Bioptome-assisted blunt dissection facilitates endovascular retrieval of a chronically embolized stent.

We present a 19-year-old female with history of d-transposition of the great arteries status post-arterial switch operation.

Hot-snare assisted percutaneous mechanical aspiration of a calcified amorphous tumor.

A 24-year-old female with history of an atrial septal defect post-patch closure (bovine pericardium) presented 4 years postoperative with an incidentally identified mass originating from the septal patch .

Novel Left Ventricular Unloading Strategies in Patients on Peripheral Venoarterial Extracorporeal Membrane Oxygenation Support.

The purpose of this study was to evaluate left ventricular (LV) unloading strategies in patients supported with peripheral venoarterial extracorporeal membrane oxygenation (VA-ECMO). A retrospective review was conducted of all consecutive patients requiring VA-ECMO support for any indication, who underwent novel LV unloading strategies with either direct left atrial venoarterial (LAVA) cannulation or pulmonary artery venoarterial (PAVA) venting, in comparison to Impella and intra-aortic balloon pump (IABP). The primary outcome was successful bridge to transplant, LV assist device, or myocardial recovery. Forty-six patients (63% male, mean age 52.8 ± 17.6 years) were included. Fourteen patients (30%) underwent novel unloading with either LAVA or PAVA, 11 patients (24%) underwent IABP placement, and 21 patients (46%) underwent Impella insertion. In the novel LV unloading cohort, 10 patients (71%) survived to hospital discharge. Four patients (29%) were weaned from ECMO and eight patients (57%) underwent cardiac transplantation. Although a trend favoring cannula-based unloading for the primary outcome was noted, the cohort was too small for statistical significance (79% LAVA/PAVA, 57% Impella, 45% IABP; p = 0.21). However, probability of survival was greater in the LAVA/PAVA cohort compared to Impella and IABP ( p < 0.05). Thus, we demonstrate the efficacy of LA and PA cannulation as an alternative LV unloading strategy for patients supported with peripheral VA-ECMO.

Right Heart Reverse Remodeling and Prosthetic Valve Function After Transcatheter vs Surgical Pulmonary Valve Replacement.

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

Cardiac Reverse Remodeling and Changes in Heart Failure Indices After Transcatheter Tricuspid Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: There are limited data about changes in cardiac function (cardiac reverse remodeling) and heart failure indices after transcatheter tricuspid valve-in-valve replacement (TT-VIVR). The purpose of this study was to evaluate cardiac reverse remodeling and temporal changes in heart failure indices after TT-VIVR in adults with congenital heart disease. METHODS: Retrospective cohort study of adults with congenital heart disease that underwent TT-VIVR and had >6 months of follow-up (January 1, 2011, to April 30, 2023). Echocardiographic indices of cardiac remodeling and heart failure indices (New York Heart Association class, NT-proBNP (N-terminal pro-brain natriuretic peptide), glomerular filtration rate, and model for end-stage liver disease excluding international normalized ratio score) were assessed preintervention and at 1-, 3-, and 5-year postintervention. RESULTS: Of 39 patients (age 39 [32-46] years), 14 (36%) and 25 (64%) received Melody valve and Sapien valve prosthesis, respectively. At 1-year post-TT-VIVR, there was a temporal improvement in right atrial reservoir strain (17±8% versus 22±8%, P<0.001), right atrial volume (81 [59-108] versus 63 [48-82] mL/m2, P<0.001), right atrial pressure (12±4% versus 6±4%, P<0.001), and right ventricular global longitudinal strain (-15±7% versus -20±7%, P<0.001). Similarly, there was a temporal improvement in NT-proBNP, glomerular filtration rate, model for end-stage liver disease excluding international normalized ratio score, and New York Heart Association class. The temporal improvements in heart failure indices and valve function were maintained at 3- and 5-year post-TT-VIVR. CONCLUSIONS: Considering the significant mortality risk associated with reoperations for tricuspid valve replacement, these data suggest favorable outcomes after TT-VIVR, and support TT-VIVR as a viable alternative to surgical tricuspid valve replacement, especially in high-risk patients.

