Novel Cardiovascular and Pulmonary Findings in the Noninvasive Ischemic Assessment of Patients With Coronavirus 2019 (COVID-19).

In the past several years, the coronavirus pandemic has introduced multiple medical disciplines to various new forms of disease previously unknown and has shown us a unique presentation of already existing diseases. We continue to understand the long-term effects of the pandemic on the population's health and continue to find new unique features previously unknown. This paper presents the unique feature of lung uptake abnormalities discovered on nuclear stress testing for cardiac perfusion defects, a consistent finding in multiple individuals with recent COVID-19 or ongoing infection.

Novel Device Therapies for Heart Failure.

Heart failure (HF) therapeutics have advanced significantly over the past few years [...].

Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy resulting from a mutation in one of several cardiac sarcomeric proteins [...].

The Roles of Coronary Computed Tomography Angiography in Characterizing Coronary Plaque: Screening, Treatment, and Prevention.

One of the major risk factors for coronary atherosclerosis is the gradual formation and maturation of coronary atherosclerotic plaque (CAP) [...].

Prevalence of lymphedema among Anderson-Fabry disease patients: A report from the Fabry registry.

BACKGROUND: Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease due to a genetic variation in the α-galactosidase A (GLA) gene. As a result, the activity of the α-galactosidase A (AGAL-A) enzyme is reduced or absent, which causes sphingolipid deposition within different body parts. AFD typically manifests with cardiovascular, renal, cerebrovascular, and dermatologic involvement. Lymphedema is caused by sphingolipid deposition within lymphatics. Lymphedema can cause intolerable pain and limit daily activities. Very limited data exist on lymphedema in AFD patients. METHODS: Using data from the Fabry Registry (NCT00196742) with 7671 patients included (44% males and 56% females), we analyzed the prevalence of lymphedema among AFD patients who were ever assessed for lymphedema and studied the age of first reported lymphedema. Additionally, we assessed whether patients received AFD-specific treatment at some point during their clinical course. The data was stratified by gender and phenotype. RESULTS: Our study showed that lymphedema occurred in 16.5% of the Fabry Registry patients who were ever assessed for lymphedema (n = 5487). Male patients when compared to female patient have higher prevalence (21.7% vs 12.7%) and experienced lymphedema at a younger age (median age at first reported lymphedema of 43.7 vs 51.7 years). When compared to other phenotypes, classic phenotype has the highest prevalence of lymphedema with the earliest reported lymphedema. Among those who reported lymphedema, 84.5% received AFD-specific treatment during their clinical course. CONCLUSIONS: Lymphedema is a common manifestation of AFD in both genders, with a tendency to present later in female patients. Recognition of lymphedema can offer an important opportunity for intervention and potential impact on associated morbidity. Additional future studies are needed to characterize the clinical implications of lymphedema in AFD patients and identify additional treatment options for this growing population.

Cardiac Amyloidosis: A Rare TTR Mutation Found in an Asian Female.

BACKGROUND: Transthyretin cardiac amyloidosis (ATTR) is a life-threatening, debilitating disease caused by abnormal formation and deposit of transthyretin (TTR) protein in multiple tissues, including myocardial extracellular matrix. It can be challenging to diagnose due to the myriad of presenting signs and symptoms. Additionally, numerous TTR mutations exist in certain ethnicities. Interestingly, our patient was discovered to have a very rare Gly67Ala TTR mutation typically not found in individuals of Asian descent. Recent advances in cardiovascular imaging techniques have allowed for earlier recognition and, therefore, management of this disease. Although incurable, there are now new, emerging treatments that are available for symptom control of cardiac amyloidosis, making early diagnosis vital for these patients, specifically their quality of life. CASE SUMMARY: We outline a case of a 50-year-old Asian female who was initially hospitalized for nausea and vomiting and persistent orthostatic hypotension. She underwent a multitude of laboratory and imaging tests, resulting in a diagnosis of cardiac amyloidosis, which was confirmed to be due to a rare TTR mutation via genetic testing. CONCLUSIONS: Our objective is to describe various TTR mutations, existing diagnostic imaging modalities, and available treatments, as well as highlight the importance of early screening and awareness of cardiac amyloidosis, allowing for quicker diagnosis and treatment of this disease.

A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment.

