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Background and Aims Patients with repaired tetralogy of Fallot remain at risk of life-threatening ventricular tachycardia related to slow-conducting anatomical isthmuses (SCAIs). Preventive ablation of SCAI identified by invasive electroanatomical mapping is increasingly performed. This study aimed to non-invasively identify SCAI using 3D late gadolinium enhancement cardiac magnetic resonance (3D-LGE-CMR). Methods Consecutive tetralogy of Fallot patients who underwent right ventricular electroanatomical mapping (RV-EAM) and 3D-LGE-CMR were included. High signal intensity threshold for abnormal myocardium was determined based on direct comparison of bipolar voltages and signal intensity by co-registration of RV-EAM with 3D-LGE-CMR. The diagnostic performance of 3D-LGE-CMR to non-invasively identify SCAI was determined, validated in a second cohort, and compared with the discriminative ability of proposed risk scores. Results The derivation cohort consisted of 48 (34 ± 16 years) and the validation cohort of 53 patients (36 ± 18 years). In the derivation cohort, 78 of 107 anatomical isthmuses (AIs) identified by EAM were normal-conducting AI, 22 were SCAI, and 7 blocked AI. High signal intensity threshold was 42% of the maximal signal intensity. The sensitivity and specificity of 3D-LGE-CMR for identifying SCAI or blocked AI were 100% and 90%, respectively. In the validation cohort, 85 of 124 AIs were normal-conducting AI, 36 were SCAI, and 3 blocked AI. The sensitivity and specificity of 3D-LGE-CMR were 95% and 91%, respectively. All risk scores showed an at best modest performance to identify SCAI (area under the curve ≤.68). Conclusions 3D late gadolinium enhancement cardiac magnetic resonance can identify SCAI with excellent accuracy and may refine non-invasive risk stratification and patient selection for invasive EAM in tetralogy of Fallot. [ABSTRACT FROM AUTHOR]

Background and Aims Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. Methods Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. Results Data for 840 consecutive patients treated in 2014–2021 at a median age of 29.2 (19.0–41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%–99.2%] of patients. Median follow-up was 20.3 (7.1–38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P =.009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%–1.0%), 0.9% (0.2%–1.6%), and 3.8% (0.0%–8.4%); pulmonary valve replacement, 0.4% (0.0%–0.8%), 1.3% (0.2%–2.4%), and 8.0% (1.2%–14.8%); and pulmonary valve thrombosis, 0.4% (0.0%–0.9%), 0.7% (0.0%–1.3%), and 0.7% (0.0%–1.3%), respectively. Conclusions Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs. [ABSTRACT FROM AUTHOR]

Background Heart transplantation is the gold standard therapy for end-stage heart failure; however, it is limited by a shortage of available donors. In recent years, heart transplantations have been performed using marginal donor hearts with valvular and/or congenital cardiac abnormalities. Case summary A 60-year-old woman with acromegalic cardiomyopathy underwent left ventricular assist device implantation and aortic valve (AV) closure 4 years prior. After 2 months, repeat AV closure and omental flap transposition were performed. During the outpatient follow-up, the patient developed recurrent severe AV regurgitation and bacteraemia-induced subarachnoid haemorrhage. She underwent urgent heart transplantation using a marginal donor heart with preserved cardiac function, mild pulmonary valve stenosis, and regurgitation after pulmonary valve-sparing tetralogy of Fallot (TOF) repair. An anatomical anastomosis was possible. She had no signs of infection, heart failure, arrhythmia, or immune rejection 15 months after the heart transplantation. Discussion In this case, the donor heart with repaired TOF did not require pulmonary valve replacement and was anatomically intact. Donor hearts with repaired TOF that are expected to have long-term durability in terms of cardiac function may be used for successful heart transplantations. The repair of marginal donor hearts creates an opportunity to increase the number of viable donors. [ABSTRACT FROM AUTHOR]

The article discusses the results of a large European registry on the Sapien S3 transcatheter pulmonary valve replacement (TPVR). The registry data shows that the Sapien S3 valve performs well in the short- and intermediate-term, especially when treating dysfunctional conduits or bioprosthetic valves. However, there are challenges when using the Sapien S3 in patients with native right ventricular outflow tract (RVOT) anatomy, with a 12% complication rate related to pre-stent migration/embolization and valve malposition. The article also highlights concerns about infective endocarditis rates and leaflet thrombosis in TPVR patients. Overall, while the Sapien S3 is a promising option, it is not a panacea and there are still important considerations and risks to be aware of. [Extracted from the article]

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]

Risk-benefit assessment Pre-emptive catheter ablation for repaired TOF before native RVOT transcatheter PVR is reasonable in the context of conventional risk/benefit analysis. Catheter ablation of monomorphic ventricular tachycardia for repaired Tetralogy of Fallot Catheter ablation of monomorphic VT for repaired TOF is unlike other forms of congenital heart disease. [Extracted from the article]

