rd and 4th intercostal spaces. After incision of the pulmonary artery, the pulmonary cusps were resected. A 27-mm St Jude Medical Epic heart valve was implanted in the intra-annular position. Subsequently, the left atrial appendage was resected. The patient was discharged without complications. To our knowledge, this is the first case of totally endoscopic pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

The pulmonary valve is the most frequently replaced cardiac valve in congenital heart diseases. Whether the valve alone or part of the right ventricular outflow tract have to be repaired or replaced depends on the specific pathological anatomy of the malformation. Once the decision to replace the pulmonary valve has been made, two options are available: the isolated transcatheter pulmonary valve replacement and the surgical implantation of a prosthetic valve either isolated or in combination with a procedure on the right ventricular outflow tract. In this paper, we will focus on the different past and present surgical options and present a new concept called “endogenous tissue restoration,” a promising alternative to the hitherto existing implants. From a general point of view, neither the transcatheter nor the surgical valvular implants are magic bullets in the arsenal for the management of valvular diseases. Smaller valves have to be frequently replaced because of outgrowth of the patients, larger tissue valves may present late structural valve deterioration, while xenograft and homograft conduits may calcify and therefore become narrowed within unpredictable incidence and interval following implantation. Based on long-term research efforts combining the knowledge of supramolecular chemistry, electrospinning, and regenerative medicine, endogenous tissue restoration has emerged most recently as a promising option to create long-term functioning implants. This technology is appealing because following resorption of the polymer scaffold and timely replacement through autologous tissue, no foreign material remain at all in the cardiovascular system. Proof-of-concept studies as well as small first-in-man series have been completed and have demonstrated favorable anatomic and hemodynamic results, comparable to currently available implants in the short term. Based on the initial experience, important modifications to improve the pulmonary valve function have been initiated.

Background: Nikaidoh translocation operation is increasingly being used for patients with TGA, VSD and LVOTO. The early results of this operation are excellent and possibly better than the Rastelli repair. However, the long-term results remain inadequately defined. Methods and results: Detailed follow up data of a patient, 12 years after Nikaidoh operation, using computerized image analysis are reported. The patient complained of chest pain on exertion. Imaging showed severe dilatation of the non-coronary sinus of the aortic root, compressing the RVOT and pulmonary artery branches resulting in RV dilatation. The dilated aortic sinus showed marked pulsatility with expansion during systole and diastole. As the child was symptomatic, with a risk of aneurysm rupture, excision was performed. This complication is thought to be due to interruption of the innervation of the root as well as cutting vasa vasorum. Conclusion: Nikaidoh operation is a viable solution for the repair of TGA, VSD, PS. Dilatation of the neo-aortic root should be carefully followed and causes of the dilatation investigated.

Background: The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). Methods: A total of 38 patients with severe PR (mean age 24.2 ± 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scanning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. Results: All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed underexpanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3-8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8-94.7) and 83.5% (95%CI 73.8-93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. Conclusion: TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture. [ABSTRACT FROM AUTHOR]

Many patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution. In a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003–2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up. Pulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery. [ABSTRACT FROM AUTHOR]

Background: To assess immediate and intermediate outcomes of percutaneous balloon pulmonary valvuloplasty (BPV) in children and adults. Materials and Methods: This retrospective, single-center study included patients who had undergone balloon valvuloplasty for the treatment of moderate to severe pulmonary stenosis at a tertiary care center in India between May 2011 and July 2018. Clinical profile, echocardiographic details, procedural details, complications, short term, and intermediate results were assessed. Results: A total of 43 patients were assessed. The mean age of the study population was 13.87 ± 11.71 years. Of them, 21 (48.8%) patients were men and 30 (69.8%) patients were children/adolescents. Single-balloon technique was used in all the cases. Balloon/annulus ratio was 1.28 ± 0.04. Immediate procedural success (Group 1) and partial procedural success (Group 2) were achieved in 26 (60.5%) and 17 (39.5%) patients, respectively. The right ventricular systolic pressure reduced from 117.70 ± 31.77 mmHg to 53.56 ± 13.29 mmHg postprocedure (P < 0.001). Peak-to-peak transvalvular gradient reduced from 102.81 ± 31.66 mmHg to 35.56 ± 12.47 mmHg postprocedure (P < 0.001). Intermediate follow-up was conducted for 2.61 ± 0.75 years (range: 2–4 years). At intermediate follow-up, peak-to-peak instantaneous gradient was 27.21 ± 5.80 mmHg. Restenosis, moderate, and severe pulmonary regurgitation were reported in 2 (7.1%), 4 (14.3%), and 2 (7.1%) patients, respectively. Conclusion: Percutaneous BPV is a safe and efficacious procedure for the treatment of moderate to severe pulmonary valve stenosis in children and adults. The procedure had excellent immediate and intermediate follow-up results.