Endovascular rehabilitation of acutely thrombosed fontan conduit in a patient with multiorgan failure.

A 32-year-old man with history of hypoplastic left heart syndrome status post-Fontan palliation (20-mm aortic homograft conduit) had previously undergone Fontan conduit (FC) and left pulmonary artery (LPA) stenting to relieve conduit obstruction.

Infective Endocarditis Risk with Melody versus Sapien Valves Following Transcatheter Pulmonary Valve Implantation: A Systematic Review and Meta-Analysis of Prospective Cohort Studies.

BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) is an effective non-surgical treatment method for patients with right ventricle outflow tract dysfunction. The Medtronic Melody and the Edwards Sapien are the two valves approved for use in TPVI. Since TPVI patients are typically younger, even a modest annual incidence of infective endocarditis (IE) is significant. Several previous studies have shown a growing risk of IE after TPVI. There is uncertainty regarding the overall incidence of IE and differences in the risk of IE between the valves. METHODS: A systematic search was conducted in the MEDLINE, EMBASE, PubMed, and Cochrane databases from inception to 1 January 2023 using the search terms 'pulmonary valve implantation', 'TPVI', or 'PPVI'. The primary outcome was the pooled incidence of IE following TPVI in Melody and Sapien valves and the difference in incidence between Sapien and Melody valves. Fixed effect and random effect models were used depending on the valve. Meta-regression with random effects was conducted to test the difference in the incidence of IE between the two valves. RESULTS: A total of 22 studies (including 10 Melody valve studies, 8 Sapien valve studies, and 4 studies that included both valves (572 patients that used the Sapien valve and 1395 patients that used the Melody valve)) were used for the final analysis. Zero IE incidence following TPVI was reported by eight studies (66.7%) that utilized Sapien valves compared to two studies (14.3%) that utilized Melody valves. The pooled incidence of IE following TPVI with Sapien valves was 2.1% (95% CI: 0.9% to 5.13%) compared to 8.5% (95% CI: 4.8% to 15.2%) following TPVI with Melody valves. Results of meta-regression indicated that the Sapien valve had a 79.6% (95% CI: 24.2% to 94.4%, p = 0.019; R2 = 34.4) lower risk of IE incidence compared to the Melody valve. CONCLUSIONS: The risk of IE following TPVI differs significantly. A prudent valve choice in favor of Sapien valves to lower the risk of post-TPVI endocarditis may be beneficial.

Prognostic Value of the Anatomic-Physiologic Classification in Adults With Congenital Heart Disease.

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.

Clinical benefits of angiotensin receptor-Neprilysin inhibitor in adults with congenital heart disease.

BACKGROUND: There are limited data about the clinical benefits of angiotensin receptor-neprilysin inhibitor (ARNI) in adults with congenital heart disease (CHD). The purpose of the study was to assess the clinical benefits (chamber function and heart failure indices) of ARNI in adults with CHD. METHOD: In this retrospective cohort study, we compared the temporal change in chamber function and heart failure indices between 35 patients that received ARNI for >6 months, and a propensity matched control group (n = 70) of patients that received angiotensin converting enzyme inhibitor or angiotensin-II receptor blocker (ACEI/ARB) within the same period. RESULTS: Of the 35 patients in the ARNI group, 21 (60%) had systemic left ventricle (LV) while 14 (40%) had systemic right ventricle (RV). Compared to the ACEI/ARB group, the ARNI group had greater relative improvement in LV global longitudinal strain (GLS) (28% versus 11% increase from baseline, p < 0.001) and RV-GLS (11% versus 4% increase from baseline, p < 0.001), and greater relative improvement in New York Heart Association functional class (-14 versus -2% change from baseline, p = 0.006) and N-terminal pro-brain natriuretic peptide levels (-29% versus -13% change from baseline, p < 0.001). These results were consistent across different systemic ventricular morphologies. CONCLUSIONS: ARNI was associated with improvement in biventricular systolic function, functional status, and neurohormonal activation, suggesting prognostic benefit. These results provide a foundation for a randomized clinical trial to empirically test the prognostic benefits of ARNI in adults with CHD, as the next step towards evidence-based recommendations for heart failure management in this population.

