A mimicker of constriction: a case report of a rare case of cardiac angiosarcoma.

Background: Cardiac angiosarcoma is an exceptionally rare primary malignant tumour with an aggressive course and typically poor prognosis. Diagnosis is difficult, and patients often present with metastatic disease. We report the rare case of a patient with cardiac angiosarcoma who presents with constrictive physiology due to tumour encasement. Case summary: A 65-year-old female with a past medical history of Hodgkin's lymphoma and limited scleroderma presented with progressive dyspnoea on exertion. Multimodality imaging and haemodynamics with echocardiography, cardiac magnetic resonance imaging (MRI), and cardiac catheterization showed findings of constrictive physiology. Cardiac MRI showed areas of pericardial enhancement, so she was initially started on colchicine, prednisone, and mycophenolate mofetil to treat pericardial inflammation. However, her symptoms progressed, and she underwent pericardiectomy with cardiac surgery. Pericardium was noted to be thickened and a mass-like substance was densely adherent and potentially invading the heart itself and could not be dissected free. Surgical pathology showed features consistent with epithelioid angiosarcoma. Patient had rapid progression of her disease and was started on chemotherapy. Her course, however, was complicated by acute gastrointestinal bleeding, atrial fibrillation with rapid rates, and persistent volume overload. She elected for comfort measures and passed away shortly after her diagnosis. Discussion: Our case shows an extremely rare diagnosis, cardiac angiosarcoma, presenting with typical findings of constrictive physiology. The case shows the typical features of constrictive physiology using multimodality imaging and haemodynamics and emphasizes the need to always think broadly in creating a differential diagnosis for constriction to ensure that rare diseases are considered.

Direct comparison of echocardiography speckle tracking and cardiac magnetic resonance feature tracking for quantification of right ventricular strain: a prospective intermodality study in functional mitral regurgitation.

BACKGROUND: Functional mitral regurgitation (FMR) is a known risk factor for right ventricular dysfunction (RVDYS). RV global longitudinal strain (GLS) is an emerging index of RV function; however, the magnitude of agreement between RV GLS by echocardiography (echo) and cardiac magnetic resonance (CMR) and the relative utility of each modality for both the diagnosis of RVDYS and prognostication of all-cause mortality and heart failure hospitalization remain unknown. RESULTS: 32% of patients had RVDYS (EF < 50%) on CMR, among whom there was more advanced NYHA class and lower LV and RV ejection fraction (all p < 0.05). RV GLS was impaired in patients with RVDYS whether quantified via STE or FT-CMR, with strong correlation between modalities (r = 0.81). Both STE and FT-CMR derived GLS yielded excellent detection of RVDYS (AUC 0.94 for both), paralleling similar performance for free wall strain by both modalities (FT-CMR AUC 0.94, STE AUC 0.92) with lower accuracy demonstrated by STE derived septal strain (STE AUC 0.78 and FT-CMR AUC 0.92). RV S' and TAPSE showed lower diagnostic accuracy (RV S' AUC 0.77 and TAPSE AUC 0.81). During median follow up of 51 months (IQR 42, 60 months), all-cause mortality or HF hospitalization occurred in 25% (n = 25). Both STE and FT-CMR derived RV GLS stratified risk for adverse prognosis (STE p = 0.007, FT-CMR p = 0.005) whereas conventional RV indices, TAPSE and RV S', did not (TAPSE p = 0.30, S' p = 0.69). CONCLUSION: RV GLS is a robust marker of RVDYS irrespective of modality which provides incremental diagnostic value and improves risk stratification for event free survival beyond conventional RV indices.

Racial disparities in the treatment of aortic stenosis: Has transcatheter aortic valve replacement bridged the gap?

BACKGROUND: Racial disparities in outcomes and utilization of surgical aortic valve replacement (SAVR) for the treatment of severe aortic stenosis (AS) is well known. While transcatheter aortic valve replacement (TAVR) has become more widespread, its impact on racial disparities remains unclear. OBJECTIVES: Our goal was to assess the utilization of SAVR and TAVR and their clinical outcomes among various racial groups. METHODS: The National Inpatient database (2009-2015) was used to study the racial distribution of admissions for SAVR and TAVR, and their impact on inpatient outcomes. Survey estimation commands were used to determine weighted national estimates. RESULTS: There were 3,445,267; 294,108; and 52,913 inpatient visits for AS, SAVR, and TAVR, respectively. SAVR visits were 86% White, 3.8% Black, 5.5% Hispanic, 1.2% Asian/Pacific Islander (A/PI), 0.4% Native American (NA), and 2.9%; TAVR were 87.7% White, 3.7% Black, 3.8% Hispanic, 1.0% A/PI, 0.2% NA, and 3.5% Other while AS visits were 83.7% White, 6.7% Black, 5.3% Hispanic, 1.7% A/PI, 0.4% NA, and 2.2% Other. Racial minorities generally had more co-morbidities compared with Whites. After SAVR, Black patients had a higher unadjusted inpatient mortality than Whites, however, there was no difference after adjustment for other variables. A/PI were more likely to require a permanent pacemaker after SAVR. Need for blood transfusion was significantly higher among the minorities compared with Whites, except for NA, but there were no racial differences in stroke rates. There was no difference in inpatient mortality, pacemaker implantation, stroke, and bleeding after TAVR, but acute kidney injury occurred more often in Hispanics, A/PI, and "others" compared with Whites. CONCLUSIONS: Racial disparities in the treatment of AS continues in the contemporary era; however it was found that TAVR resulted in comparable inpatient outcomes, despite higher comorbidities, and adverse socioeconomic factors in minorities.

Inhibition of BKCa negatively alters cardiovascular function.

Large conductance calcium and voltage-activated potassium channels (BKCa ) are transmembrane proteins, ubiquitously expressed in the majority of organs, and play an active role in regulating cellular physiology. In the heart, BKCa channels are known to play a role in regulating the heart rate and protect it from ischemia-reperfusion injury. In vascular smooth muscle cells, the opening of BKCa channels results in membrane hyperpolarization which eventually results in vasodilation mediated by a reduction in Ca2+ influx due to the closure of voltage-dependent Ca2+ channels. Ex vivo studies have shown that BKCa channels play an active role in the regulation of the function of the majority of blood vessels. However, in vivo role of BKCa channels in cardiovascular function is not completely deciphered. Here, we have evaluated the rapid in vivo role of BKCa channels in regulating the cardiovascular function by using two well-established, rapid-acting, potent blockers, paxilline and iberiotoxin. Our results show that BKCa channels are actively involved in regulating the heart rate, the function of the left and right heart as well as major vessels. We also found that the effect on BKCa channels by blockers is completely reversible, and hence, BKCa channels can be exploited as potential targets for clinical applications for modulating heart rate and cardiac contractility.