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Giulia d'Amati, Ornella Leone,
Stefano Nistri,
Alberto Roghi,
Annalisa Angelini,
Cristina Basso,
Elena Biagini,
Ettore Colombo,
Donato Mele,
Guglielmina Pepe,
Claudio Rapezzi,
Gaetano Thiene
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ABSTRACT: Improved knowledge of the structure and function of the aortic wall, as well as its pathophysiology and histopathology, will result in an increasing impact on clinical evaluation and treatment of thoracic aortic diseases. This opinion paper highlights the main "paradigmatic shifts" in this field, providing cardiologists with some novel clues for the clinical approach to patients with thoracic aortic disease.
Giornale italiano di cardiologia (2006) 02/2013; 14(2):97-109.
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Gerald Berry,
Margaret Burke,
Claus Andersen,
Annalisa Angelini,
Patrick Bruneval,
Fiorella Calbrese,
Michael C Fishbein,
Martin Goddard, Ornella Leone,
Joseph Maleszewski,
Charles Marboe,
Dylan Miller,
Desley Neil,
Robert Padera,
Doris Rassi,
Monica Revello,
Alexandra Rice,
Susan Stewart,
Samuel A Yousem
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 01/2013; 32(1):14-21. · 3.54 Impact Factor
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Candida Cristina Quarta,
Pier Luigi Guidalotti,
Simone Longhi,
Cinzia Pettinato, Ornella Leone,
Alessandra Ferlini,
Elena Biagini,
Francesco Grigioni,
Maria Letizia Bacchi-Reggiani,
Massimiliano Lorenzini,
Agnese Milandri,
Angelo Branzi,
Claudio Rapezzi
JACC. Cardiovascular imaging 07/2012; 5(7):755-8. · 14.29 Impact Factor
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Claudio Rapezzi,
Candida Cristina Quarta,
Laura Obici,
Federico Perfetto,
Simone Longhi,
Fabrizio Salvi,
Elena Biagini,
Massimiliano Lorenzini,
Francesco Grigioni, Ornella Leone,
Francesco Cappelli,
Giovanni Palladini,
Paola Rimessi,
Alessandra Ferlini,
Giorgio Arpesella,
Antonio Daniele Pinna,
Giampaolo Merlini,
Stefano Perlini
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ABSTRACT: AimsHereditary transthyretin (TTR)-related amyloidosis (ATTR) is mainly considered a neurologic disease. We assessed the phenotypic and genotypic spectra of ATTR in a Caucasian area and evaluated the prevalence, genetic background, and disease profile of cases with an exclusively cardiac phenotype, highlighting possible hints for the differential diagnosis with hypertrophic cardiomyopathy (HCM) and senile systemic amyloidosis (SSA).Methods and resultsIn this Italian multicentre study, 186 patients with ATTR were characterized at presentation. Thirty patients with SSA and 30 age-gender-matched HCM patients were used for comparison. Phenotype was classified as exclusively cardiac (n = 31, 17%), exclusively neurologic (n = 46, 25%), and mixed cardiac/neurologic (n = 109, 58%). Among the eight different mutations responsible for an exclusively cardiac phenotype, Ile68Leu was the most frequent. Five patients with an exclusively cardiac phenotype developed mild abnormalities at neurological examination, but no symptoms during a 36-month follow-up (range: 14-50). Exclusively cardiac phenotype was characterized by male gender, age >65 years, heart failure symptoms, symmetric left ventricular (LV) 'hypertrophy', and moderately depressed LV ejection fraction. This profile was similar to SSA, but relatively distinct from HCM. Compared with patients with a mixed phenotype, patients with an exclusively cardiac phenotype showed a more pronounced cardiac involvement on both echocardiogram and electrocardiogram (ECG).ConclusionA clinically relevant subset of Caucasian ATTR patients present with an exclusively cardiac phenotype, mimicking HCM or SSA. Echocardiographic and ECG findings are useful to differentiate ATTR from HCM but not from SSA. The role of liver transplantation in these patients should be reconsidered.
