Musca Dott. Francesco
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Estimation of Right Atrial Pressure by Ultrasound-Assessed Jugular Vein Distensibility in Patients With Heart Failure.
Circ Heart Fail2024 Feb;17(2):e010973. doi: 10.1161/CIRCHEARTFAILURE.123.010973.
Ammirati Enrico, Marchetti Davide, Colombo Giada, Pellicori Pierpaolo, Gentile Piero, D'Angelo Luciana, Masciocco Gabriella, Verde Alessandro, Macera Francesca, Brunelli Dario, Occhi Lucia, Musca Francesco, Perna Enrico, Bernasconi Davide P, Moreo Antonella, Camici Paolo G, Metra Marco, Oliva Fabrizio, Garascia Andrea
Abstract
BACKGROUND:
Clinical evaluation of central venous pressure is difficult, depends on experience, and is often inaccurate in patients with chronic advanced heart failure. We assessed the ultrasound-assessed internal jugular vein (JV) distensibility by ultrasound as a noninvasive tool to identify patients with normal right atrial pressure (RAP ?7 mm?Hg) in this population.
METHODS:
We measured JV distensibility as the Valsalva-to-rest ratio of the vein diameter in a calibration cohort (N=100) and a validation cohort (N=101) of consecutive patients with chronic heart failure with reduced ejection fraction who underwent pulmonary artery catheterization for advanced heart failure therapies workup.
RESULTS:
A JV distensibility threshold of 1.6 was identified as the most accurate to discriminate between patients with RAP ?7 versus >7 mm?Hg (area under the receiver operating characteristic curve, 0.74 [95% CI, 0.64-0.84]) and confirmed in the validation cohort (receiver operating characteristic, 0.82 [95% CI, 0.73-0.92]). A JV distensibility ratio >1.6 had predictive positive values of 0.86 and 0.94, respectively, to identify patients with RAP ?7 mm?Hg in the calibration and validation cohorts. Compared with patients from the calibration cohort with a high JV distensibility ratio (>1.6; n=42; median RAP, 4 mm?Hg; pulmonary capillary wedge pressure, 11 mm?Hg), those with a low JV distensibility ratio (?1.6; n=58; median RAP, 8 mm?Hg; pulmonary capillary wedge pressure, 22 mm?Hg;
CONCLUSIONS:
Ultrasound-assessed JV distensibility identifies patients with chronic advanced heart failure with normal RAP and better outcomes.
REGISTRATION:
URL: https://www.clinicaltrials.gov; Unique identifier: NCT03874312.
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Ultrasound-Assisted, Catheter-Directed Thrombolysis for Acute Intermediate/High-Risk Pulmonary Embolism: Design of the Multicenter USAT IH-PE Registry and Preliminary Results.
J Clin Med2024 Jan;13(2):. doi: 619.
Colombo Claudia, Capsoni Nicolò, Russo Filippo, Iannaccone Mario, Adamo Marianna, Viola Giovanna, Bossi Ilaria Emanuela, Villanova Luca, Tognola Chiara, Curci Camilla, Morelli Francesco, Guerrieri Rossella, Occhi Lucia, Chizzola Giuliano, Rampoldi Antonio, Musca Francesco, De Nittis Giuseppe, Galli Mario, Boccuzzi Giacomo, Savio Daniele, Bernasconi Davide, D'Angelo Luciana, Garascia Andrea, Chieffo Alaide, Montorfano Matteo, Oliva Fabrizio, Sacco Alice
Abstract
Catheter-based revascularization procedures were developed as an alternative to systemic thrombolysis for patients with intermediate-high- and high-risk pulmonary embolisms. USAT IH-PE is a retrospective and prospective multicenter registry of such patients treated with ultrasound-facilitated, catheter-directed thrombolysis, whose preliminary results are presented in this study. The primary endpoint was the incidence of pulmonary hypertension (PH) at follow-up. Secondary endpoints were short- and mid-term changes in the echocardiographic parameters of right ventricle (RV) function, in-hospital and all-cause mortality, and procedure-related bleeding events. Between March 2018 and July 2023, 102 patients were included. The majority were at intermediate-high-risk PE (86%), were mostly female (57%), and had a mean age of 63.7 ± 14.5 years, and 28.4% had active cancer. Echocardiographic follow-up was available for 70 patients, and in only one, the diagnosis of PH was confirmed by right heart catheterization, resulting in an incidence of 1.43% (CI 95%, 0.036-7.7). RV echocardiographic parameters improved both at 24 h and at follow-up. In-hospital mortality was 3.9% (CI 95%, 1.08-9.74), while all-cause mortality was 11% (CI 95%, 5.4-19.2). Only 12% had bleeding complications, of whom 4.9% were BARC ? 3. Preliminary results from the USAT IH-PE registry showed a low incidence of PH, improvement in RV function, and a safe profile.
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Case Report: A rare homozygous patient affected by systemic amyloidosis with a prominent heart involvement.
Front Cardiovasc Med2023 ;10():1164916. doi: 1164916.
Micaglio Emanuele, Santangelo Gloria, Moscardelli Silvia, Rusconi Daniela, Musca Francesco, Verde Alessandro, Campiglio Laura, Bursi Francesca, Guazzi Marco
Abstract
Hereditary transthyretin amyloidosis is a severe, adult-onset autosomal dominant inherited systemic disease predominantly affecting the peripheral and autonomic nervous system, heart, kidney, and the eyes. We present a case of a Caucasian 65-year-old man with cardiac amyloidosis and the homozygous mutation Val142Ile (classically, Val122Ile) in the transthyretin gene. We provide a genotype-phenotype correlation regarding the genetic status of both heterozygous and homozygous individuals and their clinical conditions at the time of genetic testing.
© 2023 Micaglio, Santangelo, Moscardelli, Rusconi, Musca, Verde, Campiglio, Bursi and Guazzi.
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The impact of transcatheter edge-to-edge repair on right ventricle-pulmonary artery coupling in patients with functional mitral regurgitation.
Eur J Clin Invest2023 Jan;53(1):e13869. doi: e13869.
Tua Lorenzo, Mandurino-Mirizzi Alessandro, Colombo Claudia, Morici Nuccia, Magrini Giulia, Nava Stefano, Frassica Romina, Montalto Claudio, Ferlini Marco, Sacco Alice, Musca Francesco, Moreo Antonella, Ghio Stefano, Oreglia Jacopo, Oltrona-Visconti Luigi, Oliva Fabrizio, Crimi Gabriele
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Biochemical but not imaging parameters are predictive of outcome in septic shock: a pilot study.
