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Management of patients with hypertrophic obstructive cardiomyopathy and severe mitral annular calcification can be challenging. Our cases highlight the importance of addressing all elements contributing to left ventricular outflow tract obstruction in cases of hypertrophic obstructive cardiomyopathy: hypertrophic basal interventricular septum, abnormal papillary muscles, and systolic anterior motion of the anterior mitral valve leaflet. Addressing mitral valve repair through aortotomy by performing a septal myectomy, papillary muscle realignment, and resection of aberrant chordae tendineae allows left ventricular outflow tract obstruction and systolic anterior motion to be successfully corrected. The success of these procedures depended on preoperative imaging and intraoperative provocation. [ABSTRACT FROM AUTHOR]

Background: Calcific aortic valve disease (CAVD) is the most common cause of acquired valve disease. Initial phases of CAVD include thickening of the cusps, whereas advanced stages are associated with biomineralization and reduction of the aortic valve area. These conditions are known as aortic valve sclerosis (AVSc) and aortic valve stenosis (AVS), respectively. Because of its asymptomatic presentation, little is known about the molecular determinants of AVSc. The aim of this study was to correlate plasma and tissue osteopontin (OPN) levels with echocardiographic evaluation for the identification of asymptomatic patients at risk for CAVD. In addition, our aim was to analyze the differential expression and biological function of OPN splicing variants as biomarkers of early and late stages of CAVD. Methods: From January 2010 to February 2011, 310 patients were enrolled in the study. Patients were divided into 3 groups based on transesophageal echocardiographic (TEE) evaluation: controls (56 patients), AVSc (90 patients), and AVS (164 patients). Plasma and tissue OPN levels were measured by immunohistochemical evaluation, enzyme-linked immunosorbent assay (ELISA), and real-time quantitative polymerase chain reaction (qPCR). Results: Patients with AVSc and AVS have higher OPN levels compared with controls. OPN levels are elevated in asymptomatic patients with AVSc with no appearance of calcification during TEE evaluation. OPN splicing variants OPN-a, OPN-b, and OPN-c are differentially expressed during CAVD progression and are able to inhibit biomineralization in a cell-based biomineralization assay. Conclusions: The analysis of the differential expression of OPN splicing variants during CAVD may help in developing diagnostic and risk stratification tools to follow the progression of asymptomatic aortic valve degeneration. [ABSTRACT FROM AUTHOR]

Objective: The inflammatory process of aortic stenosis involves the differentiation of aortic valve myofibroblasts into osteoblasts. Osteopontin, a proinflammatory glycoprotein, both stimulates differentiation of myofibroblasts and regulates the deposition of calcium by osteoblasts. Osteopontin levels are increased in patients with such conditions as end-stage renal disease, ectopic calcification, and autoimmune disease. We hypothesized that increased plasma osteopontin levels might be associated with the presence of aortic valve calcification and stenosis. Methods: Venous blood from volunteers older than 65 years undergoing routine echocardiographic analysis or aortic valve surgery for aortic stenosis was collected. Plasma osteopontin levels were measured by means of enzyme-linked immunosorbent assay. The presence of aortic stenosis was defined as an aortic valve area of less than 2.0 cm2. Aortic valve calcification was assessed by using a validated echocardiographic grading system (1, none; 2, mild; 3, moderate; 4, severe). Comparisons were performed with nonpaired t tests. Results: Aortic stenosis was present in 23 patients (mean age, 78 years) and was absent in 7 patients (mean age, 72 years). Aortic valve calcification scores were 3.5 ± 0.6 and 1.3 ± 0.5 in patients with and without aortic stenosis, respectively (P < .001). Patients with no or mild aortic valve calcification had lower osteopontin levels compared with patients with moderate or severe aortic valve calcification (406.1 ± 165.8 vs 629.5 ± 227.5 ng/mL, P = .01). Similarly, patients with aortic stenosis had higher osteopontin levels compared with patients without aortic stenosis (652.2 ± 218.7 vs 379.7 ± 159.9 ng/mL, P < .01). Conclusion: Increased levels of plasma osteopontin are associated with the presence of aortic valve calcification and stenosis. These findings suggest that osteopontin might play a functional role in the pathogenesis of calcific aortic stenosis. [Copyright &y& Elsevier]

Localized pericardial hematomas after cardiac surgery may have atypical clinical presentations due to regional alterations in cardiac function and hemodynamics. We report a case of extravascular thrombus that compressed the main pulmonary artery and produced acute hypoxemia due to right-to-left shunting across a patent foramen ovale. We review the pathophysiology leading to this finding and the echocardiographic studies that established the diagnosis. [Copyright &y& Elsevier]

