Incidentally Discovered Right Atrial Mass: A Rare and Unexpected Etiology.

Primary cardiac sarcoma is a rare type of intracardiac mass. This report describes a patient with atrial flutter who had a new right atrial mass incidentally discovered on transesophageal echocardiography. A thrombus was suspected based on radiographic appearance, but there was minimal change with anticoagulation. The mass was resected and found to be an undifferentiated pleomorphic cardiac sarcoma, an uncommon sub-type within the already rare category of primary cardiac neoplasms. This report highlights the importance of considering primary malignancy and thoroughly correlating radiographic and clinical evidence during the diagnostic workup of patients with intracardiac masses.

Tricuspid Regurgitation Impact on Outcomes (TRIO): A Simple Clinical Risk Score.

OBJECTIVE: To determine which clinical variables infer the highest risk for mortality in patients with notable tricuspid regurgitation (TR) and to develop a clinical assessment tool (the Tricuspid Regurgitation Impact on Outcomes [TRIO] score). PATIENTS AND METHODS: A single-center retrospective cohort of 13,608 patients with undifferentiated moderate to severe TR at the time of index echocardiography between January 1, 2005, and December 31, 2016, was included. Baseline demographic and clinical data were obtained. Patients were randomly assigned to a training (N=10,205) and a validation (N=3403) cohort. Median follow-up was 6.5 years (interquartile range, 0.8 to 11.0 years). Variables associated with mortality were identified by Cox proportional hazards methods. A geographically distinct cohort of 7138 patients was used for further validation. The primary end point was all-cause mortality over 10 years. RESULTS: The 5-year probability of death was 53% for moderate TR, 63% for moderate-severe TR (hazard ratio [HR], 1.24 [95% CI, 1.17 to 1.31]; P<.001 vs moderate), and 71% for severe TR (HR, 1.55 [95% CI, 1.47 to 1.64]; P<.001 vs moderate). Factors associated with all-cause mortality on multivariate analysis included age 70 years or older, male sex, creatinine level greater than 2 mg/dL, congestive heart failure, chronic lung disease, aspartate aminotransferase level of 40 U/L or greater, heart rate of 90 beats/min or greater, and severe TR. Variables were assigned 1 or 2 points (HR, >1.5) and added to compute the TRIO score. The score was associated with all-cause mortality (C statistic = 0.67) and was able to separate patients into risk categories. Findings were similar in the second, independent and geographically distinct cohort. CONCLUSION: The TRIO score is a simple clinical tool for risk assessment in patients with notable TR. Future prospective studies to validate its use are warranted.

Optogenetic Control of Engrafted Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes in Live Mice: A Proof-of-Concept Study.

Background: Cellular transplantation has emerged as promising approach for treating cardiac diseases. However, a poor engraftment rate limits our understanding on how transplanted cardiomyocytes contribute to cardiac function in the recipient's heart. Methods: The CRISPR/Cas9 technique was employed for stable and constitutive gene expression in human-induced pluripotent stem-cell-derived cardiomyocytes (hiPSC-CMs). Myocardial infarction was induced in adult immunodeficient mice, followed by intramyocardial injection of hiPSC-CMs expressing either CCND2/channelrhodopsin 2 (hiPSC-CCND2OE/ChR2OECMs) or CCND2/luciferase (hiPSC-CCND2OE/LuciOECMs). Six months later, hemodynamics and intramural electrocardiogram were recorded upon blue light illuminations in anesthetized, open-chest mice. Results: Blue light resets automaticity of spontaneously beating hiPSC-CCND2OE/ChR2OECMs in culture, but not that of hiPSC-CCND2OE/LuciOECMs. Response to blue light was also observed in mice carrying large (>106 cells) intracardiac grafts of hiPSC-CCND2OE/ChR2OECM but not in mice carrying hiPSC-CCND2OE/LuciOECMs. The former exhibited single premature ventricular contractions upon light illumination or ventricular quadrigeminy upon second-long illuminations. At the onset of premature ventricular contractions, maximal systolic ventricular pressure decreased while ventricular volume rose concomitantly. Light-induced changes reversed upon resumption of sinus rhythm. Conclusions: We established an in vivo model for optogenetic-based modulation of the excitability of donor cardiomyocytes in a functional, reversible, and localized manner. This approach holds unique value for studying electromechanical coupling and molecular interactions between donor cardiomyocytes and recipient hearts in live animals.

A Case of Severe Mitral Valve Regurgitation in a Patient with Leadless Pacemaker.

