Low-Gradient Severe Mitral Stenosis: Hemodynamic Profiles, Clinical Characteristics, and Outcomes.

Background Optimal management of patients with severe mitral stenosis ( MS ) and low transmitral gradient is incompletely understood. Methods and Results We examined 101 consecutive patients with severe rheumatic MS (mitral valve area ≤1.5 cm2) who underwent balloon valvuloplasty. Low gradient was defined as mean transmitral gradient <10 mm Hg and low flow as stroke volume index ≤35 mL/m2 by echocardiography. Symptoms and mortality data were collected. Systolic, diastolic, and arterial function were characterized by measuring left ventricular (LV) end-systolic elastance, LV stiffness constant (ß), diastolic capacitance (predicted LV end-diastolic volume at a common LV filling pressure of 30 mm Hg), and effective arterial elastance. Low gradient (<10 mm Hg) was present in 55 patients, including low flow/low gradient in 11 and normal flow/low gradient in 44 patients, and high gradient was present in 46 patients. Participants with low-flow/low-gradient (LG) MS were older with higher rates of atrial fibrillation (64%) and subvalvular thickening, higher afterload, and decreased LV compliance with lower ejection fraction (57±10% versus 65±4% versus 63±6%, P=0.002) but similar end-systolic elastance compared with patients with normal-flow/ LG and high-gradient MS . The normal-flow/ LG group had larger mitral valve area and lower left atrial pressure by catheterization, as well as favorable long-term outcomes compared with the low-flow/ LG and high-gradient MS group. A total of 40% of patients with LG MS had no symptomatic benefit from valvuloplasty compared with 18% of patients with high-gradient MS ( P=0.02). Conclusions Presence of low gradient in patients with severe MS was associated with lesser symptomatic benefit from valvuloplasty. In the subset with low stroke volume index, this may be related to independent ventricular-vascular uncoupling, decreased LV compliance, and high prevalence of atrial fibrillation in addition to intrinsic MS .

Echocardiographic Assessment for the Detection of Cardiotoxicity Due to Vascular Endothelial Growth Factor Inhibitor Therapy in Metastatic Renal Cell and Colorectal Cancers.

BACKGROUND: Cardio-oncology is a recently established discipline that focuses on the management of patients with cancer who are at risk for developing cardiovascular complications as a result of their underlying oncologic treatment. In metastatic colorectal cancer (mCRC) and metastatic renal cell carcinoma (mRCC), vascular endothelial growth factor inhibitor (VEGF-i) therapy is commonly used to improve overall survival. Although these novel anticancer drugs may lead to the development of cardiotoxicity, whether early detection of cardiac dysfunction using serial echocardiography could potentially prevent the development of heart failure in this patient population requires further study. The aim of this study was to investigate the role of two-dimensional speckle-tracking echocardiography in the detection of cardiotoxicity due to VEGF-i therapy in patients with mCRC or mRCC. METHODS: Patients with mRCC or mCRC were evaluated using serial echocardiography at baseline and 1, 3, and 6 months following VEGF-i treatment. RESULTS: A total of 40 patients (34 men; mean age, 63 ± 9 years) receiving VEGF-i therapy were prospectively recruited at two academic centers: 26 (65%) were receiving sunitinib, eight (20%) pazopanib, and six (15%) bevacizumab. The following observations were made: (1) 8% of patients developed clinically asymptomatic cancer therapeutics-related cardiac dysfunction; (2) 30% of patients developed clinically significant decreases in global longitudinal strain, a marker for early subclinical cardiac dysfunction; (3) baseline abnormalities in global longitudinal strain may identify a subset of patients at higher risk for developing cancer therapeutics-related cardiac dysfunction; and (4) new or worsening hypertension was the most common adverse cardiovascular event, afflicting nearly one third of the study population. CONCLUSIONS: Cardiac dysfunction defined by serial changes in myocardial strain assessed using two-dimensional speckle-tracking echocardiography occurs in patients undergoing treatment with VEGF-i for mCRC or mRCC, which may provide an opportunity for preventive interventions.

Use of speckle-tracking echocardiography-derived strain and systolic strain rate measurements to predict rejection in transplant hearts with preserved ejection fraction.

