Anderson, Jason

BACKGROUND: The current study aims to describe outcomes after transcatheter valve replacement in adults with congenital heart disease. METHODS: Retrospective study of adults with congenital heart disease who underwent transcatheter valve replacement and had a 1-year follow-up postintervention at Mayo Clinic (2010–2024). The study period was divided into early (2010–2014), mid (2015–2019), and late (2020–2024) eras. The primary outcome was valve reintervention. The secondary outcomes were all-cause mortality and temporal change in prosthetic valve gradient and clinical indices of disease severity. RESULTS: Overall, 341 patients (age 38±17 years; 175 [51%] men) received 346 prostheses (pulmonary [N=236, 68%], tricuspid [N=75, 22%], aortic [N=22, 8%], and mitral [N=7, 2%]). The prostheses were Sapien (N=181, 52%), Melody (N=142, 41%), and Harmony prostheses (N=23, 7%). The number of transcatheter valve implantations increased from the early era (N=75), mid era (N=109), to the late era (N=162). The 10-year incidence of valve reintervention was 35%, and was similar between Sapien versus Melody prosthesis (41% versus 33%; P =0.11). The 10-year incidence of all-cause mortality was 13%. There was a temporal increase in prosthetic valve Doppler mean gradient (baseline versus 10 years) for pulmonary (12±5 versus 28±11 mmHg; P <0.001), tricuspid (3±1 versus 8±3 mmHg; P <0.001), and aortic prosthesis (13±5 versus 26±12 mmHg; P <0.001). There was a temporal increase in predicted peak oxygen consumption (absolute ∆, 9% [95% CI, 4–13]; P =0.006) and a decrease in NT-proBNP (N-terminal pro-B-type natriuretic peptide) level (absolute ∆, −138 pg/mL [95% CI, −209 to −64]; P <0.001) at 1-year postintervention. CONCLUSIONS: There has been a temporal increase in the number of transcatheter valve implantations over time. Transcatheter valve replacement was associated with clinical improvement across multiple domains. However, there was a significant increase in prosthetic valve gradient within 10 years of follow-up, suggesting limited prosthesis longevity. There is a need for strategies to improve prosthetic valve longevity, which in turn may improve outcomes in this population.

ABSTRACT Background Preparation of the right ventricular outflow tract (RVOT) using covered stent placement is a common preparatory step for transcatheter pulmonary valve replacement (TPVR) to reduce the risk of disruption associated with dilating calcified RVOT conduits. Aim We present a novel method employing a self‐expanding covered aortic endoprosthesis and discuss its advantages as an alternative strategy. Methods Patients with congenital heart disease undergoing RVOT conduit preparation with the GORE TAG Thoracic Branch Endoprosthesis Extender (W.L. Gore & Associates, Newark, DE) followed by TPVR, were prospectively enrolled in a dedicated database. To date, 14 patients have been included. Data on demographics, diagnoses, procedural details, hemodynamic parameters, and outcomes were analyzed. Results Fourteen patients (median age: 35 years [range: 18–79]; 57% female) successfully underwent endograft placement immediately before TPVR. Treated conduits included pulmonary homografts ( n = 8), surgical bioprostheses ( n = 2), Hancock conduit ( n = 1), Contegra conduit ( n = 1), native RVOT ( n = 1), and Melody valve within a surgical bioprosthesis ( n = 1). Most patients presented with mixed stenosis and regurgitation ( n = 9), with the remaining having stenosis ( n = 4) or regurgitation ( n = 1). Valves implanted post‐preparation included the Sapien S3 valve (Edwards Lifesciences, Irvine, CA) ( n = 10) and the Melody valve (Medtronic, Minneapolis, MN) ( n = 5). Significant reductions in RVOT systolic gradient were observed (median 34 mmHg pre‐procedure to 3 mmHg post‐procedure), along with marked conduit expansion (median diameter 13 mm pre‐procedure to 25.5 mm post‐procedure). No complications were reported. Conclusions This study highlights the feasibility of a self‐expanding aortic endoprosthesis for RVOT preparation before TPVR. Further comparative study is warranted. Procedural success was achieved in all cases.

Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterised by multiorgan pathological histiocytic infiltration. Cardiac involvement occurs in 40–75% of patients and increases mortality. A 55-year-old woman with chest pain and dyspnoea was found to have an interatrial septal mass and aortitis suspicious for ECD without other organ involvement. After two inadequate transcatheter interatrial biopsies were obtained using ultrasound guidance alone, diagnostic samples were successfully obtained using intraprocedural frozen section examination. The patient started genotype-targeted treatment with good response. Biopsy confirmation of ECD is required to guide treatment; however, cardiac biopsies are uncommonly performed when other organs are affected. Our unique case of isolated cardiovascular involvement highlights how frozen sectioning with multimodal intraprocedural imaging guidance can improve diagnostic yield of endomyocardial biopsies. It also highlights how diagnosis of rare conditions requires careful multidisciplinary evaluation of affected organs, procedural risk and diagnostic yield. The use of frozen sectioning can improve diagnostic yield of endomyocardial biopsies.

ABSTRACT Background In recent years, transcatheter implantation devices to restrict pulmonary arterial flow have emerged as a potential alternative to surgical pulmonary artery banding. Case Presentation A term male was diagnosed with critical aortic stenosis (AS) and severely reduced left ventricle (LV) systolic function. He underwent aortic balloon valvuloplasty on day 2 of life, resulting in some antegrade flow, but LV ejection fraction only improved to 15%. He remained ductal dependent for systemic perfusion. Pulmonary over‐circulation ensued with systemic steal evident in his second week of life. On day 14, he underwent a transcatheter stage 1 procedure including the placement of bilateral pulmonary flow restrictor (PFR) devices and ductal stenting. Post‐procedure, he improved clinically, weaned off respiratory support, and transitioned to oral feeds while awaiting cardiac transplant. Twelve days later, he received a donor heart, with an uneventful recovery. He is now 3 years post‐transplant and has excellent graft function, and his transplant course has been unremarkable. Conclusion Transcatheter‐based palliation for a neonate with critical AS, utilizing endoluminal PFRs, proved effective in stabilizing the infant and successfully bridging him to transplant. This highlights an opportunity for the utilization of PFRs in neonatal conditions where control of pulmonary blood flow is imperative as a bridge to transplantation.

ABSTRACT Background Tricuspid valve‐in‐valve (TVIV) implantation has traditionally included post‐procedure overnight in‐hospital observation with next‐day discharge. Recently, our practice has shifted toward same‐day discharge for most patients after vascular access site hemostasis and adequate recovery from anesthesia. However, data on the safety of this approach remains scarce. Aims Our study aims to describe our practice change and to identify factors that may lead to overnight observation post‐TVIV. Methods We retrospectively reviewed medical records of patients who underwent uncomplicated TVIV implantation as an outpatient procedure at Mayo Clinic between 2011 and 2023. Results Of the 83 patients who underwent TVIV from 2011 to 2023, 65 (78%) were included in the study. The first 39 patients in our institutional series were observed overnight as standard practice. 18 (69%) of the most recent 26 patients in our series were discharged the same day; three patients who remained in the hospital overnight were because of provider preference and/or patient preference without any specific medical concern. Of all 47 patients who were observed overnight, one patient was treated for fluid overload, one patient had non‐sustained ventricular tachycardia on telemetry that resulted in adjustment of antiarrhythmic therapy, two patients had continued bleeding from the access sites and was observed for another day. Among the two of them, one also developed atrial tachyarrhythmias requiring initiation of additional antiarrhythmic therapy. Out of 18 patients discharged the same day, one patient had an ED presentation within 24 h due to access site bleeding which was resolved promptly. There was no mortality or procedural‐related readmission. Conclusion Same‐day discharge may be considered for adult patients who have undergone uncomplicated TVIV with adequate hemostasis and recovery after anesthesia.