Pulmonary valve (PV) fibroelastomas are a rare pathology, with limited anecdotal literature surrounding them. Consequently, the natural history is unclear; however, two features have remained salient; they are asymptomatic and found incidentally. Here, we describe a 52-year-old female, presenting with symptoms suggestive pulmonary embolism (PE). Pulmonary angiography revealed a filling deficit in the pulmonary trunk (PT), adjacent to the PV. Subsequent investigation found a large PV fibroelastoma. The presence of symptoms is likely secondary to right ventricular outflow tract obstruction from the lesions large size. We describe our investigation and management of the lesion. The reporting of this case challenges the existing knowledge of PV fibroelastomas. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Right ventricle to pulmonary artery anatomic discontinuity is common in complex congenital heart malformations. Handsewn conduits are a practised method of repair. In a proof-of-concept study, we evaluated pulmonary valve replacement with a handsewn pericardial valved pulmonary conduit in vitro and in vivo. METHODS: A pulsatile flow-loop model (in vitro) and an acute 60-kg porcine model (in vivo) were used. With echocardiography and pressure catheters, baseline geometry and fluid dynamics were measured. The pulmonary valve was replaced with a handsewn glutaraldehyde-treated pericardial valved pulmonary conduit corresponding to a 21-mm prosthetic valve, after which geometric measurements and fluid dynamics were reassessed. RESULTS: In vitro, 15 pulmonary trunks at 4 l/min and 13 trunks at 7 l/min, and in vivo, 11 animals were investigated. The valved pulmonary conduit was straightforward to produce at the operating table and easy to suture in place. All valves were clinically sufficient in vitro and in vivo. The mean transvalvular pressure gradient in the native valve and the conduit was 8 mmHg [standard deviation (SD): 2] and 7 mmHg (SD: 2) at 4 l/min in vitro, 19 mmHg (SD: 3) and 17 mmHg (SD: 4) at 7 l/min in vitro and 3 mmHg (SD: 2) and 6 mmHg (SD: 3) in vivo. CONCLUSIONS: Our proof-of-concept demonstrates no early evidence of structural damage to the conduit, and the fluid dynamic data were acceptable. The handsewn conduit can be produced at the operating table. Conduits to restore the right ventricle to pulmonary artery (RV–PA) anatomic discontinuity are used routinely in complex congenital malformations that involve the right ventricular outflow tract. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Our goal was to evaluate the risk-adjusted effects of operative and non-operative repair on long-term outcomes in patients with congenitally corrected transposition of the great arteries and left ventricular outflow tract obstruction (CCTGA/LVOTO). METHODS We retrospectively reviewed 391 patients with CCTGA/LVOTO from 2001 to 2020 (operative group, 282; non-operative group, 109) in 3 centres in China. The operative group included 73 patients who underwent anatomical repair and 209 patients who underwent non-anatomical repair. The median follow-up time was 8.5 years. The inverse probability of treatment weighted-adjusted Cox regression and a Kaplan–Meier analysis were used to evaluate long-term outcomes. RESULTS Operative repair did not reduce the hazard ratio (HR) of death, tricuspid regurgitation or New York Heart Association functional class III/IV, but a significantly increased HR was observed for pulmonary valve regurgitation [HR, 2.84; 95% confidence interval (CI), 1.10–7.33; P  = 0.031]. Compared with that in the non-operative group, anatomical repair resulted in significantly increased HRs for death (HR, 2.94; 95% CI, 1.10–7.87; P  = 0.032) and pulmonary valve regurgitation (HR, 9.71; 95% CI, 3.66–25.77; P  < 0.001). Subgroup analysis showed that in patients with CCTGA/LVOTO with moderate or worse tricuspid regurgitation (≥ moderate), anatomical repair significantly reduced the HR of death. An inverse probability of treatment weighting-adjusted Kaplan–Meier analysis showed that the survival rates at 5 and 10 days postoperatively were 88.2 ± 4.0% and 79.0 ± 7.9%, respectively, in the anatomical repair group; these rates were significantly lower than the rates in the non-operative group (95.4 ± 2.0% and 91.8 ± 2.8%; P  = 0.032). CONCLUSIONS For patients with CCTGA/LVOTO, operative repair does not provide superior long-term outcomes, and anatomical repair results in a higher incidence of death. However, in patients with CCTGA/LVOTO with tricuspid regurgitation ≥ moderate, anatomical repair can reduce the risk of death in the long term. [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]

Objectives: Amoxicillin is the drug of choice in the management of streptococcal and enterococcal infective endocarditis (IE) but little is known regarding amoxicillin diffusion into infected heart valves. Herein, we assessed amoxicillin valvular distribution and related pharmacokinetic/pharmacodynamic (PK/PD) target attainment in IE patients undergoing heart valve surgery.Patients and Methods: In this 2-year prospective study, patients with IE treated by continuous infusion of amoxicillin and undergoing a surgical valve replacement were included. Both amoxicillin plasma and tissue concentrations were measured the day of surgery. Amoxicillin concentration in plasma and crushed heart valves were measured by a validated liquid chromatography method coupled with ultra-violet and tandem mass spectrometry, respectively. MIC and MBC of amoxicillin were determined for all available isolates. The rate of achievement of PK/PD efficacy parameters were assessed.Results: Twenty-two heart valves were removed from 20 patients. Bacterial aetiology was streptococcal (n = 17) and enterococcal (n = 3). Amoxicillin mean daily dose was 12 ± 3 g/24 h, mean plasma concentration was 29 ± 21 mg/L (n = 15), mean tissue concentration was 23 ± 15 mg/L (n = 22). Median diffusion rate was 62%. Patients reached a plasma concentration target >4XCMI (n = 13). Tissue concentrations were bactericidal for all streptococcal IE but not for enterococcal IE.Conclusions: Amoxicillin intravalvular measurements in IE treated patients showed significant penetration into the infectious site. These data are reassuring that in situ bactericidal concentrations can be largely achieved in the management of streptococcal IE and support the need for combination antibiotic therapy for enterococcal IE. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Early results from the prospective ESPOIR Trial have indicated excellent results for pulmonary valve replacement using decellularized pulmonary homografts (DPH). METHODS A 5-year analysis of ESPOIR Trial patients was performed to provide an insight into the midterm DPH performance. ESPOIR Trial and Registry patients were matched with cryopreserved homografts (CH) patients considering patient age, type of heart defect and previous procedures to present the overall experience with DPH. RESULTS A total of 121 patients (59 female) were prospectively enrolled (8/2014–12/2016), median age 16.5 years (interquartile range 11.2–29.8), and median DPH diameter 24 mm. One death (73 year-old) occurred during a median follow-up of 5.9 years (5.4–6.4), in addition to 2 perioperative deaths resulting in an overall mortality rate of 2.5%. One case of endocarditis in 637 patient-years was noticed, resulting in an incidence of 0.15% per patient-year. At 5 years, the mean peak gradient was 19.9 mmHg (9.9), mean regurgitation 0.9 (0.6, grade 0–3) and freedom from explantation/any reintervention 97.5% (1.5). The combined DPH cohort, n  = 319, comprising both Trial and Registry data, showed significantly better freedom from explantation for DPH 95.5% (standard deviation 1.7) than CH 83.0% (2.8) (P  < 0.001) and less structural valve degeneration at 10 years when matched to 319 CH patients [DPH 65.5% (standard deviation 4.4) and CH 47.3% (3.7), P  = 0.11]. CONCLUSIONS The 5-year data of the prospective ESPOIR Trial show excellent performance for DPH and low rates of adverse events. ESPOIR Registry data up to 15 years, including a matched comparison with CH, demonstrated statistically significant better freedom from explantation. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The emergence of big cardio-thoracic surgery datasets that include not only short-term and long-term discrete outcomes but also repeated measurements over time offers the opportunity to apply more advanced modelling of outcomes. This article presents a detailed introduction to developing and interpreting linear mixed-effects models for repeated measurements in the setting of cardiothoracic surgery outcomes research. METHODS A retrospective dataset containing serial echocardiographic measurements in patients undergoing surgical pulmonary valve replacement from 1986 to 2017 in Erasmus MC was used to illustrate the steps of developing a linear mixed-effects model for clinician researchers. RESULTS Essential aspects of constructing the model are illustrated with the dataset including theories of linear mixed-effects models, missing values, collinearity, interaction, nonlinearity, model specification, results interpretation and assumptions evaluation. A comparison between linear regression models and linear mixed-effects models is done to elaborate on the strengths of linear mixed-effects models. An R script is provided for the implementation of the linear mixed-effects model. CONCLUSIONS Linear mixed-effects models can provide evolutional details of repeated measurements and give more valid estimates compared to linear regression models in the setting of cardio-thoracic surgery outcomes research. [ABSTRACT FROM AUTHOR]

