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Background: Isolated pulmonary valve endocarditis (IPE) accounts for less than 2% of all infective endocarditis patients. It is commonly associated with several predisposing factors, including intravenous drug use (IVDU) and congenital heart disease. The most common causative pathogens of IPE are Staphylococcus aureus and Streptococcus viridans. We report a Down's syndrome patient with IPE and with no standard risk factors caused by the rare pathogen Acinetobacter spp. This led to respiratory failure and systemic infection due to septic pulmonary emboli. Early elective surgery was decided upon as the patient was no longer responding to medical therapy, and his clinical condition was worsening over time. Case presentation: A 15-year-old male with Down syndrome and no underlying heart defect presented with a 3-month history of episodic fever, nausea, vomiting, and diarrhea. Transthoracic echocardiography (TTE) revealed large vegetation on the pulmonary valve leaflet, another mobile mass at the pulmonary artery bifurcation, and severe pulmonary regurgitation. Serial blood cultures isolated Acinetobacter spp. Despite initial antibiotic therapy, the patient continued to have sepsis, unresolved vegetations, and developed life-threatening complications and respiratory distress, which convinced us to perform a pulmonary valve replacement surgery with a homograft. After surgery, the patient recovered and was discharged on the ninth postoperative day (POD). Conclusion: This report highlights IPE's diagnostic and therapeutic challenges, alongside the importance of a comprehensive cardiopulmonary workup in patients with unexplained fever, sepsis, and pulmonary symptoms, even without typical risk factors. Based on the patient's aggravating condition despite medical treatment, early surgical intervention and pulmonary valve replacement were deemed crucial. However, there still needs to be a definitive guideline on when and how surgery should be performed in patients with complicated IPE, especially in pediatric patients. [ABSTRACT FROM AUTHOR]

Objectives Patients with congenital heart disease frequently require surgical or percutaneous interventional valve replacement after initial congenital heart defect (CHD) repair. In some of these patients, simultaneous replacement of both semilunar valves is necessary, resulting in increased procedural complexity, morbidity, and mortality. In this study, we analyze the outcomes of simultaneous aortic and pulmonary valve replacements following multiple surgical interventions for CHD. Methods This was a retrospective study of 24 patients who after initial repair of CHD underwent single-stage aortic and pulmonary valve replacement at our institution between 2003 and 2021. Results The mean age of the patients was 28 ± 13 years; the mean time since the last surgery was 15 ± 11 years. Decellularized valved homografts (DVHs) were used in nine patients, and mechanical valves were implanted in seven others. In eight patients, DVHs, biological, and mechanical valves were implanted in various combinations. The mean cardiopulmonary bypass time was 303 ± 104 minutes, and aortic cross-clamp time was 152 ± 73 minutes. Two patients died at 12 and 16 days postoperatively. At a maximum follow-up time of 17 years (mean 7 ± 5 years), 95% of the surviving patients were categorized as New York Heart Association heart failure class I. Conclusion Single-stage aortic and pulmonary valve replacement after initial repair of CHD remains challenging with substantial perioperative mortality (8.3%). Nevertheless, long-term survival and clinical status at the latest follow-up were excellent. The valve type had no relevant impact on the postoperative course. The selection of the valves for implantation should take into account operation-specific factors—in particular reoperability—as well as the patients' wishes. [ABSTRACT FROM AUTHOR]

Pulmonary valve replacement (PVR) consists of substituting a patient's original valve with a prosthetic one, primarily addressing pulmonary valve insufficiency, which is crucially relevant in Tetralogy of Fallot repairment. While extensive clinical and computational literature on aortic and mitral valve replacements is available, PVR's post‐procedural haemodynamics in the pulmonary artery and the impact of prosthetic valve dynamics remain significantly understudied. Addressing this gap, we introduce a reduced Fluid–Structure Interaction (rFSI) model, applied for the first time to the pulmonary valve. This model couples a three‐dimensional computational representation of pulmonary artery haemodynamics with a one‐degree‐of‐freedom model to account for valve structural mechanics. Through this approach, we analyse patient‐specific haemodynamics pre and post PVR. Patient‐specific geometries, reconstructed from CT scans, are virtually equipped with a template valve geometry. Boundary conditions for the model are established using a lumped‐parameter model, fine‐tuned based on clinical patient data. Our model accurately reproduces patient‐specific haemodynamic changes across different scenarios: pre‐PVR, six months post‐PVR, and a follow‐up condition after a decade. It effectively demonstrates the impact of valve implantation on sustaining the diastolic pressure gradient across the valve. The numerical results indicate that our valve model is able to reproduce overall physiological and/or pathological conditions, as preliminary assessed on two different patients. This promising approach provides insights into post‐PVR haemodynamics and prosthetic valve effects, shedding light on potential implications for patient‐specific outcomes. [ABSTRACT FROM AUTHOR]