Right ventricular dysfunction is a major determinant of outcome in patients with complex congenital heart disease, as in tetralogy of Fallot. In these patients, right ventricular dysfunction emerges after initial pressure overload and hypoxemia, which is followed by chronic volume overload due to pulmonary regurgitation after corrective surgery. Myocardial adaptation and the transition to right ventricular failure remain poorly understood. Combining insights from clinical and experimental physiology and myocardial (tissue) data has identified a disease phenotype with important distinctions from other types of heart failure. This phenotype of the right ventricle in tetralogy of Fallot can be described as a syndrome of dysfunctional characteristics affecting both contraction and filling. These characteristics are the end result of several adaptation pathways of the cardiomyocytes, myocardial vasculature and extracellular matrix. As long as the long-term outcome of surgical correction of tetralogy of Fallot remains suboptimal, other treatment strategies need to be explored. Novel insights in failure of adaptation and the role of cardiomyocyte proliferation might provide targets for treatment of the (dysfunctional) right ventricle under stress.

Double-chamber right ventricle repair surgery requires the excision of anomalous obstructive muscular or fibromuscular bundles in the right ventricular outflow tract. Because of the close proximity of key structures in the right ventricular outflow tract, the surgery is extremely challenging and requires precise resection. Underresection of the muscle bands can lead to significant residual gradients in the postoperative period, whereas overenthusiastic resection can cause iatrogenic injury to surrounding structures. Various techniques like Hegar sizing by the surgeons, direct chamber pressure measurement, transesophageal echocardiography, and epicardial echocardiography can guide the surgeons about the adequacy of repair. Transesophageal echocardiography is crucial at each step, as it can precisely determine the exact site of obstruction in the preoperative period. Postoperatively, it helps determine the adequacy of surgical repair and identification of inadvertent iatrogenic complications. [ABSTRACT FROM AUTHOR]

The pulmonary circulation after one and one-half ventricle repair is complex because of the direct connection of two sources of blood flow. Associated pulmonary regurgitation further complicates the pulmonary circulation. We report the complex hemodynamics depicted by phase-contrast magnetic resonance imaging in a patient with one and one-half ventricle circuit and severe pulmonary regurgitation. Antegrade superior vena caval flow occurred almost exclusively during diastole and regurgitated into the right ventricle. Consequently, the entire systemic venous return reached the right ventricle during diastole and is pumped back into the pulmonary arteries in systole. The case highlights the importance of analyzing flow patterns phase-by phase throughout the cardiac cycle instead of relying on the net flow volumes through each source of pulmonary blood flow. [ABSTRACT FROM AUTHOR]

This study aimed to investigate the effect of pulmonary regurgitation (PR) on left ventricular ventriculo–arterial (VA) coupling in patients with repaired tetralogy of Fallot (ToF). It was hypothesised that increasing PR severity results in a smaller forward compression wave (FCW) peak in the aortic wave intensity, because of right-to-left ventricular interactions. The use of cardiovascular magnetic resonance (CMR)-derived wave-intensity analysis provided a non-invasive comparison between patients with varying PR degrees. A total of n = 201 patients were studied and both hemodynamic and wave-intensity data were compared. Wave-intensity peaks and areas of the forward compression and forward expansion waves were calculated as surrogates of ventricular function. Any extent of PR resulted in a significant reduction in the FCW peak. A correlation was found between aortic distensibility and the FCW peak, suggesting unfavourable (VA) coupling in patients that also present stiffer ascending aortas. Data suggest that VA coupling is affected by increased impedance. [ABSTRACT FROM AUTHOR]

The pulmonary valve (PV) is the least imaged among the heart valves. However, pulmonary regurgitation (PR) and pulmonary stenosis (PS) can occur in a variety of patients ranging from fetuses, newborns (e.g., tetralogy of Fallot) to adults (e.g., endocarditis, carcinoid syndrome, complications of operated tetralogy of Fallot). Due to their complexity, PR and PS are studied using multimodality imaging to assess their mechanism, severity, and hemodynamic consequences. Multimodality imaging is crucial to plan the correct management and to follow up patients with pulmonary valvulopathy. Echocardiography remains the first line methodology to assess patients with PR and PS, but the information obtained with this technique are often integrated with cardiac magnetic resonance (CMR) and computed tomography (CT). This state-of-the-art review aims to provide an updated overview of the usefulness, strengths, and limits of multimodality imaging in patients with PR and PS. [ABSTRACT FROM AUTHOR]

Carcinoid heart disease is an important complication of neuroendocrine tumors that may lead to significant morbidity and mortality. Right-sided heart valve involvement with consequent valve dysfunction is one of the common manifestations. Patients often have multiple, significant comorbidities with advanced metastatic disease and, as such, may not be suitable for surgical valve replacement due to excessive risk. Transcatheter valve replacement using balloon-expandable valves has been the mainstay of management for these patients, particularly with stenotic lesions but there is limited experience in patients with pulmonary regurgitation and dilated pulmonary arteries outside the dimensions that would be suitable for these valves. We report three successful cases of percutaneous pulmonary valve implantation with the VenusP-valve, a large self-expanding valve platform, and highlight the technical aspects and challenges specific to treatment of pulmonary regurgitation in patients with carcinoid heart disease. Percutaneous pulmonary valve implantation, using the VenusP-valve, may be considered an effective treatment in this patient group., (© 2024 Wiley Periodicals LLC.)

Competing Interests: Disclosures Dr O’Leary previously received research grant support from Abbott Cardiovascular. Dr Maschietto is a consultant and proctor for Medtronic Inc and Edwards Lifesciences.