PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Long-term follow-up after endovascular treatment of aortic coarctation with bare and covered Cheatham platinum stents.

BACKGROUND: Endovascular treatment of aortic coarctation (CoA) in children and adults frequently requires stent implantation. The aim of this study was to analyze long-term results after CoA treatment with bare and covered Cheatham-PlatinumTM (CP) stents in our institution and to derive recommendations for the differential use of these stent types. METHODS: In this retrospective single institution study, 212 patients received endovascular CoA treatment with bare (n = 71) and covered (n = 141) CP stents between September 1999 and July 2021, respectively. The indications for treatment were native CoA in 110/212 patients (51.9%) and re-coarctation after primary surgical or interventional treatment in 102/212 patients (48.1%). Median patient age at endovascular CoA treatment was 18.8 years [IQR 11.9; 35.8]. Long-term follow-up was available in 158/212 patients (74.5%) with a median follow-up of 7.3 years [IQR 4.3; 12.6]. RESULTS: Procedural success was achieved in 187/212 (88.2%) patients. Survival rate was 98.1% after 5, and 95.6% after 10 and 15 years, respectively. The probability of freedom from re-intervention was 93.0% after 5, 82.3% after 10 and 77.8% after 15 years, respectively. Freedom from re-interventions (44/158, 27.8%) did not differ between patients who received bare or covered CP stents (p = 0.715). Multivariable risk factor analysis identified previous CoA surgery (HR: 2.0, 95% confidence interval (CI): 1.1-3,9, p = 0.029), postdilatation (HR: 2,9, 95% CI: 1.1-6.3, p = 0.028) and age at intervention (HR: 0.96, 95% CI: 0.94-0.99, p = 0.002) as independent risk factors for re-intervention. Peri-procedural complications occurred in 15/212 (7.1%) patients (dissection/thrombosis of vascular access vessel: n = 9; bleeding: n = 1; stent dislocation: n = 2; aortic dissection/aortic wall rupture: n = 3). Long-term complications were observed in 36 patients and included stent fracture (n = 19), aneurysm formation (n = 14), endoleak (n = 1) and subclavian artery stenosis (n = 2). Peri-procedural and long-term complications did not differ between patients who received CoA treatment with bare or covered CP stents (all p > 0.05). CONCLUSION: Endovascular treatment of CoA using bare or covered CP stents can be performed safely and effectively with excellent long-term results. Survival, re-intervention and complication rate did not significantly differ between both stent types. However, individual stent selection is advisable with regard to CoA morphology and severity as well as patient age.

Left atrial disc device thrombosis after atrial defect closure with the Gore® Cardioform septal occluder: A case series.

Device thromboses after patent foramen ovale (PFO) and atrial septal defect (ASD) closure are rare but serious complications. They have been reported in different devices of virtually all manufacturers. Here, we report three cases of left atrial device thrombosis after atrial defect closure with the Gore® Cardioform septal occluder (GSO) in our recent institutional experience. All patients were symptomatic with new-onset neurological impairments and evidence of cerebral thromboembolism. Device thromboses have occurred despite antiplatelet therapy in two patients and moreover were observed late, at approximately 2 years after implantation, in two patients. One device was surgically explanted while in two cases, thrombi completely resolved with initiated anticoagulation. All patients had a favorable neurological recovery. Our observations suggest that regular follow-up echocardiography beyond 6 months after device implantation might be advisable in patients with GSO devices to rule out late device thromboses. More long-term follow-up data considering safety and late complications of contemporary PFO and ASD devices is required to establish confident evidence-based recommendations on long-term follow-up management and antithrombotic therapy regimen after PFO and ASD closure.

Serum dihydrotestosterone levels are associated with adverse myocardial remodeling in patients with severe aortic valve stenosis before and after aortic valve replacement.

