A Case Series of Minimally Invasive Robotic-Assisted Resection of Cardiac Papillary Fibroelastoma: The Mayo Clinic Experience.

Papillary fibroelastomas (PFEs) are small, slowly growing benign cardiac tumors with clinically significant risk of embolization. Surgical excision is the definitive treatment of symptomatic PFE and is conventionally performed through a median sternotomy. In this study, we report a series of 12 patients, who underwent robotic-assisted PFE removal at the Mayo Clinic. PFE involved the mitral valve, left atrium, and tricuspid valve. No major complications occurred after the procedure, and most patients were discharged 4 days after the surgery. On follow-up, 1 patient demonstrated pericarditis.

Case Report: A myxoma with a far reach.

A 73-year-old woman presented to the emergency department with a syncopal episode and a history of dizzy spells. A transthoracic echocardiogram demonstrated a large left atrial mass extending into the right upper pulmonary veins. Subsequently, cardiac magnetic resonance imaging and coronary computed tomography angiography with three-dimensional reconstruction and printing of the heart and mass were performed, which demonstrated a high index of suspicion for an atypical left atrial myxoma. The mass was excised robotically, and the pathology report confirmed a diagnosis of myxoma.

Case report: New is not always better: treatment of non-bacterial thrombotic endocarditis.

An elderly female with metastatic adenocarcinoma of the lung and atrial fibrillation presented with multiple embolic strokes while on anticoagulation with Apixaban. After further investigation, a TEE showed lesions of non-bacterial thrombotic endocarditis on the mitral valve. A decision to switch the patient to LMWH for anticoagulation was then made and a follow-up TEE showed resolution of the NBTE. In this abstract, we show that heparin should remain as the anticoagulation agent of choice in the setting of NBTE associated with malignancy.

Impact of atrial fibrillation on outcomes in asymptomatic severe aortic stenosis: a propensity-matched analysis.

Background: Atrial fibrillation (AF) portends poor prognosis in patients with aortic stenosis (AS). Objectives: This study aimed to study the association of AF vs. sinus rhythm (SR) with outcomes in asymptomatic severe AS during routine clinical practice. Methods: We identified 909 asymptomatic patients from 3,208 consecutive patients with aortic valve area ≤1.0 cm2 and left ventricular ejection fraction ≥50% at a tertiary academic center. Patients were grouped by rhythm at the time of transthoracic echocardiogram [SR: 820/909 (90%) and AF: 89/909 (10%)]. Propensity-matched analyses (2 SR:1 AF) matching 174 SR to 89 AF patients by age, sex, and clinical comorbidities were used to compare outcomes. Results: In the propensity-matched cohort, median age (82 ± 8 vs. 81 ± 9 years, p = 0.31), sex distribution (male 58% vs. 52%, p = 0.30), and Charlson comorbidity index (4.0 vs. 3.0, p = 0.26) were not different in AF vs. SR. Median follow-up duration was 2.6 (IQR: 1.0-4.4) years. The 1-year rate of aortic valve replacement (AVR) was not different (AF: 32% vs. SR: 37%, p = 0.31). All-cause mortality was higher in AF [hazard ratio (HR): 1.68 (1.13-2.50), p = 0.009]. Independent predictors of mortality were age [HR: 1.92 (1.40-2.62), p < 0.001], Charlson comorbidity index [1.09 (1.03-1.15), p = 0.002], aortic valve peak velocity [HR: 1.87 (1.20-2.94), p = 0.006], stroke volume index [HR: 0.75 (0.60-0.93), p = 0.01], moderate or more mitral regurgitation [HR: 2.97 (1.43-6.19), p = 0.004], right ventricular systolic dysfunction [HR: 2.39 (1.29-4.43), p = 0.006], and time-dependent AVR [HR: 0.36 (0.19-0.65), p = 0.0008]. There was no significant interaction of AVR and rhythm (p = 0.57). Conclusions: Lower forward flow, right ventricular systolic dysfunction, and mitral regurgitation identified increased risk of subsequent mortality in asymptomatic patients with AF and AS. Additional studies of risk stratification of asymptomatic AS in AF vs. SR are needed.

Uric Acid Is Associated with Worsening of Diastolic Function and Adverse Outcomes in Patients with Coronary Slow Flow.