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 Background Isolated pulmonary valve endocarditis (IPE) accounts for less than 2% of all infective endocarditis patients. It is commonly associated with several predisposing factors, including intravenous drug use (IVDU) and congenital heart disease. The most common causative pathogens of IPE are Staphylococcus aureus and Streptococcus viridans. We report a Down’s syndrome patient with IPE and with no standard risk factors caused by the rare pathogen Acinetobacter spp. This led to respiratory failure and systemic infection due to septic pulmonary emboli. Early elective surgery was decided upon as the patient was no longer responding to medical therapy, and his clinical condition was worsening over time. Case presentation A 15-year-old male with Down syndrome and no underlying heart defect presented with a 3-month history of episodic fever, nausea, vomiting, and diarrhea. Transthoracic echocardiography (TTE) revealed large vegetation on the pulmonary valve leaflet, another mobile mass at the pulmonary artery bifurcation, and severe pulmonary regurgitation. Serial blood cultures isolated Acinetobacter spp. Despite initial antibiotic therapy, the patient continued to have sepsis, unresolved vegetations, and developed life-threatening complications and respiratory distress, which convinced us to perform a pulmonary valve replacement surgery with a homograft. After surgery, the patient recovered and was discharged on the ninth postoperative day (POD). Conclusion This report highlights IPE’s diagnostic and therapeutic challenges, alongside the importance of a comprehensive cardiopulmonary workup in patients with unexplained fever, sepsis, and pulmonary symptoms, even without typical risk factors. Based on the patient’s aggravating condition despite medical treatment, early surgical intervention and pulmonary valve replacement were deemed crucial. However, there still needs to be a definitive guideline on when and how surgery should be performed in patients with complicated IPE, especially in pediatric patients.

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.

Abstract Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

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Abnormalities of the right ventricular outflow tract are common within the spectrum of congenital heart disease. Reconstruction of this outflow with or without the use of prosthetic material forms an integral part of many surgical procedures. Consequently, this part of the heart constitutes an important locus for infective endocarditis. Focused literature on infective endocarditis of the right ventricular outflow is sparse. This narrative review therefore attempts to collate the currently available data on a subject that is gaining importance because of the increasing numbers of surgical and catheter-based interventions on the right ventricular outflow. [ABSTRACT FROM AUTHOR]

Surgical treatment is recommended in patients with symptomatic severe tricuspid regurgitation and pulmonary regurgitation. Although renal transplant patients are a high-risk patient group for cardiac surgery, heart valve surgeries can be performed successfully. There are a limited number of studies published on this subject in the literature. Therefore, we present a case who underwent tricuspid ring annuloplasty (TRA) before being followed up with renal transplantation and then successfully performed redo tricuspid valve replacement (TVR) and pulmonary valve replacement (PVR). [ABSTRACT FROM AUTHOR]

Background There are limited data about the impact of timing of pulmonary valve replacement (PVR) on right heart reverse remodeling in patients with pulmonary regurgitation following intervention for isolated pulmonary valve stenosis (PS). This study compared differences in postprocedural right heart reverse remodeling after early versus late PVR (defined as PVR before versus after attainment of the conservative consensus criteria proposed by Bokma et al, 2018) in patients with prior intervention for PS, using patients with tetralogy of Fallot as the reference group. Method and Results Right atrial reservoir strain and right ventricular free wall strain was measured at baseline, 1 and 3 years after PVR. There were 114 patients with PS (early PVR, 87 [76%]; late PVR, 27 [24%]) and 291 patients with tetralogy of Fallot (early PVR, 197 [67%]; late PVR, 96 [33%]). The PS group had greater improvement in right atrial reservoir strain at 1 year (12%±4% versus 8%±4%; P

This case series presents two young patients with severe pulmonary valve regurgitation and pectus excavatum. Both patients underwent surgical repair of tetralogy of Fallot and pulmonary valvulotomy, respectively, during infancy and remained under close cardiological monitoring thereafter. After the diagnosis of severe pulmonary regurgitation was confirmed, both were referred to our center for pulmonary valve replacement. Minimally invasive pulmonary valve replacement was performed through a left anterior minithoracotomy. The swift recovery and return to daily activities observed in the presented cases suggest that minimally invasive pulmonary valve replacement through a left anterior minithoracotomy could indeed be considered the procedure of choice for patients with pectus excavatum. [ABSTRACT FROM AUTHOR]

Abstract Background Recently, improvements in the repair of tetralogy of Fallot have increased the need for reoperation in adulthood, and it’s not rare that these reoperation candidates suffer from biventricular failure. However, there are no firm treatment guidelines, and each country, and even each facility, treats each case individually. Case presentation We report the successful staged treatment of pulmonary regurgitation and pacemaker-induced cardiomyopathy with biventricular failure in adulthood in a case of complete atrioventricular block after tetralogy of Fallot repair in childhood. We planned a staged therapeutic strategy with preoperative left ventricular volume reduction with medication, following surgical pulmonary valve replacement concomitant epicardial lead implantation on the lateral basal wall, placed just beneath the generator pocket through 3rd intercostal space. in addition to postoperative intervention with a defibrillator to adjust cardiac resynchronization therapy, resulted in improvement of symptoms. Conclusion In a patient with biventricular failure after TOF repair, a staged treatment strategy involving medication, PVR, and CRT with a combination of epicardial and intravenous leads could be a useful treatment worth trying before heart transplantation.

Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (−) of PVR and presence (+) versus absence (−) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. Results: In total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication–). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR–/indication–). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P

Abstract Background Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients. Methods 130 rTOF patients who underwent CMR (2006–2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome. Results Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p

Background: Recently, improvements in the repair of tetralogy of Fallot have increased the need for reoperation in adulthood, and it's not rare that these reoperation candidates suffer from biventricular failure. However, there are no firm treatment guidelines, and each country, and even each facility, treats each case individually. Case presentation: We report the successful staged treatment of pulmonary regurgitation and pacemaker-induced cardiomyopathy with biventricular failure in adulthood in a case of complete atrioventricular block after tetralogy of Fallot repair in childhood. We planned a staged therapeutic strategy with preoperative left ventricular volume reduction with medication, following surgical pulmonary valve replacement concomitant epicardial lead implantation on the lateral basal wall, placed just beneath the generator pocket through 3rd intercostal space. in addition to postoperative intervention with a defibrillator to adjust cardiac resynchronization therapy, resulted in improvement of symptoms. Conclusion: In a patient with biventricular failure after TOF repair, a staged treatment strategy involving medication, PVR, and CRT with a combination of epicardial and intravenous leads could be a useful treatment worth trying before heart transplantation. [ABSTRACT FROM AUTHOR]

There are limited data about postprocedural right heart reverse remodeling and long-term prosthesis durability after transcatheter pulmonary valve replacement (TPVR) and how these compare to surgical pulmonary valve replacement (SPVR). This study sought to compare right heart reverse remodeling, pulmonary valve gradients, and prosthetic valve dysfunction after TPVR vs SPVR. Patients with TPVR were matched 1:2 to patients with SPVR based on age, sex, body surface area, congenital heart lesion, and procedure year. Right heart indexes (right atrial [RA] reservoir strain, RA volume index, RA pressure, right ventricular [RV] global longitudinal strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline (preintervention), 1 year postintervention, and 3 years postintervention. Pulmonary valve gradients were assessed at 1, 3, 5, 7, and 9 years postintervention. There were 64 and 128 patients in the TPVR and SPVR groups, respectively. Among patients with TPVR, 46 (72%) and 18 (28%) received Melody (Medtronic) vs SAPIEN (Edwards Lifesciences) valves, respectively. The TPVR group had greater postprocedural improvement in RA reservoir strain and RV global longitudinal strain at 1 and 3 years. The TPVR group had a higher risk of prosthetic valve dysfunction mostly because of a higher incidence of prosthetic valve endocarditis compared to SPVR but a similar risk of pulmonary valve reintervention because some of the patients with endocarditis received medical therapy only. Both groups had similar pulmonary valve mean gradients at 9 years postintervention. These data suggest a more favorable right heart outcome after TPVR. However, the risk of prosthetic valve endocarditis and prosthetic valve dysfunction remains a major concern. [Display omitted] [ABSTRACT FROM AUTHOR]

Robust data on changes in pulmonary valve replacement (PVR) procedural volume and predictors of bioprosthetic pulmonary valve (BPV) durability in patients with tetralogy of Fallot (TOF) are scarce. This study sought to assess temporal trends in PVR procedural volume and BPV durability in a nationwide, retrospective TOF cohort. Data were obtained from patient records. Robust linear regression was used to assess temporal trends in PVR procedural volume. Piecewise exponential additive mixed models were used to estimate BPV durability, defined as the time from implantation to redo PVR with death as a competing risk, and to assess risk factors for reduced durability. In total, 546 PVR were performed in 384 patients from 1976 to 2021. The annual number of PVR increased from 0.4 to 6.0 per million population (P < 0.001). In the last decade, the transcatheter PVR volume increased by 20% annually (P < 0.001), whereas the surgical PVR volume did not change significantly. The median BPV durability was 17 years (Q1: 10-Q3: 10 years-not applicable). There was no significant difference in the durability of different BPV after adjustment for confounders. Age at PVR (HR: 0.78 per 10 years from <1 year; 95% CI: 0.63-0.96; P = 0.02) and true inner valve diameter (9-17 mm vs 18-22 mm HR: 0.40; 95% CI: 0.22-0.73; P = 0.003 and 18-22 mm vs 23-30 mm HR: 0.59; 95% CI: 0.25-1.39; P = 0.23) were associated with reduced BPV durability in multivariate models. The PVR procedural volume has increased over time, with a greater increment in transcatheter than surgical PVR during the last decade. Younger patient age at PVR and a smaller true inner valve diameter predicted reduced BPV durability. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients. Methods: 130 rTOF patients who underwent CMR (2006–2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome. Results: Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p <.001; p =.037), HF (p =.049; p =.005), composite outcome (p =.039; p =.009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p <.001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy. Conclusions: Besides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role. [ABSTRACT FROM AUTHOR]

The aim of this study is to analyze the relationship between QRS duration after pulmonary valve replacement (PVR) and ventricular arrhythmias (VA) in patients with repaired tetralogy of Fallot (ToF). ToF patients may face complications such as heart failure and VA after primary repair, often mitigated by PVR. Prior studies have shown a decrease in QRS duration and right ventricular (RV) size following PVR. It remains unclear whether a lack of QRS duration reduction identifies patients at risk of VA. We retrospectively identified adult patients with repaired ToF who underwent surgical or transcatheter PVR. EKG data (pre-PVR, 30 days to 1-year post-PVR, and closest to CMR) was collected. The primary endpoint was sustained ventricular tachycardia (VT), ICD shock for sustained VT, or inducible VT on EP study. 85 patients were included (median follow-up 3.6 years; median age 34 years; 51% females). The primary outcome was noted in 8 patients. Mean QRS duration decreased by 5 ms following PVR (p = 0.0001). Increased age at PVR, QRS ≥ 180 ms post-PVR, no reduction in QRS after PVR, and a history of VT were associated with higher risk of the primary endpoint. The change in QRS was linearly correlated with the change in RVEDVi (R = 0.66). Adults with repaired ToF experience a reduction in QRS duration post-PVR that correlates with the change of the RV size. Patients with QRS ≥ 180 ms post-PVR, no reduction in QRS, increased age at repair, and a history of VT are at risk for recurrent VT and warrant closer monitoring/ICD consideration. [ABSTRACT FROM AUTHOR]