BACKGROUND: Noninvasive diagnosis of allograft rejection in heart transplant recipients is challenging. The utility of 2-dimensional speckle-tracking echocardiography (2D-STE) to predict severe rejection in heart transplant recipients with preserved left ventricular ejection fraction (LVEF) was evaluated. METHODS: Adult heart transplant patients with preserved LVEF (> 55%) and severe rejection by biopsy (Rejection Grade ≥ 2R) or no rejection between 1997 and 2011 at the Mayo Clinic in Rochester, Minnesota were evaluated. Transthoracic echocardiography was performed within 1 month of the biopsy. LV global longitudinal and circumferential strain and strain rates (GLS, GLSR, GCS, and GCSR) were analyzed retrospectively. RESULTS: Of 65 patients included, 25 had severe rejection and 40 were normal transplant controls without rejection. Both groups had more men than women (64 and 75%, respectively). Baseline clinical variables were similar between the groups. Both groups had normal LVEF (64.3% vs 64.5%; P = .87). All non-strain echocardiographic variables were similar between the 2 groups. Strain analysis showed significantly increased early diastolic longitudinal strain rate (P = .02) and decreased GCS (P < .001) and GCSR (P = .02) for the rejection group compared with the control group. The area under the receiver operating characteristic curve for GCS was 0.77. With a GCS cutoff of - 17.60%, the sensitivity and specificity of GCS to detect severe acute rejection were 81.8 and 68.4%, respectively. CONCLUSIONS: 2D-STE may be useful in detecting severe transplant rejection in heart transplant patients with normal LVEF.

Concomitant acute stroke, pulmonary and myocardial infarction due to in-transient thrombus across a patent foramen ovale

Non-atherosclerotic myocardial infarction (MI) is an important but often misdiagnosed cause of acute MI. Furthermore, non-atherosclerotic MI with concomitant acute stroke and pulmonary embolism due to in-transit thrombus across a patent foramen ovale (PFO) is a rare but potentially fatal combination (1, 2, 3). Early detection of this clinical entity can facilitate delivery of targeted therapies and avoid poor outcome (1, 2). Here, we describe a 68-year-old female with hypertension, tobacco abuse and chronic obstructive pulmonary disease presenting with facial droop, right arm weakness and aphasia. Head computed tomography (CT) without contrast was unremarkable. ECG showed an acute inferolateral ST-elevation MI (Fig. 1, Panel A). As patient presented with both an acute neurological deficit and MI, clinical suspicion of non-atherosclerotic MI was raised and the patient underwent concurrent emergency coronary angiography (CAG) and transesophageal echocardiogram (TEE). TEE revealed highly mobile masses in the left and right atrium (Fig. 1, Panel B and Video 1). The large mass (thrombus or cast of a deep venous thrombus) was caught in a PFO (Fig. 1, Panel C, D, E and Videos 2, 3). A second smaller mass/thrombus was seen on the Eustachian valve near the right atrial/inferior vena cava junction (Fig. 1, Panel F and Video 4). CAG confirmed a 100% occluded distal right posterolateral artery suggestive of an embolic phenomenon. The patient underwent successful thrombectomy, retrieving a large thrombus burden (Fig. 1, Panel G and Videos 5, 6, 7). CT angiography showed occluded internal carotid artery (Fig. 1, Panel H). Pathology from thrombectomy confirmed fibrin-rich thrombus. The patient had bilateral lower extremity deep vein thrombosis and bilateral diffuse pulmonary embolisms.

Evaluation of Left Ventricular Diastolic Function by Echocardiography with Tissue Doppler in Systemic Sclerosis / Avaliação da Função Diastólica do Ventrículo Esquerdo com Ecocardiografia Associada a Doppler Tecidual na Esclerose Sistêmica

Abstract Background: Systemic sclerosis (SS) is a connective tissue abnormality characterized by fibrosis of the skin and internal organs. Cardiac involvement with consequent myocardial dysfunction in SS is associated with increased morbidity and mortality. Objective: To investigate the left ventricular (LV) diastolic function in patients with SS and preserved systolic function. Methods: Patients with SS were evaluated with two-dimensional echocardiography with tissue Doppler for analysis of chamber diameters, LV mass index (LVMI), indexed left atrial volume (iLAV), systolic function of both ventricles, and presence and degree of diastolic dysfunction (DD). Results: We evaluated 50 patients, divided according to the presence of DD into Group 1 (n = 25; normal diastolic function, E/A ratio ≥ 0.8, deceleration time [DT] > 150 ms and < 200 ms, and septal e' > 8 cm/s) and Group 2 (n = 25; with DD, subdivided into type I DD [E/A < 0.8, DT > 200 ms], type II [E/A ≥ 0.8, septal e' < 8 cm/s, iLAV > 34 mL/m2], and type III [E/A > 2, DT < 150 ms, septal e' < 8 cm/s]). Type I DD was the most frequent (34%), followed by type II DD (16%). LVMI and iLAV were similar in both groups, but septal and lateral e' were reduced only in Group 2. In Group 2, we observed that patients with moderate DD had longer disease duration (p = 0.02). Conclusion: The prevalence of type I DD was elevated in SS and associated with aging. Disease duration emerged as an important factor in moderate DD.