Cardiovasc Ultrasound2022 Mar;20(1):6. doi: 6.
Belli Oriana E, Campolo Jonica, Vallerio Paola, Musca Francesco, Moreo Antonella, Maloberti Alessandro, Parolini Marina, Bonacchini Luca, Monti Gianpaola, De Gasperi Andrea, Fumagalli Roberto, Giannattasio Cristina
Abstract
BACKGROUND:
Septic shock is a severe form of sepsis marked by hypotension with an ominous outcome despite the introduction of modern intensive care. The aim of the present study is to obtain a panel with biomarkers, echocardiographic and vascular parameters to better risk stratify patients and identify those at higher risk of ominous outcome.
METHODS:
Between May 2013 and April 2016, 35 consecutive patients admitted at the Intensive Care Unit (ICU) of ASST Great Metropolitan Hospital Niguarda with the diagnosis of severe sepsis or septic shock were enrolled. All patients underwent rest echocardiography and several circulating biomarkers of myocardial damage or oxidative stress.
RESULTS:
The multivariate Cox's proportional hazard model showed that the only independent prognostic predictor for 30-day mortality was the angiopoietin-2, (HR 1.017, 95% CI 1.000-1.034; P?=?0.049). An angiopoietin-2 concentrations ? of 33,418?pg/mL was identified as the optimal threshold for the discrimination between survivors and non survivors at the time of admission in ICU, with a sensitivity of 80% and a specificity of 68%.
CONCLUSIONS:
Septic shock has a poor in-hospital outcome even when the best of care is implemented. Among the biochemical parameters angiopoietin was able to identify patients at risk of death. The lowest the value at admission, the highest the risk of in-hospital death. No echocardiographic nor vascular parameter was able to predict outcome in this setting.
© 2022. The Author(s).
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Prevalence and Rate of Resolution of Left Atrial Thrombus in Patients with Non-Valvular Atrial Fibrillation: A Two-Center Retrospective Real-World Study.
J Clin Med2022 Mar;11(6):. doi: 1520.
Faggiano Pompilio, Dinatolo Elisabetta, Moreo Antonella, De Chiara Benedetta, Sbolli Marco, Musca Francesco, Curnis Antonio, Belli Oriana, Giannattasio Cristina, Tomasi Cesare, Metra Marco, Santangelo Gloria
Abstract
BACKGROUND AND AIM:
Thromboembolic events due to left atrial appendage (LAA) thrombosis are the main complication of non-valvular atrial fibrillation (NVAF). Although anticoagulants are effective in patients with NVAF, a minimal residual thromboembolic risk persists. Little is known about the prevalence of LAA thrombus and the rate of resolution after the recommended period of anticoagulation therapy, including vitamin K antagonists (VKA), heparin, and non-vitamin K antagonist oral anticoagulants (NOACs).
METHODS AND RESULTS:
We aimed to study the prevalence of LAA thrombus in an unselected cohort of patients undergoing transesophageal echocardiogram (TEE), and the determinants of LAA thrombus resolution. We retrospectively analyzed 8888 consecutive TEEs performed over five years in two high-volume centers and included all patients with LAA thrombus. A total of 265 patients (3%) had an LAA thrombus. Among these, 97% presented with AF. Fifty-eight percent of patients were on anticoagulants at least three weeks before the diagnosis. After the LAA thrombus diagnosis, VKAs were prescribed in 52%, heparin in 18.5%, and NOAC in 27% of patients. Among the 183 patients with repeat TEE, performed at (25-75th) 39 days (21-84), 67% showed resolution of the LAA thrombus. Although the rate of thrombus resolution was higher in patients treated with NOACs (NOACs 71%, VKA 66%, Heparin 60%) the difference between anticoagulants was statistically non-significant (VKA, OR 0.9, = 0.83; NOAC, OR 1.23, = 0.42; heparin, OR 0.69, = 0.35). Thus, NOACs were demonstrated to be at least as effective as other anticoagulants in the rate of LAA thrombus resolution. Upon multivariate-adjusted analysis, higher LAA emptying velocities were the only predictor of thrombus resolution. In conclusion, the majority of patients were already on anticoagulants. NOACs could be at least as effective as other anticoagulants, yielding an LAA thrombus resolution in two-thirds of patients. This may have clinical relevance, especially in patients undergoing cardioversion or catheter ablation.
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Primary mediastinal large B-cell lymphoma and pregnancy: a challenging clinical scenario.
Monaldi Arch Chest Dis2022 Feb;92(4):. doi: 10.4081/monaldi.2022.2198.
Intravaia Rita, De Chiara Benedetta, Musca Francesco, Casadei Francesca, Santambrogio Gloria, Spanò Francesca, Belli Oriana, Quattrocchi Giuseppina, Giannattasio Cristina, Moreo Antonella
Abstract
A 26-weeks pregnant woman presented with progressively worsening dyspnoea and poor general conditions. Using low-dose radiation multi-imaging techniques and thoracic biopsy a primary mediastinal large B cell was diagnosed. A multidisciplinary approach identified the correct hemodynamic management, the best therapeutic strategy and the timing for delivery.
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Prevalence of hypertension mediated organ damage in subjects with high-normal blood pressure without known hypertension as well as cardiovascular and kidney disease.
J Hum Hypertens2022 Jul;36(7):610-616. doi: 10.1038/s41371-021-00604-6.
Maloberti Alessandro, Rebora Paola, Occhino Giuseppe, Alloni Marta, Musca Francesco, Belli Oriana, Spano Francesca, Santambrogio Gloria Maria, Occhi Lucia, De Chiara Benedetta, Casadei Francesca, Moreo Antonella, Valsecchi Maria Grazia, Giannattasio Cristina
Abstract
Purpose of our study was to assess the prevalence of hypertension mediated organ damage (HMOD) in healthy subjects with high-normal Blood Pressure (BP) comparing them with subjects with BP values that are considered normal (
© 2021. The Author(s), under exclusive licence to Springer Nature Limited.
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Gamma Knife Radiosurgery for Treatment of Brain Metastases during the COVID-19 Outbreak.
Stereotact Funct Neurosurg2020 ;98(5):319-323. doi: 10.1159/000510271.
Crisà Francesco Maria, Leocata Filippo, Arienti Virginia Maria, Picano Marco, Berta Luca, Mainardi Hae Song, Monti Angelo Filippo, Musca Francesco, Colombo Silvia, Palazzi Mauro, La Camera Alessandro
Abstract
INTRODUCTION:
The WHO declared 2019 severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) a public health emergency of international concern. The National and Regional Health System has been reorganized, and many oncological patients died during this period or had to interrupt their therapies. This study summarizes a single-centre experience, during the COVID-19 period in Italy, in the treatment of brain metastases with Gamma Knife stereotactic radiosurgery (GKRS).