Animal studies show a pivotal role of dihydrotestosterone (DHT) in pressure overload-induced myocardial hypertrophy and dysfunction. The aim of our study was to evaluate the role of DHT levels and myocardial hypertrophy and myocardial protein expression in patients with severe aortic valve stenosis (AS). Forty-three patients [median age 68 (41-80) yr] with severe AS and indication for surgical aortic valve replacement (SAVR) were prospectively enrolled. Cardiac magnetic resonance imaging including analysis of left ventricular muscle mass (LVM), fibrosis and function, and laboratory tests including serum DHT levels were performed before and after SAVR. During SAVR, left ventricular (LV) biopsies were performed for proteomic profiling. Serum DHT levels correlated positively with indexed LVM (LVMi, R = 0.64, P = 0.0001) and fibrosis (R = 0.49, P = 0.0065) and inversely with LV function (R = -0.42, P = 0.005) in patients with severe AS. DHT levels were associated with higher abundance of the hypertrophy (moesin, R = 0.52, P = 0.0083)- and fibrosis (vimentin, R = 0.41, P = 0.039)-associated proteins from LV myocardial biopsies. Higher serum DHT levels preoperatively were associated with reduced LV function (ejection fraction, R = -0.34, P = 0.035; circulatory efficiency, R = -0.46, P = 0.012; and global longitudinal strain, R = 0.49, P = 0.01) and increased fibrosis (R = 0.55, P = 0.0022) after SAVR. Serum DHT levels were associated with adverse myocardial remodeling and higher abundance in hypertrophy- and fibrosis-associated proteins in patients with severe AS. DHT may be a target to prevent or attenuate adverse myocardial remodeling in patients with pressure overload due to AS.NEW & NOTEWORTHY Serum dihydrotestosterone (DHT) levels correlated positively with the degree of hypertrophy, fibrosis, and dysfunction from cardiac magnetic resonance imaging in female and male patients with aortic valve stenosis. Left ventricular proteome profiling had been performed in this patient cohort and an association between serum DHT levels and the abundance of the hypertrophy-associated protein moesin and the fibrosis-associated protein vimentin was found.

Transcatheter Closure of Atrial Septal Defect with Carag Bioresorbable Septal Occluder™: First-in-Child Experience with 12-MonthFollow-Up.

Background: Nowadays, transcatheter device closure of an atrial septal defect (ASD) is a standard approach in children. Potential early and long-term side effects or complications related to the metal framework of the devices are a known issue. A bioresorbable device such as the Carag Bioresorbable Septal Occluder™ (CBSO) could resolve such complications. Material and Results. The Carag Bioresorbable Septal Occluder™ (CBSO; Carag AG, Baar, Switzerland) is a self-centering double disk, repositionable, and retractable device with a bioresorbable framework (polylactic-co-glycolic acid), which is almost completely resorbed by 18-24 months postimplantation. This manuscript reports the four first-in-child ASD device closures using a CBSO. The patients' age was median (IQ1-IQ3), 4.5 years (4-7.25). Weight was 21.3 kg (17.6-32.7). We demonstrated procedural feasibility and safety. Effective defect closure with the device was 100%. Echocardiographic measurements of the thickness of the interatrial septum did not show any relevant increase over a 12-monthfollow-up period. There were no residual defects found after the procedure or later during the resorption process. The patients showed no evidence of any local or systemic inflammatory reaction. Conclusions: The CBSO device system could offer a new treatment option for transcatheter ASD device closure in the pediatric and adult fields. In our first-in-child experience, it was effectively and safely implanted. During the first 12 months of follow-up, no complications occurred.

Coronary Interventions in Pediatric Congenital Heart Disease.

Coronary artery lesions represent rare conditions in pediatric congenital heart disease and mainly include coronary artery stenoses (CAS) or coronary artery fistulae (CAF). Due to the small vessel size, pediatric percutaneous coronary interventions (PCI) are demanding and studies concerning long-term results are missing. In this retrospective study, we analyzed indications, procedural details, and post-procedural outcomes in pediatric patients who underwent PCI in our institution. For CAS treatment, procedural success was defined as efficient coronary revascularization with a significant improvement of coronary perfusion. CAF treatment was considered successful, when no residual shunt was detectable. From 1995 to 2020, 32 pediatric patients aged ≤ 18 years received interventional treatment for CAS (n = 24/32) or CAF (n = 8/32). Reasons for CAS were post-surgical (n = 15/24) or post-transplant (n = 9/24). Interventional treatment strategies included coronary angioplasty (20/43), stent placement (10/43), and a combination of both (13/43). In-hospital mortality occurred in 6/24 patients and late mortality in 5/24 patients leading to an overall 5-year survival of 62.5%. Early mortality mainly occurred due to post-ischemic myocardial failure. CAF occlusion was performed using coil embolization (n = 3), placement of vascular plugs (n = 3), a combination of both (n = 1), or a combination of coil embolization and a covered stent (n = 1). Treatment of coronary fistulae was successful in all patients with excellent post-procedural results and no follow-up death. PCI in pediatric patients with congenital heart disease can be performed safely and effectively. However, the overall 5-year survival probability of patients with CAS is reduced due to severe ischemic myocardial damage.