- Isaac Newton The pulmonary valve (PV) has an essential role as any other heart valve. Incremental value of three-dimensional echocardiography for evaluating pulmonary valve In patients with good-quality 2DE of the PV, it is possible to acquire transthoracic 3DE data sets of the PV from either the parasternal or the subcostal short-axis views. Apart from anatomical considerations, the longer-than-expected learning curve supports the fact that operator skill plays a pivotal role in the success of PV imaging.[4] Pulmonary valve stenosis is often associated with valve thickening, which increases the 3D echogenicity and image quality. [Extracted from the article]

Background Pulmonary artery sarcoma (PAS) is an extremely rare tumour, preferably treated by surgery. However, the surgical management remains largely debatable, as only less than half of patients with PAS can undergo thorough excision. Case summary A 32-year-old man with a tumour involving the right ventricle outflow tract, pulmonary trunk extending into the bifurcation, and right pulmonary arteries underwent complete resection using a homologous pulmonary valve and vascular grafts for reconstruction, combined with right pulmonary endarterectomy (PEA) for potential seeding metastasis. Histopathological examination demonstrated undifferentiated pleomorphic sarcoma with surgical margins free of disease. The patient remains asymptomatic, and follow-up computed tomography 5 months after surgery indicated no recurrence or metastasis. Discussion Radical resection of a PAS with reconstruction using pulmonary valve allograft and polytetrafluoroethylene vascular grafts is technically feasible and successful. Additionally, PEA may eliminate the potential intima implantation metastasis. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Many centres have recently adopted pulmonary valve (PV) preservation (PVP) during tetralogy of Fallot (ToF) repair. We sought to identify the midterm functional outcomes and risk factors for pulmonary regurgitation after this procedure. METHODS All patients undergoing PVP during transatrial–transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and December 2020 were reviewed. RESULTS Overall, 73 patients were included. At the index surgery, the body surface area was 0.31 ± 0.04 m2, the age was 4.9 ± 2.9 months and the preoperative PV z -score was -3.02 ± 1.11. At a mean follow-up of 5.3 ± 2.7 years, the fractional area change of the right ventricle (RV) was 47.1 ± 5.2%, and the tricuspid annular plane systolic excursion z -score was -3.31 ± 1.89%. The 5-year freedom from moderate/severe PV regurgitation was 61.3% [95% confidence interval (CI): 48, 73%]. There was a significant correlation between RV function and moderate/severe PR at follow-up (R2: 0.08; P = 0.03). A comparison with a group of patients undergoing a transannular patch procedure (N = 33) showed superior outcomes for patients with PVP. The preoperative PV z -score and the degree of PR at discharge were risk factors for the early development of moderate/severe PR at follow-up [hazard ratio (HR): 0.64; 95% CI: 0.48, 0.86, P = 0.01 and HR: 2.31; 95% CI: 1.00, 5.36, P = 0.04, respectively]. A preoperative PV annulus z -score ≤ -2.85 was found to be predictive for moderate/severe PR at 5 years after PVP (HR: 2.56; 95% CI: 1.31, 5.01, P  = 0.002). CONCLUSIONS A pulmonary valve preservation strategy during tetralogy of Fallot repair should always be attempted. However, a preoperative PV annulus z -score < -2.85 and moderate/severe regurgitation upon discharge are risk factors for midterm pulmonary regurgitation. [ABSTRACT FROM AUTHOR]

Valvular regurgitation represents an important cause of cardiovascular morbidity and mortality. Imaging is pivotal in the evaluation of native valve regurgitation and echocardiography is the primary imaging modality for this purpose. The imaging assessment of valvular regurgitation should integrate quantification of the regurgitation, assessment of the valve anatomy and function, and the consequences of valvular disease on cardiac chambers. In clinical practice, the management of patients with valvular regurgitation largely relies on the results of imaging. It is crucial to provide standards that aim at establishing a baseline list of measurements to be performed when assessing native valve regurgitation. The present document aims to present clinical guidance for the multi-modality imaging assessment of native valvular regurgitation. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot is the most common cyanotic congenital heart defect, accounting for 10% of all CHD. Despite most patients now surviving well into adulthood, morbidity and mortality rates continue to be high. Surgical and percutaneous pulmonary valve replacement are procedures that are performed to prevent long-term complications from occurring. Unfortunately, pulmonary valve replacement based on current CMR criteria does not prevent postoperative ventricular arrhythmia, heart failure, and sudden cardiac death. Thus, a more advanced and comprehensive hemodynamic evaluation is needed to better understand right ventricular (dys)function in tetralogy of Fallot patients and to optimize the timing of valve replacement. Recently, four-dimensional flow CMR has emerged as a promising and non-invasive imaging technique that can provide comprehensive quantitative evaluation of flow in an entire volume within the chest in a single imaging session. With velocity-encoding in all three spatial directions throughout the complete cardiac cycle, it can provide analysis of cardiac, pulmonary artery and aortic flow volumes, flow velocities, flow patterns, as well as more advanced hemodynamic parameters. Four-dimensional flow CMR could therefore provide insights into the complex hemodynamics of tetralogy of Fallot and could potentially provide novel criteria for pulmonary valve replacement in these patients. The aim of this review is to provide an overview of available research on four-dimensional flow CMR research in tetralogy of Fallot patients. [ABSTRACT FROM AUTHOR]

Background Infective endocarditis (IE) is one of the common causes of life-threatening infections. Compared to left-sided endocarditis, right-sided infective endocarditis is rarer, with pulmonary valve endocarditis much rarer than the tricuspid valve. Its diagnosis poses a challenge, owing to its rarity, low index of clinical suspicion, and lack of availability of appropriate diagnostic measures. Risk factors include indwelling central venous catheter, sepsis, intravenous drug use, pacemaker with lead infection, or ventricular septal defect (VSD). Case summary We describe a case of pulmonary valve endocarditis that led to septic pulmonary emboli in a patient scheduled for elective bypass surgery for triple vessel disease. There was an incidental finding of VSD on echocardiography, which is also a risk factor for pulmonary valve endocarditis owing to the jet of VSD to the pulmonary valve. The patient was given 4 weeks of antibiotics and subsequently underwent coronary artery bypass graft, pulmonary valve replacement, and VSD closure. Discussion Our case demonstrated the importance of high clinical suspicion and vigilance of diagnosing pulmonary valve endocarditis when dealing with pyrexia of unknown origin in a patient with a congenital VSD as VSD-associated pulmonary valve endocarditis remained a rare disease. Besides, an active search for clinical and radiological signs of pulmonary embolization is necessary in patients with right-sided endocarditis especially those with large and mobile vegetation. A conservative approach or valve repair is recommended for most patients with right sided IE affecting the tricuspid or pulmonary valve. [ABSTRACT FROM AUTHOR]