An 85-year-old man with cardiac history notable for atrial fibrillation diagnosed 10 years ago which was being treated with atenolol and warfarin presented to our institution with persistent atrial fibrillation. His echocardiogram showed ejection fraction (EF) of 56%, no regional wall motion abnormalities, mild mitral and pulmonary regurgitation, and trivial tricuspid regurgitation. Despite this treatment, he had recurrent episodes of paroxysmal symptomatic atrial fibrillation with a rapid rate requiring multiple emergency department visits and hospital admissions. Given difficulty to control the rate, he underwent atrioventricular (AV) nodal ablation and leadless pacemaker insertion. Fifteen days after the procedure, he was found to have a severe mitral regurgitation murmur.

Unusual Cause of Heart Failure in a Patient with Marfan Syndrome: A Late Complication of Bioprosthetic Valved Graft Replacement.

A high-velocity gradient across the left ventricular outflow tract is most often caused by aortic valve stenosis. We describe the unusual case of a high-velocity gradient caused by a kinked ascending aortic graft in a 69-year-old man who had Marfan syndrome. The patient had a history of ascending aortic aneurysm and had previously undergone replacement of the aortic root and ascending aorta with use of a bioprosthetic valved graft. The kinking was caused by dilation of the native aortic arch. The patient underwent successful hemi-arch replacement and repair of the kinked graft. Late complications and reoperation after proximal aortic surgery in patients with Marfan syndrome are rare, and a high-velocity left ventricular outflow tract gradient caused by the kinking of the aorta is unusual.

Microbubbles in the Aorta and Left Ventricle of a Patient with a Left Ventricular Assist Device: A Unique Presentation of Pump Thrombosis Leading to Urgent Surgery.

Microbubble formation occurs due to the cavitation phenomenon. We report a rare echocardiographic finding of microbubbles in a patient's aorta and left ventricle due to pump thrombosis in the left ventricle assist device, requiring pump exchange surgery.

Choke Attack: Atypical Cardiomyopathy Subsequent to Postnasal Drip.

Midventricular ballooning syndrome, an atypical presentation of takotsubo cardiomyopathy (TCM), presents with transient wall motion abnormalities of the midsegment of the left ventricle with apical sparing. In midventricular TCM, apical contractility is unaffected or may be hyperkinetic in contrast to the typical form of TCM. We report a case of atypical TCM, wherein the patient presented with chest pain following choking and coughing spells due to a postnasal drip.

Left Ventricular Septal Hypertrophy in Elderly Patients With Aortic Stenosis.

OBJECTIVES: Left ventricular (LV) septal hypertrophy in aortic stenosis raises diagnostic and therapeutic questions. However, the etiology and clinical consequences of this finding have not been well studied. The aim of this study was to perform a morphologic evaluation of the LV in aortic stenosis and to investigate the contributing factors and consequences of septal hypertrophy. METHODS: Patients with moderate or severe aortic stenosis were prospectively enrolled. Patients with previous myocardial infarction, wall motion abnormalities, at least moderate valvular regurgitation, known cardiomyopathy, an LV ejection fraction of less than 50%, and age younger than 65 years were excluded. RESULTS: Forty-one patients underwent a final analysis. Septal hypertrophy (LV septal wall thickness ≥15 mm) was confirmed in 21 of 41 patients. The septal hypertrophy group had higher peak aortic valve velocity, a higher diabetes mellitus rate, and a higher rate and longer duration of hypertension than those without septal hypertrophy. The peak aortic valve velocity (odds ratio, 7.1; 95% confidence interval, 1.4-37.1) and diabetes mellitus (odds ratio, 7.4; 95% confidence interval, 1.2-46.2) were the significant factors associated with septal hypertrophy by multivariate analysis. Intraventricular conduction disturbance on electrocardiography was more frequent in the septal hypertrophy group (P = .021). CONCLUSIONS: Left ventricular septal hypertrophy was commonly observed in elderly patients with aortic stenosis, and a higher aortic valve velocity, hypertension, and diabetes mellitus were associated factors. Intraventricular conduction disturbance occurred more often in patients with septal hypertrophy than those without, which implies the pathophysiologic consequence. Further studies are needed to determine the impact of septal hypertrophy and intraventricular conduction disturbance on the prognosis of patients after aortic valve interventions.

Effects of Pectus Excavatum Repair on Right and Left Ventricular Strain.