Pathogenesis, Evaluation, and Management of Pulmonary Vein Stenosis: JACC Review Topic of the Week.

Pulmonary vein stenosis (PVS) can arise from several etiologies, including congenital, acquired, and iatrogenic sources. PVS presents insidiously, leading to significant delays in diagnosis. A high index of suspicion and dedicated noninvasive evaluation are key to diagnosis. Once diagnosed, both noninvasive and invasive evaluation may afford further insights into the relative contribution of PVS to symptoms. Treatment of underlying reversible pathologies coupled with transcatheter balloon angioplasty and stenting for persistent severe stenoses are established approaches. Ongoing refinements in diagnostic modalities, interventional approaches, postintervention monitoring, and medical therapies hold promise to further improve patient outcomes.

Left ventricular adaptation to aortic regurgitation in adults with repaired coarctation of aorta.

BACKGROUND: Aortic regurgitation (AR) can develop in adults with repaired coarctation of aorta (COA), but there are limited data about left ventricular (LV) remodeling and clinical outcomes in this population. The purpose of the study was to compare LV remodeling (LV mass index [LVMI], LV ejection fraction [LVEF], and septal E/e') and onset of symptoms before aortic valve replacement, and LV reverse remodeling (%-change in LVMI, LVEF and E/e') after aortic valve replacement in patients with versus without repaired COA presenting with AR. METHODS: Asymptomatic adults with repaired COA presenting with moderate/severe AR (AR-COA group) were matched 1:2 to asymptomatic adults without COA and similar severity of AR (control group). RESULTS: Although both groups (AR-COA n = 52, and control n = 104) had similar age, sex, body mass index, aortic valve gradient, and AR severity, the AR-COA group had higher LVMI (124 ± 28 versus 102 ± 25 g/m2, p < 0.001) and E/e' (12.3 ± 2.3 versus 9.5 ± 2.1, p = 0.02) but similar LVEF (63 ± 9% versus 67 ± 10%, p = 0.4). COA diagnosis (adjusted HR 1.95, 95%CI 1.49-2.37, p < 0.001), older age, E/e', and LV hypertrophy were associated with onset of symptoms. Of 89 patients (AR-COA n = 41, and control n = 48) with echocardiographic data at 1-year post- aortic valve replacement, the AR-COA group had less regression of LVMI (-8% [95%CI -5 to -11] versus -17% [95%CI -15 to -21], p < 0.001) and E/e' (-5% [95% CI -3 to -7] versus -16% [95% CI -13 to -19], p < 0.001). CONCLUSIONS: Patients with COA and AR had a more aggressive clinical course, and perhaps may require a different threshold for surgical intervention.

Clinical Outcomes of Percutaneous Fontan Stenting in Adults.