Aims The Leiden Convention coronary coding system structures the large variety of coronary anatomical patterns; isolated and in congenital heart disease. It is widely used by surgeons but not by cardiologists as the system uses a surgeons' cranial view. Since thoracic surgeons and cardiologists work closely together, a coronary coding system practical for both disciplines is mandatory. To this purpose, the 'surgical' coronary coding system was adapted to an 'imaging' system, extending its applicability to different cardiac imaging techniques. Methods and results The physician takes place in the non-facing sinus of the aortic valve, oriented with the back towards the pulmonary valve, looking outward from the sinus. From this position, the right-hand sinus is sinus 1, and the left-hand sinus is sinus 2. Next, a clockwise rotation is adopted starting at sinus 1 and the encountered coronary branches described. Annotation of the normal anatomical pattern is 1R-2LCx, corresponding to the 'surgical' coding system. The 'imaging' coding system was made applicable for Computed Tomography (CT), Magnetic Resonance Imaging (MRI), echocardiography, and coronary angiography, thus facilitating interdisciplinary use. To assess applicability in daily clinical practice, images from different imaging modalities were annotated by cardiologists and cardiology residents and results scored. The average score upon evaluation was 87.5%, with the highest scores for CT and MRI images (average 90%). Conclusion The imaging Leiden Convention is a coronary coding system that unifies the annotation of coronary anatomy for thoracic surgeons, cardiologists, and radiologists. Validation of the coding system shows it can be easily and reliably applied in clinical practice. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Since percutaneous pulmonary valve implantation (PPVI) was introduced to prolong the lifetime of surgically placed right ventricular to pulmonary artery conduits, valve technology has evolved and the indications for PPVI expanded to native and larger right ventricular outflow tracts. We explore how indications, patient populations and outcomes compare to surgical pulmonary valve replacement (PVR). METHODS This is a retrospective cohort study of PPVI and PVR procedures between 1998 and 2020 at a single UK centre. One hundred and twenty-eight patients underwent PPVI and 365 patients PVR. Primary outcome measures were survival, infective endocarditis and reintervention. RESULTS The most common indication for PVR was replacement of the native pulmonary valve for pulmonary regurgitation whereas PPVI was more commonly used to treat pulmonary stenosis in a previously placed bioprosthetic conduit or valve. Treatment indications for PPVI expanded over the study to include the native right ventricular outflow tract. Survival was similar for PPVI and PVR (92% PPVI and 96.8% PVR at 5 years; 85.8% PPVI and 95.1% PVR at 10 years). Preprocedural New York Heart Association class 3 and 4 was the most important predictor of poor outcome. Annualized infective endocarditis rate was significantly higher for the Melody PPVI (0.024 vs 0.0024/person/year for PVR, P  < 0.05). Both groups showed significant symptomatic improvement postprocedure with remodelling of ventricular volumes and improvement in cardiac output. Long-term follow-up for PVR showed half of patients will need replacement at 10–15 years post-index procedure. CONCLUSIONS An increasing number of patients requiring PVR can now be treated percutaneously. A lifetime strategy for re-valving should be considered at the first valve implant. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7–106.9 ml/m2, P  <   0.001), end-systolic volume (101.4–64.9 ml/m2, P  <   0.001), stroke volume (66.8–48.0 ml/m2, P  =   0.007) and cardiac output (5.1–3.6 l/m2, P  =   0.040). Twenty-eight percentage of patients achieved normalization of the right ventricular volume after pulmonary valve replacement. In the exercise test, the maximum rate of oxygen consumption (72.5–69.5%) and oxygen pulse (95.0–83.0%) changed without statistical significance. CONCLUSIONS Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status. [ABSTRACT FROM AUTHOR]

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

Aims Right ventricular (RV) function assessment is crucial in congenital heart disease patients, especially in atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) patients with pulmonary regurgitation (PR). In this study, we aimed to analyse both 3D RV shape and deformation to better characterize RV function in ASD and TOF-PR. Methods and results We prospectively included 110 patients (≥16 years old) into this case–control study: 27 ASD patients, 28 with TOF, and 55 sex- and age-matched healthy controls. Endocardial tracking was performed on 3D transthoracic RV echocardiographic sequences and output RV meshes were post-processed to extract local curvature and deformation. Differences in shape and deformation patterns between subgroups were quantified both globally and locally. Curvature highlights differences in RV shape between controls and patients while ASD and TOF-PR patients are similar. Conversely, strain highlights differences between controls and TOF-PR patients while ASD and controls are similar [global area strain: −31.5 ± 5.8% (controls), −34.1 ± 7.9% (ASD), −24.8 ± 5.7% (TOF-PR), P  < 0.001, similar significance for longitudinal and circumferential strains]. The regional and local analysis highlighted differences in particular in the RV free wall and the apical septum. Conclusion Chronic RV volume loading results in similar RV shape remodelling in both ASD and TOF patients while strain analysis demonstrated that RV strain is only reduced in the TOF group. This suggests a fundamentally different RV remodelling process between both conditions. [ABSTRACT FROM AUTHOR]