Amyloidosis is a group of disorders that can affect almost any organ due to the misfolding of proteins with their subsequent deposition in various tissues, leading to various disease manifestations based on the location. When the heart is involved, amyloidosis can manifest with a multitude of presentations such as heart failure, arrhythmias, orthostatic hypotension, syncope, and pre-syncope. Diagnosis of cardiac amyloidosis can be difficult due to the non-specific nature of symptoms and the relative rarity of the disease. Amyloidosis can remain undiagnosed for years, leading to its high morbidity and mortality due to this delay in diagnosis. Newer imaging modalities, such as cardiac magnetic resonance imaging, advanced echocardiography, and biomarkers, make a timely cardiac amyloidosis diagnosis more feasible. Many treatment options are available, which have provided new hope for this patient population. This manuscript will review the pathology, diagnosis, and treatment options available for cardiac amyloidosis and provide a comprehensive overview of this complicated disease process.

A Review of the Role of Imaging Modalities in the Evaluation of Viral Myocarditis with a Special Focus on COVID-19-Related Myocarditis.

Viral myocarditis is inflammation of the myocardium secondary to viral infection. The clinical presentation of viral myocarditis is very heterogeneous and can range from nonspecific symptoms of malaise and fatigue in subclinical disease to a more florid presentation, such as acute cardiogenic shock and sudden cardiac death in severe cases. The accurate and prompt diagnosis of viral myocarditis is very challenging. Endomyocardial biopsy is considered to be the gold standard test to confirm viral myocarditis; however, it is an invasive procedure, and the sensitivity is low when myocardial involvement is focal. Cardiac imaging hence plays an essential role in the noninvasive evaluation of viral myocarditis. The current coronavirus disease 2019 (COVID-19) pandemic has generated considerable interest in the use of imaging in the early detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related myocarditis. This article reviews the role of various cardiac imaging modalities used in the diagnosis and assessment of viral myocarditis, including COVID-19-related myocarditis.

Arrhythmogenic Ventricular Cardiomyopathy: Challenges With Complex Genetics and Variable Phenotypes.

After a 20-year-old woman suddenly died, autopsy showed characteristic findings of biventricular arrhythmogenic cardiomyopathy. Screening of her family members revealed the same desmoplakin gene mutation and imaging abnormalities predominantly involving the left ventricle. We describe the variable phenotypic expression in a family that shares a common gene variant. (Level of Difficulty: Advanced.).

Impact of left ventricular diastolic dysfunction on liver transplantation outcomes based on the latest American Society of Echocardiography/European Association of Cardiovascular Imaging recommendations.

Aim of the study: Cirrhotic cardiomyopathy encompasses systolic dysfunction, left ventricular diastolic dysfunction (LVDD), and conduction abnormalities. This study aims to investigate the impact of LVDD on mortality in patients undergoing liver transplantation (LT). Material and methods: A retrospective review of 400 consecutive patients who underwent LT at our institution was performed. Patient demographics, clinical data, and transthoracic echocardiogram (TTE) were reviewed to identify LVDD. The total cohort consisted of 266 patients after excluding patients with insufficient TTE data (n = 56), patients with indeterminate LVDD (n = 71), and patients with ejection fraction (EF) < 55% (n = 7). Statistical analysis was performed using descriptive statistics. Cox regressions with hazard ratios (HRs) and 95% confidence intervals (CI) were applied to predict 5-year all-cause mortality. Kaplan-Meier survival analysis was conducted to understand the impact of LVDD on 5-year all-cause mortality. Results: Patients with LVDD have higher incidence of hyperlipidemia (36% vs. 17%, p = 0.003), hypertension (50% vs. 27%, p = 0.001) and diabetes (52% vs. 30%, p = 0.003). In addition, patients with non-alcoholic steatohepatitis (NASH) were more likely to have LVDD (48% vs. 24%, p = 0.001). A multivariate logistic regression analysis was performed with age, body mass index (BMI), NASH, alcoholic cirrhosis, hepatitis C, history of diabetes, history of hyperlipidemia, and history of hypertension. In this multivariate logistic regression analysis, NASH (odds ratio [OR] = 4.43 [1.10-17.8], p = 0.04), and history of hypertension (OR = 2.33 [1.16-4.66], p = 0.01) were independent predictors of LVDD. The Kaplan-Meier survival analysis and multivariate Cox regression demonstrated that the presence of LVDD had no impact on 5-year all-cause mortality (log-rank test nonsignificant). Conclusions: This study indicates that LVDD in end-stage liver disease (ESLD) patients does not affect immediate post-transplant outcomes or 5-year all-cause mortality.