Long-term experience using CNI-free immunosuppression in selected paediatric heart transplant recipients.

BACKGROUND: CNI-free immunosuppression with conversion to mTORi-based immunosuppression has been demonstrated to reduce CNI-toxicity and to exhibit anti-proliferative properties. However, the experience of CNI-free immunosuppression in paediatric heart transplantation is limited. METHODS: A retrospective analysis was conducted of 129 paediatric heart transplants performed between 1997 and 2015. Fifteen patients with clinically indicated conversion from CNI-based to CNI-free immunosuppression were identified. Survival data, rejection episodes, renal function, post-transplantation lymphoproliferative disorder and CAV, including examination with OCT were analysed. RESULTS: Immunosuppression conversion was successful in all patients. Fourteen of 15 patients (93%) are currently living with good graft function. Median post-transplant survival was 15 years (range, 5-23 years), and median follow-up since conversion was 6 years (range, 1-11 years). Mild (grade 1R) ACR was present in three patients after discontinuation of CNIs. The recovery of renal function with a significant increase in eGFR was observed at 1 and 3 years after conversion. No patient had angiographic signs of macroscopic CAV according to the current ISHLT classification; however, OCT showed the signs of angiographically silent CAV in all patients. CAV did not progress in any patient, implying CAV was stabilised by mTORi-based CNI-free immunosuppression. CONCLUSIONS: CNI-free immunosuppression based on mTORis is a safe and appropriate strategy for maintenance therapy in selected paediatric patients, significantly improves renal function and stabilises CAV. OCT revealed early development of angiographically silent CAV.

Acute and midterm outcomes of the post-approval MELODY Registry: a multicentre registry of transcatheter pulmonary valve implantation.

AIMS: The post-approval MELODY Registry aimed to obtain multicentre registry data after transcatheter pulmonary valve implantation (TPVI) with the Melody™ valve (Medtronic plc.) in a large-scale cohort of patients with congenital heart disease (CHD). METHODS AND RESULTS: Retrospective analysis of multicentre registry data after TPVI with the Melody™ valve. Eight hundred and forty-five patients (mean age: 21.0 ± 11.1 years) underwent TPVI in 42 centres between December 2006 and September 2013 and were followed-up for a median of 5.9 years (range: 0-11.0 years). The composite endpoint of TPVI-related events during follow-up (i.e. death, reoperation, or reintervention >48 h after TPVI) showed an incidence rate of 4.2% per person per year [95% confidence interval (CI) 3.7-4.9]. Transcatheter pulmonary valve implantation infective endocarditis (I.E.) showed an incidence rate of 2.3% per person per year (95% CI 1.9-2.8) and resulted in significant morbidity and in nine deaths. In multivariable Cox proportional hazard models, the invasively measured residual right ventricle (RV)-to-pulmonary artery (PA) pressure gradient (per 5 mmHg) was associated with the risk of the composite endpoint (adjusted hazard ratio: 1.21, 95% CI 1.12-1.30; P < 0.0001) and the risk of TPVI I.E. (adjusted hazard ratio: 1.19, 95% CI 1.07-1.32; P = 0.002). Major procedural complications (death, surgical, or interventional treatment requirement) occurred in 0.5%, 1.2%, and 2.0%, respectively. Acutely, the RV-to-PA pressure gradient and the percentage of patients with pulmonary regurgitation grade >2 improved significantly from 36 [interquartile range (IQR) 24-47] to 12 (IQR 7-17) mmHg and 47 to 1%, respectively (P < 0.001 for each). CONCLUSION: The post-approval MELODY Registry confirms the efficacy of TPVI with the Melody™ valve in a large-scale cohort of CHD patients. The residual invasively measured RV-to-PA pressure gradient may serve as a target for further improvement in the composite endpoint and TPVI I.E. However, TPVI I.E. remains a significant concern causing significant morbidity and mortality.

Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period.

OBJECTIVES: The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season. METHODS: We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October). RESULTS: Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06). CONCLUSION: Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

Successful exclusion of an aortic aneurysm with a novel PTFE-tube covered cobalt-chromium stent in a pediatric patient with native coarctation of the aorta.

Treatment of aneurysmal lesions in the context of coarctation of the aorta (CoA) is a challenging task and these lesions are rare in children. An 11-year-old boy was incidentally diagnosed with native CoA and concomitant complex aneurysmal lesions during medical check-up for arterial hypertension. Pre-catheterization imaging was performed with computed tomography (CT), which showed a mild CoA and two native aneurysms that were juxtaposed to the origin of the left subclavian artery. For planning and guidance of the catheter procedure, image fusion software was used with an overlay from pre-registered three-dimensional reconstruction images on live fluoroscopy. Here, we report the first case of successful treatment of an aneurysmal lesion in the context of native coarctation with a novel PTFE-tube covered cobalt-chromium stent (BeGraft, Bentley) in a pediatric patient.

Presence of reduced regional left ventricular function even in the absence of left ventricular wall scar tissue in the long term after repair of an anomalous left coronary artery from the pulmonary artery.

BACKGROUND: We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery. METHODS: A total of 20 patients aged 12.8±7.4 years were assessed 10 (0.5-17) years after the repair of an anomalous origin of the left coronary artery from the pulmonary artery; of them, 10 (50%) patients showed left ventricular wall scar tissue on current cardiac MRI. Left ventricular regional function was assessed by two-dimensional speckle-tracking echocardiography in 10 patients with scar tissue and 10 patients without scar tissue and in 10 age-matched controls. RESULTS: In patients with scar tissue, MRI-derived left ventricular ejection fraction was significantly reduced compared with that in patients without scar tissue (51 versus 61%, p<0.05), and echocardiography-derived longitudinal strain was significantly reduced in five of six left ventricular areas compared with that in healthy controls (average relative reduction, 46%; p<0.05). In patients without scar tissue, longitudinal strain was significantly reduced in two of six left ventricular areas (average relative reduction, 23%; p<0.05) and circumferential strain was reduced in one of six left ventricular areas (relative reduction, 56%; p<0.05) compared with that in healthy controls. CONCLUSIONS: Regional left ventricular function is reduced even in patients without left ventricular wall scar tissue late after successful repair of an anomalous origin of the left coronary artery from the pulmonary artery. This highlights the need for meticulous lifelong follow-up in all patients with a repaired anomalous origin of the left coronary artery from the pulmonary artery.

Four-dimensional velocity-encoded magnetic resonance imaging improves blood flow quantification in patients with complex accelerated flow.

PURPOSE: To evaluate the use of four-dimensional (4D) velocity-encoded magnetic resonance imaging (VEC MRI) for blood flow quantification in patients with semilunar valve stenosis and complex accelerated flow. MATERIALS AND METHODS: Peak velocities (Vmax) and stroke volumes (SV) were quantified by 2D and 4D VEC MRI in volunteers (n = 7) and patients with semilunar valve stenosis (n = 18). Measurements were performed above the aortic and pulmonary valve with both techniques and, additionally, at multiple predefined planes in the ascending aorta and in the pulmonary trunk within the 4D dataset. In patients, 4D VEC MRI streamline analysis identified flow patterns and regions of highest flow velocity (4D(max-targeted)) for further measurements and Vmax was also measured by Doppler-echocardiography. RESULTS: In patients, 4D VEC MRI showed higher Vmax than 2D VEC MRI (2.7 ± 0.6 m/s vs. 2.4 ± 0.5 m/s, P < 0.03) and was more comparable to Doppler-echocardiography (2.8 ± 0.7 m/s). 4D(max-targeted) revealed highest Vmax values (3.1 ± 0.6 m/s). SV measurements showed significant differences between different anatomical levels in the ascending aorta in patients with complex accelerated flow, whereas differences in volunteers with laminar flow patterns were negligible (P = 0.004). CONCLUSION: 4D VEC MRI improves MRI-derived blood flow quantification in patients with semilunar valve stenosis and complex accelerated flow.