Background To the best of our knowledge, this is the first reported case of transcatheter pulmonary valve replacement (TPVR) with extracorporeal membrane oxygenation (ECMO) support with successful decannulation as a bridge to recovery in a young adult with complex congenital heart disease. Case summary We describe a 24-year-old male patient with a history of D-transposition of the great arteries with ventricular septal defect status post-Rastelli repair at age three lost to follow-up and presenting with severe biventricular failure, left ventricular thrombus, and critical pulmonary conduit stenosis, deemed non-surgical and non-transplant candidate, who underwent conduit stenting and TPVR in the setting of cardiogenic shock. Upon intubation for general anaesthesia, the patient suffered from ventricular tachycardia arrest requiring cardiopulmonary resuscitation and veno-arterial ECMO. Once stabilized, conduit stenting and TPVR was performed with significant haemodynamic improvement and immediate ECMO decannulation with subsequent biventricular function improvement. Discussion In critically ill patients with complex congenital heart disease that are neither surgical nor transplant candidates, ECMO support can be used as a means of support during a transcatheter intervention to improve haemodynamics and a bridge to recovery, allowing time for future potential candidacy for surgery or transplantation as indicated. Patients with congenital heart disease need regular follow-up in specialty clinics to prevent the development of such critical illness. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The aim of this study was to investigate the long-term outcomes following right ventricle-to-pulmonary artery (RV-to-PA) conduit insertion of Medtronic Freestyle® porcine valve (MFV) or pulmonary allograft valve (PAV) in adult patients with congenital heart disease. METHODS Retrospective medical record review of consecutive RV-to-PA conduit insertion, using either PAV or MFV from 1991 to 2017. Perioperative data and clinic reports were collected. Cause and date of death were obtained from the Australian National Death Index to obtain survival function. RESULTS In total, 232 patients (median age 31.5 years, interquartile range 25–41 years) underwent RV-to-PA conduit insertion (PAV = 84 and MFV = 148) and were eligible for inclusion [63.8% tetralogy of Fallot (TOF); 11.6% congenital pulmonary stenosis (PS); 24.6% other diagnoses]. The overall median follow-up time was 9.1 years (interquartile range 5.3–12.6 years). The mean gradient was 11.8 ± 7.1 mmHg in PAV and 16.6 ± 9.6 mmHg in MFV patients. Congenital PS patients had 100% survival at 20 years, TOF patients at 5, 10, 15 and 20 years had 99%, 97%, 96% and 96% survival, respectively. Patients with other primary diagnoses at 5, 10, 15 and 20 years had 93%, 91%, 87% and 87% respectively. Freedom from reintervention did not differ significantly at 5 and 10 years between pulmonary allograft (98.6%, 98.6%) and Freestyle® porcine bioprosthesis (97.5%, 93%). CONCLUSIONS Both valves perform equally well with regard to patients' freedom from reoperation, although transvalvular gradient was higher for Freestyle® patients. Congenital PS and TOF patients had better survival than patients with other primary diagnoses. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The aims of this study were to evaluate and compare the outcomes after pulmonary valve replacement (PVR) with a mechanical prosthesis (MP) and a bioprosthesis (BP). METHODS From 2004 through 2017, a total of 131 patients, who had already been repaired for tetralogy or Fallot or its variants, underwent their first PVR with an MP or a BP. Outcomes of interests were prosthesis failure (stenosis >3.5 m/s, regurgitation >mild or infective endocarditis) and reintervention. RESULTS The median age at PVR was 19 years. BP and MP were used in 88 (67.2%) and 43 (32.8%) patients, respectively. The median follow-up duration was 7.4 years, and the 10-year survival rate was 96.4%. Risk factors for prosthesis failure were smaller body surface area [hazard ratio (HR) 0.23 per 1 m2, P  =   0.047] and smaller prosthesis size (HR 0.73 per 1 mm, P  =   0.039). Risk factors for prosthesis reintervention were smaller body surface area (HR 0.11 per 1 m2, P  =   0.011) and prosthesis size (HR 0.67 per 1 mm, P  =   0.044). Probability of prosthesis failure and reintervention at 10 years were 24.6% (19.5% in BP vs 34.8% in MP, P  =   0.34) and 7.8% (5.6% in BP vs 11.9% in MP, P  =   0.079), respectively. Anticoagulation-related major thromboembolic events were observed in 4 patients receiving an MP. CONCLUSIONS MP might not be superior to BP in terms of prosthesis failure or reintervention. MP should be carefully considered for highly selected patients in the era of transcatheter PVR. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The present study was conducted to investigate the decrease in left ventricular stroke volume index (LVSVI) that is caused by pulmonary regurgitation-induced right heart dysfunction and its clinical implications before and after pulmonary valve replacement (PVR). METHODS Between January 2010 and December 2019, 30 adults who underwent surgical PVR for chronic pulmonary regurgitation with right ventricular dilation late after tetralogy of Fallot (TOF) repair were included. All patients were evaluated using cardiac magnetic resonance before PVR. The median interval from TOF repair to PVR was 29 [25th, 75th percentile: 25, 37] years. The median pulmonary regurgitation fraction and right ventricular end-diastolic volume index were 56 [48, 66] % and 203 [187, 239] ml/m2. Twenty-three patients (76.7%) were re-evaluated 1 year after PVR. RESULTS Before PVR, the median LVSVI was 40 [35, 46] ml/beat/m2. A lower LVSVI was associated with a longer interval from TOF repair to PVR (r = −0.40, P  =   0.029) and a lower right ventricular ejection fraction (r  =   0.52, P  =   0.004). A lower LVSVI was not associated with a higher right ventricular end-diastolic volume index. LVSVI remained unchanged after PVR. The patients were subdivided into Normal-stroke volume index (SVI) and Subnormal-SVI groups using the preoperative LVSVI cut-off value of 35 mL/beat/m2. Compared with the Normal-SVI group, the Subnormal-SVI group had a higher incidence of ablation therapy before PVR (4.7 vs 2.3 patient-years, P  =   0.044). After PVR, LVSVI in the Subnormal-SVI group was still lower (40 [34, 42] vs 44 [42, 47] ml/beat/m2, P  =   0.038) despite the right ventricular end-diastolic volume index normalization. There was no difference in the clinical event incidence between the 2 groups during the follow-up period. Brain natriuretic peptide level in the Subnormal-SVI group was higher within 3 years after PVR (P  =   0.046). CONCLUSIONS Reduced left ventricular stroke volume did not fully recover after PVR. PVR for patients with repaired TOF should be performed before the left ventricular stroke volume begins to decrease. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The clinical significance of persistent end-diastolic forward flow (EDFF) after pulmonary valve replacement (PVR) remains unclear in patients with repaired tetralogy of Fallot. This study aimed to identify the characteristics of these patients and the impact of persistent EDFF on outcomes. METHODS Of 46 consecutive patients who underwent PVR for moderate to severe pulmonary regurgitation between 2003 and 2019, 23 (50%) did not show EDFF before PVR [group (−)]. In the remaining 23 patients with EDFF before PVR, EDFF was diminished after PVR in 13 (28%) [group (+, −)] and persisted in 10 (22%) [group (+, +)]. The following variables were compared between these 3 groups: (i) preoperative right ventricular (RV) and right atrial volumes measured by magnetic resonance imaging, haemodynamic parameters measured by cardiac catheterization and the degree of RV myocardial fibrosis measured by RV biopsy obtained at PVR and (ii) the post-PVR course, development of atrial arrhythmia and need for intervention. RESULTS A high RV end-diastolic pressure, a greater right atrial volume index and a greater RV end-systolic volume index before PVR and a high degree of RV fibrosis were significantly associated with persistent EDFF 1 year after PVR. Persistent EDFF was a significant risk factor for postoperative atrial tachyarrhythmia, and catheter ablation and pacemaker implantation were required more frequently in these patients. CONCLUSIONS Persistent EDFF after PVR could predict a worse prognosis, especially an increased risk of arrhythmia. Close follow-up is required in patients with persistent EDFF for early detection of arrhythmia and prompt reintervention if necessary. Clinical trial registration number Institutional review board of Osaka University Hospital, number 16105 [ABSTRACT FROM AUTHOR]