Objective Previous studies have demonstrated that bilateral internal mammary artery (BIMA) grafts lead to superior outcomes compared with single internal mammary artery grafts. This study examines whether cardiopulmonary bypass affects conduit-dependent outcomes of coronary artery bypass grafting (CABG) surgery. Methods From 1994 to 2013, a total of 6666 patients underwent isolated CABG surgery at our institution. Of these procedures, 3548 (53.2%) were performed off pump. A BIMA–saphenous vein graft (SVG) was used in 1544, and 5122 had left internal mammary artery–SVGs. These 2 conduit groups differed significantly in baseline characteristics. Propensity matching based on 22 preoperative variables and using a nearest-neighbor matching algorithm was used to make balanced cohorts, resulting in 2 groups of 1006. To account for the influence of pump status on conduit selection, a second propensity score was developed for pump use. These cases were matched to create 4 patient cohorts of 353 patients each (a total of 1412), balanced for both conduit use and pump status. Late mortality was determined using the Social Security Death Index. Results No difference was found in survival between patients receiving BIMA-SVGs on or off pump (78.9% vs 79.1%). BIMA-SVGs outperformed the left internal mammary artery–SVGs regardless of whether the procedure was performed off pump (73.9%) or on pump (69.9%). Conclusions This study demonstrates that the use of cardiopulmonary bypass does not significantly affect the long-term outcomes in these patients as long as full revascularization is achieved. In addition, these results are consistent with prior research showing that the use of BIMAs produces better outcomes than use of a single internal mammary artery when performing CABG. [ABSTRACT FROM AUTHOR]

• Remodeling in SERT-/- mice occurs earlier in the mitral valve than in other cardiac substructures. • Left side heart valve interstitial cells are more sensitive than right side to 5HT stimulation. • TGFβ1 upregulated HTR2B expression in valve interstitial cells from all valves, and fibroblasts. Increased serotonin (5HT) concentration and signaling, can lead to pathological remodeling of the cardiac valves. We previously showed that a reduction of the 5HT transporter (SERT) expression in the mitral valve (MV) contributes to the progression of degenerative MV regurgitation (MR). We sought to investigate the myocardial and valvular phenotype of SERT-/- mice in order to identify remodeling mechanisms specific to the MV and left ventricular (LV) remodeling. Using 8- and 16-week-old WT and SERT-/- mice we show that male and female animals deficient of SERT have pathological remodeling of the cardiac valves, myocardial fibrosis, diminished ejection fraction and altered left ventricular dimensions. In the MV and intervalvular area of the aortic valve (AV)-MV, gene expression, including Col1a1 mRNA, was progressively altered with age up until 16 weeks of age. In contrast, in the AV and myocardium, most gene expression changes occurred earlier and plateaued by 8 weeks. To explore basal differences in susceptibility to remodeling stimuli among cardiac valves, valve interstitial cells (VIC) were isolated from AV, MV, tricuspid valve (TV), pulmonary valve (PV) and fibroblasts (Fb) from the myocardial apex from 16 weeks old wild type (WT) mice. After 24h stimulation with 10 µM of 5HT, the gene expression of Col1a1 and Acta2 were upregulated in MVIC to a higher degree than in VIC from other valves and Fb. Treatment with TGFβ1 similarly upregulated Cola1 and Acta2 in MVIC and AVIC, while the increase was milder in right heart VIC and Fb. Experiments were also carried out with human VIC. In comparison to mice, human left heart VIC were more sensitive to 5HT and TGFβ1, upregulating COL1A1 and ACTA2; TGFβ1 upregulated HTR2B expression in all VIC. Our results support the hypothesis that a deleterious cardiac effect of SERT downregulation may be mediated by increased susceptibility to HTR2B-dependent pro-fibrotic mechanisms, which are distinct among VIC populations and cardiac fibroblasts, regardless of SERT activity. Given that HTR2B mechanisms involved in VIC and myocardial remodeling response are due to both 5HT and also to downstream related TGFβ1 and TNFα activity, targeting HTR2B could be a therapeutic strategy for dual treatment of MR and LV remodeling. [ABSTRACT FROM AUTHOR]

Abstract: Juvenile xanthogranuloma is the most frequent type of non-Langerhans cell histiocytosis. It most commonly presents in infancy and early childhood; manifesting as cutaneous lesions on the head, neck, and trunk that suddenly appear and usually undergo spontaneous regression. Extracutaneous involvement, although rare, may occur along with the cutaneous form or in isolation. It most frequently involves the eye, deep subcutaneous tissues, lung, and liver. Involvement of the heart is exceptionally rare, with only seven reports found in the English literature, all affecting infants. We present the first report of an intracardiac juvenile xanthogranuloma in an adult. [Copyright &y& Elsevier]