Impact of reduction in right ventricular pressure and/or volume overload by percutaneous pulmonary valve implantation on biventricular response to exercise: an exercise stress real-time CMR study.

AIMS: To assess the impact of relief of pulmonary stenosis (PS) and pulmonary regurgitation (PR) by percutaneous pulmonary valve implantation (PPVI) on biventricular function during exercise stress. METHODS AND RESULTS: Seventeen patients, who underwent PPVI for PS or PR, were included. Magnetic resonance imaging was performed at rest and during supine exercise stress pre- and within 1-month post-PPVI, using a radial k - t SENSE real-time sequence. In patients with PS (n = 9), there was no reserve in right ventricular (RV) ejection fraction (EF) in response to exercise prior to PPVI (48.2 ± 12.1% at rest vs. 48.4 ± 14.8% during exercise, P = 0.87). Post-PPVI, reserve in RVEF in response to exercise was re-established (53.4 ± 15.0% at rest vs. 59.6 ± 17.3% during exercise, P = 0.003) with improvement in left ventricular stroke volume (LVSV) (45.4 ± 6.2 mL/m(2) at rest vs. 52.8 ± 8.8 mL/m(2) during exercise, P = 0.001). In patients with PR prior to PPVI (n = 8), LVSV during exercise increased (43.0 ± 8.5 vs. 54.3 ± 6.6 mL/m(2), P < 0.001) due to reduction in PR fraction during exercise (29.2 ± 5.2 vs. 13.6 ± 6.1%, P < 0.001). After PPVI, LVSV increased from rest to exercise (48.4 ± 8.8 vs. 57.2 ± 8.1 mL/m(2), P < 0.001) due to improved RVEF (45.5 ± 8.3 vs. 50.4 ± 6.9%, P = 0.001). There was a significantly higher increase in LVSV at exercise from pre- to post-PPVI in PS patients than in PR patients (ΔLVSV 8.2 ± 4.1 vs. Δ2.9 ± 4.1 mL/m(2), P = 0.01). The reduction in the RV outflow tract gradient correlated significantly with the improvement in LVSV during exercise (r = -0.73, P < 0.001). CONCLUSION: Percutaneous pulmonary valve implantation in patients with PS leads to restoration of reserve in RVEF during exercise stress. In patients with PR, SV augmentation improves only mildly post-PPVI. Improvement in SV augmentation during exercise stress after PPVI is dependent mainly on afterload reduction.

Modified approach for transcatheter correction of superior sinus venosus atrial septal defect: a case series.

Background: Transcatheter correction of superior sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous drainage (PAPVD) of the right upper and/or right middle pulmonary vein (RUPV/RMPV) has recently been described as an alternative to surgical approach in a substantial number of patients. We describe a modified technical approach for transcatheter correction of SVASD using transoesophageal echocardiography (TOE) to confirm adequate stent landing zone and apposition and evaluate its feasibility, safety, and procedural success. Case summary: From 2019 to 2021, three consecutive patients received a transcatheter correction of SVASD with PAPVD by redirecting the superior vena cava and RUPV/RMPV to the left atrium by implantation of a custom-made covered Cheatham platinum stent (10-zig, length: 60-80 mm). Prior to stent implantation, a balloon testing was performed in the anticipated landing zone using TOE to confirm complete defect closure and unobstructed pulmonary venous drainage. Stent deployment and flaring of the interior stent portion were performed with TOE guidance to confirm adequate landing zone and apposition and to avoid residual shunt or pulmonary vein obstruction. Conclusion: Transcatheter correction of SVASD with PAPVD was performed without any complications. The follow-up period was 7.8, 13.6, and 29.8 months, respectively. During follow-up, no mortality, stent embolization, or obstruction of pulmonary venous drainage occurred. The TOE-guided modified transcatheter approach for correction of SVASD with PAPVD is safe and feasible with excellent post-procedural results and represents an alternative to surgical treatment in a pre-selected patient cohort.

A Multimodal Score Accurately Classifies Fontan Failure and Late Mortality in Adult Fontan Patients.