Can Computed Fractional Flow Reserve Coronary CT Angiography (FFRCT) Offer an Accurate Noninvasive Comparison to Invasive Coronary Angiography (ICA)? "The Noninvasive CATH." A Comprehensive Review.

Invasive coronary angiography (ICA) serves as a very important tool in the diagnosis of coronary artery disease (CAD) and provides information for further intervention. Fractional Flow Reserve (FFR) at the time of ICA is the gold standard to analyze the hemodynamic and physiologic significance of moderate coronary stenosis. The dawn of coronary CT angiography (CTA) has helped in visualizing the anatomy of coronary arteries. Computed Fractional Flow Reserve (FFRCT) from such an imaging study shows promise in providing valuable data about physiology on top of the anatomy noninvasively; which can guide decision-making process for revascularization. This manuscript aims to review the accuracy of FFRCT obtained from a coronary CTA in the diagnosis of hemodynamically significant coronary artery stenosis and ruling out nonsignificant coronary artery stenosis when compared to the Gold standard of FFR obtained during ICA. We conducted a Medline search using various combinations of "FFRCT," "ICA" "noninvasive," "significant stenosis," and "CAD" to identify pivotal randomized trials published before May 1, 2020, for inclusion in this review. Concurrently, major practice guidelines, trial bibliographies, and pertinent reviews were examined to ensure inclusion of relevant trials. A consensus among the authors was used to choose items for narrative inclusion. The following section reviews data from pivotal trials to determine a noninvasive strategy in appropriate patients to accurately detect functionally significant stenosis. For these trials, the sensitivity, specificity, and accuracy are compared. Trials reviewed: CTA, FFRCT, ICA, CT-myocardial perfusion imaging. FFRCT is a novel noninvasive modality which localizes significant "ischemia-causing" stenosis (≤0.80) by means of crystal fluid dynamics eliminating the need for vasodilators. The analysis of FFRCT by DISCOVER FLOW, DeFACTO, NXT trials revealed high sensitivity, negative predictive value, and good accuracy. The ADVANCE registry showed significantly lower events of CV death or myocardial infarction with a negative FFRCT (>0.80 study). The PLATFORM trial showed significant reduction in negative ICA with negative FFRCT, thus ultimately reducing the number of unnecessary percutaneous coronary intervention. Decrease in healthcare costs was noted with FFRCT, decreasing downstream testing, and invasive procedures. FFRCT is a novel modality for analyzing significant stenosis in CAD noninvasively. The high sensitivity of this modality could make it a good rule out tool to avoid unnecessary intervention in physiologically insignificant lesions. Limitations of this modality include low specificity, double exposure to contrast, turnaround time, and upfront costs. Further query into this matter is warranted.

Outcomes of Coronary Artery Revascularization Procedures in Patients with Antiphospholipid Syndrome.

BACKGROUND: Published data on the outcome of coronary artery revascularization in patients with antiphospholipid syndrome (APS) are limited. Because APS is associated with a high rate of arterial thrombosis, there is concern that coronary revascularization in this group may be complicated by increased need for repeat revascularization. We aimed to determine the incidence and timing of repeat revascularization performed in patients with APS undergoing percutaneous coronary interventions (PCI) or coronary artery bypass grafting (CABG). METHODS: Our institutional database was queried for individuals (n = 575) testing positive for antiphospholipid antibodies between 2000 and 2012. From this group, 46 patients underwent cardiac catheterization. Charts were reviewed to identify subsequent revascularization procedures. RESULTS: The study sample consisted of 15 patients (67 ±â€¯11 years, 11 females) who underwent revascularization. All of the study subjects had prior history of arterial (stroke, TIA n = 7) or venous (n = 10) thrombosis. Ten of the subjects had initial revascularization (6 CABG, 4 PCI) at an outside facility, while another five underwent initial PCI at our hospital. Repeat revascularization occurred in five patients (33%) at a median of 6 years (range 4, 13) following the initial revascularization. The median follow-up for patients who did not require repeat revascularization (n = 10) was 10 years (range 2, 15). CONCLUSION: Amongst patients with APS who underwent CABG or PCI the need for repeat revascularization was infrequent and occurred several years after initial procedure. Based on this small sample size the periprocedural risk associated with coronary artery revascularization in subjects with APS is not prohibitively high.

From Tick Bite to Heart Failure: Ehrlichial Myocarditis.