Background Early detection of left and right ventricular systolic dysfunction (LVSD and RVSD respectively) in children can lead to intervention to reduce morbidity and death. Existing artificial intelligence algorithms can identify LVSD and RVSD in adults using a 12‐lead ECG; however, its efficacy in children is uncertain. We aimed to develop novel artificial intelligence–enabled ECG algorithms for LVSD and RVSD detection in pediatric patients. Methods and Results We identified 10 142 unique pediatric patients (age≤18) with a 10‐second, 12‐lead surface ECG within 14 days of a transthoracic echocardiogram, performed between 2002 and 2022. LVSD was defined quantitatively by left ventricular ejection fraction (LVEF). RVSD was defined semiquantitatively. Novel pediatric models for LVEF ≤35% and LVEF <50% achieved excellent test areas under the curve of 0.93 (95% CI, 0.89–0.98) and 0.88 (95% CI, 0.83–0.94) respectively. The model to detect LVEF <50% had a sensitivity of 0.85, specificity of 0.80, positive predictive value of 0.095, and negative predictive value of 0.995. In comparison, the previously validated adult data‐derived model for LVEF <35% achieved an area under the curve of 0.87 (95% CI, 0.84–0.90) for LVEF ≤35% in children. A novel pediatric model for any RVSD detection reached a test area under the curve of 0.90 (0.87–0.94). Conclusions An artificial intelligence–enabled ECG demonstrates accurate detection of both LVSD and RVSD in pediatric patients. While adult‐trained models offer good performance, improvements are seen when training pediatric‐specific models.

AbstractBackgroundTranscatheter tricuspid valve‐in‐valve (ViV) replacement has yielded good hemodynamic outcomes in the treatment of dysfunctional bioprosthetic valves (BPVs). Intentional fracture of certain rigid BPV frames, if feasible, allows a larger implanted valve when compared with implant into an unfractured BPV. There remains limited data on the feasibility of tricuspid valve frame fracture.AimsEvaluate the feasibility of transcatheter tricuspid ViV replacement with fracture of the underlying BPV ring.MethodsAn international multicenter registry of tricuspid ViV replacement with intentional tricuspid valve frame fracture was created. Demographic data along with procedural characteristics, outcomes, and follow‐up data were collected. Comparison was made to the pre‐ and post‐ViV replacement with fracture of the tricuspid valve frame conditions.ResultsTen patients from six centers were included with a median age and weight of 29 years and 67.3 kg respectively. Tricuspid valve frame fracture was performed using a median balloon diameter 3 mm (IQR 3–5) larger than the true inner diameter (ID). The final ID was a mean of 1.5 mm (95% CI: 0.35, 2.64: p < 0.05), and median 1.1 mm (0.5, 2.1) larger than the reported true ID of the surgical BPV after ViV replacement. The mean tricuspid inflow gradient by echocardiogram decreased by 6.65 mmHg (95% CI: 4.14, 9.15: p < 0.001). All procedures were without complication, specifically there was no heart block, pericardial effusion, or right coronary disruption.ConclusionIntentional tricuspid valve frame fracture with tricuspid ViV replacement is feasible and can increase the valve orifice potentially reducing the risk of ViV patient prosthesis mismatch and is not associated with significant complications.

