Real-world experience with mavacamten in obstructive hypertrophic cardiomyopathy: Observations from a tertiary care center.

BACKGROUND: In symptomatic obstructive hypertrophic cardiomyopathy (oHCM) patients, mavacamten is commercially approved to help improve left ventricular (LV) outflow tract (LVOT) gradients, symptoms, and reduce eligibility for septal reduction therapy (SRT) under the risk evaluation and mitigation strategy (REMS) program. We sought to prospectively report the initial real-world clinical experience with the use of commercially available mavacamten in a multi-hospital tertiary healthcare system. METHODS: We studied the first 150 consecutive oHCM patients (mean age 65 years, 53% women, 83% on betablockers and 61% in New York Heart Association [NYHA] class III) who were initiated on 5 mg of mavacamten with dose titrations using symptom assessment and echocardiographic measurements of LVOT gradient and LV ejection fraction (LVEF) measurements. We measured changes in NYHA class, LVEF, LVOT gradients (resting and Valsalva) at baseline, 4, 8 and 12 weeks. RESULTS: At 261 ± 143 days (range of 31-571 days), 69 (46%) patients had ≥1 NYHA class, and 27 (18%) additional patients had ≥2 NYHA class improvement. The mean Valsalva LVOT gradient decreased from 72 ± 43 mmHg at baseline to 29 ± 31 mmHg at 4 weeks, 29 ± 28 mmHg at 8 weeks and 30 ± 29 mmHg at 12 weeks (p < 0.001). At baseline, 100% patients had Valsalva LVOT gradients ≥30 mmHg, which reduced to 29% at 4 weeks, 28% at 8 weeks and 30% at 12 weeks. In 40 patients who reported no symptomatic improvement, the mean Valsalva LVOT gradient decreased from 73 ± 39 mmHg at baseline to 34 ± 27 mmHg at 4 weeks, 35 ± 28 mmHg at 8 weeks and 30 ± 24 mmHg at 12 weeks (P < 0.001). The mean LVEF at baseline was 66 ± 6% and changed to 64 ± 5% at 4 weeks, 63 ± 5% at 8 weeks and 62 ± 7% at 12 weeks (p < 0.0001). No patient underwent SRT, developed LVEF ≤30% or developed heart failure requiring admission. Three (2%) patients needed temporary interruption of mavacamten due to LVEF<50%. CONCLUSIONS: In a real-world study in symptomatic oHCM patients at a multi-hospital tertiary care referral center, we demonstrate the efficacy and safety, along with the logistic feasibility of prescribing mavacamten under the REMS program.

Outcomes of Octogenarian Patients Treated with Tafamidis for Transthyretin Amyloid Cardiomyopathy.

Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) benefit from disease-modifying agents such as tafamidis. However, the survival benefit of tafamidis in elderly patients (age ≥80 years) is not reported. This study aimed to assess the survival of patients with ATTR-CM aged 80 years and older who were treated with tafamidis compared with patients aged <80 years. We conducted a retrospective analysis of patients with ATTR-CM who underwent tafamidis treatment, aged 45 to 97 years at the time of diagnosis between January 1, 2008, and May 31, 2021. A total of 484 patients were included, with 208 in the ≥80 years group and 276 in the <80 years group. The cohort was followed up for mortality outcomes, and hazard ratios with 95% confidence intervals were calculated. After a median follow-up of 18.5 months, 72 deaths were recorded in the entire cohort. Kaplan-Meier curves showed no differences in survival probability between the 2 groups at 30 months (p for log-rank test = 0.76). The survival rates for patients aged ≥80 years who underwent treatment at 1, 2, 3, 4, and 5 years were 94.7%, 86.0%, 77.0%, 77.0%, and 38.5%, respectively. The corresponding rates for patients aged <80 years who underwent treatment were 93.2, 84.8, 74.4, 68.2, and 64.6%, respectively. In the multivariable analysis, the hazard ratio (95% confidence interval) of the mortality comparing treatment patients aged ≥80 years with those aged <80 years was 0.81 (0.41 to 1.61). In conclusion, tafamidis treatment is associated with similar reductions in mortality in older and younger patients with ATTR-CM.

Multimodality Imaging Approach Evaluation of the Congenital Pericardial Defect: a Contemporary Review.