OBJECTIVE: The impact of uric acid on worsening of diastolic function and clinical outcomes in patients with coronary slow flow remains unclear. This study aims to investigate possible associations between serum uric acid, worsening of diastolic function, and major adverse cardiovascular events in coronary slow flow patients. METHODS: Blood samples were obtained prospectively from 537 patients who had been angiographically diagnosed with coronary slow flow. Of those, 425 patients underwent comprehensive cardiac function assessment both before and after maximal treadmill exertion by stress echocardiography. The association between serum uric acid and major adverse cardiovascular events was examined using Cox proportional hazards regression model. RESULTS: Among the 425 patients (mean age: 58 ± 11 years; 52.2% men), worsening of diastolic function occurred in 176 (41.4%) after exercise stress. Patients with worsening of diastolic function had elevated levels of serum uric acid compared to those without (5.7 [4.1, 6.7] vs 4.3 [3.6, 5.3] mg/dL, respectively; P <.001). Higher serum uric acid levels were also significantly associated with neutrophil counts and high-sensitive C-reactive protein in patients with worsening of diastolic function but not in those without. Multivariate regression analysis found serum uric acid to be an independent predictor of worsening of diastolic function (odds ratio = 1.87 [1.17-3.82], P =.023). Moreover, serum uric acid remained associated with major adverse cardiovascular events even after adjusting for echocardiographic and clinical variables (hazard ratio = 1.56 [1.03-2.89], P =.016). CONCLUSION: Serum uric acid is associated with worsening of diastolic function and may be mediated by inflammation. These findings indicate that uric acid is a risk factor for major adverse cardiovascular events in patients with coronary slow flow.

Underestimation of Aortic Stenosis Severity by Doppler Mean Gradient during Atrial Fibrillation: Insights from Aortic Valve Weight.

BACKGROUND: Doppler mean gradient (MG) can underestimate aortic stenosis (AS) severity when obtained during atrial fibrillation (AF) compared with sinus rhythm (SR). Aortic valve weight (AVW) is a flow-independent measure of AS severity. The objective of this study was to determine whether AVW or AVW/MG ratio was increased in AF versus SR in patients with AS. METHODS: Excised native aortic valves from 495 consecutive patients (median age, 77 years; interquartile range [IQR], 71-82 years; 40% women), with left ventricular ejection fractions ≥50% who underwent surgical aortic valve replacement for native valve severe AS (aortic valve area ≤ 1 cm2 or indexed aortic valve area ≤ 0.6 cm2/m2) were weighed. Excised AVW/MG ratios were compared in AF versus SR in patients with high-gradient AS (aortic peak velocity ≥ 4 m/sec or MG ≥ 40 mm Hg) and low-gradient AS (aortic peak velocity < 4 m/sec and MG < 40 mm Hg) in sex-specific analyses. RESULTS: AF was present in 51 patients (10%; 11 of 51 [22%] had low-gradient AS) and SR in 444 (90%; 23 of 444 [5%] had low-gradient AS). There was no difference in sex distribution between AF and SR. Aortic valve area was not different, but forward stroke volume index and transaortic valve flow rate were lower in AF (P ≤ .002 for all); MG was lower in AF versus SR (median, 46 mm Hg [IQR, 37-50 mm Hg] vs 50 mm Hg [IQR, 44-61 mm Hg]; P < .0001). Overall AVW was not different (median, 2,290 mg [IQR, 1,830-3,063 mg] vs 2,140 mg [IQR, 1,530-2,958 mg]; P = .31), but overall AVW/MG ratio was higher in AF (median, 55 [IQR, 41-67] vs 42 [IQR, 30-55]; P = .001). In sex- and MG-specific analyses, the AVW/MG ratio was higher in AF compared with SR in men with high-gradient AS (median, 58 [IQR, 41-75] vs 51 [IQR, 39-61]; P = .03), but the differences were not statistically significant between AF and SR in other groups. CONCLUSIONS: AVW was discordant to Doppler MG in AF compared with SR in men with high-gradient AS. Additional studies of the relationship of MG to other measures of AS severity, such as leaflet fibrosis, are needed.