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. This review includes (1) Fetal diagnosis, associated anomalies, and factors affecting outcomes in fetal congenitally corrected transposition of the great arteries (ccTGA), (2) The impact of Fontan-associated liver disease on heart and heart/liver transplant, (3) 1-year outcomes of the Harmony transcatheter pulmonary valve, (4) Risk factors associated with major adverse cardiac events in patients with pulmonary atresia and intact ventricular septum undergoing intervention, and (5) Benefits of pulmonary valve replacement in tetralogy of Fallot patients. [ABSTRACT FROM AUTHOR]

Patients with repaired tetralogy of Fallot (TOF) are at risk for ventricular tachycardia (VT) related to well-described anatomical isthmuses. The purpose of this study was to explore QRS morphology as an indicator of anatomical isthmus conduction. Patients with repaired TOF and complete right bundle branch block referred for transcatheter pulmonary valve replacement (PVR) or presenting with sustained VT underwent comprehensive 3-dimensional mapping in sinus rhythm. Electrocardiographic characteristics were compared to right ventricular (RV) activation and anatomical isthmus conduction properties. Twenty-two patients (19 pre–pulmonary valve replacement and 3 clinical VT) underwent comprehensive 3-dimensional mapping (median 39 years; interquartile range [IQR] 27–48 years; 12 [55%] male). Septal RV activation (median 40 ms; IQR 34–46 ms) corresponded to the nadir in lead V 1 and free wall activation (median 71 ms; IQR 64–81 ms) to the transition point in the upstroke of the Rʹ wave. Patients with isthmus block between the pulmonary annulus and the ventricular septal defect patch and between the ventricular septal defect patch and the tricuspid annulus (when present), were more likely to demonstrate lower amplitude Rʹ waves in lead V 1 (5.8 mV vs 9.4 mV; P =.005), QRS fragmentation in lead V 1 (15 [94%] vs 2 [13%]; P <.001), and terminal S waves in lead aVF (15 [94%] vs 6 [40%]; P <.001) than those with intact conduction. During catheter ablation, these QRS changes developed during isthmus block. For patients with repaired TOF, the status of septal isthmus conduction was evident from sinus rhythm QRS morphology. Low-amplitude, fragmented Rʹ waves in lead V 1 and terminal S waves in the inferior leads were related to septal isthmus conduction abnormalities, providing a mechanistic link between RV activation and common electrocardiographic findings. [ABSTRACT FROM AUTHOR]

Background: Right atrial (RA) dysfunction and atrial arrhythmias are relatively common in adults with repaired tetralogy of Fallot. The purpose of this study was to determine whether RA function improved after surgical pulmonary valve replacement (PVR), and the association between postoperative RA reverse remodeling and late postoperative atrial arrhythmias. Method: RA reverse remodeling (ΔRA reservoir strain based speckle tracking echocardiography) was calculated as: ([postoperative RA reservoir strain – preoperative RA reservoir strain]/preoperative RA reservoir strain)x100. Optimal RA reverse remodeling was defined as ΔRA reservoir strain >15%. Results: Of 411 patients (age 36 ± 13 years), preoperative RA reservoir strain was 31 ± 13%, postoperative RA reserve remodeling was 13 ± 9%, and 171 (42%) had optimal RA reserve remodeling. Preoperative RA reservoir strain (β±SE 1.12 ± 0.09, p

Abstract Background Patients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF. Methods Thirty two rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3–6 months post-surgery. Results RV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI) ≥ 160 ml/m2 or right ventricular end-systolic volume index (RVESVI) ≥ 80 ml/m2 in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI = 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI 2.35 were diagnosed with RV moderate-severe dilatation (AUC = 0,882; Sensitivity = 94.1%; Specificity = 77.3%). Conclusions RV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score > 2.35, the patient is indicated for further CMR examination and treatment.

Tetralogy of Fallot was repaired previously in a preschooler through right ventriculotomy, providing excellent exposure for ventricular septal defect closure and right ventricular outflow tract obstruction relief. Herein, we describe the preschooler as a 50-year-old man with untreated atrial septal defect found at pulmonary valve replacement in the remote period after tetralogy of Fallot repair. It was inferred intraoperatively that the previous tetralogy of Fallot was repaired only through right ventriculotomy, and atrial septal defect closure was performed together with pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

[Display omitted] [ABSTRACT FROM AUTHOR]

Objectives: Atrial arrhythmias are a significant cause of late morbidity and mortality in patients after tetralogy of Fallot repair. However, reports on their recurrence following atrial arrhythmia surgery are limited. We aimed to identify the risk factors for atrial arrhythmia recurrence after pulmonary valve replacement (PVR) and arrhythmia surgery. Methods: We reviewed 74 patients with repaired tetralogy of Fallot who underwent PVR for pulmonary insufficiency at our hospital between 2003 and 2021. Twenty-two patients (mean age, 39 years) underwent PVR and atrial arrhythmia surgery. A modified Cox-maze III was performed in 6 patients with chronic atrial fibrillation, and a right-sided maze was performed in 12 with paroxysmal atrial fibrillation, 3 with atrial flutter, and 1 with atrial tachycardia. Atrial arrhythmia recurrence was defined as any documented sustained atrial tachyarrhythmia requiring intervention. The influence of preoperative parameters on recurrence was assessed with the Cox proportional-hazards model. Results: The median follow-up period was 9.2 years (interquartile range, 4.5-12.4). Cardiac death and redo-PVR due to prosthetic valve dysfunction were not observed. Eleven patients had atrial arrhythmia recurrence after discharge. Atrial arrhythmia recurrence-free rates were 68% at 5 years and 51% at 10 years after PVR and arrhythmia surgery. Multivariable analysis revealed that right atrial volume index (hazard ratio, 1.04; 95% confidence interval, 1.01-1.08, P = .009) was a significant risk factor for atrial arrhythmia recurrence after arrhythmia surgery and PVR. Conclusions: Preoperative right atrial volume index was associated with atrial arrhythmia recurrence, which may assist in planning the timing of atrial arrhythmia surgery and PVR.