Infective endocarditis remains a medical challenge among urgent cases of cardiac disease. Multi-valvular endocarditis is uncommon and simultaneous right and left-sided valvular involvement, particularly affecting the pulmonary valve, is scarcely reported. A rare case of a patient with subacute myelodysplastic syndrome, who presented with endocarditis involving both aortic and pulmonary valves, complicated with new-onset heart failure, is described. The patient presented prompt recovery of both right and left ventricular function after combined aortic and pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Percutaneous pulmonary valve prostheses and right ventricle-to-pulmonary artery conduits are at risk for infective endocarditis (IE). In children and adults with a congenital heart disease, a pulmonary valve implant is frequently necessary. Prosthetic valve endocarditis is a conservatively barely manageable, serious life-threatening condition. We investigated the results of surgical pulmonary valve replacements in patients with IE. METHODS A total of 20 patients with congenital heart disease with the definite diagnosis of IE between March 2013 and July 2020 were included in this single institutional, retrospective review. Infected conduits were 11 Melody, 5 Contegra, 3 homografts and 1 Matrix P Plus. All of the infected prosthetic material was removed from the right ventricular outflow tract up to the pulmonary bifurcation. Pulmonary homografts were implanted after pulmonary root resection as right ventricle-to-pulmonary artery conduits. RESULTS All patients survived and were discharged infection-free. The mean time from the conduit implant to the operation for IE was 4.9 years [95% confidence interval (CI), 3.0–6.9]. The median intensive care unit stay was 3.0 days (95% CI, 2.0–4.7), and the median hospital time was 25.0 days (95% CI, 19.2–42.0). Median follow-up time was 204.5 days (range 30 days to 5 years) without death or recurrent endocarditis. CONCLUSIONS The surgical treatment of IE of percutaneous pulmonary valve prostheses and right ventricle-to-pulmonary artery conduits is a safe and effective therapeutic concept. Early surgical referral of patients with suspicion of IE should be pursued to avoid sequelae such as right ventricular failure, septic emboli, intracardiac expansion and antibiotic resistance. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Indications for and timing of pulmonary valve replacement (PVR) after tetralogy of Fallot repair are controversial. Among magnetic resonance imaging indices proposed to time valve replacement, a right ventricular (RV) end-diastolic volume index greater than 160 ml/m2 is often used. Recent evidence suggests that this value may still identify patients with irreversible RV dysfunction, thus hindering recovery. Our goal was to define, using intraoperative video kinematic evaluation, whether a relationship exists between timing of PVR and early functional recovery after surgery. METHODS Between November 2016 and November 2018, a total of 12 consecutive patients aged 27.1 ± 19.1 years underwent PVR on average 22.2 ± 13.3 years after tetralogy of Fallot repair. Mean RV end-diastolic volume evident on the magnetic resonance images was 136.9 ± 35.7 ml/m2. Intraoperative cardiac kinematics were assessed by video kinematic evaluation via a high-speed camera acquiring videos at 200 fps before and after valve replacement. RESULTS Patients presenting with RV end-diastolic volume <147 ml/m2 were significantly younger (11.2 ± 5.0 vs 38.4 ± 17.0; P  = 0.005) and had a shorter time interval to valve replacement (11.0 ± 5.2 vs 30.1 ± 11.3; P  = 0.03). The entire population showed a moderate correlation among energy expenditure, cardiac fatigue, perimeter of contraction and preoperative RV end-diastolic volume index. Both groups showed a reduction in all kinematic parameters after PVR, but those with end-diastolic volume >147 ml/m2 showed an unpredictable outcome. CONCLUSIONS Video kinematic evaluation provides insight into intraoperative RV recovery in patients with tetralogy of Fallot undergoing PVR. Accordingly, functional recovery can be expected in patients with preoperative end-diastolic volume <147 ml/m2. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Transcatheter pulmonary valve replacement has become a valid treatment option for right ventricular outflow tract diseases. However, some limitations occur in patients with wide, compliant right ventricular outflow tracts that might be amenable to treatment with self-expanding valved protheses. An experimental ovine study was designed to evaluate a novel dip-coated, low-profile trileaflet polycarbonate urethane (PCU) heart valve mounted into a self-expandable nitinol stent. METHODS The PCU valves were produced by a dip-coating technique, mounted in a conical-shaped nitinol stent and provided with a leaflet thickness of 100–150 µm. The valved stents were implanted percutaneously via transfemoral access in 6 consecutive sheep divided into 2 groups. Three animals were followed up for 1 month and the remainder, for 6 months. Angiographic measurements and transthoracic echocardiography were performed before and after implantation and at the end of the 1- or 6-month observation period, respectively. RESULTS Orthotopic positioning of the valve was achieved in all animals. All except 1 had competent valves during the follow-up period. The peak-to-peak gradient across the PCU valved stents was 4.6 ± 1.0 mmHg after 1 month and 4.4 ± 2.3 mmHg after 6 months of follow-up. Macroscopic and microscopic post-mortem evaluation indicated good morphological and structural results. There were no stent fractures, leaflet calcification or thrombus formation. CONCLUSIONS This study demonstrates successful transcatheter pulmonary valve replacement with a novel dip-coated valved nitinol stent. The trileaflet PCU prostheses indicated good functional and biocompatible properties after a 6-month observation period. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Hybrid approach to pulmonary valve replacement (PVR) in the paediatric population has been reported, although data in infants and small children are limited. Several strategies are now possible. The aim of this study is to review our hybrid PVR strategy in a complex patient cohort, outlining a variety of approaches employed in our centre. METHODS We performed a retrospective review of infants and small children who underwent hybrid PVR between May 2017 and April 2019 in a single tertiary cardiology centre. Medical records were reviewed to ascertain demographic, clinical and outcome data. RESULTS Ten patients with a median (interquartile range) age of 1.5 years (1.1–1.9) and weight of 8.8 kg (8–10.6) were managed with hybrid pulmonary valve insertion. Eight patients had perventricular approach (4 sternotomy and 4 subxiphoid) and 2 patients had surgically sutured valve. Six patients underwent cardiopulmonary bypass for associated lesions. Three had insertion of the valve into conduits and 7 were deployed into native right ventricular outflow tracts. The pulmonary valve was successfully inserted in all 10 patients with no mortality. Postprocedural complications included paravalvar leak in 2 patients, suspected endocarditis in 1 patient who developed early valve regurgitation and wound infection in 1 patient. CONCLUSIONS Several approaches to hybrid PVR may be employed in small children with a high success rate. Follow-up studies are required to evaluate longer term durability of these approaches compared to standard surgical replacement. [ABSTRACT FROM AUTHOR]

OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients. [ABSTRACT FROM AUTHOR]

Native pulmonary valve endocarditis is a rare phenomenon as native valve endocarditis tends to typically affect the left sided heart valves. However, the right-sided heart valves can be affected in patients with a history of intravenous drug use, whereby the tricuspid valve is most commonly affected. We present two cases who were diagnosed with native pulmonary valve endocarditis in the absence of congenital heart disease. In the first case, the native pulmonary valve endocarditis was probably a derivative of compounding factors of an enlarged underlying pulmonary artery and staphylococcal bacteraemia. In the second case, a common causal organism of native valve endocarditis following dental treatment and the resultant echocardiography findings was of significant interest. In summary, native pulmonary valve endocarditis is relatively rare complication in the adult population, especially in the absence of congenital heart disease. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Our goal was to compare results between a standard computed tomography (CT)-based strategy, the 'three-step preoperative sequential planning' (3-step PSP), for pulmonary valve replacement in repaired tetralogy of Fallot versus a conventional planning approach. METHODS We carried out a retrospective study with unmatched and matched groups. The 3-step PSP comprised the planning of mediastinal re-entry, cannulation for cardiopulmonary bypass (CPB) and the main procedure, using standard 3-dimensional videos. Operative times (skin incision to CPB, CPB time, end of CPB to skin closure and cross-clamp time) as well as postoperative length of stay and in-hospital mortality were compared. RESULTS Eighty-two patients (49% classical tetralogy of Fallot) underwent an operation (85% with pulmonary homograft) with 1.22% in-hospital mortality. The 3-step PSP (n  = 14) and the conventional planning (n  = 68) groups were compared. There were no statistically significant differences in the preoperative characteristics. Differences were observed in the total operative time (P  =   0.009), skin incision to CPB (P  =   0.034) and cross-clamp times (74 ± 33 vs 108 ± 47 min; P  =   0.006), favouring the 3-step PSP group. Eight matched pairs were compared showing differences in the total operative time (263 ± 44 vs 360 ± 66 min; P  =   0.008), CPB time (123 ± 34 vs 190 ± 43 min; P  =   0.008) and postoperative length of stay (P  =   0.031), favouring the 3-step PSP group. CONCLUSIONS In patients with repaired tetralogy of Fallot undergoing pulmonary valve replacement, preoperative planning using a standard CT-based strategy, the 3-step PSP, is associated with shorter operative times and shorter postoperative length of stay. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent. [ABSTRACT FROM AUTHOR]