PURPOSE OF REVIEW: This review presents the etiology, clinical manifestations, diagnostic approach, and treatment of congenital pericardial defects. It also highlights the critical role of echocardiography, cardiac computed tomography (CCT), and cardiac magnetic resonance (CMR) in the diagnosis and management approach. RECENT FINDINGS: Congenital pericardial defects are rare. Although most cases are found incidentally, some cases could potentially be associated with serious outcomes including sudden cardiac death. The diagnosis is often challenging due to non-specific clinical manifestations and electrocardiogram findings. Echocardiography is the first-line imaging investigation for the evaluation of this condition. Advanced cardiac imaging modalities, including CCT and CMR, play important adjuvant roles in establishing the diagnosis and assists with prognostication.

Challenges in diagnosis and therapeutic options for metastatic prostate cancer to the right ventricle.

Our patient presented with right-sided heart failure symptoms and found to have a large mass protruding into the heart's right ventricle. Cardiac MRI delineated the morphological and tissue characteristics of the tumour. Although 18-fluorodeoxyglucose positron emission tomography (PET) did not reveal an intracardiac mass, the lesion was well demonstrated by Fluciclovine F18 PET/CT.

Utility of Intracardiac Echocardiography for Infective Endocarditis and Cardiovascular Device-Related Endocarditis: A Contemporary Systematic Review.

The diagnosis of infective endocarditis (IE) can pose a significant challenge, particularly in cases of prosthetic valve endocarditis (PVE) or cardiac device-related endocarditis (CDIE) (1). While echocardiography remains a crucial diagnostic tool for identifying IE, including PVE and CDIE, there are certain circumstances where transesophageal echocardiography (TEE) may not be conclusive or practically feasible (2). Recently, intracardiac echocardiography (ICE) has emerged as a promising alternative for diagnosing IE and evaluating intracardiac infections, especially in cases where transthoracic echocardiography (TTE) has not been revealing, and TEE has been contraindicated. Furthermore, ICE has been found to be useful in guiding transvenous lead extractions in infected implantable cardiac devices (3). This systematic review aims to comprehensively explore the various applications of ICE in the diagnosis of IE and assess its efficacy in comparison to traditional diagnostic methods.

Association of ACEI/ARB and statin prescribing patterns with mortality after Transcatheter Aortic Valve Replacement (TAVR): Findings from real-world claims data.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) has become the standard of care for most patients with severe aortic stenosis (AS), but the impact of medical therapy prescribing patterns on post-TAVR patients has not been thoroughly investigated. METHODS: We analyzed Optum claims data from 9,012 adults who received TAVR for AS (January 2014-December 2018). Pharmacy claims data were used to identify patients who filled ACEI/ARB and/or statin prescriptions during the study's 90-day landmark period post-TAVR. Kaplan-Meier and adjusted Cox Proportional Hazards models were used to evaluate the association of prescribing patterns with mortality during the 3-year follow-up period. Subgroup analyses were performed to examine the impact of 11 potential confounders on the observed associations. RESULTS: A significantly lower adjusted 3-year mortality was observed for patients with post-TAVR prescription for ACEI/ARBs (hazard ratio [HR] = 0.82, 95% confidence interval [CI] 0.74-0.91, P = .0003) and statins (HR = 0.85, 95% CI 0.77-0.94, P = .0018) compared to patients who did not fill prescriptions for these medications post-TAVR. Subgroup analyses revealed that the survival benefit associated with ACEI/ARB prescription was not affected by any of the potential confounding variables, except preoperative ACEI/ARB prescription was associated with significantly lower risk of mortality vs postoperative prescription only. No other subgroup variables had significant interactions associated with survival benefits, including preoperative use of statins. CONCLUSIONS: In this large-scale, real-world analysis of patients undergoing TAVR, the prescription of ACEI/ARB and statins was associated with a significantly lower risk of mortality at 3-years, especially in those where the medications were initiated preoperatively.

Trends and Outcomes of Left Atrial Appendage Occlusion in Renal and Liver Transplant Recipients: Insights From the United States National Inpatient and Readmission Database.