METHODS:
We retrospectively analysed our series of patients with brain metastases who underwent GKRS at the Niguarda Hospital from February 24 to April 24, 2020.
RESULTS:
We treated 30 patients with 66 brain metastases. A total of 22 patients came from home and 8 patients were admitted to the emergency room for urgent neurological symptoms. Duration of stay was limited to 0-1 day in 17 patients. We chose to treat a cluster of 9 patients, whose greater lesion exceeded 10 cm3, with 2-stage modality GKRS to minimize tumour recurrence and radiation necrosis.
CONCLUSION:
Due to the COVID-19 pandemic, the whole world is at a critical crossroads about the use of health care resources. During the COVID-19 outbreak, the deferral of diagnostic and therapeutic procedures and a work backlog in every medical specialty are the natural consequences of reservation of resources for COVID-19 patients. GKRS improved symptoms and reduced the need for open surgeries, allowing many patients to continue their therapeutic path and sparing beds in ICUs. Neurosurgeons have to take into account the availability of stereotactic radiosurgery to reduce hospital stay, conciliating safety for patients and operators with the request for health care coming from the oncological patients and their families.
© 2020 S. Karger AG, Basel.
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Thoracic radiotherapy as a risk factor for heart ischemia in subjects treated with chest irradiation and chemotherapy and without classic cardiovascular RISK factors.
Radiother Oncol2020 Nov;152():146-150. doi: 10.1016/j.radonc.2020.07.004.
Vallerio Paola, Maloberti Alessandro, Palazzini Matteo, Occhi Lucia, Peretti Alessio, Nava Stefano, Soriano Francesco, Musca Francesco, De Chiara Benedetta, Belli Oriana, Moreo Antonella, Bisceglia Irma, Lestuzzi Chiara, Giannattasio Cristina
Abstract
BACKGROUND AND PURPOSE:
Radiation Induced Heart Disease (RIHD) represents a late effect of chest irradiation, contributing in increasing mortality rate in oncological patients by affecting pericardium, myocardium, valvs and coronaries. Currently, regarding the risk of Coronary Artery Disease (CAD), a cardiological screening involving exercise stress electrocardiography after 5-10 years from radiotherapy is advised. We sought to determine the rate of ischemia at exercise stress electrocardiography in a population of patients without cardiovascular risk factors who sustained radiotherapy, using a cohort of patients presenting with at least one cardiovascular risk factor as control group.
DESIGN AND METHODS:
A population of 115 patients who sustained chest irradiation (and associated chemotherapy), presenting without classic cardiovascular risk factors or typical symptoms suggesting CAD, was evaluated with exercise stress electrocardiography. 135 patients with at least one risk factor for cardiovascular disease candidate to stress testing for primary prevention or for atypical symptoms served as control group.
RESULTS:
The cohort of irradiated patients without classical cardiovascular risk factors is younger (48.7 ± 10.1 vs 60.5 ± 10.8 years, p
CONCLUSIONS:
Chest irradiation represents a strong cardiovascular risk factor. In fact, prevalence of positive ECG-stress test is not different (nor higher and nor lower) in irradiated subjects without cardiovascular risk and not irradiated patients with classic cardiovascular risk.
Copyright © 2020 Elsevier B.V. All rights reserved.
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Assessment of right ventricular function in advanced heart failure with nonischemic dilated cardiomyopathy: insights of right ventricular elastance.
J Cardiovasc Med (Hagerstown)2020 Feb;21(2):134-143. doi: 10.2459/JCM.0000000000000921.
Bianco Francesco, Bucciarelli Valentina, Ammirati Enrico, Occhi Lucia, Musca Francesco, Tonti Giovanni, Frigerio Maria, Gallina Sabina
Abstract
BACKGROUND:
The right ventriculoarterial coupling (R-V/A), a measure of right ventricular systolic dysfunction (RVSD) adaptation/maladaptation to chronic overload, and consequent pulmonary hypertension, has been little investigated in nonischemic dilated cardiomyopathy (NIDCM). We examined the correlates of R-V/A and traditional echocardiographic indices of RVSD, over the spectrum of pulmonary hypertension and tertiles of mean pulmonary artery pressures (PAPm).
METHODS:
In 2016-2017, we studied 81 consecutive patients for heart transplant/advanced heart failure. Inclusion criteria were NIDCM, reduced ejection fraction (?40%) and sinus rhythm. R-V/A was computed as the RV/pulmonary elastances ratio (R-Elv/P-Ea), derived from a combined right heart catheterization/transthoracic- echocardiographic assessment [right heart catheterization/transthoracic-echocardiographic (RHC/TTE)].
RESULTS:
A total of 68 patients (mean age 64?±?7 years, 82% men) were eligible. After adjustments, R-Elv and P-Ea were higher in isolated postcapillary-pulmonary hypertension (Ipc-PH) than combined-pulmonary hypertension (Cpc-PH) (P?=?0.004 and P?=?0.002, respectively), whereas R-V/A progressively decreased over Ipc-PH and Cpc-PH (P?=?0.006). According to PAPm increment, P-Ea congruently increased (P-Trend?=?0.028), R-Elv progressively decreased (P-Trend
CONCLUSION:
Among NIDCM HF patients, in a small cohort study, RHC/TTE-derived R-V/A assessment demonstrated good correlations with pulmonary hypertension types and RV functional status. These data suggest that R-V/A encloses comprehensive information of the whole cardiopulmonary efficiency, better clarifying the amount of RVSD, with good reliability.
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Could two-dimensional radial strain be considered as a novel tool to identify pre-clinical hypertrophic cardiomyopathy mutation carriers?
Int J Cardiovasc Imaging2019 Dec;35(12):2167-2175. doi: 10.1007/s10554-019-01668-9.