OBJECTIVES Open in new tab Download slide Open in new tab Download slide Aortic stenosis or regurgitation that requires operations in children often results from unicuspid valve morphology. In all paediatric patients with this anomaly, we have performed unicuspid valve repair by bicuspidization, creating a new commissure via adding patch material. This study reviewed our experience with this procedure. METHODS All patients with a unicuspid aortic valve who underwent bicuspidization at ≤18 years of age between 2003 and 2018 were evaluated. Autologous pericardium had initially been used for cusp augmentation. Since 2014, decellularized xenogeneic tissue or expanded polytetrafluoroethylene membrane has been applied. RESULTS There were 60 consecutive patients. The median operative age was 13 (1–18) years. Thirty patients had prior surgical or catheter valvuloplasties. Aortic regurgitation, aortic stenosis and their combination were present in 22, 11 and 27 patients, respectively. Autologous pericardium decellularized tissue and expanded polytetrafluoroethylene were used in 45, 11 and 4 patients, respectively, without intraoperative conversion to valve replacement. The overall survival was 96% at 10 years with 2 late deaths. Twenty patients underwent aortic valve reoperation due to patch degeneration (n  = 11), suture dehiscence of the patch (n  = 3), subaortic stenosis (n  = 3) or other reasons (n  = 3). Freedom from aortic valve reoperation was 73% and 50% at 5 and 10 years, respectively. Fifteen patients (25%) required valve replacement with pulmonary autograft (n  = 14) or prosthesis (n  = 1) 5.2 (0.2–13) years after bicuspidization at the age of 19 (10–32) years. CONCLUSIONS Bicuspidization is a safe and reproducible alternative to valve replacement with acceptable freedom from reoperation. It can serve as a bridge to valve replacement providing superior outcomes in adults. [ABSTRACT FROM AUTHOR]

In the past decade, balloon-expandable percutaneous pulmonary valves have been developed and applied in clinical practice. However, all the existing products of pulmonary artery interventional valves in the market have a straight structure design, and they require a preset support frame and balloon expansion. This shape design of the valve limits the application range. In addition, the age of the population with pulmonary artery disease is generally low, and the existing products cannot meet the needs of anti-calcification properties and valve material durability. In this study, through optimization of the support frame and leaflet design, a self-expanding pulmonary valve product with a double bell-shaped frame was designed to improve the match of the valve and the implantation site. A loading and deployment study showed that the biomaterial of the valve was not damaged after being compressed. Pulsatile flow and fatigue in vitro tests showed that the fabricated pulmonary valve met the hydrodynamic requirements after 2 × 108 accelerated fatigue cycles. The safety and efficacy of the pulmonary valve product were demonstrated in studies of pulmonary valve implantation in 11 pigs. Angiography and echocardiography showed that the pulmonary valves were implanted in a good position, and they had normal closure and acceptable valvular regurgitation. The 180 days' implantation results showed that the calcium content was 0.31–1.39 mg/g in the anti-calcification treatment group, which was significantly lower than that in the control valve without anti-calcification treatment (16.69 mg/g). Our new interventional pulmonary valve product was ready for clinical trials and product registration. [ABSTRACT FROM AUTHOR]

Background  Isolated pulmonary valve endocarditis is a very rare form of right-sided infective endocarditis. Due to the anatomy, in most cases, just the tricuspid valve is involved. Diagnosis can be challenging because of non-specific symptoms (fever, dyspnoea, haemoptysis, and pleuritic chest pain) and difficulty of detection by echocardiography. Risk factors include intravenous drug abuse, congenital heart disorders, alcohol abuse, male sex and central venous catheters, or pacemaker leads. Case summary  A 39-year-old homeless male patient, who was a current intravenous drug user, presented with fever, dyspnoea, and haemoptysis. The chest X-ray showed bilateral infiltrates. Empiric antibiotic treatment was initiated. Blood cultures showed the presence of Streptococcus dysgalactiae. Atypical causes of pneumonia were excluded. Systemic embolism was suspected, and a computed tomography scan of brain, thorax, and abdomen was performed. Multiple septic embolic lesions were detected in both lungs. Echocardiography revealed an isolated pulmonary valve endocarditis. Penicillin G and gentamycin were administered intravenously for a duration of 6 and 2 weeks, respectively. The patient was discharged in stable condition but did not return for outpatient clinical appointments. Discussion  To detect rare causes of right-sided infective endocarditis, repeated echocardiograms with special focus on the pulmonary valve may be required. Usually, antibiotic treatment alone leads to recovery. In special situations (heart failure, septic shock, or large vegetation size) surgery is required. Due to the high risk of postoperative complications, surgery in intravenous drug users should be avoided if possible. [ABSTRACT FROM AUTHOR]

OBJECTIVES Open in new tab Download slide Open in new tab Download slide We aimed to investigate the safety, feasibility and early and mid-term outcomes of total repair of elective tetralogy of Fallot through a right subaxillary thoracotomy. METHODS Between May 2010 and September 2015, a retrospective review of patients with elective tetralogy of Fallot undergoing primary repair through a right subaxillary thoracotomy was performed. This study included 47 patients [27 males, 20 females; the mean age of 1.7 ± 1.5 years (ranging from 3 to 106 months); body weight of 9.7 ± 2.8 kg (ranging from 5 to 16 kg); McGoon ratio ≥1.5]. The ventricular septal defects were approached through a right atriotomy in 30 patients, a right ventriculotomy in 8 patients and the combination of a right atriotomy and ventriculotomy in 9 patients. Transannular and non-transannular patches were used in 20 and 27 patients, respectively. RESULTS There was 1 operative death (2.1%), and the others recovered uneventfully. The median cardiopulmonary bypass and aortic cross-clamping times was 79 ± 20.8 (45–136) and 50 ± 15.7 (25–94) min, respectively. The mechanical ventilation time, length of intensive care unit and postoperative hospital stay were 0.9 ± 0.6, 2.6 ± 1.1 and 10.7 ± 2.7 days, respectively. The main complications involved low cardiac output syndrome in 5 patients, arrhythmia in 6 patients, pulmonary infection in 4 patients, small residual shunt in 2 patients, mild residual right ventricular outflow tract obstruction in 2 patients, mild tricuspid regurgitation in 3 patients and atelectasis in 1 patient. The mean follow-up time was 82 ± 22.0 months, and no late deaths occurred during the follow-up. One (2.1%) patient received reintervention for residual significant pulmonary artery stenosis. That was in addition to 2 (4.3%) patients having mild residual right ventricular outflow tract, 1 (2.1%) patient with moderate tricuspid regurgitation and 3 (6.4%) patients with moderate pulmonary valve regurgitation to be followed up. CONCLUSIONS The right subaxillary thoracotomy, providing a safe and feasible alternative, can be performed with a low mortality rate and satisfactory early and mid-term outcomes for total repair of elective tetralogy of Fallot. [ABSTRACT FROM AUTHOR]