BACKGROUND: The prognostic role of the congenital heart disease (CHD) anatomic/physiologic classification has not been systematically studied. The purpose of this study was to determine whether CHD physiologic stage provided improvement in prognostic power (to predict all-cause mortality) beyond conventional clinical risk models. METHODS: Retrospective study of adults with CHD at the Mayo Clinic (2003–2019). The CHD physiologic stage was assessed at baseline and 36 (24–48) months, and patients were classified into stages A to D at these time points. Clinical stability (remaining in the same stage), clinical improvement (moving to less advanced stage), and clinical deterioration (moving to more advanced stage) were determined at 36 months. We defined conventional clinical risk indices as age/sex, functional class, comorbidities, cardiac procedures, hepatorenal dysfunction, and ventricular/valvular dysfunction. RESULTS: Of 5321 patients, 1649 (31%), 1968 (37%), 1224 (23%), and 480 (9%) were in stages A, B, C, and D at baseline. Of 5321 patients, 4588 (86%) also had assessments at 36 months, and of these patients, 3347 (73%), 386 (8%), and 855 (19%) had clinical stability, deterioration, and improvement, respectively. Patients with clinical improvement were more likely to have undergone cardiac procedures between both assessments. Both baseline CHD physiologic stage (hazard ratio, 1.13 [95% CI, 1.09–1.17]; P <0.001, per unit increase in stage) and change in CHD physiologic stage (hazard ratio, 1.46 [95% CI, 1.32–1.61]; P =0.007, per unit increase in stage) were associated with mortality after adjustments for conventional risk indices and provided incremental improvement in prognostic power beyond conventional clinical risk models as evidence by an increase in C statistic from 0.702 (0.681–0.724) to 0.769 (0.754–0.787). CONCLUSIONS: The CHD physiologic stage can potentially be used for risk stratification, as well as to monitor disease progression and response to therapy.

Background: There are limited data about the stage D heart failure (advanced HF) in adults with congenital heart disease. Our study objectives were (1) to determine the incidence of new-onset advanced HF in patients and the relationship between advanced HF and all-cause mortality and (2) to determine the relationship between therapies for advanced HF and all-cause mortality. Methods: Retrospective cohort study of adults with congenital heart disease at Mayo Clinic (2003–2019). We defined advanced HF using the European Society of Cardiology diagnostic criteria for advanced HF. Therapies received by the patients with advanced HF were classified into 3 mutually exclusive groups (treatment pathways): (1) conventional cardiac intervention, (2) transplant listing, and (3) palliative care. Results: Of 5309 patients without advanced HF at baseline assessment, 432 (8%) developed advanced HF during follow-up (1.1%/y), and the incidence of advanced HF was higher in patients with severe or complex congenital heart disease. Onset of advanced HF was associated with 6-fold increase in the risk of mortality. Conventional cardiac intervention was associated with significantly higher risk of mortality as compared to transplant listing. The longer the interval from the initial onset of advanced HF to transplant evaluation, the lower the odds of being listed for transplant. Conclusions: Based on these data, we postulate that early identification of patients with advanced HF, and a timely referral for transplant evaluation (instead of conventional cardiac intervention) may offer the best chance of survival for these critically ill patients. Further studies are required to validate this postulation.

Background: Left ventricular global longitudinal strain (LVGLS) has been shown to improve risk stratification in patients with LV systolic dysfunction and subsequent recovery of LV ejection fraction (LVEF) in the acquired heart disease population. The purpose of this study was to assess the relationship between LVGLS and cardiovascular events (heart failure hospitalization, sustained ventricular tachycardia/appropriate shock, heart transplant, or cardiovascular death) and deterioration in LVEF (absolute decrease in LVEF ≥10% to LVEF <50%) in adults with congenital heart disease. Methods: Retrospective cohort study of congenital heart disease patients with previous diagnosis of LV systolic dysfunction (LVEF <50%) and subsequent recovery of LVEF (absolute increase in LVEF of ≥10% to LVEF ≥50%) on subsequent echocardiogram (index echocardiogram). Based on the index echocardiogram, patients were divided into normal LVGLS (absolute LVGLS >18%) versus abnormal LVGLS (absolute LVGLS ≤18%) groups. Results: Of 193 patients with recovered LVEF, 86 (45%) had normalization of LVGLS at index echocardiogram. A higher absolute LVGLS and use of renin angiotensin aldosterone system antagonist was associated with a lower risk of cardiovascular events and subsequent deterioration in LVEF, while hypertension was associated with higher risk of cardiovascular events and deterioration in LVEF. Conclusions: These results suggest that patients with congenital heart disease with recovered LVEF remained at risk for adverse outcomes, and LVGLS can be used to identify patients at risk for adverse outcomes. Medical therapy for heart failure and treatment of hypertension may reduce the risk of adverse outcome, but these findings require empirical validation, hence the need for a clinical trial.