Left atrial appendage occlusion using the Watchman device has emerged as an alternative treatment strategy for preventing strokes in patients with atrial fibrillation. However, there is no data on its safety and clinical outcomes in prior renal or liver transplant recipients. We included a total of 61,995 patients from the National Inpatient Sample (NIS, in-hospital outcomes) and 55,048 patients from the National Readmission Database (NRD, 30-day outcomes) who underwent percutaneous left atrial appendage occlusion (LAAO). From this group, 0.65% (n=405) and 0.62% (n=339) were renal and liver transplant recipients in NIS and NRD respectively. Transplant recipients were younger compared with non-transplant recipients (mean age 69 vs 77 years, P=<0.01). There was little difference in terms of in-hospital mortality (0% vs 0.2%, P=0.43), major complications (6.2% vs 5.6%, P=0.61), cardiovascular complications (2.5% vs 2.8%, P=0.73), neurological complications (1.2% vs 0.7%, P=0.21) or bleeding complications (1.2% vs 0.7%, P=0.99) between transplant vs. non-transplant patients. Based on the NRD database, 30-day readmission rate was not meaningfully different for transplant recipients undergoing LAAO (9.44%) when compared to non-transplant patients (8.12%, [log-rank, P=0.56]). There was no difference between 30-day major or cardiovascular complications, however vascular complication rates were significantly higher for transplant recipients (OR 2.56, 95% CI [(1.66-3.47]). Our study findings suggest that LAAO may be safe for patients with a prior renal or liver transplant in terms of major complications, cardiovascular complications, and all-cause readmission rates. However vascular complications may be higher in transplant recipients. Further large-scale studies are needed to confirm these findings.

Advanced Cardiovascular Imaging for the Diagnosis of Mycobacterium chimaera Prosthetic Valve Infective Endocarditis After Open-heart Surgery: A Contemporary Systematic Review.

Mycobacterium chimaera is an opportunistic and emerging pathogen, which has been recognized to cause prosthetic valve infective endocarditis and disseminated infection following open-chest cardiac surgery with certain contaminated heater-cooler systems. Diagnostic evaluation of suspected prosthetic valve infective endocarditis due to M chimaera is challenging and requires a very high index of suspicion. This systematic review aims to evaluate prosthetic valve infective endocarditis due to M chimaera. Based on the current literature review, transesophageal echocardiography and 18F-fluorodeoxyglucose positron emission tomography/computed tomography are the most common imaging modalities used to establish the diagnosis. Based on 22 published cases, the reported cases of M chimaera endocarditis have occurred almost entirely in males. Within this cohort, the patients developed endocarditis on average 2.7 years after exposure to contaminated heater-cooler systems during cardiac surgery. M chimaera infection is associated with significant morbidity and mortality.

5-Year Outcomes Comparing Surgical Versus Transcatheter Aortic Valve Replacement in Patients With Chronic Kidney Disease.

OBJECTIVES: The aim of this study was to compare 5-year cardiovascular, renal, and bioprosthetic valve durability outcomes in patients with severe aortic stenosis (AS) and chronic kidney disease (CKD) undergoing transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR). BACKGROUND: Patients with severe AS and CKD undergoing TAVR or SAVR are a challenging, understudied clinical subset. METHODS: Intermediate-risk patients with moderate to severe CKD (estimated glomerular filtration rate <60 mL/min/m2) from the PARTNER (Placement of Aortic Transcatheter Valve) 2A trial (patients randomly assigned to SAPIEN XT TAVR or SAVR) and SAPIEN 3 Intermediate Risk Registry were pooled. The composite primary outcome of death, stroke, rehospitalization, and new hemodialysis was evaluated using Cox regression analysis. Patients with and without perioperative acute kidney injury (AKI) were followed through 5 years. A core laboratory-adjudicated analysis of structural valve deterioration and bioprosthetic valve failure was also performed. RESULTS: The study population included 1,045 TAVR patients (512 SAPIEN XT, 533 SAPIEN 3) and 479 SAVR patients. At 5 years, SAVR was better than SAPIEN XT TAVR (52.8% vs 68.0%; P = 0.04) but similar to SAPIEN 3 TAVR (52.8% vs 58.7%; P = 0.89). Perioperative AKI was more common after SAVR than TAVR (26.3% vs 10.3%; P < 0.001) and was independently associated with long-term outcomes. Compared with SAVR, bioprosthetic valve failure and stage 2 or 3 structural valve deterioration were significantly greater for SAPIEN XT TAVR (P < 0.05) but not for SAPIEN 3 TAVR. CONCLUSIONS: In intermediate-risk patients with AS and CKD, SAPIEN 3 TAVR and SAVR were associated with a similar risk for the primary endpoint at 5 years. AKI was more common after SAVR than TAVR, and SAPIEN 3 valve durability was comparable with that of surgical bioprostheses.