European Heart Journal 06/2012; · 10.48 Impact Factor
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Ornella Leone,
Simone Longhi,
Candida C Quarta,
Teresa Ragazzini,
Lucilla Badiali De Giorgi,
Ferdinando Pasquale,
Luciano Potena,
Luigi Lovato,
Agnese Milandri,
Giorgio Arpesella,
Claudio Rapezzi
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ABSTRACT: Knowledge of the patterns of myocardial amyloid accumulation could improve the interpretation of electrocardiographic, echocardiographic and magnetic resonance imaging findings of amyloidosis. We assessed the extent and pattern of myocardial amyloid infiltration in explanted or autopsied hearts of patients with cardiomyopathy related to acquired monoclonal immunoglobulin light-chain (AL) or hereditary transthyretin (TTR) related amyloidosis (ATTR).
We analyzed nine explanted/autopsied hearts from patients with AL (n = 4) and ATTR (n = 5) cardiac amyloidosis. For each heart, a biventricular histological macrosection was obtained at mid-ventricular level and analyzed with both inspective and computer-assisted histologic and histomorphometric analysis aimed in particular at quantifying muscle cells, fibrosis and amyloid infiltration.
The extent of amyloid infiltration of the left ventricle (LV) ranged from 45 to 76% (median [interquartile range (IQR)] = 57% [51-64]) of the overall surface. Although LV trabecular and subendocardial were the most infiltrated layers (45-94%, median [IQR] = 73% [67-84] and from 44 to 71%, median [IQR] = 57% [49-59], respectively), intra- and inter-patient heterogeneity was high. Three main patterns of amyloid infiltration of the LV were identified: diffuse (five cases), mainly subendocardial (two cases), and mainly segmental (two cases). The extent of amyloid infiltration of the right ventricle ranged from 48 to 93% (median [IQR] = 61% [59-83]); contributions of parietal and trabecular layers ranged from 32 to 99% (median [IQR] = 63% [47-88]) and from 49 to 93% (median [IQR] = 74% [64-79]), respectively.
In amyloidotic cardiomyopathy, amyloid deposition is highly heterogeneous. Different patterns of infiltration are identifiable, including diffuse, mainly segmental and mainly subendocardial. Awareness of this variability can help the interpretation of ECGs, echocardiograms and magnetic resonance imaging.
Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 05/2012; 19(2):99-105. · 2.12 Impact Factor
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Giovanni Monego,
Vincenzo Arena,
Sandra Pasquini,
Egidio Stigliano,
Roberta Fiaccavento, Ornella Leone,
Giorgio Arpesella,
Luciano Potena,
Franco Oreste Ranelletti,
Paolo Di Nardo,
Arnaldo Capelli
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ABSTRACT: The PTHrP/PTH1R signalling system induces calciotropic and myorelaxant effects on the vascular system and plays critical roles
in the development of the cardiovascular system. In animal models, PTHrP exerts important effects on heart rate and contractility,
particularly under ischemic conditions, while, in human hearts, the expression of PTHrP by cardiomyocytes remains to be defined
in both normal and ischemic conditions. The present study has been conducted on 101 autoptical cases and confirmed on nine
explanted hearts in order to analyze the expression of the PTHrP/PTH1R system by ventricular myocardium in respect to morphological
aspects of the myocardial ischemic damage, myofiber hypertrophy and disarray, coronarosclerosis, age and sex. Immunohistochemistry
showed positive cytoplasmic immunostaining for both PTHrP and PTH1R in ventricular cardiomyocytes. The expression levels of
the PTHrP/PTH1R system resulted significantly increased (P=0.0008 and P<0.0001, respectively) in association with the myocardial ischemic damage and the presence of cardiomyocyte hypertrophy
(P=0.02 and P=0.009 respectively). Conversely, increased expression levels of PTHrP alone were observed in myofiber disarray (P=0.04), whereas PTH1R was augmented in coronarosclerosis (P=0.004) and age (P=0.001). Taken together, these results demonstrate that human ventricular cardiomyocytes express PTHrP and PTH1R and suggest
that the activation of the PTHrP/PTH1R system could represent an aspect of the embryonic gene program typically reactivated
by the myocardium when subjected to ischemia and/or hypertrophy.