Background: Patients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF. Methods: Thirty two rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3–6 months post-surgery. Results: RV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI) ≥ 160 ml/m2 or right ventricular end-systolic volume index (RVESVI) ≥ 80 ml/m2 in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI = 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI < 160 ml/m2 and RVESVI < 80 ml/m2, and the two parameters were higher than normal values. Compared with the RV mild dilatation group, patients of RV moderate-severe dilatation have worse cardiac function before surgery (right ventricular ejection fraction, 38.92(9.19) % versus 48.31(5.53) %, p < 0.001; Left ventricular ejection fraction, 59.80(10.26) versus 66.41(4.15), p = 0.021). Patients with RV moderate-severe dilatation faced longer operation time and more blood transfusion during operation (operation time, 271.53(08.33) min versus 170.53(72.36) min, p < 0.01; Intraoperative blood transfusion, 200(175) ml versus 100(50) ml, p = 0.001). Postoperative RV moderate-severe dilatation patients have poor short-term prognosis, which was reflected in a longer postoperative hospital stay (6.59 [2.12] days versus 9.80 [5.10] days, p = 0.024) and a higher incidence of hypohepatia (0[0] % versus 4[26.7] %, p = 0.023). Patients with RV dilatation score > 2.35 were diagnosed with RV moderate-severe dilatation (AUC = 0,882; Sensitivity = 94.1%; Specificity = 77.3%). Conclusions: RV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score > 2.35, the patient is indicated for further CMR examination and treatment. [ABSTRACT FROM AUTHOR]

Background: Pulmonary valve replacement is recommended in patients with repaired tetralogy of Fallot based on cardiac magnetic resonance imaging (MRI) criteria. This procedure is performed by surgical or transcatheter approaches. Objective: We aimed to investigate the differences in preprocedural MRI characteristics (volume, function, strain) and morphology of the right ventricular outflow tract and branch pulmonary arteries in patients for whom surgical or transcatheter pulmonary valve replacement was planned. Materials and methods: Cardiac MRI of 166 patients with tetralogy of Fallot were analyzed. Of these, 36 patients for whom pulmonary valve replacement was planned were included. Magnetic resonance imaging characteristics, right ventricular outflow tract morphology, branch pulmonary artery flow distribution and diameter were compared between surgical and transcatheter groups. Spearman correlation and Kruskal–Wallis tests were performed. Results: Circumferential and radial MRI strain for the right ventricle were lower in the surgical group (P=0.045 and P=0.046, respectively). The diameter of the left pulmonary artery was significantly lower (P=0.021) and branch pulmonary artery flow and diameter ratio were higher (P=0.044 and P = 0.002, respectively) in the transcatheter group. There was a significant correlation between right ventricular outflow tract morphology and right ventricular end-diastolic volume index and global circumferential and radial MRI strain (P=0.046, P=0.046 and P= 0.049, respectively). Conclusion: Preprocedural MRI strain, right-to-left pulmonary artery flow, diameter ratio and morphological features of the right ventricular outflow tract were significantly different between the two groups. A transcatheter approach may be recommended for patients with branch pulmonary artery stenosis, since both pulmonary valve replacement and branch pulmonary artery stenting can be performed in the same session. [ABSTRACT FROM AUTHOR]

Objectives: In pulmonary valve replacement (PVR) after tetralogy of Fallot (TOF) repair, the right ventricular end-diastolic and end-systolic volume index (RVEDVI and RVESVI) of cardiac magnetic resonance imaging (cMRI) are often used as indicators of the RV volume. We examined the utility of QRS duration, cardiothoracic ratio (CTR), and plasma brain natriuretic peptide (BNP) as indicators of the appropriate timing of cMRI to assess the RV volume and function before PVR. Methods: We assessed the correlation of QRS duration, CTR, and BNP with RVEDVI and RVESVI on cMRI in 26 patients after TOF repair. Fifteen underwent PVR (age, 45.2 ± 11.4 years). Twelve underwent post-PVR cMRI. The RV volume change from before to after PVR was investigated. Results: QRS duration, BNP, and CTR were positively correlated with RVEDVI and RVESVI after TOF repair. The post-PVR QRS duration was also positively correlated with post-PVR RVEDVI (p = 0.017) and RVESVI (p = 0.001). From before to after PVR, in 5 cases with QRS duration ≤ 160 ms, the QRS duration decreased from 110.4 ± 28.9 to 101.8 ± 30.5 ms (p = 0.063). Both RVEDVI and RVESVI decreased to the normal range in 4 of 5 cases. In contrast, in 7 cases with QRS duration > 160 ms, the QRS duration decreased from 183.0 ± 17.4 to 160.3 ± 23.8 ms (p = 0.013); however, RVESVI did not normalize in 6 of 7 cases. Conclusions: A prolonged QRS duration is a useful marker of RVEDVI and RVESVI enlargement after TOF repair. We recommend performing cMRI before the QRS duration reaches 160 ms due to normalization of the RV volume after PVR. [ABSTRACT FROM AUTHOR]

Background To evaluate long‐term outcome of tetralogy of Fallot repair analyzing an unbiased country‐wide surgically treated population with tetralogy of Fallot. Methods and Results Retrospective analysis of consecutive patients aged 90% at 30 years. Each reintervention significantly incrementally increased the risk of mortality. Type of initial repair predicted the need for specific surgical or catheter reinterventions.