Comparison of and Frequency of Mortality, Left Ventricular Assist Device Implantation, Ventricular Arrhythmias, and Heart Transplantation in Patients With Familial Versus Nonfamilial Idiopathic Dilated Cardiomyopathy.

We postulated that familial idiopathic dilated cardiomyopathy (F-IDC) is associated with a worse prognosis than nonfamilial IDC (nonF-IDC). Patients with F-IDC had either a strong family history and/or proved genetic mutations. We studied long-term prognosis (mean follow-up: 6.1 ± 4.1 years) of 162 patients with IDC (age: 55.5 ± 17.9 years, men: 57.8%, 50% F-IDC) with an implantable cardioverter-defibrillator or cardiac resynchronization therapy. The primary end point was a composite of death, left ventricular (LV) assist device implant, or heart transplantation. The secondary end point was a ventricular arrhythmia event. There was no significant difference in the prevalence of diabetes, hypertension, New York Heart Association class, medical therapy, and years of follow-up between the F-IDC and nonF-IDC groups. Patients with F-IDC were younger than patients with nonF-IDC (49.1 ± 17.0 years vs 61.6 ± 16.5 years, p <0.001). Mean LV ejection fraction was significantly lower in F-IDC group than in the nonF-IDC group (26 ± 12% vs 31 ± 12%, p = 0.022). The primary end point was achieved in 54 patients in F-IDC group (66.7%) versus 19 in the nonF-IDC group (23.5%) (p <0.001). The Kaplan-Meier survival estimates for the composite end point and for ventricular arrhythmia were significantly lower in the F-IDC versus nonF-IDC (log-rank p ≤0.001 and 0.04, respectively). F-IDC was the only multivariable predictor of the primary composite end point (hazard ratio 3.419 [95% confidence interval 1.845 to 6.334], p <0.001). The likelihood of LV remodeling manifested by LV ejection fraction improvement (≥10%) was significantly lower in F-IDC than nonF-IDC (27.1% vs 44.8%, p = 0.042). In conclusion, F-IDC is a predictor of mortality, need for LV assist device, or heart transplantation. F-IDC is associated with significantly lower event-free survival for primary end point and ventricular arrhythmia than nonF-IDC. F-IDC has significantly lower likelihood of LV reverse remodeling than nonF-IDC.

Recurrent Parotid Adenocarcinoma Presenting as Diffuse Myocardial Metastatic Disease.

A 64-year-old man who had undergone treatment for left parotid adenocarcinoma presented with progressive exertional shortness of breath. Evaluation revealed metastatic invasion of the myocardium as a rare presentation of recurrent parotid adenocarcinoma. This case highlights the importance of using multimodal imaging methods in diagnostic evaluation and a collaborative multidisciplinary approach in managing patient care.

Clinical and echocardiographic characteristics of patients with pathology proven right-sided papillary fibroelastomas.

BACKGROUND: Papillary fibroelastoma (PFE) are one of the most common primary cardiac neoplasms. They are most commonly found on cardiac valves especially on the left side of the heart and have been associated with an increased risk of embolization. To date, no large studies looked at the clinical significance of right-sided PFE (R-PFE). Therefore, we sought to better characterize patients with R-PFE and its clinical sequelae. METHODS: We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with R-PFE were analyzed. Medical records and echocardiograms were reviewed for clinical and PFE characteristics. RESULTS: Twenty-nine patients with R-PFE (mean age 70 ± 11 years; 62% women) were included in the analysis. PFEs were located on the right atrial (RA) wall in 31%, tricuspid valve in 34%, right ventricular (RV) wall in 7%, RV outflow tract in 4%, and pulmonary valve in 10% of patients. The remaining patients (14%) had multifocal PFEs. Around 38% of patients had cancer diagnosed before PFE diagnosis, and 34% had associated congenital heart disease. Seventeen (59%) patients had angina or dyspnea at the time of the presentation, and 3 (10%) had embolic symptoms. One patient with PFE located on the RA with associated patent foramen ovale had a stroke at time of presentation. Two patients, one with PFE on the RA wall and another patient with PFE at the tricuspid valve, had pulmonary embolism at the time of presentation. Median maximal length for PFE varied by location, ranging from a media of 8 mm to 16 mm. Of the 12 patients with follow up echocardiogram 1 year after PFE removal; 3/12 (25%) had documented PFE recurrence. CONCLUSION: R-PFE can be present in patients thromboembolic events when there is a right-to-left shunt. They can be a rare cause of pulmonary embolism, and at least in those that had follow-up echocardiograms, had a significant recurrence rate.