Resumo Fundamento: A esclerose sistêmica (ES) é uma anomalia do tecido conjuntivo caracterizada por fibrose da pele e dos órgãos internos. O envolvimento cardíaco com consequente disfunção miocárdica na ES está associado a uma maior morbimortalidade. Objetivo: Investigar a função diastólica do ventrículo esquerdo (VE) em pacientes com ES e função sistólica preservada. Método: Pacientes com ES foram estudados com ecocardiografia bidimensional com Doppler tecidual para análise dos diâmetros cavitários, índice de massa do VE (IMVE), volume indexado do átrio esquerdo (VAEi), função sistólica biventricular e presença, além de grau de disfunção diastólica (DD) pelo Doppler convencional e tecidual. Resultados: Estudamos 50 pacientes, divididos conforme presença de DD em Grupo 1 (n = 25; função diastólica normal, razão E/A ≥ 0,8, tempo de desaceleração [TD] > 150 ms e < 200 ms e e' septal > 8 cm/s) e Grupo 2 (n = 25; com presença de DD, subdividido em DD tipo I [E/A < 0,8, TD > 200 ms], tipo II [E/A ≥ 0,8, e' septal < 8 cm/s, VAEi > 34 mL/m2] e tipo III [E/A > 2, TD < 150 ms, e' septal < 8 cm/s]. A DD tipo I foi a mais frequente (34%), seguida de DD tipo II (16%). O IMVE e o VAEi foram similares entre os grupos, porém a e' septal e lateral estiveram reduzidas apenas no Grupo 2. No Grupo 2, observamos que os pacientes com DD moderada apresentavam maior duração da doença (p = 0,02). Conclusão: A prevalência de DD tipo I foi elevada na ES e esteve associada ao envelhecimento. No caso da DD moderada, a duração da doença despontou como um fator importante.

A rash with a heavy heart.

Cardiac amyloidosis (CA) is relatively rare and frequently misdiagnosed. Other disorders presenting with increased left ventricular (LV) mass can mimic its diagnosis. This case illustrates unique findings of primary light chain (AL) amyloidosis in a patient with remarkable signs of CA. Here, we report a 49-year-old man with prior diagnosis of hypertrophy cardiomyopathy (HCM) based on an echocardiogram performed 1 year earlier that presented with 8 weeks of periorbital rash. He had numbness in the past 3 years. More recently, he presented with shortness of breath. Physical examination was remarkable for periorbital purpura, macroglossia, and orthostatic hypotension. Cardiac auscultation showed S3 and S4. Electrocardiography (ECG) showed diffuse low-voltage QRS complexes. Echocardiography revealed severe diastolic impairment; granular "sparkling" pattern of the myocardium with thickened walls, interatrial septum, and valves; and pericardial effusion. Diastolic dysfunction and thick walls with low ECG voltage are compelling diagnostic findings. Laboratory work up showed increased free light chain-differential (FLC-diff), N-terminal fragment of brain natriuretic peptide (NT-BNP), and cardiac Troponin T (cTnT). Bone marrow biopsy confirmed AL amyloidosis. A diagnosis of AL amyloidosis with cardiac involvement mimicking HCM was made. The patient died during hospitalization due to sudden cardiac death. This cases illustrates the importance of the combination of clinical, serological, electro- and echocardiographic findings to establish the diagnosis of CA.

Prognosis of Light Chain Amyloidosis With Preserved LVEF: Added Value of 2D Speckle-Tracking Echocardiography to the Current Prognostic Staging System.