BACKGROUND: The cardiopulmonary benefits of pectus excavatum repair have been debated. Echocardiographic speckle-tracking strain and strain rate have been used to evaluate and detect subclinical myocardial dysfunction in patients receiving cardiotoxic chemotherapy, and patients with valvular heart disease. This technology was applied to evaluate the effects of pectus excavatum surgery on left ventricular (LV) and right ventricular (RV) function. METHODS: Speckle tracing strain evaluation was performed on intraoperative transesophageal echocardiographic images acquired immediately before and after Nuss repair in adult patients (aged 18 years or more) from 2011 to 2014. Standard severity and compression indices were measured on chest imaging performed before pectus excavatum repair. RESULTS: In total, 165 patients with transesophageal echocardiographic images during repair were reviewed (71.5% male; mean age 33.0 years; range, 18 to 71; Haller index 5.7; range, 2.3 to 24.3). Significant improvement after repair was seen in global RV longitudinal strain (-13.5% ± 4.1% to -16.7% ± 4.4%, p < 0.0001) and strain rate (-1.3 ± 0.4 s-1 to -1.4 ± 0.4 s-1, p = 0.0102); LV global circumferential strain (-18.7% ± 5.7% to -23.5% ± 5.8%, p < 0.0001) and strain rate (-1.5 ± 0.5 s-1 to -1.9 ± 0.8 s-1, p = 0.0003); and LV radial strain (24.1% ± 13.5% to 31.1% ± 16.4%, p = 0.0050). There was a strong correlation between preoperative right atrial compression on transesophageal echocardiogram and improvement in RV global longitudinal strain rate immediately after pectus repair. CONCLUSIONS: Mechanical compression and impaired RV and LV strain is improved by Nuss surgical repair of pectus deformity.

Role of Transesophageal Echocardiography in the Diagnosis of Paradoxical Low Flow, Low Gradient Severe Aortic Stenosis.

BACKGROUND AND OBJECTIVES: Prior studies indicate that up to 35% of cases of severe aortic stenosis (AS) have paradoxical low flow, low gradient despite preserved left ventricular ejection fraction (LVEF). However, error in left ventricular outflow tract (LVOT) diameter may lead to misclassification. Herein, we determined whether measurement of LVOT diameter by transesophageal echocardiography (TEE) results in reclassification of cases to non-severe AS. SUBJECTS AND METHODS: Patients with severe AS with aortic valve area (AVA) <1 cm2 by transthoracic echocardiography (TTE) within 6 months were studied. Paradoxical low flow, low gradient was defined as mean Doppler gradient (MG) <40 mm Hg and stroke volume index (SVI) ≤35 mL/m2. Preserved LVEF was defined as ≥0.50. RESULTS: Among 108 patients, 12 (15%) had paradoxical low flow, low gradient severe AS despite preserved LVEF based on TTE measurement. When LVOT diameter by TEE in 2D was used, only 5 (6.3%) patients had low flow, low gradient severe AS (p<0.001). Coefficients of variability for intraobserver and interobserver measurement of LVOT were <10%. However, the limits of agreement between TTE and TEE measurement of LVOT ranged from 0.43 cm (95% confidence interval [CI]: 0.36 to 0.5) to -0.31 cm (95% CI: -0.38 to -0.23). CONCLUSION: TEE measured LVOT diameter may result in reclassification to moderate AS in some patients due to low prevalence of true paradoxical low flow, low gradient (PLFLG) severe AS.

Predictors of Elevated Cardiac Enzyme Levels in Hospitalized Patients with Atrial Fibrillation and No Known Coronary Artery Disease.

We retrospectively studied the predictive capabilities of elevated cardiac enzyme levels in terms of the prognosis of patients who were hospitalized with atrial fibrillation and who had no known coronary artery disease. Among 321 patients with atrial fibrillation, 60 without known coronary artery disease had their cardiac enzyme concentrations measured during hospitalization and underwent stress testing or cardiac catheterization within 12 months before or after hospitalization. We then compared the clinical and electrocardiographic characteristics of the 20 patients who had elevated cardiac enzyme levels and the 40 patients who had normal levels. Age, sex, and comorbidities did not differ between the groups. In the patients with elevated cardiac enzyme levels, the mean concentrations of troponin T and creatine kinase-MB isoenzymes were 0.08 ± 0.08 ng/mL and 6.49 ± 4.94 ng/mL, respectively. In univariate analyses, only peak heart rate during atrial tachyarrhythmia was predictive of elevated enzyme levels (P <0.0001). Mean heart rate was higher in the elevated-level patients (146 ± 22 vs 117 ± 29 beats/min; P=0.0007). Upon multivariate analysis, heart rate was the only independent predictor of elevated levels. Coronary artery disease was found in only 2 patients who had elevated levels and in one patient who had normal levels (P=0.26). Increased myocardial demand is probably why the presenting heart rate was predictive of elevated cardiac enzyme levels. Most patients with elevated enzyme levels did not have coronary artery disease, and none died of cardiac causes during the 6-month follow-up period. To validate our findings, larger studies are warranted.