Recurrence of Pathologically Proven Papillary Fibroelastoma.

BACKGROUND: Papillary fibroelastoma (PFE) is the most common primary benign cardiac tumor. Because PFEs have the potential to embolize, they often are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. We aimed to determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. METHODS: We retrospectively identified all patients from a single center with pathologically proven PFE, treated from January 1995 through December 2018. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. We compared echocardiographic images obtained intraoperatively (after excision) and at dismissal with those of the most recent examination to assess the possibility of PFE recurrence. RESULTS: We included 98 patients in the study. The mean duration of follow-up was 5.4 (SD 3.7) years (range, 1 to 17); the median duration of follow-up was 4.3 years (interquartile range, 1.9 to 7.7). Twelve patients (12.2%) had echocardiographically supported PFE recurrence. Three patients had the recurrent lesion surgically reexcised, and pathologic analysis showed that two were recurrent PFEs and one was a Lambl excrescence. Initial clinical presentation of stroke or transient ischemic attack was more common for the recurrence group (for the first PFE) than for the nonrecurrence group (83% vs 26%; P < .001). CONCLUSIONS: Contrary to findings from previous studies, PFEs do recur after surgical excision. These findings emphasize the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses.

Case Report: A Tale of a Cardiac Mass: Looks Like a Papillary Fibroelastoma, Acts Like a Non-bacterial Thromboendocarditis.

Introduction: Benign cardiac tumors and tumor like conditions are a heterogeneous collection of mass lesions that vary widely in their characteristics, such as presentation, size, and location. In some instances, these tumors are found incidentally, and therefore a broad differential diagnosis should be considered. Case: An elderly male with significant unintentional weight loss and a high risk for cancer presented with an incidental valvular cardiac mass. The mass was thought to be a non-bacterial thromboendocarditis on initial clinical evaluation. After multiple imaging modalities, the mass was suspected to be a papillary fibroelastoma (PFE), which was resected due to high stroke risk and multiple previous chronic infarcts on brain MRI. Conclusion: This case highlights the need for a comprehensive cardiac evaluation of a valvular tumor to discern the etiology and rule out other underlying pathophysiological processes that may require alternative interventions to cardiac surgery.

Blood lead level in Chinese adults with and without coronary artery disease.

BACKGROUND: The Trial to Assess Chelation Therapy study found that edetate disodium (disodium ethylenediaminetetraacetic acid) chelation therapy significantly reduced the incidence of cardiac events in stable post-myocardial infarction patients, and a body of epidemiological data has shown that accumulation of biologically active metals, such as lead and cadmium, is an important risk factor for cardiovascular disease. However, limited studies have focused on the relationship between angiographically diagnosed coronary artery disease (CAD) and lead exposure. This study compared blood lead level (BLL) in Chinese patients with and without CAD. METHODS: In this prospective, observational study, 450 consecutive patients admitted to Beijing Anzhen Hospital with suspected CAD from November 1, 2018, to January 30, 2019, were enrolled. All patients underwent coronary angiography, and an experienced heart team calculated the SYNTAX scores (SXscore) for all available coronary angiograms. BLLs were determined with atomic absorption spectrophotometry and compared between patients with angiographically diagnosed CAD and those without CAD. RESULTS: In total, 343 (76%) patients had CAD, of whom 42% had low (0-22), 22% had intermediate (23-32), and 36% had high (≥ 33) SXscore. BLLs were 36.8 ± 16.95 µg/L in patients with CAD and 31.2 ± 15.75 µg/L in those without CAD (P = 0.003). When BLLs were categorized into three groups (low, middle, high), CAD prevalence increased with increasing BLLs (P < 0.05). In the multivariate regression model, BLLs were associated with CAD (odds ratio (OR): 1.023, 95% confidence interval (CI): 1.008-1.039; P = 0.0017). OR in the high versus low BLL group was 2.36 (95% CI: 1.29-4.42,P = 0.003). Furthermore, BLLs were independently associated with intermediate and high SXscore (adjusted OR: 1.050, 95% CI: 1.036-1.066; P < 0.0001). CONCLUSION: BLLs were significantly associated with angiographically diagnosed CAD. Furthermore, BLLs showed excellent predictive value for SXscore, especially for complex coronary artery lesions.