Santambrogio Gloria Maria, Maloberti Alessandro, Vallerio Paola, Peritore Angelica, Spanò Francesca, Occhi Lucia, Musca Francesco, Belli Oriana, De Chiara Benedetta, Casadei Francesca, Facchetti Rita, Turazza Fabio, Manfredini Emanuela, Giannattasio Cristina, Moreo Antonella
Abstract
Treatment of overt form of hypertrophic cardiomyopathy (HCM) is often unsuccessful. Efforts are focused on a possible early identification in order to prevent or delaying the development of hypertrophy. Our aim was to find an echocardiographic marker able to distinguish mutation carriers without left ventricular hypertrophy (LVH) from healthy subjects. We evaluated 28 patients, members of eight families. Three types of mutation were recognized: MYBPC3 (five families), MYH7 (two families) and TNNT2 (one family). According to genetic (G) and phenotypic (Ph) features, patients were divided in three groups: Group A (10 patients), mutation carriers with LVH (G+/Ph+); Group B (9 patients), mutation carriers without LVH (G+/Ph-); Group C (9 patients), healthy subjects (G-/Ph-). Echocardiography examination was performed acquiring standard 2D, DTI and 2D-strain imaging. Global longitudinal strain (GLS) and global radial strain (GRS) at basal and mid-level were measured. GRS was significantly different between group B and C at basal level (32.18%?±?9.6 vs. 44.59%?±?12.67 respectively; p-value?0.0001). In basal posterior and basal inferior segments this difference was particularly evident. ROC curves showed for both the involved segments good AUCs (0.931 and 0.861 for basal posterior and inferior GRS respectively) with the best predictive cut-off for basal posterior GRS at 43.65%, while it was 38.4% for basal inferior GRS. Conversely, GLS values were similar in the three group. 2D longitudinal strain is a valid technique to study HCM. Radial strain and particularly basal posterior and inferior segmental reduction could be able to identify mutation carriers in a pre-clinical phase of disease.
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Safety of centrifugal left ventricular assist device in patients previously treated with MitraClip system.
Int J Cardiol2019 May;283():131-133. doi: 10.1016/j.ijcard.2019.02.039.
Ammirati Enrico, Van De Heyning Caroline M, Musca Francesco, Brambatti Michela, Perna Enrico, Cipriani Manlio, Cannata Aldo, Mondino Michele, Moreo Antonella, De Bock Dina, Pretorius Victor, Claeys Marc J, Adler Eric D, Russo Claudio F, Frigerio Maria
Abstract
INTRODUCTION:
No data regarding the safety of continuous-flow left ventricular assist device (CF-LVAD) implantation in patients with previous MitraClip have been reported. Thus, it remains unknown whether an initial treatment strategy with MitraClip therapy might complicate future heart failure management in patients who are also considered for CF-LVAD.
METHODS:
We retrospectively identified 6 patients (median age of 62?years; 2 women) who had been treated with MitraClip, that were eventually implanted with a CF-LVAD (all Heartware HVAD) in 3 hospitals between 2013 and 2018.
RESULTS:
Patients were treated in 4 cases with 2 clips, and in 2 cases with 1 clip. Median time from MitraClip implantation to CF-LVAD implant was 282?days (interquartile range 67 to 493), and median time on CF-LVAD support was 401?days (interquartile range 105 to 492?days). Two patients underwent a heart transplant, 3 patients died on support, and 1 is alive on support. In all cases, there was a reduction of functional mitral regurgitation without MitraClip-related complications.
CONCLUSIONS:
Based on this small case series, implantation of a CF-LVAD appears safe in patients with a previously positioned MitraClip system, at least, with 1 or 2 clips in place, with no need for additional mitral valve surgery.
Copyright © 2019 Elsevier B.V. All rights reserved.
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Portico Sheathless Transcatheter Aortic Valve Implantation via Distal Axillary Artery.
Ann Thorac Surg2017 Feb;103(2):e175-e177. doi: 10.1016/j.athoracsur.2016.07.065.
Bruschi Giuseppe, Colombo Paola, Botta Luca, Nava Stefano, Merlanti Bruno, Belli Oriana, Musca Francesco, Soriano Francesco, Russo Claudio F, Oliva Fabrizio
Abstract
Transcatheter aortic valve implantation has been designed to treat older patients affected by severe aortic stenosis who are considered high-risk surgical candidates because of multiple comorbidities. The least invasive approach for transcatheter aortic valves implantation should be considered the transfemoral retrograde route, because it is minimally invasive and is feasible with local anesthesia and mild sedation. Despite significant technical improvements in recent years, the transfemoral approach is contraindicated in cases of severe peripheral artery disease. We describe the first case of a Portico transcatheter aortic valve implantation system (St. Jude Medical, Minneapolis, MN) made through the distal axillary artery in a 90-year-old patient affected by severe aortic stenosis.
Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
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Annexin A5 in treated hypertensive patients and its association with target organ damage.
J Hypertens2017 Jan;35(1):154-161.
Maloberti Alessandro, Meani Paolo, Vallerio Paola, Varrenti Marisa, Casadei Francesca, Musca Francesco, Facchetti Rita, Di Blasio Anna M, Ravassa Susanna, Mancia Giuseppe, Giannattasio Cristina
Abstract
OBJECTIVE:
Annexin A5 (AnxA5) has been previously linked to the presence of carotid and cardiac target organ damage (TOD) in the context of heart failure and rheumatologic patients. However, information is scant in the context of hypertension. Aim of our study was to evaluate AnxA5 in treated hypertension patients compared with normotensive controls and to determine whether it is associated with vascular and heart TOD evaluated as arterial stiffness, carotid plaque and left ventricular hypertrophy.
METHODS:
We enrolled 123 consecutive treated hypertension and 124 normotensive controls. TOD was evaluated as pulse wave velocity (PWV, complior), left ventricular hypertrophy (echocardiography) and intima-media thickness and carotid plaque presence (ecographic methods). AnxA5 levels was dosed and compared in patients with and without hypertension and with and without TOD.
RESULTS:
With similar age hypertension patients showed higher SBP, DBP and AnxA5 levels (13.9?±?11.1 vs 10.1?±?8.4?ng/ml, P?0.001) compared with controls. Regarding TOD hypertension showed higher PWV (8.5?±?1.8 vs 7.6?±?1.5?m/s, P?0.001) and LVMI (121.7?±?29.3 vs 113.5?±?21.1?g/m, P?0.05), whereas carotid intima-media thickness was superimposable. AnxA5 correlates with PWV (r?=?0.13, P?0.05) and DBP (r?=?0.15, P?0.01), whereas it has never been found as a significant independent predictor of TOD in linear regression analysis.
CONCLUSION:
Our data have shown that AnxA5 levels are increased in treated hypertension patients. In this condition, it is probably released in the plasma as a defensive mechanism through its anti-inflammatory and anticoagulants effects. We found a significant association with arterial stiffness, but AnxA5 was not found to be a significant predictor of TOD.
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Evolut R Implantation to Treat Severe Pure Aortic Regurgitation in a Patient With Mitral Bioprosthesis.
