Ortner's syndrome due to aneurysmal dilatation of the left pulmonary artery secondary to mixed pulmonary valve disease.

A young female patient was referred by her general practitioner to the ear, nose and throat outpatient clinic with a 5-year history of progressive hoarseness and loss of voice. A chest radiograph was reported as showing marked dilatation of the left pulmonary artery without any other features to suggest underlying pulmonary arterial hypertension. A provisional diagnosis of left recurrent laryngeal nerve palsy secondary to its compression between the left pulmonary artery and the undersurface of the aortic arch-Ortner's or cardiovocal syndrome-was suggested and confirmed on further investigation including CT, microlaryngoscopy and echocardiography.

Structural Degeneration of a 9-Year-Old Tendyne: Treatment by Transfemoral Transseptal SAPIEN in Tendyne.

An 83-year-old woman presented 9 years after Tendyne transcatheter mitral valve replacement with acute severe decompensated heart failure. Transesophageal echocardiography reported severe transvalvular mitral regurgitation caused by degenerated Tendyne leaflets. A transfemoral transseptal valve-in-Tendyne procedure using a 26-mm SAPIEN device resulted in significant mitral regurgitation reduction and symptomatic recovery.

Percutaneous Closure of an Iatrogenic Intracardiac Shunt in Treating Torrential Tricuspid Regurgitation.

A patient presented with severe right heart failure due to a large LV-to-RA shunt with left-to-right shunting and torrential tricuspid regurgitation 6-weeks following surgical sub-aortic stenosis resection. Retrograde delivery of an Occlutech ventricular septal defect device produced instantaneous resolution of shunt, reduction in tricuspid regurgitation, and impressive diuresis of 28 kg.

Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows:While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.

Prognostic Value of Cardiac Magnetic Resonance Feature Tracking Strain in Aortic Stenosis.

BACKGROUND: Recent data have suggested that global longitudinal strain (GLS) could be useful for risk stratification of patients with severe aortic stenosis (AS). In this study, we aimed to investigate the prognostic role of GLS in patients with AS and also its incremental value in relation to left ventricular ejection fraction (LVEF) and late gadolinium enhancement (LGE). METHODS: We analysed all consecutive patients with AS and LGE-CMR in our institution. Survival data were obtained from office of national statistics, a national body where all deaths in England are registered by law. Death certificates were obtained from the general register office. RESULTS: Some 194 consecutive patients with aortic stenosis were investigated with CMR at baseline and followed up for 7.3 ± 4 years. On multivariate Cox regression analysis, only increasing age remained significant for both all-cause and cardiac mortality, while LGE (any pattern) retained significance for all-cause mortality and had a trend to significance for cardiac mortality. Kaplan-Meier survival analysis demonstrated that patients in the best and middle GLS tertiles had significantly better mortality compared to patients in the worst GLS tertiles. Importantly though, sequential Cox proportional-hazard analysis demonstrated that GLS did not have significant incremental prognostic value for all-cause mortality or cardiac mortality in addition to LVEF and LGE. CONCLUSIONS: Our study has demonstrated that age and LGE but not GLS are significant poor prognostic indicators in patients with moderate and severe AS.

Concomitant Transapical Aortic/Mitral Transcatheter Valve Replacement for Severe Aortic Stenosis and Mitral Annular Calcification.

Mitral regurgitation (MR) associated with mitral annular calcification (MAC) is surgically challenging, and valve-in-MAC procedures using transcatheter aortic valve replacement (TAVR) devices have poor outcomes. Transcatheter mitral valve replacement (TMVR) may be an option. Concomitant TAVR and TMVR are limited to 2 reports. We describe the first case of concomitant TAVR and TMVR-in-MAC procedures. (Level of Difficulty: Advanced.).

Time to TAVI: streamlining the pathway to treatment.

INTRODUCTION: Severe aortic stenosis is a major cause of morbidity and mortality. The existing treatment pathway for transcatheter aortic valve implantation (TAVI) traditionally relies on tertiary Heart Valve Centre workup. However, this has been associated with delays to treatment, in breach of British Cardiovascular Intervention Society targets. A novel pathway with emphasis on comprehensive patient workup at a local centre, alongside close collaboration with a Heart Valve Centre, may help reduce the time to TAVI. METHODS: The centre performing local workup implemented a novel TAVI referral pathway. Data were collected retrospectively for all outpatients referred for consideration of TAVI to a Heart Valve Centre from November 2020 to November 2021. The main outcome of time to TAVI was calculated as the time from Heart Valve Centre referral to TAVI, or alternative intervention, expressed in days. For the centre performing local workup, referral was defined as the date of multidisciplinary team discussion. For this centre, a total pathway time from echocardiographic diagnosis to TAVI was also evaluated. A secondary outcome of the proportion of referrals proceeding to TAVI at the Heart Valve Centre was analysed. RESULTS: Mean±SD time from referral to TAVI was significantly lower at the centre performing local workup, when compared with centres with traditional referral pathways (32.4±64 to 126±257 days, p<0.00001). The total pathway time from echocardiographic diagnosis to TAVI for the centre performing local workup was 89.9±67.6 days, which was also significantly shorter than referral to TAVI time from all other centres (p<0.003). Centres without local workup had a significantly lower percentage of patients accepted for TAVI (49.5% vs 97.8%, p<0.00001). DISCUSSION: A novel TAVI pathway with emphasis on local workup within a non-surgical centre significantly reduced both the time to TAVI and rejection rates from a Heart Valve Centre. If adopted across the other centres, this approach may help improve access to TAVI.