Cardioform Septal Occluder for the Closure of a Peri-Device Leak After Initial Watchman Implantation.

Left atrial appendage occlusion has become a safe and effective alternative for stroke-risk reduction among patients with non-valvular atrial fibrillation (AF). Although complete closure is ideal, residual peri-device leaks (PDL) are not uncommon and have been associated with an increased residual risk of stroke. PDL closure has been proposed as an alternate strategy to allow for the safe discontinuation of oral anticoagulation. We describe the safety and feasibility of successful PDL closure using a non-fenestrated Cardioform (Gore Medical, Flagstaff, Arizona) septal occluder after initial Watchman (Boston Scientific, Marlborough, Massachusetts) implantation.

Management of peri-device leak following left atrial appendage closure: A systematic review.

OBJECTIVE: This study aimed to examine the cumulative experience of peri-device leak (PDL) closure following left atrial appendage (LAA) closure. BACKGROUND: The management of PDL following LAA closure remains controversial. While PDL closure has been proposed, procedural features and clinical outcomes have not been well established. METHODS: A systematic review of all published cases of PDL closure with available anatomical, procedural, and clinical outcomes was performed. RESULTS: We identified 18 indexed publications and 110 cases between April 2013 and March 2020. 71 patients (mean age 72 ± 8 yrs), met study criteria and were included. PDL closure was most common in males, bilobar LAA morphology, and after Watchman procedures. The mean PDL size was 7.6 ± 5.8 mm (range 2-26 mm). Leaks were classified according to size: small (<5 mm; 45%), moderate (≥5-9 mm; 25%), and large (≥10 mm; 30%). Endovascular coils and endovascular plugs were used to close both small and moderate sized leaks, and second LAA closure devices were exclusively used for large PDLs. Successful PDL closure occurred in 90%, and was similar between PDL sizes and types of occluder used. Procedural complication rates were uncommon (2.8%). No strokes were reported following PDL closure at 6 months. CONCLUSIONS: In patients with PDL for whom discontinuation of OAC may be considered unsafe, percutaneous closure using a tailored approach with either endovascular coils, plugs, or second occluder represents a safe, and feasible alternative associated with favorable clinical outcomes.

Impact of Transcatheter Aortic Valve Replacement on Severity of Chronic Kidney Disease.

BACKGROUND: The effect of transcatheter aortic valve replacement (TAVR) on kidney function stage in patients with aortic stenosis remains poorly understood. We hypothesized that in some patients, TAVR results in improved kidney function by alleviating cardiorenal syndrome. OBJECTIVES: The purpose of this study was to assess change in chronic kidney disease (CKD) stage following TAVR, identify variables associated with pre- and post-TAVR estimated glomerular filtration rate (eGFR), and assess association of post-TAVR eGFR with mortality. METHODS: Patients (n = 5,190) receiving TAVR in the PARTNER (Placement of Aortic Transcatheter Valves) 1, 2, and PARTNER 2 S3 trials between April 2007 and October 2014 were included. Pre-TAVR and procedural variables associated with post-TAVR eGFR, change in CKD stage at ≤7 days post-TAVR, and association of post-TAVR eGFR on intermediate-term mortality were assessed. RESULTS: At baseline, CKD stage ≥2 was present in 91% of patients. CKD stage either improved or was unchanged following TAVR in the majority of patients (77% stage 1, 90% stage 2, 89% stage 3A, 94% stage 3B, and 99% stage 4). Progression to CKD stage 5 occurred in 1 (0.035%) of 2,892 patients within 7 days post-TAVR. Of 3,546 patients in whom data were available, 70 (2.0%) underwent post-TAVR dialysis. Higher pre-TAVR eGFR and transfemoral approach were strongly associated with higher post-TAVR eGFR. Lower baseline and longitudinal post-TAVR eGFR were associated with lower intermediate-term survival. CONCLUSIONS: In patients with severe aortic stenosis undergoing TAVR, even with baseline impaired eGFR, CKD stage is more likely to stay the same or improve than worsen. Aortic stenosis may contribute to cardiorenal syndrome that improves with TAVR.