BackgroundTetralogy of Fallot is a common congenital heart disease characterized by cyanosis. The primary treatment approach involves corrective surgery typically performed within the first year of life to achieve complete resolution. However, certain patients may undergo surgery at an older age. This study seeks to assess the efficacy of surgery by examining the midterm outcomes of total correction of Tetralogy of Fallot when performed in older individuals.MethodsThis interventional-longitudinal study focused on patients who underwent complete surgery to correct tetralogy of Fallot at an advanced age of over 15 years. All of the participants were referred to the Shahid Rajaei Heart and Vascular Center, which is a referral center for congenital heart diseases in Iran, between 2010 and 2020. The surgical procedures for these patients involved primary total correction of tetralogy of Fallot or surgery following by shunt implantation. Prior to the surgery, the necessary information was gathered from the patients' medical records. The patients were then monitored over a 5-year period, during which they received regular check-ups from cardiologist with fellowship in adult congenital heart disease.ResultsA total of 94 participants were enrolled in the study, with an average age of 26.7 ± 9.6 years. Notably, the majority of the participants were male. The study reported a late mortality rate of 3.2%. Furthermore, 17 patients, constituting 18% of the cohort, underwent a secondary surgical procedure. This secondary surgery encompassed 14 cases of Pulmonary Valve Replacement (14.8%) and 3 cases of Ventricular Septal Defect repair (3.1%).ConclusionWhile the optimal age for total correction of Tetralogy of Fallot is conventionally considered to be within the first year of life, this study demonstrated that surgical intervention performed at a later stage of life can yield favorable midterm prognoses. It is imperative to emphasize that individuals unable to undergo surgery at the ideal age due to a multitude of factors should not be deprived of the potential benefits associated with surgical intervention.

Congenital heart diseases (CHDs) frequently impact the right ventricular outflow tract, resulting in a significant incidence of pulmonary valve replacement in the pediatric population. While contemporary pediatric pulmonary valve replacements (PPVRs) allow satisfactory patient survival, their biocompatibility and durability remain suboptimal and repeat operations are commonplace, especially for very young patients. This places enormous physical, financial, and psychological burdens on patients and their parents, highlighting an urgent clinical need for better PPVRs. An important reason for the clinical failure of PPVRs is biofouling, which instigates various adverse biological responses such as thrombosis and infection, promoting research into various antifouling chemistries that may find utility in PPVR materials. Another significant contributor is the inevitability of somatic growth in pediatric patients, causing structural discrepancies between the patient and PPVR, stimulating the development of various growth‐accommodating heart valve prototypes. This review offers an interdisciplinary perspective on these challenges by exploring clinical experiences, physiological understandings, and bioengineering technologies that may contribute to device development. It thus aims to provide an insight into the design requirements of next‐generation PPVRs to advance clinical outcomes and promote patient quality of life. [ABSTRACT FROM AUTHOR]

Objective: This review aims to make a brief overview of blood biomarkers’ clinical decision limits, possibly aiding in outcome prediction in all-aged Tetralogy of Fallot patients. Potentially, these biomarkers could also assist in necessity and timing of pulmonary valve replacement.

Abstract Congenital heart diseases (CHDs) frequently impact the right ventricular outflow tract, resulting in a significant incidence of pulmonary valve replacement in the pediatric population. While contemporary pediatric pulmonary valve replacements (PPVRs) allow satisfactory patient survival, their biocompatibility and durability remain suboptimal and repeat operations are commonplace, especially for very young patients. This places enormous physical, financial, and psychological burdens on patients and their parents, highlighting an urgent clinical need for better PPVRs. An important reason for the clinical failure of PPVRs is biofouling, which instigates various adverse biological responses such as thrombosis and infection, promoting research into various antifouling chemistries that may find utility in PPVR materials. Another significant contributor is the inevitability of somatic growth in pediatric patients, causing structural discrepancies between the patient and PPVR, stimulating the development of various growth‐accommodating heart valve prototypes. This review offers an interdisciplinary perspective on these challenges by exploring clinical experiences, physiological understandings, and bioengineering technologies that may contribute to device development. It thus aims to provide an insight into the design requirements of next‐generation PPVRs to advance clinical outcomes and promote patient quality of life.

