Pubblicazioni - Musca Dott. Francesco

• Could two-dimensional radial strain be considered as a novel tool to identify pre-clinical hypertrophic cardiomyopathy mutation carriers?
  Int J Cardiovasc Imaging

  2019 Jul;():. 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?
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• Safety of centrifugal left ventricular assist device in patients previously treated with MitraClip system.
  Int J Cardiol

  2019 05;283():131-133. doi: S0167-5273(18)36049-2.
  
  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 Surg

  2017 Feb;103(2):e175-e177. doi: S0003-4975(16)30974-2.
  
  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 Hypertens

  2017 01;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?
  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 Surg

  2016 Dec;102(6):e521-e524. doi: S0003-4975(16)30549-5.
  
  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 Cardiol

  2016 Nov;223():810-812. doi: S0167-5273(16)32008-3.
  
  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 Cardiol

  2016 09;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.
  EuroIntervention

  2015 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 Hypertens

  2016 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 < 0.001) and central BP (systolic BP +14±14.2mm Hg, diastolic BP +7.3±10.4mm Hg, P < 0.001) and a significant raise of cfPWV (+1.3±1.8 m/sec, P = 0.003). There was also a significant alteration of markers of diastolic and subclinical left ventricular systolic function, including global longitudinal strain (-19.9±3.8% at T0, -17.8±2.6% at T2, P < 0.05). All the changes were already evident at T1, worsened at T2 in patients who maintained oncological treatment, but disappeared at T2 in patients in whom treatment was stopped.
  
  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 Cases

  2015 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. < The diagnosis of cardiac metastases is challenging and histologic characterization is necessary to guide therapy. Multimodality imaging and minimally invasive thoracoscopy are key tools to achieve these goals.>.
  
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• Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis.
  J Hypertens

  2014 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?<0.001), reduced end-diastolic left ventricular volumes (P?<0.001), and diastolic dysfunction (P?<0.001). In patients with preserved ejection fraction, mFS was markedly depressed [10.6% (8.7-13.5) vs. 17.8% (15.9-19.5) P?<0.001]. At multivariable analysis, mFS, troponin I, and NT-pro-brain natriuretic peptide were the only significant prognostic determinants (P?<0.001), whereas other indices of diastolic (E/E' ratio, transmitral and pulmonary vein flow velocities) and systolic function (tissue Doppler systolic indices, ejection fraction), or the presence/absence of congestive heart failure did not enter the model.
  
  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 Electrocardiol

  2013 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?
  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 Cardiol

  2013 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 Electrocardiol

  2013 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 Cardiol

  2013 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 J

  2013 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 (<40 mm Hg) who underwent a comprehensive 2-dimensional and Doppler echocardiography and graded bicycle semisupine exercise Doppler echocardiography. Pulmonary artery systolic pressure, cardiac output, and pulmonary vascular resistance (PVR) were estimated noninvasively. Cutoff values of PASP ?50 mm Hg and PVR ?3.0 Wood Units at peak exercise were considered a significant exercise-induced increase in PASP and PVR, respectively.
  
  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 Med

  2014 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 < 0.001 vs. baseline). Rather unexpectedly, before the tutorial, the performance of VI-year students and of residents was not significantly different from their III-year colleagues, since the two groups correctly identified 14.2 and 16.2 % of the diagnoses, respectively. After the tutorial, the VI-year students and the residents also improved their overall performance (to 73.1 and 76.1 %, respectively; p < 0.001 for both when compared to before the tutorial). The persistence of this capability after 3 years was remarkable, since the 85 students who repeated the test without any further exposure to the 10-h teaching session with Harvey(©) correctly identified 68.4 % of the possible cardiac diagnoses (p < 0.001 vs. baseline). These data underscore the importance of clinical training in order to improve auscultation skills in our academic setting, prompting to redesign teaching curricula. Simulation-based cardiac auscultation should be considered as the "missing link" between formal lecturing and bedside teaching of heart sounds and murmurs.
  
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• Prognostic value of fragmented QRS in cardiac AL amyloidosis.
  Int J Cardiol

  2013 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 Imaging

  2012 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< 0.0001). Compared with controls and NCA patients, traditional echocardiography, TDI, and speckle-tracking evaluation detected significantly (P< 0.0001) depressed RV longitudinal systolic function in CA patients. No difference was observed between groups at Doppler diastolic evaluation, whereas at tricuspidal annulus TDI analysis, CA subject showed significantly lower E' and A' values with increased E/E' ratio (P< 0.0001). Over a 19 months median follow-up period, 18 patients died. Cox multivariate analysis showed that N-terminal pro-Brain natriuretic peptide and RV longitudinal strain were the strongest death predictor.
  
  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 Interv

  2011 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, <45%) at the baseline, EF significantly increased from 37 ± 6% to 48 ± 7% after TAVI (P = 0.003). Before TAVI, 42 patients had no or mild MR, 13 mild-to-moderate, and 3 moderate or moderate-to-severe. During follow-up, the MR degree was unchanged in the majority of patients (55%), 12% reduced, and 33% worsened. Variables associated with worsening in MR were depth of aortic prosthesis (P = 0.02 for the distance between the ventricular end and the right coronary cusp; P = 0.04 for mean distance right-left coronary cusps) and left atrium area at the baseline (P = 0.02). After TAVI, six patients (10%) developed mild or moderate mitral stenosis, often in a native valve with anterior calcifications.
  
  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.
  Circulation

  2009 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<0.0001) or low voltage-to-mass ratio (1.1+/-0.5 versus 0.9+/-0.5; P<0.0001). AL patients appeared to have greater hemodynamic impairment. On multivariate analysis, ATTRm was a strongly favorable predictor of survival, and ATTRwt predicted freedom from major cardiac events.
  
  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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