OBJECTIVES: This study evaluated whether 2-dimensional speckle-tracking echocardiography (2D-STE) has incremental value for prognosis over traditional clinical, echocardiographic, and serological markers-with main focus on the current prognostic staging system-in light-chain (AL) amyloidosis patients with preserved left ventricular ejection fraction. BACKGROUND: Cardiac amyloidosis (CA) is the major determinant of outcome in AL amyloidosis. The current prognostic staging system is based primarily on serum levels of cardiac troponin T (cTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain differential (FLC-diff). METHODS: Consecutive patients with biopsy-proven AL amyloidosis and left ventricular ejection fraction ≥55% were divided into group 1 with CA (n = 63) and group 2 without CA (n = 87). Global longitudinal strain (GLS) by 2D-STE was performed with Vivid E9 (GE Healthcare Co., Milwaukee, Wisconsin) and syngo Velocity Vector Imaging (VVI) software (Siemens Medical Solutions USA, Inc., Malvern, Pennsylvania) (GLSGE and GLSVVI, respectively). RESULTS: Thirty-two deaths (51%) occurred in group 1 and 13 (15%) in group 2 (p ≤ 0.001). Group 1 had thicker walls, lower early diastolic tissue Doppler velocity at septal mitral annulus, and greater left ventricular mass, left atrial volume, glomerular filtration rate, FLC-diff, cTnT, and NT-proBNP (p < 0.001). For the entire cohort, GLSGE ≥ -14.81, GLSVVI ≥-15.02, cTnT, NT-proBNP, FLC-diff, age, left ventricular wall thickness, early diastolic tissue Doppler velocity at septal mitral annulus, diastolic dysfunction grade, glomerular filtration rate, deceleration time, and left atrial volume were univariate predictors of death. In a multivariate Cox model, GLSGE ≥-14.81 (hazard ratio [HR]: 2.68; 95% confidence interval [CI]: 1.07 to 7.13; p = 0.03), FLC-diff, NT-proBNP, and age were independent predictors of survival. There was also a strong trend for GLSVVI ≥-15.02 (HR: 2.44; 95% CI: 0.98 to 6.33; p = 0.055). Using a nested logistic regression model, GLSGE (p = 0.03) and GLSVVI (p = 0.05) provided incremental prognostic value over cTnT, NT-proBNP, and FLC-diff. For survival analysis limited to group 2 (non-CA), GLSGE and GLSVVI both predicted all-cause mortality (GLSGE HR: 1.23; 95% CI: 1.03 to 1.47 [p = 0.02]; GLSVVI HR: 1.22; 95% CI: 1.01 to 1.49 [p = 0.04], respectively). CONCLUSIONS: 2D-STE predicted outcome and provided incremental prognostic information over the current prognostic staging system, especially in the group without CA.

Rationale for setting up a cardio-oncology unit: our experience at Mayo Clinic.

BACKGROUND: The diagnosis and management of cardiovascular complications have become a clinical concern for oncologists, cardiologists, surgeons, interventional radiologists, radiation therapy physicians, internists, nurses, pharmacists, administrators, and all the stakeholders involved in the care of cancer patients. Anticancer therapies have extended the lives of patients with cancer, but for some this benefit is attenuated by adverse cardiovascular effects. METHODS: This review article aims to provide an overview of the rationale of setting up a cardio-oncology unit and reflect on our own experience establishing this service, and conclude with some fundamental aspects of consideration for evaluation and management of patients with cancer and cardiovascular diseases. RESULTS: Cardiotoxicity can lead to congestive heart failure and cardiac death. In fact, chemotherapy-related cardiac dysfunction may carry one of the worst prognoses of all types of cardiomyopathies, and has a profound impact on morbidity and mortality in oncology patients. Other complex clinical situations involve cancer patients who might benefit from a highly curative drug in terms of cancer survival but face limitations of its administration because of concomitant cardiovascular diseases. Indeed, the balance between the benefits and risks of the cancer therapy regimen in the context of the cardiovascular status of the individual patient can sometimes be extraordinarily challenging. A subspecialty with a multidisciplinary integrative approach between oncologists, hematologists, cardiologists, among others has thus emerged to address these issues, termed cardio-oncology. Cardio-oncology addresses the spectrum of prevention, detection, monitoring and treatment of cancer patients with cardiovascular diseases, or at risk for cardiotoxicity, in a multidisciplinary manner. In this field, cardiologists assist oncologists and hematologists with cardiovascular recommendations. This can be mediated through e-consultations or face-to-face evaluations. CONCLUSION: Cardio-oncology is a subspecialty that assists in the overall care of cancer patients with and without cardiovascular disease in an interdisciplinary fashion. We believe that this partnership of sharing responsibilities and experiences among health-care team members can potentially decrease cancer therapeutics-related cardiovascular complications and improve clinical outcomes.