Aim: Ventricular arrhythmias (VAs) are the most common cause of death in patients with repaired Tetralogy of Fallot (rTOF). However, risk stratifying remains challenging. We examined outcomes following programmed ventricular stimulation (PVS) with or without subsequent ablation in patients with rTOF planned for pulmonary valve replacement (PVR). Methods: We included all consecutive patients with rTOF referred to our institution from 2010 to 2018 aged ≥18 years for PVR. Right ventricular (RV) voltage maps were acquired and PVS was performed from two different sites at baseline, and if non‐inducible under isoproterenol. Catheter and/or surgical ablation was performed when patients were inducible or when slow conduction was present in anatomical isthmuses (AIs). Postablation PVS was undertaken to guide implantable cardioverter‐defibrillator (ICD) implantation. Results: Seventy‐seven patients (36.2 ± 14.3 years old, 71% male) were included. Eighteen were inducible. In 28 patients (17 inducible, 11 non‐inducible but with slow conduction) ablation was performed. Five had catheter ablation, surgical cryoablation in 9, both techniques in 14. ICDs were implanted in five patients. During a follow‐up of 74 ± 40 months, no sudden cardiac death occurred. Three patients experienced sustained VAs, all were inducible during the initial EP study. Two of them had an ICD (low ejection fraction for one and important risk factor for arrhythmia for the second). No VAs were reported in the non‐inducible group (p <.001). Conclusion: Preoperative EPS can help identifying patients with rTOF at risk for VAs, providing an opportunity for targeted ablation and may improve decision‐making regarding ICD implantation. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES To mitigate the shortage of homograft sources, the use of handmade trileaflet expanded polytetrafluoroethylene valves in pulmonary valve replacement has shown excellent results from multicentre studies conducted in Japan. However, world-wide data outside Japan are relatively insufficient. This study presents the long-term results of a single surgeon's use of flipped-back trileaflet method in a 10-year case series. METHODS We have developed an efficient way to make a trileaflet-valved conduit utilizing flipped-back method for pulmonary valve replacement and have employed the technique since 2011. Retrospective data were studied between October 2010 and January 2020. Echocardiography, electrocardiogram, Pro-Brain Natriuretic Peptide and Magnetic Resonance Imaging data were analysed. RESULTS Fifty-five patients were reviewed and median follow-up duration was 2.9 years. The majority of diagnoses was Tetralogy of Fallot (n  = 41), and these patients subsequently underwent secondary pulmonary valve replacement at a median age of 15.6 years. Survival was 92.7% with the longest follow-up period being 10 years. There was no need for reoperation, and freedom from reintervention was 98.0% at 10 years. There were 4 deaths (3 in-hospital and 1 outpatient). One patient eventually received transcatheter pulmonary valve implantation. Postoperative echocardiography showed mild or less pulmonary stenosis and pulmonary regurgitation degree in 92.2% and 92.0% of patients, respectively. Comparable magnetic resonance imaging data (n  = 25) showed significant reduction in right ventricular volumes but not in ejection fractions. CONCLUSIONS Our series showed satisfactory long-term function of handmade flipped-back trileaflet-valved conduit used in our patients. The simple design is efficiently reproducible without complex fabrication process. [ABSTRACT FROM AUTHOR]

The impact of pulmonary valve replacement (PVR) on major adverse clinical outcomes in patients with repaired tetralogy of Fallot (rTOF) is unknown. The purpose of this study was to determine whether PVR is associated with improved survival and freedom from sustained ventricular tachycardia (VT) in rTOF. A PVR propensity score was created to adjust for baseline differences between PVR and non-PVR patients enrolled in INDICATOR (International Multicenter TOF Registry). The primary outcome was time to the earliest occurrence of death or sustained VT. PVR and non-PVR patients were matched 1:1 on PVR propensity score (matched cohort) and in the full cohort, modeling was performed with propensity score as a covariate adjustment. Among 1,143 patients with rTOF (age 27 ± 14 years, 47% PVR, follow-up 8.3 ± 5.2 years), the primary outcome occurred in 82. The adjusted HR for the primary outcome for PVR vs no-PVR (matched cohort n = 524) was 0.41 (95% CI: 0.21-0.81; multivariable model P = 0.010). Full cohort analysis revealed similar results. Subgroup analysis suggested beneficial effects in patients with advanced right ventricular (RV) dilatation (interaction P = 0.046; full cohort). In patients with RV end-systolic volume index >80 mL/m2, PVR was associated with a lower primary outcome risk (HR: 0.32; 95% CI: 0.16-0.62; P < 0.001). There was no association between PVR and the primary outcome in patients with RV end-systolic volume index ≤80 mL/m2 (HR: 0.86; 95% CI: 0.38-1.92; P = 0.70). Compared with rTOF patients who did not receive PVR, propensity score-matched individuals receiving PVR had lower risk of a composite endpoint of death or sustained VT. [Display omitted] [ABSTRACT FROM AUTHOR]

ABSTRACT Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non‐valved conduit to connect the right ventricle (RV) to the pulmonary artery (PA) or just patch enlargement of the native RVOT. With time, this RV‐PA conduit will degenerate with deterioration of function, either causing pulmonary stenosis or pulmonary regurgitation. This RVOT dysfunction may result in RV dilation, RV dysfunction, and eventual RV failure and arrhythmias. Multiple surgical pulmonary valve replacement (PVR) is often required throughout the patient's lifetime. Patients are subjected to increased risks with each additional cardiac operation. Transcatheter PVR (TPVR) has been developed over the past two decades as a valuable non‐surgical alternative to restore the RVOT and RV function, and hence reduce patients’ lifetime risks related to surgery. This article will discuss the long‐term results of TPVR which are demonstrated to be comparable to surgical results and the latest development of large pulmonary valves which will allow TPVR to be performed on native or larger RVOT.

