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.

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.

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.

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.

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.

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.

Valvular heart disease in patients with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) or "brittle bone disease" is a rare connective tissue hereditary disorder. The most common clinical presentation of OI is bone fractures. OI also involves extraskeletal structures; however, cardiovascular manifestations are rare. In this report, we describe the cardiovascular anomalies of patients with OI who underwent valve surgery and review the literature on this subject.

Effect of mitral inflow pattern on diagnosis of severe mitral regurgitation in patients with chronic organic mitral regurgitation.

BACKGROUND: To determine sensitivity and specificity of E wave velocity in patients with severe chronic organic mitral regurgitation (MR) and normal left ventricular ejection fraction (EF) and to evaluate prevalence of A wave dominance in patients with severe MR. METHODS: We compared 35 patients with quantified severe, chronic, quantified, organic MR due to flail/prolapsed leaflets who had reparative surgery with 35 age-matched control subjects. EXCLUSION CRITERIA: EF < 60%, atrial fibrillation, and more than mild aortic regurgitation. RESULTS: Mean [standard deviation (SD)] age [70 (8) years vs. 69 (8) years; p = 0.94] and mean (SD) EF [66% (6%) vs. 65% (4%); p = 0.43] were not different between the two groups. Mean (SD) E wave velocity was greater in case patients than control subjects [1.2 (0.3) m/sec vs. 0.7 (0.15) m/sec; p < 0.001]. However, E wave velocity of 1.2 m/sec had a sensitivity of only 57% [95% confidence interval (CI), 41-7 and a specificity of 100% (95% CI, 90-100%) in identifying severe MR. E wave velocity of 0.9 m/sec had a more optimal combined sensitivity (89%; 95% CI, 74-95%) and specificity (86%; 95% CI, 71-94%). A wave dominance was seen in 18% of case patients and 66% of control subjects (p < 0.001). CONCLUSION: E wave velocity of 1.2 m/sec is specific not sensitive for severe organic MR; E wave velocity of 0.9 m/sec has better sensitivity and specificity. A wave dominance pattern alone cannot exclude patients with severe organic MR. Our findings highlight the importance of a comprehensive echocardiographic exam rather than relying on a few Doppler parameters in diagnosing MR.

Aorto-right ventricular fistula: a complication of aortic valve replacement.

The occurrence of aorto-right ventricular (aorto-RV) fistula after prosthetic aortic valve replacement is rare. Transthoracic echocardiography (TTE) with color-flow Doppler, transesophageal echocardiography (TEE), or both may be required for diagnosis. A 42-year-old woman sought care for palpitations and dyspnea due to atrial flutter 2 weeks after prosthetic aortic valve replacement and graft replacement of the ascending aorta. TTE and TEE revealed left-to-right shunt due to aorto-RV fistula.

Valvular heart disease with the use of fenfluramine-phentermine.

Exposure to the anorectic drug fenfluramine, alone or in combination with phentermine, a noradrenergic central nervous system stimulant, has been associated with unusual cardiac valvular morphology and resultant regurgitation of the left- and right-sided heart valves. The prevalence of significant valvular disease associated with the use of these anorectic drugs is reported to be as high as 23%. Herein, we report the occurrence of multivalvular disease and pulmonary hypertension associated with fenfluramine-phentermine use, discovered in an obese 59-year-old woman before expected gastric bypass surgery.

Symptomatic pericardial cyst: a case series.

Pericardial cysts are most commonly located at the cardiophrenic angle or, rarely, in the posterior or anterior superior mediastinum. The majority of pericardial cysts are asymptomatic and are found incidentally. Symptomatic pericardial cysts present with dyspnoea, chest pain, or persistent cough. We describe four patients with symptomatic pericardial cysts who were treated with either echocardiographically guided percutaneous aspiration or video-assisted thoracoscopic surgery, or both; thoracotomy; or conservative therapy.

An unusual presentation of hemolytic anemia in a patient with prosthetic mitral valve.

Although rare, periprosthetic valvular regurgitation can cause hemolytic anemia. We present the case of a 63-year-old man who had an unusual presentation of hemolytic anemia due to periprosthetic mitral valve regurgitation (PMVR) in the presence of cold agglutinins. Due to high surgical risk, PMVR was percutaneously closed with three Amplatzer devices under the guidance of three-dimensional transesophageal echocardiography.