Lipocalin 2: could it be a new biomarker in pediatric pulmonary hypertension associated with congenital heart disease?

The role of lipocalin 2 (LCN2) in pulmonary hypertension (PH) in pediatric patients with congenital heart disease (CHD) remains unclear. We sought to investigate whether LCN2 could be a potential biomarker for PH in pediatric patients who underwent surgery for CHD. From December 2018 to February 2020, patients undergoing surgical repair for congenital defects with and without PH were identified. Healthy children without CHD and PH served as controls. A mean pulmonary artery pressure (mPAP) >20 mmHg was used as the definition of PH. Blood samples and echocardiograms were obtained in all patients and right heart catheterization was performed in 79 patients. Multivariable logistic regression analysis was used to determine potential predictors for PH. Among 102 patients, the median age was 10 [Interquartile range (IQR) 7.0-13] months, and 37.5% were female. Compared to non-PH patients and controls, PH patients showed elevated levels of LCN2 (P < 0.001). In addition, LCN2 levels positively correlated with the invasive haemodynamic indices of PH. In univariate regression, LCN2 (odds ratio = 2.69 [1.06-5.31], P < 0.001), N-Terminal pro Brain Natriuretic Peptide (NT-proBNP) (OR = 1.91 [1.21-7.56], P = 0.03) and high-sensitive troponin T (hsTnT) (OR = 1.36 [1.01-3.57], P = 0.01) were associated with PH; however, only LCN2 (OR = 1.68 [1.04-4.52], P = 0.03) was significantly associated with PH on multivariate analysis. In conclusion, children with PH had increased LCN2 expression. LCN2 levels positively correlated with invasive indices of PH. These results indicate LCN2 could be a useful biomarker for prediction of PH in pediatric CHD cases.

High Prevalence of Severe Aortic Stenosis in Low-Flow State Associated With Atrial Fibrillation.

BACKGROUND: Atrial fibrillation (AF) is a low-flow state and may underestimate aortic stenosis (AS) severity. Single-high Doppler signals (HS) consistent with severe AS (peak velocity ≥4 m/s or mean gradient ≥40 mm Hg) are averaged down in current practice. The objective for the study was to determine the significance of HS in AF low-gradient AS (LGAS). METHODS: One thousand five hundred forty-one patients with aortic valve area ≤1 cm2 and left ventricular ejection fraction ≥50% were identified and classified as high-gradient AS (HGAS) (≥40 mm Hg) and LGAS (<40 mm Hg), and AF versus sinus rhythm (SR). Available computed tomography aortic valve calcium scores (AVCS) were retrieved from the medical record. Outcomes were assessed. RESULTS: Mean age was 76±11 years, female 47%. Mean gradient was 51±12 in SR-HGAS, 48±10 in AF-HGAS, 31±5 in SR-LGAS, and 29±7 mm Hg in AF-LGAS, all P≤0.001 versus SR-HGAS; HS were present in 33% of AF-LGAS. AVCS were available in 34%. Compared with SR-HGAS (2409 arbitrary units; interquartile range, 1581-3462) AVCS were higher in AF-HGAS (2991 arbitrary units; IQR1978-4229, P=0.001), not different in AF-LGAS (2399 arbitrary units; IQR1817-2810, P=0.47), and lower in SR-LGAS (1593 arbitrary units; IQR945-1832, P<0.001); AVCS in AF-LGAS were higher when HS were present (P=0.048). Compared with SR-HGAS, the age-, sex-, comorbidity index-, and time-dependent aortic valve replacement-adjusted mortality risk was higher in AF-HGAS (hazard ratio=1.82 [1.40-2.36], P<0.001) and AF-LGAS with HS (hazard ratio=1.54 [1.04-2.26], P=0.03) but not different in AF-LGAS without HS or SR-LGAS (both P=not significant). CONCLUSIONS: Severe AS was common in AF-LGAS. AVCS in AF-LGAS were not different from SR-HGAS. AVCS were higher and mortality worse in AF-LGAS when HS were present.