Diagnosis and management of patients with asymptomatic severe aortic stenosis.

Aortic stenosis (AS) is a disease that progresses slowly for years without symptoms, so patients need to be carefully managed with appropriate follow up and referred for aortic valve replacement in a timely manner. Development of symptoms is a clear indication for aortic valve intervention in patients with severe AS. The decision for early surgery in patients with asymptomatic severe AS is more complex. In this review, we discuss how to identify high-risk patients with asymptomatic severe AS who may benefit from early surgery.

Visualization of atrial septal aneurysm and patent foramen ovale by three-dimensional transesophageal echocardiography.

: Transesophageal echocardiography is frequently performed for further evaluation of sources of embolism and better evaluation of atrial septum in patients with cerebral ishemic events. Although two-dimensional transesophageal echocardiography can depict atrial septal aneurysm and patent foramen ovale, the full extent of the patent foramen ovale cannot be easily discerned in some cases. We describe a patient with transient cerebral ischemia where three-dimensional echocardiography provided incremental value when compared to two-dimensional transesophageal echocardiography in the assessment of atrial septal aneurysm and patent foramen ovale.

Visual Estimation of the Severity of Aortic Stenosis and the Calcium Burden by 2-Dimensional Echocardiography: Is It Reliable?

OBJECTIVES: Guidelines have recommended aortic valve surgery in asymptomatic patients with severe aortic stenosis and a large aortic valve calcium burden. The purpose of this study was to determine whether visual assessment of aortic valve calcium and stenosis severity are reliable based on 2-dimensional echocardiography alone. METHODS: We prospectively enrolled 68 patients with aortic stenosis and compared them with 30 control participants without aortic stenosis. All had aortic valve calcium score assessment by computed tomography. In a random order, 2-dimensional images without hemodynamic data were independently reviewed by 2 level 3-trained echocardiographers, who then classified these patients into categories based on aortic valve calcium and stenosis severity. RESULTS: The 68 patients (mean age ± SD, 74 ± 10 years) were classified as having mild (n = 28), moderate (n = 22), and severe (n = 18) aortic stenosis. When the observers were asked to grade the degree of valve calcification, the agreement between them was poor (κ = 0.33-0.39). The visual ability to determine stenosis severity compared with Doppler echocardiography had high specificity (81% and 88% for observers 1 and 2). However, sensitivity was unacceptably low (56%-67%), and the positive predictive value was poor (44%-50%). Agreement was fair (κ= 0.58-0.69) between the observers for determining severe stenosis. CONCLUSIONS: Our results suggest that visual assessment of aortic valve calcium has high interobserver variability; the visual ability to determine severe aortic stenosis has low sensitivity but high specificity. Our results may have important implications for treatment of patients with aortic stenosis and guiding the use of handheld echocardiography. Further research with larger cohorts is needed to validate the variability, sensitivity, and specificity reported in our study.

Does valvuloarterial impedance impact prognosis after surgery for severe aortic stenosis in the elderly?

BACKGROUND: Valvuloarterial impedance (Zva) was introduced as a prognostic measure in patients with aortic stenosis (AS). However, it is unclear whether Zva has a prognostic impact on survival after surgical aortic valve replacement (AVR) in patients with severe AS with preserved ejection fraction (EF). METHODS: We retrospectively reviewed 929 consecutive patients who had AVR. We investigated 170 elderly patients (age >65 years, mean 76 years) who had AVR secondary to severe AS (mean gradient ≥40 mm Hg; aortic valve area ≤1 cm(2); peak velocity ≥4 m/s). Patients with EF <50%, greater than moderate aortic regurgitation, prior heart surgery and concomitant mitral or tricuspid valve surgery were excluded. Zva was calculated and the patients were divided into two groups; low Zva, Zva <4.3 (n=82) and high Zva, Zva ≥4.3 (n=88). The end point was all-cause of death. Survival curves were calculated according to Kaplan-Meier method. RESULTS: Age, prevalence of hypertension, diabetes, chronic kidney disease (CKD), atrial fibrillation, symptoms, EF, E/e' and concomitant coronary artery bypass graft were not different between the groups. Survival was not different between the groups at 5 years (70% in low Zva and 81% in high Zva; p=0.21) and for the entire follow-up period (p=0.23). Only age was a significant factor in predicting survival by multivariate analyses in Cox proportional hazards model after adjusting for Zva, CKD, atrial fibrillation and hypertension. CONCLUSIONS: Our results suggest that preoperative Zva does not have a prognostic impact on postoperative survival in elderly patients with severe AS with preserved EF. Further investigation is needed to elucidate the controversial results.