The range of valve choices available to the cardiac surgeon for placement in the pulmonary position continues to expand. This article will provide a brief compendium of the most clinically relevant among these choices and review the contemporary literature regarding their relative durability as well as risk factors for structural valve deterioration and reintervention. The unique advantages and disadvantages of each of these valve choices will be discussed as they pertain to unique patient-specific factors, including patient size and the anatomy of the right ventricular outflow tract, that inform the choice of one prosthesis over another. Finally, general principles regarding the approach to valve choice, and future directions will be discussed. [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]

Open in new tab Download slide OBJECTIVES To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. METHODS Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977–1991), intermediate (1992–2006) and late (2007–2021). RESULTS The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7–30.5). There was a temporal trend towards less shunt palliation (–0.3% per year, 95% CI –0.05 to –0.1). Median age at intracardiac repair was 2.9 years (1.8–5.0), 0.8 years (0.5–1.3) and 0.5 years (0.4–0.7) (P  < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (–0.7% per year, 95% CI –0.5 to –1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5–1.0). Survival at 10 years was 79% (64–76), 90% (87–93) and 95% (92–98) (P  < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1–6), 12% (8–16) and 21% (13–29) (P  < 0.001) in the early, intermediate and late era, respectively. CONCLUSIONS There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair. [ABSTRACT FROM AUTHOR]

Resumen: Según las series, aproximadamente un 30% de los pacientes corregidos de Fallot mediante parche transanular pueden tener comprometida la funcionalidad de la válvula tricúspide en el momento del reemplazo valvular pulmonar (RVP). Esta insuficiencia tricuspídea (IT) es secundaria a la dilatación progresiva del ventrículo derecho, pudiendo existir causas intrínsecas de la cirugía correctora inicial. En la actualidad, está claramente establecido que la IT ensombrece el pronóstico de los pacientes con afección izquierda y la tendencia es a corregir cada vez más esta válvula en aras de una mejor supervivencia y clase funcional. En el caso de los pacientes corregidos de Fallot con parche transanular en el momento del RVP no existe una evidencia tan clara al respecto. Diversos estudios tratan de abordar la cuestión, todos ellos retrospectivos, la mayoría con escaso número de pacientes, con una potencia estadística limitada y susceptibles a errores y sesgos, llegando a conclusiones dispares.Aprovechando un caso clínico típico repasaremos los estudios realizados hasta el momento, intentando esclarecer algunos aspectos en el manejo de este tipo de pacientes. ¿Reparar o no reparar la tricúspide? Abstract: According to the series, approximately 30% of corrected Fallot with transannular patch surgery may have compromised tricuspid valve functionality at the time of pulmonary valve replacement. This tricuspid regurgitation is secondary to the progressive dilation of the right ventricle, and there may be intrinsic causes of the initial corrective surgery. At present it is clearly established that tricuspid regurgitation darkens the prognosis of patients with left pathology and the trend is to increasingly correct this valve in the interest of better survival and functional class. In the case of patients corrected for Fallot at the time of pulmonary valve replacement, there is not such clear evidence in this regard. Various studies try to address the issue, all of them retrospective, most with a small number of patients, with limited statistical power and susceptible to errors and biases, reaching disparate conclusions.Taking advantage of a typical clinical case, we will review the studies carried out so far, trying to clarify some aspects in the management of this type of patients. To repair or not to repair the tricuspid?

Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non‐valved conduit to connect the right ventricle (RV) to the pulmonary artery (PA) or just patch enlargement of the native RVOT. With time, this RV‐PA conduit will degenerate with deterioration of function, either causing pulmonary stenosis or pulmonary regurgitation. This RVOT dysfunction may result in RV dilation, RV dysfunction, and eventual RV failure and arrhythmias. Multiple surgical pulmonary valve replacement (PVR) is often required throughout the patient's lifetime. Patients are subjected to increased risks with each additional cardiac operation. Transcatheter PVR (TPVR) has been developed over the past two decades as a valuable non‐surgical alternative to restore the RVOT and RV function, and hence reduce patients' lifetime risks related to surgery. This article will discuss the long‐term results of TPVR which are demonstrated to be comparable to surgical results and the latest development of large pulmonary valves which will allow TPVR to be performed on native or larger RVOT. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Pulmonary regurgitation (PR) required pulmonary valve replacement (PVR) is usually seen after surgically repaired tetralogy of Fallot (TOF). Assessment by cardiac magnetic resonance imaging (CMR) plays a crucial role in the decision of PVR. Herein, we presented our 3-year interdisciplinary CMR experience in the assessment of repaired TOF. METHODS: CMR examinations of 196 patients with repaired TOF performed between 2016 and 2018 were enrolled in this retrospective study. Only 165 were included in the study. CMR findings were assessed according to the American College of Cardiology/American Heart Association guideline and recommendations of Geva. RESULTS: Among those 165 patients (median age 14 years [mean age 15.62±7.42 years], M/F=114/61; 1.86/1), 73 patients were found eligible for PVR (59 patients for transcatheter while 14 patients for surgical). The mean QRS duration was 170.2±16.89 ms. On CMR assessment, mean indexed right ventricular end-diastolic volume, end-systolic volume, right, and left ventricular ejection fraction were 187.64±45.07 ml/m2, 39.90±6.60%, and 47.83±6.12%, respectively. The PR fraction was as 50.10±2.54% and 2.25±1.92. Balloon dilatation and/or stenting of branch pulmonary arteries in 12 patients and ventricular septal defect closure in four patients were performed at the same session of percutaneous PVR. At the time of the surgical PVR, repair of partial anomalous pulmonary venous return in one patient, ventricular septal defect in two patients, and subaortic membrane in one patient were performed. An implantable cardioverter-defibrillator was also performed in one patient. CONCLUSION: Our CMR experience has the largest patient population in our country and may contribute to the national data pool. We believe that our collaborative experience between radiologists, cardiologists, and cardiovascular surgeons may also enhance the use of CMR in determining the appropriate technique or timing for PVR. [ABSTRACT FROM AUTHOR]