Archiv für Kreislaufforschung 04/2012; 104(4):427-434. · 7.35 Impact Factor
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ABSTRACT: Hereditary transthyretin-related amyloidosis remains a widely underdiagnosed condition, owing to its extreme phenotypic variability: the clinical spectrum of the disease ranges from an almost exclusive neurologic involvement to strictly cardiac manifestations. This heterogeneity is linked to several factors including specific transthyretin mutations, geographic distribution and endemic vs. non-endemic aggregation type. The existence of exclusively or predominantly cardiac phenotypes makes the recognition of the disease very challenging since it can mimic other more common causes of left ventricular "hypertrophy". Assessment of such patients should include an active search for possible red flags that can indicate the correct final diagnosis.
Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 04/2012; 19 Suppl 1:16-21. · 2.12 Impact Factor
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European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 01/2012; · 2.40 Impact Factor
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ABSTRACT: Primary heart tumours are rare lesions with variegated histological types. We reviewed our 35 years experience with a significant number of primary benign cardiac tumour cases.
The patient database at University of Bologna was searched to identify patients with primary cardiac tumours between 1974 and 2009. Benign tumours were classified as myxomas and non-myxomas. Ninety-four were myxomas (mean age of 59.1 ± 15.6), and 13 were benign non-myxomas tumours (mean age of 39.7 ± 24.9; P = 0.0001). Complete resection of the masses was performed in all cases except in one.
In-hospital mortality was 3% in the myxoma group and 8% in the non-myxoma group. The mean follow-up was 15.1 and 7.4 years for the myxoma and non-myxoma groups, respectively. The long-term survival of discharged patients was 68 ± 7% for the myxoma group and 100% for the non-myxoma group at 20 years, respectively. Recurrence of a tumour occurred only in the myxoma group (four cases) after 1, 3, 5 and 8 years, respectively. Twenty patients had an extracardiac tumour that was diagnosed before operation in 12 (11 in the myxoma group) and during the follow-up in 8 patients (only in the myxoma group).
Primary cardiac tumours can be surgically treated with good short- and long-term results. Mortality and morbidity are mainly due to the status of preoperative patients'. An accurate follow-up is mandatory in order to detect the recurrence of a cardiac tumour such as to exclude the presence or the development of extracardiac tumours that we found frequently associated with the myxoma.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 12/2011; 41(4):812-9. · 2.40 Impact Factor
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Ornella Leone,
John P Veinot,
Annalisa Angelini,
Ulrik T Baandrup,
Cristina Basso,
Gerald Berry,
Patrick Bruneval,
Margaret Burke,
Jagdish Butany,
Fiorella Calabrese, [......],
Angela Pucci,
E René Rodriguez,
Jeffrey E Saffitz,
Mary N Sheppard,
Charles Steenbergen,
James R Stone,
Carmela Tan,
Gaetano Thiene,
Allard C van der Wal,
Gayle L Winters
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ABSTRACT: The Association for European Cardiovascular Pathology and the Society for Cardiovascular Pathology have produced this position paper concerning the current role of endomyocardial biopsy (EMB) for the diagnosis of cardiac diseases and its contribution to patient management, focusing on pathological issues, with these aims: • Determining appropriate EMB use in the context of current diagnostic strategies for cardiac diseases and providing recommendations for its rational utilization • Providing standard criteria and guidance for appropriate tissue triage and pathological analysis • Promoting a team approach to EMB use, integrating the competences of pathologists, clinicians, and imagers.
Cardiovascular pathology: the official journal of the Society for Cardiovascular Pathology 12/2011; 21(4):245-74. · 1.63 Impact Factor
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ABSTRACT: Prediction of aortic dissection or rupture is extremely difficult in patients with bicuspid aortic valve. We aimed to identify clinical and echocardiography predictors of histological abnormalities of the aortic wall in patients with bicuspid aortic valve undergoing aortic surgery.