Objectives: Despite the outstanding success of the Fontan operation, it is a palliative procedure and a substantial number of patients experience late failure of the Fontan circulation. Clinical presentation and hemodynamic phenotypes of Fontan failure are considerably variable. While various parameters have been identified as risk factors for late Fontan failure, a feasible score to classify Fontan failure and possibly allow timely risk stratification is lacking. Here, we explored the possibility of developing a score based on hemodynamic, clinical and laboratory parameters to classify Fontan failure and mortality. Methods: We performed a retrospective study in our cohort of adult Fontan patients from two institutions [n = 198, median follow-up after Fontan 20.3 (IQR 15.6-24.3) years], identifying those patients with clinical Fontan failure (n = 52, 26.3%). Various hemodynamic, echocardiographic, laboratory and clinical data were recorded and differences between patients with and without Fontan failure were analyzed. We composed a Fontan Failure Score containing 15 parameters associated with Fontan failure and/or mortality and assessed its accuracy to discriminate between patients with and without late Fontan failure as well as late mortality and survival. Results: Late failure occurred at a median of 18.2 (IQR 9.1-21.1) years after Fontan completion. Mortality associated with Fontan failure was substantial (25/52, 48.1%) with freedom of death/transplantation/take-down of 64% at 5 years and 36% at 10 years after onset of Fontan failure, respectively. Patients with Fontan failure had a significantly higher median Fontan Failure Score compared to non-failing Fontan patients [8 points (IQR 5-10) vs. 2 points (IQR 1-5), p < 0.001]. The score accurately classifies Fontan failure as well as mortality as assessed with receiver operating characteristic analysis. Area under the curve of the Fontan Failure Score was 0.963 (95% CI 0.921; 0.985, p < 0.001) to discriminate failure and 0.916 (95% CI 0.873; 0.959, p < 0.001) to classify mortality. Conclusion: We have developed an uncomplex yet remarkably accurate score to classify Fontan failure and late mortality in adult Fontan patients. Prospective validation and most likely refinement and calibration of the score in larger and preferably multi-institutional cohorts is required to assess its potential to predict the risk of Fontan failure and late mortality.

Revascularization of Left Subclavian to Common Carotid Artery Prepares for Covered Stent Implantation in Patients With Complex Aortic Coarctation.

Seven adult patients underwent a two-stage treatment of complex coarctation (CoA), including surgical revascularization of the left subclavian artery (LSA) to left common carotid artery (LCCA), followed by transcatheter covered stent implantation. The majority of patients (5 of 7, 71%) received 1 covered stent (covered Cheatham Platinum stent: 8 zig/45 mm [n = 2], 10 zig/60 mm [n = 1], 10 zig/65 mm [n = 1]; BeGraft: 24/48 mm [n = 2]). In 1 patient (14%), the implantation of 2 covered stents (BeGraft 20/48 mm) was necessary. During a median follow-up of 2.4 years (interquartile range, 0.1 to 4.9 years), complications occurred in 3 of 7 patients (43%), including an asymptomatic but severe stenosis of the LSA bypass (n = 1), a recoarctation with a mild endoleak (n = 1), and a severe endoleak (n = 1). Surgical revascularization of the LSA to the LCCA can successfully prepare for covered stent implantation in complex CoA in adult patients. This two-stage approach was feasible and safe with complications occurring in 3 of 7 patients (43%). All complications were managed by catheter reintervention only.

Nationwide Registry-Based Analysis of Infective Endocarditis Risk After Pulmonary Valve Replacement.