CAD-LT score effectively predicts risk of significant coronary artery disease in liver transplant candidates.

BACKGROUND & AIMS: Patients with cirrhosis and significant coronary artery disease (CAD) are at risk of peri-liver transplantation (LT) cardiac events. The coronary artery disease in liver transplantation (CAD-LT) score and algorithm aim to predict the risk of significant CAD in LT candidates and guide pre-LT cardiac evaluation. METHODS: Patients who underwent pre-LT evaluation at Indiana University (2010-2019) were studied retrospectively. Stress echocardiography (SE) and cardiac catheterization (CATH) reports were reviewed. CATH was performed for predefined CAD risk factors, irrespective of normal SE. Significant CAD was defined as CAD requiring percutaneous or surgical intervention. A multivariate regression model was constructed to assess risk factors. Receiver-operating curve analysis was used to compute a point-based risk score and a stratified testing algorithm. RESULTS: A total of 1,771 pre-LT patients underwent cardiac evaluation, including results from 1,634 SE and 1,266 CATH assessments. Risk-adjusted predictors of significant CAD at CATH were older age (adjusted odds ratio 1.05; 95% CI 1.03-1.08), male sex (1.69; 1.16-2.50), diabetes (1.57; 1.12-2.22), hypertension (1.61; 1.14-2.28), tobacco use (pack years) (1.01; 1.00-1.02), family history of CAD (1.63; 1.16-2.28), and personal history of CAD (6.55; 4.33-9.90). The CAD-LT score stratified significant CAD risk as low (≤2%), intermediate (3% to 9%), and high (≥10%). Among patients who underwent CATH, a risk-based testing algorithm (low: no testing; intermediate: non-invasive testing vs. CATH; high: CATH) would have identified 97% of all significant CAD and potentially avoided unnecessary testing (669 SE [57%] and 561 CATH [44%]). CONCLUSIONS: The CAD-LT score and algorithm (available at www.cad-lt.com) effectively stratify pre-LT risk for significant CAD. This may guide more targeted testing of candidates with fewer tests and faster time to waitlist. LAY SUMMARY: The coronary artery disease in liver transplantation (CAD-LT) score and algorithm effectively stratify patients based on their risk of significant coronary artery disease. The CAD-LT algorithm can be used to guide a more targeted cardiac evaluation prior to liver transplantation.

Increased Risk of Valvular Heart Disease in Systemic Sclerosis: An Underrecognized Cardiac Complication.

OBJECTIVE: Cardiac involvement is a poor prognostic marker in systemic sclerosis (SSc). While diastolic dysfunction, myocardial fibrosis, and arrhythmias are traditionally considered features of primary cardiac involvement in SSc, the incidence of valvular heart disease (VHD) is not well reported. Our objective was to examine the prevalence of VHD at the time of SSc diagnosis and incidence of VHD during follow-up compared to non-SSc subjects. METHODS: Medical records of patients with suspicion of SSc were reviewed to identify incident cases. SSc subjects were matched 1:2 by age and sex to non-SSc subjects. RESULTS: The study included 78 incident SSc cases and 156 non-SSc comparators (56 yrs [± 15.7], 91% female). A nearly 4-fold increase in the prevalence of moderate/severe VHD prior to SSc diagnosis compared to non-SSc subjects (6% vs 0%; P = 0.004) was identified. During follow-up, 18 SSc and 12 non-SSc patients developed moderate/severe VHD. The cumulative incidence of VHD at 10 years after SSc incidence/index was 17.9% (95% CI 10.7-29.9) in patients with SSc compared with 2.3% (95% CI 0.7-7.0) in non-SSc subjects (HR 4.23, 95% CI 2.03-8.83). Coronary artery disease was the only significant risk factor for VHD. CONCLUSION: Patients with SSc have a 4-fold increase in the prevalence of moderate/severe VHD at diagnosis compared to non-SSc patients. They also have a 4-fold increased risk of developing moderate/severe VHD after diagnosis of SSc. Aortic stenosis and mitral regurgitation have a much higher prevalence in patients with SSc, besides secondary tricuspid regurgitation. Underlying mechanisms for this association require further elucidation.