Ann Thorac Surg2016 Dec;102(6):e521-e524. doi: 10.1016/j.athoracsur.2016.05.054.
Bruschi Giuseppe, Colombo Paola, Nava Stefano, Musca Francesco, Merlanti Bruno, Belli Oriana, Soriano Francesco, Botta Luca, De Caria Danile, Giannattasio Cristina, Russo Claudio F
Abstract
Transcatheter aortic valves have been designed to treat high-risk surgical candidates affected by severe aortic stenosis, but little is known about the use of transcatheter valves in patients with severe pure aortic regurgitation. We describe the implantation of Medtronic CoreValve Evolut R (Medtronic, Minneapolis, MN) to treat an 82-year-old patient affected by severe pure aortic regurgitation who underwent prior mitral valve replacement with a biological valve protruding into the left ventricular outflow tract.
Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
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A new access for transcatheter aortic valve implantation: Distal axillary artery.
Int J Cardiol2016 Nov;223():810-812. doi: 10.1016/j.ijcard.2016.08.290.
Bruschi Giuseppe, Colombo Paola, Merlanti Bruno, Nava Stefano, Belli Oriana, Musca Francesco, Soriano Francesco, Botta Luca, Calini Angelo, De Caria Daniele F, Oliva Fabrizio, Russo Claudio F
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Long-Term Effects of Radiotherapy on Arterial Stiffness in Breast Cancer Women.
Am J Cardiol2016 Sep;118(5):771-6. doi: 10.1016/j.amjcard.2016.06.001.
Vallerio Paola, Sarno Laura, Stucchi Miriam, Musca Francesco, Casadei Francesca, Maloberti Alessandro, Lestuzzi Chiara, Mancia Giuseppe, Moreo Antonella, Palazzi Mauro, Giannattasio Cristina
Abstract
Radiotherapy for breast cancer may expose heart and vessels to late radiation-induced complications. Although recent technical progress in radiation therapy (RT) has been associated with drastic reduction in cardiovascular (CV) mortality, the prolonged life expectancy of patients with cancer requires CV evaluation for many years. The aim of our study was to evaluate local changes in vascular and cardiac function because of previous breast RT. We enrolled 43 patients treated with RT 15 years ago for breast cancer. CV risk factors and atherosclerotic carotid damage were investigated in all women. We divided patients into 2 groups: R (n = 25) treated to right breast and L (n = 18) to left breast. All subjects were submitted to standard echocardiography and functional arteries evaluation by carotid-radial pulse-wave velocity (crPWV; Complior) and AIx (Sphygmocor; Atcor Medical). Global mean age was 69.5 ± 8 years old. CV risk factors were equally allocated in 2 groups. No patients had history of cardiac or artery disease. R had a significantly increased crPWV (9.9 ± 1.4 vs 8.9 ± 1.1, p = 0.001) on right arm compared with left arm, and in L group, crPWV was similarly higher on the left arm than on right arm (9.6 ± 1.5 vs 8.9 ± 1.4, p = 0.011). AIx was significantly increased in the ipsilateral arm only in L (32.1 ± 7.6 vs 28.3 ± 6.8, p = 0.05). Central blood pressure estimation was not different in the right and left arms. No correlations were found with hormone therapy or chemotherapy. Our data show a local arterial stiffening because of radiation that can be involved in increased CV risk in breast cancer-treated patients.
Copyright © 2016 Elsevier Inc. All rights reserved.
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A life-threatening presentation of eosinophilic granulomatosis with polyangiitis.
J Cardiovasc Med (Hagerstown)2016 Dec;17 Suppl 2():e109-e111. doi: 10.2459/JCM.0000000000000330.
Ammirati Enrico, Cipriani Manlio, Musca Francesco, Bonacina Edgardo, Pedrotti Patrizia, Roghi Alberto, Astaneh Arash, Schroeder Jan W, Nonini Sandra, Russo Claudio F, Oliva Fabrizio, Frigerio Maria
Abstract
: Necrotizing eosinophilic myocarditis (NEM) is a life-threatening condition that needs rapid diagnosis by endomyocardial biopsy and hemodynamic support usually by mechanical circulatory systems. We present the case of a 25-year-old Caucasian man who developed a refractory cardiogenic shock due to a NEM that was supported with a peripheral veno-arterial extracorporeal membrane oxygenation associated with intravenous steroids and recovered after 2 weeks. Further instrumental investigations lead to the final diagnosis of NEM as first presentation of eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), remarking the importance of identifying the systemic disorder that usually triggers the eosinophilic damage of the myocardium.
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CoreValve Evolut R implantation as valve-in-valve in an Edwards SAPIEN 3 to treat paravalvular regurgitation.
EuroIntervention2015 Sep;11(5):e1. doi: 10.4244/EIJV11I5A116.
Bruschi Giuseppe, Soriano Francesco, Musca Francesco, Nava Stefano, Einaudi Arturo, Garascia Andrea, Belli Oriana, Barosi Alberto, Fratto Pasquale, Colombo Paola, Russo Claudio Francesco, Gagliardone Maria Pia, Klugmann Silvio
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Effects of Cancer Therapy Targeting Vascular Endothelial Growth Factor Receptor on Central Blood Pressure and Cardiovascular System.
Am J Hypertens2016 Feb;29(2):158-62. doi: 10.1093/ajh/hpv077.
Moreo Antonella, Vallerio Paola, Ricotta Riccardo, Stucchi Miriam, Pozzi Mattia, Musca Francesco, Meani Paolo, Maloberti Alessandro, Facchetti Rita, Di Bella Sara, Giganti Maria Olga, Sartore-Bianchi Andrea, Siena Salvatore, Mancia Giuseppe, Giannattasio Cristina
Abstract
BACKGROUND:
In the last 2 decades, new drugs that oppose the effects of vascular endothelial growth factor receptor (VEGFR), and thus angiogenesis, have considerably improved treatment of solid tumors. These anti-VEGFR drugs, however, are burdened by several side effects, particularly relevant on heart and vessels. The aim of this study was to analyze the changes in cardiovascular structure and function associated with use of anti-VEGFR drugs.
METHODS:
Twenty-nine patients (27 affected by renal and 2 by thyroid cancer), received treatment with anti-VEGFR drugs. Brachial blood pressure (BP), central BP, carotid-femoral pulse wave velocity (cfPWV), augmentation index (Aix), and several echocardiographic markers of systolic and diastolic left ventricular functions including global longitudinal strain were measured before starting treatment (T0), after 2 (T1), and 6 weeks (T2) of treatment.