BACKGROUND: Fontan pathway stenosis is a well-known complication after palliation. Percutaneous stenting is effective for angiographic/hemodynamic relief of Fontan obstruction, but its clinical impact in adults remains unknown. METHODS: This was a retrospective cohort of 26 adults undergoing percutaneous stenting for Fontan obstruction from 2014 to 2022. Procedural details, functional capacity, and liver parameters were reviewed at baseline and during follow-up. RESULTS: Median age was 22.5 years (interquartile range [IQR] 19-28.8 y); 69% were male. After stenting, Fontan gradient significantly decreased (2.0 ± 1.9 vs 0 [IQR 0-1] mm Hg; P < 0.005), and minimal Fontan diameter increased (11.3 ± 2.9 vs 19.3 [IQR 17-20] mm; P < 0.001). One patient developed acute kidney injury periprocedurally. During a follow-up of 2.1 years (IQR 0.6-3.7 y), 1 patient had thrombosis of the Fontan stent and 2 underwent elective Fontan re-stenting. New York Heart Association functional class improved in 50% of symptomatic patients. Changes in functional aerobic capacity on exercise testing were directly related to pre-stenting Fontan gradient (n = 7; r = 0.80; P = 0.03) and inversely related to pre-stenting minimal Fontan diameter (r = -0.79; P = 0.02). Thrombocytopenia (platelet count < 150 109/L) was present in 42.3% of patients before and in 32% after the procedure (P = 0.08); splenomegaly (spleen size > 13 cm) was present in 58.3% and 58.8% (P = 0.57), respectively. Liver fibrosis (aspartate transaminase to platelet ratio index and Fibrosis-4) scores were unchanged after the procedure compared with baseline. CONCLUSIONS: Percutaneous stenting in adults is safe and effective in relieving Fontan obstruction, resulting in subjective improvement in functional capacity in some. A subset of patients demonstrated improvement in markers of portal hypertension, suggesting that Fontan stenting could improve Fontan-associated liver disease in select individuals.

Transcatheter Treatment of Native Coarctation of the Aorta in a Child Using a Balloon-Expandable Endoprosthesis.

A 7-year-old child with native coarctation of the aorta was treated by transcatheter therapy using a 29-mm balloon-expandable stent. The procedure was successful and free of complications, and the patient was discharged home the same day. This stent has several features making it uniquely advantageous for treating this condition. (Level of Difficulty: Advanced.).

Right Ventricular Systolic Dysfunction in Adults With Anatomic Repair of d-Transposition of Great Arteries.

The purpose of this study was to assess the prevalence of right ventricular (RV) systolic dysfunction in adults with anatomic repair of dextro-transposition of great arteries (d-TGAs), and to determine its relation to clinical outcomes across multiple domains (functional status, peak oxygen consumption, N-terminal pro-brain natriuretic peptide, and heart failure hospitalization). Adults with anatomic repair for d-TGA and with echocardiographic images for strain analysis were divided into 2 groups: (1) d-TGA status after an arterial switch operation (d-TGA-ASO group) and (2) d-TGA status after a Rastelli operation (d-TGA-Rastelli group). RV systolic function was assessed using RV global longitudinal strain (RVGLS), and RV systolic dysfunction was defined as RVGLS >-18%. We identified 151 patients (median age 21 years [19 to 28]; d-TGA-ASO group 89 [59%], and d-TGA-Rastelli group 62 [41%]). The mean RVGLS was -22 ± 4%, and 47 patients (31%) had RV systolic dysfunction. The d-TGA-Rastelli group had lower (less negative) RVGLS than that of the d-TGA-ASO group (-19 ± 3% vs -25 ± 3%, p <0.001) and higher prevalence of RV systolic dysfunction (48% vs 19%, p <0.001). RVGLS (absolute value) was associated with peak oxygen consumption (r = 0.58, p <0.001; adjusted R2 = 0.28), log-N-terminal pro-brain natriuretic peptide (r = -0.41, p = 0.004; adjusted R2 = 0.21), New York Heart Association class III/IV (odds ratio 2.29, 1.56 to 3.19, p = 0.01), and heart failure hospitalization (hazard ratio 0.93, 0.88 to 0.98, p = 0.009). RV systolic dysfunction was common in adults with d-TGA and anatomic repair and was associated with clinical outcomes. Longitudinal studies are required to determine the risk factors for progressive RV systolic dysfunction and to identify strategies for preventing RV systolic dysfunction in this population.

Risk of pulmonary artery dissection in adults with congenital heart disease.