Background Pulmonary valve (PV) endocarditis is a frequent complication during follow-up in patients with repaired right ventricular outflow tract (RVOT) obstruction and poses relevant diagnostic and treatment challenges. We aimed to describe in details the possible different clinical presentations of this rare condition and to highlight the role of both transthoracic and transoesophageal echocardiography which, in experienced hands, may provide comprehensive useful information for the clinicians. Case summary We below describe the clinical presentation and the echo findings of three cases of pulmonary valve endocarditis complicating disease course after different repair modalities of congenital right ventricular outflow tract obstruction. Discussion The present case series outlines the diagnostic challenges of this increasingly frequent complication during follow-up of patients with congenital RVOT dysfunction after both surgical and percutaneous repair. Despite the diffusion of multimodality imaging, echocardiography with PV-dedicated views play a pivotal role in diagnosing such condition and guiding clinical management. Furthermore, this case series highlight that the suspicion of infective endocarditis should be raised whenever a sudden increase in transvalvular gradient is found during follow-up. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Many adults with repaired tetralogy of Fallot will require a pulmonary valve replacement (PVR), but there is no consensus on the best timing. In this study, we aim to evaluate the impact of age at PVR on outcomes. METHODS This is a national multicentre retrospective study including all patients >15 years of age with repaired tetralogy of Fallot who underwent their first PVR between 2000 and 2013. The optimal age cut-off was identified using Cox regression and classification and regression tree analysis. RESULTS A total of 707 patients were included, median age 26 (15–72) years. The mortality rate at 10 years after PVR was 4.2%, and the second PVR rate of 6.8%. Age at PVR of 35 years was identified as the optimal cut-off in relation to late mortality. Patients above 35 years of age had a 5.6 fold risk of death at 10 years compared with those with PVR under 35 years (10.4% vs 1.3%, P  < 0.001), more concomitant tricuspid valve repair/replacement (15.1% vs 5.7%, P  < 0.001) and surgical arrhythmia treatment (18.4% vs 5.9%, P  < 0.001). In those under 50 years, there was an 8.7 fold risk of late death compared with the general population, higher for those with PVR after 35 than those with PVR below 35 years (hazard ratio 9.9 vs 7.4). CONCLUSIONS Patients above 35 years of age with repaired tetralogy of Fallot have significantly worse mortality after PVR, compared with younger patients and a higher burden of mortality relative to the general population. This suggests that there are still cases where the timing of initial PVR is not optimal, warranting a re-evaluation of criteria for intervention. [ABSTRACT FROM AUTHOR]

OBJECTIVES Timing of pulmonary valve replacement (PVR) remains one of the most heavily debated topics in congenital cardiac surgery. We aimed to analyse the temporal evolution of QRS duration before and after PVR. METHODS We included 158 consecutive patients who underwent PVR after previous correction with transannular patch. All 3549 available serial standard 12-lead surface QRS measurements of 158 (100%) patients were analysed with linear mixed-effect modelling. RESULTS PVR was performed at a mean age of 28.0 ± 10.7 years, 23.4 ± 8.4 years after correction. Hospital survival was 98.1%. A longer time interval between ToF correction and PVR (P  < 0.001), and an older age at correction (P  = 0.015) were predictive of progressive QRS prolongation after PVR. Women on average had a shorter QRS duration (P  = 0.005) after PVR. The model predicted that in patients corrected early (model age 0.5 years), PVR within 17 years after correction leads to narrowing or stabilization of QRS width. PVR beyond 17 years was associated with prolongation of QRS duration. In a patient corrected late (model age 5 years), PVR has to be performed within 15 years after correction to prevent prolongation. Finally, a longer time period between correction and PVR was associated with an increased hazard of cardiac death (hazard ratio 1.097, 95% confidence interval 1.002–1.200). CONCLUSIONS Prolongation of QRS duration after PVR was associated with a longer time between correction and PVR, older age at correction and male sex. Prevention of progressive QRS prolongation by earlier PVR can potentially reduce the hazard of adverse events after PVR. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Our goal was to report the long-term serial follow-up after transatrial–transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome. METHODS We included all patients with TOF who had undergone transatrial–transpulmonary repair between 1970 and 2012. Records were reviewed for patient demographics, operative details and events during the follow-up period (death, pulmonary valve replacement, cardiac reinterventions and hospitalization/intervention for arrhythmias). In patients with elective early primary repair of TOF after 1990, a subanalysis of the optimal timing of TOF repair was performed. RESULTS A total of 453 patients were included (63% male patients; 65% had transannular patch); 261 patients underwent primary elective repair after 1990. The median age at TOF repair was 0.7 years (25th–75th percentile 0.3–1.3) and decreased from 1.7 to 0.4 years from before 1990 to after 2000, respectively (P  < 0.001). The median follow-up duration after TOF repair was 16.8 years (9.6–24.7). Events developed in 182 (40%) patients. In multivariable analysis, early repair of TOF (<6 months) [hazard ratio (HR) 3.06; P  < 0.001] and complications after TOF repair (HR 2.18; P  = 0.006) were found to be predictive for an event. In a subanalysis of the primary repair of TOF after 1990, the patients (n  = 125) with elective early repair (<6 months) experienced significantly worse event-free survival compared to patients who had elective repair later (n  = 136). In multivariable analysis, early repair (HR 3.00; P  = 0.001) and postoperative complications (HR 2.12; P  = 0.010) were associated with events in electively repaired patients with TOF. CONCLUSIONS Transatrial–transpulmonary repair of TOF before the age of 6 months may be associated with more events during the long-term follow-up period. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique. METHODS This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV. RESULTS We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7–11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1–21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P  = 0.006) and PV preservation was a significant protective factor against postoperative complications (P  = 0.009, odds ratio 0.5) and late adverse events (P  = 0.022). CONCLUSIONS Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity. [ABSTRACT FROM AUTHOR]