Background Infective endocarditis (IE) after pulmonary valve replacements in congenital heart disease is a significant concern. This study aimed to identify specific long-term risk factors for IE after percutaneous pulmonary valve implantation or surgical pulmonary valve replacement. Methods and Results All patients with congenital heart disease from the National Register for Congenital Heart Defects with at least 1 pulmonary valve replacement before January 2018 were included. A total of 1170 patients (56.3% men, median age at study inclusion 12 [interquartile range {Q1-Q3} 5-20 years]) received 1598 pulmonary valve replacements. IE occurred in 4.8% of patients during a follow-up of total 9397 patient-years (median 10 [Q1-Q3, 6-10] years per patient). After homograft implantation 7 of 558 (1.3%) patients developed IE, after heterograft implantation 31 of 723 (4.3%) patients, and after Melody valve implantation 18 of 241 (7.5%) patients. Edwards Sapien and mechanical valves were used less frequently and remained without IE. The incidence of IE in heterografts excluding Contegra valves was 7 of 278 (2.5%), whereas the incidence of IE in Contegra valves was 24 of 445 (5.4%). The risk of IE was not increased compared with homografts if Contegra valves were excluded from the heterografts (hazard ratio [HR], 2.60; P=0.075). The risk of IE was increased for bovine jugular vein valves, Contegra valves (HR, 6.72; P<0.001), and Melody valves (HR, 5.49; P<0.001), but did not differ between Melody valves and Contegra valves (HR, 1.01; P=0.978). Conclusions Bovine jugular vein valves have the highest risk of IE, irrespective of the mode of deployment, either surgical or percutaneous.

Four-dimensional computed tomography: a method of assessing right ventricular outflow tract and pulmonary artery deformations throughout the cardiac cycle.

OBJECTIVE: To characterise 3D deformations of the right ventricular outflow tract (RVOT)/pulmonary arteries (PAs) during the cardiac cycle and estimate the errors of conventional 2D assessments. METHODS: Contrast-enhanced, ECG-gated cardiovascular computed tomography (CT) findings were retrospectively analysed from 12 patients. The acquisition of 3D images over 10 phases of the cardiac cycle created a four-dimensional CT (4DCT) dataset. The datasets were reconstructed and deformation measured at various levels of the RVOT/PAs in both space and time. Section planes were either static or dynamic relative to the motion of the structures. RESULTS: 4DCT enabled measurement and characterisation of in vivo 3D changes of patients' RVOT/PA during the cardiac cycle. The studied patient population showed a wide range of RVOT/PA morphologies, sizes and dynamics that develop late after surgical repair of congenital heart disease. There were also significant differences in the measured cross-sectional areas of the structures between static and dynamic section planes (up to 150%, p<0.05) secondary to large 3D displacements and rotations. CONCLUSIONS: 4DCT imaging data suggest high variability in RVOT/PA dynamics and significant errors in deformation measurements if 3D analysis is not carried out. These findings play an important role for the development of novel percutaneous approaches to pulmonary valve intervention.

Persisting and reoccurring cyanosis after Fontan operation is associated with increased late mortality.

OBJECTIVES: The Fontan operation aims at reducing cyanosis and cardiac volume overload in patients with complex univentricular heart malformations. However, persisting or reoccurring cyanosis is frequently observed. We sought to systematically determine the prevalence and clinical consequences of persisting and secondary cyanosis after Fontan operation. METHODS: A total of 331 Fontan patients, operated between 1984 and 2016 with a median postoperative follow-up of 7.9 (interquartile range 2.6-15.8) years, were studied retrospectively. Cyanosis was defined as transcutaneous oxygen saturation ≤93% at rest measured by pulse oximetry. Prevalence of cyanosis was analysed at 3 different time points (t1 = post-Fontan operation, t2 = post-Fontan cardiac catheterization, t3 = last follow-up) and the association of cyanosis with mortality was examined. RESULTS: Prevalence of cyanosis was 50% at t1 and 39% at t3. Fenestration was patent in 71% and 33% of all cyanotic patients at t1 and t3, respectively. In patients with clinical indication for catheterization (t2; n = 178/331), prevalence of cyanosis was 72%. At t2, patent fenestration (33%), veno-venous collaterals (24%) or both (32%) were present. Thirty-six (11%) patients died during follow-up. In a time-varying multivariable Cox regression analysis, cyanosis was the strongest predictor for late mortality (P < 0.001, hazard ratio 12.2, 95% confidence interval 3.7-40.5). CONCLUSIONS: Prevalence of cyanosis was considerable during long-term follow-up after Fontan operation and-as a surrogate parameter for unfavourable Fontan haemodynamics-is associated with increased late mortality. Accordingly, particular attention should be directed towards the persistence or reoccurrence of cyanosis during follow-up since it may indicate haemodynamic attrition and development of Fontan failure.