RESULTS:
Anti-VEGFR treatment was accompanied by a significant increase of both peripheral (systolic BP +13±15.5mm Hg, diastolic BP +7.1±9.3mm Hg, P
CONCLUSIONS:
All the changes regarding BP and cfPWV appear early after treatment initiation and seem to be reversible if treatment is stopped, instead diastolic and systolic left ventricular function are persistently altered by anti-VEGFR drugs.
© American Journal of Hypertension, Ltd 2015. All rights reserved. For Permissions, please email: journals.permissions@oup.com.
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Cardiac metastatic melanoma: Imaging diagnostic clues.
J Cardiol Cases2015 Aug;12(2):33-36. doi: 10.1016/j.jccase.2015.03.001.
Pedrotti Patrizia, Musca Francesco, Torre Massimo, Pirola Roberto, De Biase Anna Maria, Fieschi Stefano, Quattrocchi Giuseppina, Roghi Alberto, Giannattasio Cristina
Abstract
A 47-year-old male was admitted to hospital for severe pericardial effusion; he had undergone surgical removal of cutaneous melanoma 10 years before. Echocardiography-guided pericardiocentesis revealed the presence of intramyocardial masses, which were better defined and characterized, together with pericardial involvement, by cardiac magnetic resonance. Pericardial fluid drained was negative for malignant cells, so video-assisted thoracoscopy was performed and pathologic tissue was biopsied, leading to the diagnosis of metastatic melanoma. Multidisciplinary approach and multimodality imaging played a key role in allowing the diagnostic workup in this complex case. .
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Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis.
J Hypertens2014 May;32(5):1121-31; discussion 1131. doi: 10.1097/HJH.0000000000000120.
Perlini Stefano, Salinaro Francesco, Musca Francesco, Mussinelli Roberta, Boldrini Michele, Raimondi Ambra, Milani Paolo, Foli Andrea, Cappelli Francesco, Perfetto Federico, Palladini Giovanni, Rapezzi Claudio, Merlini Giampaolo
Abstract
BACKGROUND:
Cardiac amyloidosis represents an archetypal form of restrictive heart disease, characterized by profound diastolic dysfunction. As ejection fraction is preserved until the late stage of the disease, the majority of patients do fulfill the definition of diastolic heart failure, that is, heart failure with preserved ejection fraction (HFpEF). In another clinical model of HFpEF, that is, pressure-overload hypertrophy, depressed midwall fractional shortening (mFS) has been shown to be a powerful prognostic factor.
OBJECTIVE AND METHODS:
To assess the potential prognostic role of mFS in cardiac light-chain amyloidosis with preserved ejection fraction, we enrolled 221 consecutive untreated patients, in whom a first diagnosis of cardiac light-chain amyloidosis was concluded between 2008 and 2010. HFpEF was present in 181 patients. Patients in whom cardiac involvement was excluded served as controls (n?=?121). Prognosis was assessed after a median follow-up of 561 days.
RESULTS:
When compared with light-chain amyloidosis patients without myocardial involvement, cardiac light-chain amyloidosis was characterized by increased wall thickness (P?
CONCLUSION:
In cardiac light-chain amyloidosis with normal ejection fraction, depressed circumferential mFS, a marker of myocardial contractile dysfunction, is a powerful predictor of survival.
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Prevalence and prognostic value of conduction disturbances at the time of diagnosis of cardiac AL amyloidosis.
Ann Noninvasive Electrocardiol2013 Jul;18(4):327-35. doi: 10.1111/anec.12032.
Boldrini Michele, Salinaro Francesco, Mussinelli Roberta, Raimondi Ambra, Alogna Alessio, Musca Francesco, Palladini Giovanni, Merlini Giampaolo, Perlini Stefano
Abstract
BACKGROUND AND PURPOSE:
To evaluate the prevalence and the prognostic implications of conduction delays in a large cohort of cardiac AL patients.
METHODS:
Echo Doppler and 12-lead ECG were collected in 344 consecutive patients in whom diagnosis of AL amyloidosis was concluded between 2008 and 2010. Patients were subdivided according to the presence (n?=?240) or absence (n?=?104) of cardiac involvement.
RESULTS:
When compared with patients without myocardial involvement, cardiac AL was associated with prolonged PQ, QRS, QT and QTc intervals (P?0.05), and with higher prevalence of intraventricular blocks (27.5% vs. 16.5%, P?0.05), that was associated with higher wall thickness, worse diastolic and regional systolic function, higher NT-proBNP values (all P?0.05), and higher mortality (P?=?0.0001; median follow-up: 402 days).
CONCLUSION:
Intraventricular conduction delays have a negative prognostic impact in patients with cardiac AL amyloidosis. Their presence should not be overlooked in the diagnostic workup, prompting a more accurate cardiological support.
©2013, Wiley Periodicals, Inc.
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Levosimendan reverted severe pulmonary hypertension in one patient on waiting list for heart transplantation.
Int J Cardiol2013 Oct;168(4):4518-9. doi: 10.1016/j.ijcard.2013.06.106.
Ammirati Enrico, Musca Francesco, Oliva Fabrizio, Garascia Andrea, Pacher Valentina, Verde Alessandro, Cipriani Manlio, Moreo Antonella, Martinelli Luigi, Frigerio Maria
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Diagnostic and prognostic value of low QRS voltages in cardiac AL amyloidosis.
Ann Noninvasive Electrocardiol2013 May;18(3):271-80. doi: 10.1111/anec.12036.
Mussinelli Roberta, Salinaro Francesco, Alogna Alessio, Boldrini Michele, Raimondi Ambra, Musca Francesco, Palladini Giovanni, Merlini Giampaolo, Perlini Stefano
Abstract
BACKGROUND:
In cardiac AL amyloidosis, myocardial infiltration is typically associated with "low QRS voltages" at the 12-lead electrocardiogram (ECG). Although considered as one of the hallmarks of the disease, its reported prevalence varies from 45% to 70%, mainly because of nonhomogeneous definitions.
METHODS:
To identify the "low QRS voltage" parameter having the best diagnostic value in identifying cardiac amyloidosis, and to assess its possible prognostic role, ECG and echocardiographic data were collected at diagnosis in 337 consecutive never-treated AL patients (233 with, 104 without cardiac involvement). Prognosis was assessed after a median follow-up of 14.5 months.