Midterm Outcomes in Patients With Aortic Stenosis Treated With Contemporary Balloon-Expandable and Self-Expanding Valves: Does Valve Size Have an Impact on Outcome?

Background No data currently exist comparing the contemporary iterations of balloon-expandable (BE) Edwards SAPIEN 3/Ultra and the self-expanding (SE) Medtronic Evolut PRO/R34 valves. The aim of the study was the comparison of these transcatheter heart valves with emphasis on patients with small aortic annulus. Methods and Results In this retrospective registry, periprocedural outcomes and midterm all-cause mortality were analyzed. A total of 1673 patients (917 SE versus 756 BE) were followed up for a median of 15 months. A total of 194 patients died (11.6%) during follow-up. SE and BE groups showed similar survival at 1 (92.6% versus 90.6%) and 3 (80.3% versus 85.2%) years (Plog-rank=0.136). Compared with the BE group, patients treated with the SE device had lower peak (16.3±8 mm Hg SE versus 21.9±8 mm Hg BE) and mean (8.8±5 mm Hg SE versus 11.5±5 mm Hg BE) gradients at discharge. Conversely, the BE group demonstrated lower rates of at least moderate paravalvular regurgitation postoperatively (5.6% versus 0.7% for SE and BE valves, respectively; P<0.001). In patients treated with small transcatheter heart valves (≤26 mm for SE and ≤23 mm for BE; N=284 for SE and N=260 for BE), survival was higher among patients treated with SE valves at both 1 (96.7% SE versus 92.1% BE) and 3 (91.8% SE versus 82.2% BE) years (Plog-rank=0.042). In propensity-matched patients treated with small transcatheter heart valve, there remained a trend for higher survival among the SE group at both 1 (97% SE versus 92.3% BE) and 3 years (91.8% SE versus 78.7% BE), Plog-rank=0.096). Conclusions Real-world comparison of the latest-generation SE and BE devices demonstrated similar survival up to 3 years' follow-up. In patients with small transcatheter heart valves, there may be a trend for improved survival among those treated with SE valves.

Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes.

AIMS: Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021. METHODS AND RESULTS: This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4-12) days, with a maximum dose of 120 (80-160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2-82.3%] and 43.3% (95% CI: 36-52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome. CONCLUSION: Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.

Renal dysfunction was associated with more advanced disease, higher diuretic doses, and a longer hospital inpatient stay. Chronic kidney disease was common and tripled the risk of death, transplantation, or ventricular assist device. Renal dysfunction in adults with congenital heart disease and heart failure should prompt intensified monitoring, optimization of medical therapy, and collaborative management with renal physicians.

Transition to adult care of young people with congenital heart disease: impact of a service on knowledge and self-care skills and correlates of a successful transition.

AIMS: Less than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition. METHODS AND RESULTS: Overall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P < 0.0001; describing: 14.4-42.7%, P < 0.0001; understanding: 26.1-60.7%, P < 0.0001), and from the second to the third (naming: 67.4%, P = 0.004, describing: 61.4%, P < 0.001, understanding: 71.1%, P = 0.02;). Patients with LD did not improve their disease knowledge over time (all P > 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition. CONCLUSION: A transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.

Paradoxical Left-to-Right Device Embolization Complicating Transcatheter Mitral Valve Edge-to-Edge Repair.

We describe an unusual presentation of transcatheter mitral valve edge-to-edge repair device embolization into the left common femoral vein in a patient with primary degenerative mitral regurgitation. We hypothesize a possible mechanism for this phenomenon, factors that may increase the risk of this complication, and outline the patient's clinical course. (Level of Difficulty: Intermediate.).

The Current Landscape of Transcatheter Tricuspid Valve Intervention.