BACKGROUND: There are limited data about the risk of pulmonary artery (PA) dissection in adults with congenital heart disease (CHD), and the purpose of this study was to estimate the incidence of PA dissection in this population. METHODS: Retrospective cohort study of adults with CHD that underwent cross-sectional imaging (2003-2020). PA aneurysm was defined as main or branch PA diameter > 40 mm or > 30 mm respectively, and severe PA aneurysm was defined as main or branch PA diameter > 50 mm. RESULTS: Of 1, 673 patients (41 ± 10 years; male 58%), 493 (24%), 286 (19%), and 306 (20%) had aneurysms of the main, right, and left PA respectively, while 66 (4%) had severe PA aneurysm. During a median follow-up of 8.2 (interquartile range 3.7-10.3) years, there was one PA dissection in a patient with Eisenmenger syndrome, thus the incidence of PA dissection was 14 per 100,000 patient-years. Of 779 females, 163 had one or more pregnancies during follow-up, and 41 (25%) of these patients had known PA aneurysm at the time of conception. There was no PA dissection during pregnancy. Of the 163 patients, 91 (56%) had cross-sectional imaging before and after pregnancy, there was no significant difference in PA dimension before versus after pregnancy (main PA 35 ± 5 versus 36 ± 4 mm, p = 0.6; right PA 21 ± 3 versus 33 ± 4 mm, p = 0.1; and left PA 23 ± 4 versus 22 ± 4 mm, p = 0.4). CONCLUSIONS: The risk of PA dissection was extremely low even in patients with severe PA aneurysm, or in patients with PA aneurysm that became pregnant. Collectively, these data suggest a benign natural history for patients without severe pulmonary hypertension and encourage and a conservative approach in managing patients with PA aneurysm.

Dobutamine-induced Takotsubo syndrome in a paediatric heart transplant patient.

Takotsubo syndrome is a potentially reversible cause of acute systolic dysfunction. Takotsubo syndrome is rare in children, with no reported dobutamine-induced cases to date. We present a 14-year-old male with prior history of heart transplantation, who developed Takotsubo syndrome during dobutamine stress echocardiography. We highlight the importance of its early recognition to ensure supportive measures with avoidance of inotropic medications.

Exercise-induced changes in pulmonary artery wedge pressure in adults post-Fontan versus heart failure with preserved ejection fraction and non-cardiac dyspnoea.

AIMS: Despite their universal predisposition, diagnosing diastolic dysfunction in patients post-Fontan palliation is challenging. Our aim was to compare exercise haemodynamics between adults post-Fontan and patients with heart failure with preserved ejection fraction (HFpEF) and non-cardiac dyspnoea (NCD). METHODS AND RESULTS: Twenty-four adults (age ≥18 years) post-Fontan palliation with resting and exercise pulmonary artery wedge pressure (PAWP) measured during supine biking were identified. Forty-eight patients with HFpEF and 48 with NCD diagnosed at catheterization were selected for comparison. Mean age for Fontan patients was 30.3 ± 7.5 years; median ventricular ejection fraction was 52.5% (45-55.8), being <50% in 37.5%. Resting PAWP among Fontan patients was 10.2 ± 3.5 mmHg (>12 mmHg in 25%); PAWP was lower in Fontan patients than in HFpEF but higher than NCD. During exercise, PAWP was lower in the Fontan group than HFpEF (22.5 mmHg [19.3-28] vs. 28.2 ± 6.3; p = 0.0006) but higher than NCD (11.2 ± 4.2, p ≤ 0.0001). However, there were no differences in ΔPAWP/ΔQs between Fontan and HFpEF patients (4.0 [2.1-7.3] vs. 2.7 [1.6-4.4]; p = 0.10) with the ratio being higher post-Fontan than in NCD (0.6 [0.2-1.2]; p < 0.0001). ΔPAWP/ΔQs remained similar between HFpEF and Fontan patients even when those with ejection fraction <50% were excluded (2.7 [1.6-4.4] vs. 2.7 [1.0-5.8]; p = 0.97). CONCLUSION: There were no differences in ΔPAWP/ΔQs ratios between post-Fontan and HFpEF patients, supporting markedly abnormal single ventricle compliance despite lower resting and exercise PAWP. Therefore, exercise invasive haemodynamics may represent a novel tool for the diagnosis of diastolic dysfunction in Fontan patients.