RESULTS:
"Low QRS voltage" prevalence varied from 84.12% when using Sokolow-Lyon index ?15 mm to 27.04% with the definition of low total voltages (QRS amplitude ?5 mm in each peripheral and ?10 mm in each precordial lead), the widely used definition of low peripheral voltages (?5 mm in each peripheral lead) being able to identify 66.52% cardiac AL patients. The presence of "low peripheral voltages" was associated with a more severe cardiac involvement, and was able to differentiate Mayo stage II patients' survival (i.e., AL patients with intermediate prognosis). According to receiver operator characteristic (ROC) curve analysis, sensitivity and specificity were 58.72% and 80.00%, for a peripheral QRS amplitude ?24.5 mm (the sum of QRS in all the 6 peripheral leads), and 76.26% and 65.00% for a Sokolow-Lyon index ?11 mm.
CONCLUSIONS:
In cardiac AL amyloidosis the prevalence of low QRS voltages is highly dependent on the method used for defining this ECG alteration.
©2013, Wiley Periodicals, Inc.
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Limited changes in severe functional mitral regurgitation and pulmonary hypertension after left ventricular assist device implantation: a clue to consider concurrent mitral correction?
Int J Cardiol2013 Jul;167(2):e35-7. doi: 10.1016/j.ijcard.2013.03.098.
Ammirati Enrico, Musca Francesco, Cannata Aldo, Garascia Andrea, Verde Alessandro, Pacher Valentina, Moreo Antonella, Oliva Fabrizio, Martinelli Luigi, Frigerio Maria
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Clinical and echocardiographic correlations of exercise-induced pulmonary hypertension in systemic sclerosis: a multicenter study.
Am Heart J2013 Feb;165(2):200-7. doi: 10.1016/j.ahj.2012.10.020.
Gargani Luna, Pignone Alberto, Agoston Gergely, Moreo Antonella, Capati Eugenia, Badano Luigi P, Doveri Marica, Bazzichi Laura, Costantino Marco Fabio, Pavellini Andrea, Pieri Francesco, Musca Francesco, Muraru Denisa, Epis Oscar, Bruschi Eleonora, De Chiara Benedetta, Perfetto Federico, Mori Fabio, Parodi Oberdan, Sicari Rosa, Bombardieri Stefano, Varga Albert, Cerinic Marco Matucci, Bossone Eduardo, Picano Eugenio
Abstract
BACKGROUND:
Patients with systemic sclerosis (SSc) are at risk for developing pulmonary hypertension, which is associated with a poor prognosis. Exercise Doppler echocardiography enables the identification of exercise-induced increase in pulmonary artery systolic pressure (PASP) and may provide a thorough noninvasive hemodynamic evaluation.
AIM:
The aim of this study was to evaluate the clinical and echocardiographic determinants of exercise-induced increase in PASP in a large population of patients with SSc.
METHODS:
We selected 164 patients with SSc (age 58 ± 13 years, 91% female) with normal resting PASP (
RESULTS:
Sixty-nine (42%) patients showed a significant exercise-induced increase in PASP. Among them, peak PVR ?3 Wood Units was present only in 11% of patients, about 5% of the total population. Univariate analysis showed that age, presence of interstitial lung disease, and both right and left diastolic dysfunction are predictors of peak PASP ?50 mm Hg, but none of these parameters predict elevated peak PVR.
CONCLUSIONS:
Exercise-induced increase in PASP occurs in almost one-half of patients with SSc with normal resting PASP. Peak exercise PASP is affected by age, interstitial lung disease, and right and left ventricular diastolic dysfunction and, only in 5% of the patients, is associated with an increase in PVR during exercise, suggesting heterogeneity of the mechanisms underlying exercise-induced pulmonary hypertension in SSc.
Copyright © 2013 Mosby, Inc. All rights reserved.
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Simulation-guided cardiac auscultation improves medical students' clinical skills: the Pavia pilot experience.
Intern Emerg Med2014 Mar;9(2):165-72. doi: 10.1007/s11739-012-0811-z.
Perlini Stefano, Salinaro Francesco, Santalucia Paola, Musca Francesco
Abstract
Clinical evaluation is the cornerstone of any cardiac diagnosis, although excessive over-specialisation often leads students to disregard the value of clinical skills, and to overemphasize the approach to instrumental cardiac diagnosis. Time restraints, low availability of "typical" cardiac patients on whom to perform effective bedside teaching, patients' respect and the underscoring of the value of clinical skills all lead to a progressive decay in teaching. Simulation-guided cardiac auscultation may improve clinical training in medical students and residents. Harvey(©) is a mannequin encompassing more than 50 cardiac diagnoses that was designed and developed at the University of Miami (Florida, USA). One of the advantages of Harvey(©) simulation resides in the possibility of listening, comparing and discussing "real" murmurs. To objectively assess its teaching performance, the capability to identify five different cardiac diagnoses (atrial septal defect, normal young subject, mitral stenosis with tricuspid regurgitation, chronic mitral regurgitation, and pericarditis) out of more than 50 diagnostic possibilities was assessed in 523 III-year medical students (i.e. at the very beginning of their clinical experience), in 92 VI-year students, and in 42 residents before and after a formal 10-h teaching session with Harvey(©). None of them had previously experienced simulation-based cardiac auscultation in addition to formal lecturing (all three groups) and bedside teaching (VI-year students and residents). In order to assess the "persistence" of the acquired knowledge over time, the test was repeated after 3 years in 85 students, who did not repeat the formal 10-h teaching session with Harvey(©) after the III year. As expected, the overall response was poor in the "beginners" who correctly identified 11.0 % of the administered cardiac murmurs. After simulation-guided training, the ability to recognise the correct cardiac diagnoses was much better (72.0 %; p
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Prognostic value of fragmented QRS in cardiac AL amyloidosis.
Int J Cardiol2013 Sep;167(5):2156-61. doi: 10.1016/j.ijcard.2012.05.097.
Perlini Stefano, Salinaro Francesco, Cappelli Francesco, Perfetto Federico, Bergesio Franco, Alogna Alessio, Mussinelli Roberta, Boldrini Michele, Raimondi Ambra, Musca Francesco, Palladini Giovanni, Merlini Giampaolo
Abstract
BACKGROUND:
In light-chain (AL) cardiac amyloidosis, the 12-lead electrocardiogram (ECG) reflects myocardial amyloid infiltration with low limb voltages, pseudoinfarction patterns, and conduction abnormalities. Moreover, it is not unusual to see "aspecific" QRS complex abnormalities, such as notches and RsR' pattern in the absence of QRS prolongation, i.e. a fragmentation of QRS complexes (fQRS), that has been associated with myocardial scars and prognosis. Since cardiomyocyte damage and interstitial fibrosis are associated with cardiac amyloid deposition, aim of the present study was to analyze the prevalence and the potential prognostic value of fQRS in patients with cardiac amyloidosis.