Tricuspid regurgitation (TR) is common, and its prevalence increases with age. It was previously estimated that there are 1.6 million patients in the United States with moderate or worse TR, and more contemporary data suggest the age-adjusted prevalence of TR is 0.55%. Increasing TR severity is associated with an adverse prognosis independent of the pulmonary artery pressure and the degree of right heart failure. In heart failure with reduced ejection fraction, survival is significantly worsened when moderate or severe TR is present. The mainstay of therapy has traditionally been surgery, but outcomes are poor. There has been increasing attention on the potential role of transcatheter interventions for TR. Numerous platforms are in developmental evolution, which broadly fall into 3 categories: valve replacement, valve repair (subdivided into annular, leaflet, and chordal platforms), and caval valve implantation. In this review, we examine all these strategies and devices, including guidance on how to appropriately select patients who can benefit from intervention.

Patient monitoring and education over a tailored digital application platform for congenital heart disease: A feasibility pilot study.

BACKGROUND: Patients with adult congenital heart disease (ACHD) are a rapidly growing cardiovascular population with increasing health needs and co-morbidities. Furthermore, their management requires frequent and ongoing hospital visits which can be burdensome. Digital health and remote monitoring have been shown to have a vast potential to enhance delivery of healthcare for patients, reducing their need for travel to clinic appointments therefore reducing costs to the patient and the healthcare service. METHODS: Patients over the age of 16 with a diagnosis of ACHD were invited to use the tailored digital application too. They were monitored for a period of 6 months. Information on patient demographics, time using the application, flagged events that prompted clinical reviews and their feedback through patient surveys were collected. RESULTS: A total of 103 patients were enrolled and registered to use the digital application tool. There were 57 (56%) males, median age at the time of enrolment was 39 (16-73) years. The majority (96%) had a moderate or complex ACHD according to the ACC/AHA classification. There was a total of 7 modules that were completed on a weekly basis. The median length of a participant session was 2.2 min and the mean time to complete a module was 21 s. In total, 35 (67%) felt that the application helped them better manage their cardiac condition. Almost all (94%) of patients expressed that they would like to continue using the application beyond the pilot. There were 18 flagged events during the 6 month observation period, and 50% of received early clinical intervention. CONCLUSION: Application based remote monitoring in this select group was well received and potentially holds large benefit to patients both clinically and economically. There were no safety concerns in our pilot feasibility study. Our data may inform much needed and timely investment in digital health.

Successful percutaneous closure of aortic root-to-right ventricle fistula after transcatheter aortic valve implantation: a valuable option in high-risk surgical patients.

Background: Aortic-to-right ventricle (ARV) fistula is an uncommon complication of transcatheter aortic valve implantation (TAVI). Even though surgical closure is usually the treatment of choice in such communications, percutaneous treatment options are valuable alternatives for these high-risk surgical patients. Case summary: In this article, we present the percutaneous closure of an ARV fistula after TAVI, in a highly symptomatic patient with recurrent episodes of heart failure decompensation with worsening right ventricular function, who failed conservative medical treatment and was deemed inoperable. Successful closure of the fistula with the use of the Amplatzer atrial septal occluder was performed 6 months post-TAVI, under general anaesthesia and transoesophageal echocardiography (TOE). A detailed multi-modality imaging pre-procedural planning was performed utilizing 4D cardiac computed tomography and echocardiography. The patient has remained asymptomatic and in good health 5 months after the ARV fistula closure, with marked improvement in his clinical picture and echocardiographic parameters. Discussion: Aortic-to-right ventricle fistulas with significant shunt post-TAVI could lead to biventricular failure and are associated with increased mortality if left untreated. This case demonstrates that TOE-guided percutaneous closure of a TAVI-related ARV fistula, although technically challenging, is feasible, and can be a valuable option for the treatment of symptomatic high-risk surgical patients.

Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach.

OBJECTIVES: This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). BACKGROUND: To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. METHODS: Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers. RESULTS: The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value. CONCLUSIONS: We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Author Correction: WWP2 regulates pathological cardiac fibrosis by modulating SMAD2 signaling.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

WWP2 regulates pathological cardiac fibrosis by modulating SMAD2 signaling.

Cardiac fibrosis is a final common pathology in inherited and acquired heart diseases that causes cardiac electrical and pump failure. Here, we use systems genetics to identify a pro-fibrotic gene network in the diseased heart and show that this network is regulated by the E3 ubiquitin ligase WWP2, specifically by the WWP2-N terminal isoform. Importantly, the WWP2-regulated pro-fibrotic gene network is conserved across different cardiac diseases characterized by fibrosis: human and murine dilated cardiomyopathy and repaired tetralogy of Fallot. Transgenic mice lacking the N-terminal region of the WWP2 protein show improved cardiac function and reduced myocardial fibrosis in response to pressure overload or myocardial infarction. In primary cardiac fibroblasts, WWP2 positively regulates the expression of pro-fibrotic markers and extracellular matrix genes. TGFß1 stimulation promotes nuclear translocation of the WWP2 isoforms containing the N-terminal region and their interaction with SMAD2. WWP2 mediates the TGFß1-induced nucleocytoplasmic shuttling and transcriptional activity of SMAD2.