Cardiac involvement in myocarditis induced by Human Monocytic Ehrlichiosis infection is an incredibly uncommon complication with sparsely available literature. Also, this case highlights the importance of early recognition as a first step in management.

Une atteinte cardiaque secondaire à une myocardite induite par une ehrlichiose monocytaire humaine constitue une complication extrêmement rare et très peu documentée. Le cas présenté fait ressortir l'importance d'une reconnaissance rapide du problème comme première étape de la prise en charge.

Spontaneous Rupture of Sinus of Valsalva Aneurysm Presenting as Perivalvular Hematoma.

Acute rupture of sinus of Valsalva often presents as an acute emergency with significant hemodynamic compromise whereas contained rupture of sinus of Valsalva with a perivalvular hematoma formation is rarely seen. We describe the case of a 63-year-old male who presented with acute shortness of breath and was found to have rupture of sinus of Valsalva aneurysm (SVA) with a perivalvular hematoma and severe aortic regurgitation. We also review the presentation, diagnosis, and management of SVAs.

Pacemaker diagnostics in atrial fibrillation: limited usefulness for therapy initiation in a pacemaker practice.

AIM: We aimed to determine the practical value of pacemaker diagnostics for atrial fibrillation (AF) in an unselected general pacemaker practice, specifically workflow and initiation of anticoagulation or antiarrhythmic drug (AAD) therapy. METHODS: We prospectively followed consecutive pacemaker interrogations over a period of 1 year to identify patients with AF (burden from 1% to 99%). We contacted referring physicians with AF details, and then determined whether the information resulted in therapeutic changes. RESULTS: Of the 1,100 pacemakers interrogated, 728 were dual chamber (DDDs) with AF diagnostic capability. AF was recorded in 73 (10%) but seven had limited information, leaving 66 patients; of these, 42 (63%) patients were already anticoagulated and in five (7%) patients, anticoagulation had been stopped because of complications. Initial diagnosis of AF was made by the pacemaker in 17 patients (26% of 66; 2% of 728); four (6% of 66) patients were newly initiated on anticoagulation. Of the 66 patients, 17 patients were already on AADs; 49 (74%) had satisfactory rate control or had other issues; only two (3% of 66; 0.3% of 728) received new AADs. CONCLUSIONS: Of 728 patients with DDD pacemakers, only 17 were newly discovered to have AF, and six (0.8%) had changes in medications based on the pacemaker data. Adding pacemaker-derived data to existing clinical information had little therapeutic impact, due to a combination of cumbersome workflow, and because AF was usually known to practitioners. Developments in automated monitoring systems may provide more accessible and therapeutically useful information.

Echocardiographic left ventricular end-diastolic pressure volume loop estimate predicts survival in congestive heart failure.

BACKGROUND: The left ventricular end-diastolic pressure-volume relationship (LV-EDPVR) is a measure of LV distensibility, conveying the size the LV will assume at a given LV end-diastolic pressure (LV-EDP). Measurement of LV-EDPVR requires invasive testing with specialized equipment. Echocardiography can be used to measure LV end-diastolic volume (EDV) and to grossly estimate LV-EDP noninvasively. We therefore hypothesized that categorization of patients based on these parameters to create an estimate of the end-diastolic pressure-volume loop position (EDPVE) could predict congestive heart failure (CHF) prognosis. METHODS AND RESULTS: Echocardiograms from 968 CHF clinic patients were reviewed. LV-EDP was considered to be elevated if mitral filling pattern was pseudo-normal or restrictive. EDPVE was categorized into 3 groups. EDPVE was considered to have evidence of rightward shift if the LV was severely dilated (>97 mL/m(2)). EDPVE was considered to have evidence of leftward shift if the LV was normal size (<76 mL/m(2)) and there was Doppler evidence of increased LV-EDP. Patients who did not meet criteria for leftward or rightward shift were classified as "intermediate." Using the intermediate group for comparison, those with evidence of leftward shift in EDPVE had increased mortality (hazard ratio [HR] 1.77; 95% confidence interval [CI]: 1.23-2.54). Rightward shift only correlated with increased mortality in those older than age 70 years. Leftward shift remained an independent predictor of mortality even after adjusting for LV ejection fraction, atrial fibrillation, mitral regurgitation, and Doppler indices of diastolic dysfunction. CONCLUSION: EDPVE is a strong predictor of CHF survival which is independent of LV ejection fraction and traditional Doppler indices of LV diastolic function.