METHODS:
We enrolled 375 consecutive untreated patients in whom a first AL amyloidosis diagnosis was concluded between 2008 and 2010, 264 with and 111 without heart involvement. Patients with a positive history of coronary disease were excluded from the analysis.
RESULTS:
The prevalence of fQRS was significantly higher in patients with cardiac AL amyloidosis (28.5% vs. 11.7%; p=0.0008). After a median follow-up of 561 days, Kaplan-Meier survival analysis revealed a significantly higher mortality in the fQRS group when compared with the "normal" QRS group (p=0.0008). No association was found between the presence of fQRS and the duration of PQ, QRS, and QTc intervals, the presence of peripheral low voltages or pseudonecrosis, NT-proBNP serum levels or cardiac wall thickness.
CONCLUSIONS:
In patients with cardiac AL amyloidosis, the presence of fQRS at diagnosis has an independent prognostic value. Such a simple and cheap analysis in patients' diagnostic work-up may improve diagnosis and prognostic stratification.
Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.
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Right ventricular function in AL amyloidosis: characteristics and prognostic implication.
Eur Heart J Cardiovasc Imaging2012 May;13(5):416-22. doi: 10.1093/ejechocard/jer289.
Cappelli Francesco, Porciani Maria Cristina, Bergesio Franco, Perlini Stefano, Attanà Paola, Moggi Pignone Alberto, Salinaro Francesco, Musca Francesco, Padeletti Luigi, Perfetto Federico
Abstract
AIM:
The importance of right ventricle (RV) dysfunction in AL amyloidosis has been underestimated. This study was designed to comprehensively evaluate RV function and its prognostic role in patients with AL amyloidosis with and without echocardiographic evidence of cardiac involvement.
METHOD AND RESULTS:
Fifty-two biopsy-proven AL amyloidosis patients underwent a thorough echocardiographic evaluation. Twenty-seven patients (CA) met the international echocardiographic criteria for cardiac involvement [left ventricular (LV) wall thickness ? 12 mm] and 25 patients had no cardiac amyloidosis features (NCA). Patients were compared with a sex- age-matched control group. Patients and controls underwent traditional, tissue Doppler (TDI), speckle-tracking left and RV echocardiographic evaluation. No difference was observed between groups in RV diastolic diameter, whereas CA patients showed increased RV free wall thickness (P
CONCLUSION:
Our data show that in patients with AL amyloidosis, RV involvement develops later than LV amyloid deposition but when it occurs, prognosis dramatically worsens. Moreover RV longitudinal strain was the only echocardiographic predictor of prognosis. We suggest that RV function analysis should be performed routinely as a part of echocardiographic evaluation in these patients.
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Influence of CoreValve ReValving System implantation on mitral valve function: an echocardiographic study in selected patients.
Catheter Cardiovasc Interv2011 Oct;78(4):638-44. doi: 10.1002/ccd.23045.
De Chiara Benedetta, Moreo Antonella, De Marco Federico, Musca Francesco, Oreglia Jacopo, Lobiati Elisabetta, Bruschi Giuseppe, Belli Oriana, Mauri Francesco, Klugmann Silvio
Abstract
OBJECTIVES:
The purpose of this study is to verify whether transcatheter aortic valve implantation (TAVI) determined changes in mitral valve (MV) function, in terms of mitral regurgitation (MR) and stenosis.
BACKGROUND:
Little data is available regarding the effects of TAVI on global MV function, often derived from analysis primarily focused on clinical and aortic related outcomes.
METHODS:
From May 2008 to March 2010, 73 patients with severe symptomatic aortic stenosis underwent TAVI with the CoreValve ReValving System. The study population consisted of 58 patients (27 males, mean age 82 ± 7 years) who underwent transthoracic echocardiography at least ?1 month after implantation (mean follow-up 7.8 ± 5.4 months).
RESULTS:
In patients with a left ventricular dysfunction (ejection fraction, EF,
CONCLUSIONS:
In the majority of patients no significant changes occurred in the degree of MR in native valve, but we found that if the aortic valve was deeply implanted in the left ventricle outflow tract, a worsening in MR can be observed. A mitral stenosis development must be sought in patients with heavy calcifications of the anterior leaflet.
Copyright © 2011 Wiley-Liss, Inc.
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Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types.
Circulation2009 Sep;120(13):1203-12. doi: 10.1161/CIRCULATIONAHA.108.843334.
Rapezzi Claudio, Merlini Giampaolo, Quarta Candida C, Riva Letizia, Longhi Simone, Leone Ornella, Salvi Fabrizio, Ciliberti Paolo, Pastorelli Francesca, Biagini Elena, Coccolo Fabio, Cooke Robin M T, Bacchi-Reggiani Letizia, Sangiorgi Diego, Ferlini Alessandra, Cavo Michele, Zamagni Elena, Fonte Maria Luisa, Palladini Giovanni, Salinaro Francesco, Musca Francesco, Obici Laura, Branzi Angelo, Perlini Stefano
Abstract
BACKGROUND:
Most studies of amyloidotic cardiomyopathy consider as a single entity the 3 main systemic cardiac amyloidoses: acquired monoclonal immunoglobulin light-chain (AL); hereditary, mutated transthyretin-related (ATTRm); and wild-type transthyretin-related (ATTRwt). In this study, we compared the diagnostic/clinical profiles of these 3 types of systemic cardiac amyloidosis.
METHODS AND RESULTS:
We conducted a longitudinal study of 233 patients with clear-cut diagnosis by type of cardiac amyloidosis (AL, n=157; ATTRm, n=61; ATTRwt, n=15) at 2 large Italian centers providing coordinated amyloidosis diagnosis/management facilities since 1990. Average age at diagnosis was higher in AL than in ATTRm patients; all ATTRwt patients except 1 were elderly men. At diagnosis, mean left ventricular wall thickness was higher in ATTRwt than in ATTRm and AL. Left ventricular ejection fraction was moderately depressed in ATTRwt but not in AL or ATTRm. ATTRm patients less often displayed low QRS voltage (25% versus 60% in AL; P
CONCLUSIONS:
AL, ATTRm, and ATTRwt should be considered 3 different cardiac diseases, probably characterized by different pathophysiological substrates and courses. Awareness of the diversity underlying the cardiac amyloidosis label is important on several levels, ranging from disease classification to diagnosis and clinical management.
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