Usefulness of Interleukin-1 Receptor Antagonists in Patients With Recurrent Pericarditis.

Nonsteroidal anti-inflammatory drugs and colchicine remain the standard of care as the initial treatment of acute pericarditis. Corticosteroids and traditional immunosuppressive medications are often added if patients develop recurrent symptoms and remain medically refractory. There has been growing interest in the use of interleukin-1 receptor antagonists (IL-1RAs) in managing pericarditis, especially, in medically refractory cases. Anakinra-Treatment for Recurrent Idiopathic Pericarditis is a recent pilot trial showing a benefit of using the IL-1RA, anakinra, in recurrent pericarditis. Publications remain limited and more outcomes trials are needed. This review focuses on the mechanism of action, clinical utility, current, and future studies that investigate the role of IL-1RAs in the management of recurrent pericarditis.

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects.

Objective: Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition. Methods: We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed. Results: Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5-144.9 months) and all remained asymptomatic. Conclusion: CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.

ModelHeart: a resident-led implementation of metrics to reduce 30-day heart failure readmissions.

Hospitals have been penalised for excessive 30-day readmissions via Medicare payment penalties. As such there has been keen interest in finding ways of reducing readmissions. The basis for the study was a retrospective review of heart failure (HF) admissions at Cleveland Clinic Florida from 1 January 2010 to 31 December 2010. The result of this was a set of metrics associated with >30 day span between admissions: N-terminal pro-brain natriuretic peptide by at least 23%, fluid balance of ≤-1.3 L and sodium ≥135 mEq/L on discharge. The ModelHeart trial was a prospective resident-led validation of these criteria that consisted of education about and implementation of these metrics. A total of 200 patients carrying a diagnosis of HF, admitted between 1 November 2012 and 14 January 2014 were included in the trial. Of the 200 enrolled patients, 94% of discharged patients met at least one criteria, 58% met at least two criteria and 20% met all three. There were forty-eight all-cause 30-day readmissions. 30-day readmission rates between themore than equal to two criteria cohort and the remaining patients were not significantly different (p=0.71). Overall readmission rates were higher in the 2011-2012 retrospective patient pool (19%) versus the ModelHeart cohort (11%), and proportional differences were significant, (p<0.001). This may suggest that education provided sufficient awareness to alter discharge practices outside of the measured metrics. However, the lack of significant differences between groups with respect to discharge metrics suggests that further study is needed to refine the metrics and that reducing HF readmissions involves a continuum of care that spans the inpatient and outpatient setting.

Uremic pericarditis, pericardial effusion, and constrictive pericarditis in end-stage renal disease: Insights and pathophysiology.

A rising prevalence of end-stage renal disease (ESRD) has led to a rise in ESRD-related pericardial syndromes, calling for a better understanding of its pathophysiology, diagnoses, and management. Uremic pericarditis, the most common manifestation of uremic pericardial disease, is a contemporary problem that calls for intensive hemodialysis, anti-inflammatories, and often, drainage of large inflammatory pericardial effusions. Likewise, asymptomatic pericardial effusions can become large and impact the hemodynamics of patients on chronic hemodialysis. Constrictive pericarditis is also well documented in this population, ultimately resulting in pericardiectomy for definitive treatment. The management of pericardial diseases in ESRD patients involves internists, cardiologists, and nephrologists. Current guidelines lack clarity with respect to the management of pericardial processes in the ESRD population. Our review aims to describe the etiology, classification, clinical manifestations, diagnostic imaging tools, and treatment options of pericardial diseases in this population.

A Large Cardiac Papillary Fibroelastoma Arising from the Coumadin Ridge: Unusual Location and Presentation.

•The coumadin ridge is a very unusual location for a cardiac PFE.•Although benign, large, mobile cardiac PFEs can pose a significant embolic risk.•Multimodality CV imaging can differentiate cardiac PFE from other cardiac tumors.