We assessed the histology of the aortic wall and clinical and echocardiography variables in a cohort of patients with bicuspid aortic valve (n = 127) and a wide spectrum of valvar disease who underwent replacement of the ascending aorta (with or without aortic valve surgery). Histology was classified using a 5-grade system developed by Larson and Edward.
Histological alterations of the aortic wall were absent/mild (grade 0-1) in 77 patients (61%) and moderate/severe (grade 2-3) in 50 (39%). Patients with moderate/severe histological alterations were younger (47 ± 17 vs 53 ± 16; p = 0.042). Eighteen patients out of 48 (38%) with an ascending aorta diameter ≤ 4.5 cm had grade 2-3 aortic wall disease as did 8 out of 18 (44%) with a diameter ≤ 4 cm. Nineteen out of 46 (41%) patients with a maximal ascending aortic area/height ratio < 10 cm(2) m(-1) had moderate/severe histological alterations. Multivariate logistic regression analysis showed that the indexed diameter of the aortic annulus was significantly associated with grade 2-3 aortic wall disease (odds ratio (OR): 12.22, 95% confidence interval (CI): 1.65-90.38, p = 0.014).
A high proportion of patients with bicuspid aortic valve and mild to moderate aortic dilatation have severe histological abnormalities of the aortic wall that are not predictable by clinical and echocardiographic findings. These observations suggest that risk stratification for aortic dissection or rupture in patients with bicuspid aortic valve is so far quite suboptimal and future investigations are warranted.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 08/2011; 41(2):322-7. · 2.40 Impact Factor
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Annalisa Angelini,
Claus Boegelund Andersen,
Giovanni Bartoloni,
Fiona Black,
Paul Bishop,
Helen Doran,
Marny Fedrigo,
Jochen W U Fries,
Martin Goddard,
Heike Goebel, [......],
Francois Paraf,
Samuel Rotman,
Nesrin Turhan,
Patrick Bruneval,
Anna Chiara Frigo,
Francesco Grigoletto,
Alessio Gasparetto,
Roberto Mencarelli,
Gaetano Thiene,
Margaret Burke
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ABSTRACT: The aim of this study was to assess, at the European level and using digital technology, the inter-pathologist reproducibility of the ISHLT 2004 system and to compare it with the 1990 system We also assessed the reproducibility of the morphologic criteria for diagnosis of antibody-mediated rejection detailed in the 2004 grading system.
The hematoxylin-eosin-stained sections of 20 sets of endomyocardial biopsies were pre-selected and graded by two pathologists (A.A. and M.B.) and digitized using a telepathology digital pathology system (Aperio ImageScope System; for details refer to http://aperio.com/). Their diagnoses were considered the index diagnoses, which covered all grades of acute cellular rejection (ACR), early ischemic lesions, Quilty lesions, late ischemic lesions and (in the 2005 system) antibody-mediated rejection (AMR). Eighteen pathologists from 16 heart transplant centers in 7 European countries participated in the study. Inter-observer reproducibility was assessed using Fleiss's kappa and Krippendorff's alpha statistics.
The combined kappa value of all grades diagnosed by all 18 pathologists was 0.31 for the 1990 grading system and 0.39 for the 2005 grading system, with alpha statistics at 0.57 and 0.55, respectively. Kappa values by grade for 1990/2005, respectively, were: 0 = 0.52/0.51; 1A/1R = 0.24/0.36; 1B = 0.15; 2 = 0.13; 3A/2R = 0.29/0.29; 3B/3R = 0.13/0.23; and 4 = 0.18. For the 2 cases of AMR, 6 of 18 pathologists correctly suspected AMR on the hematoxylin-eosin slides, whereas, in each of 17 of the 18 AMR-negative cases a small percentage of pathologists (range 5% to 33%) overinterpreted the findings as suggestive for AMR.
Reproducibility studies of cardiac biopsies by pathologists in different centers at the international level were feasible using digitized slides rather than conventional histology glass slides. There was a small improvement in interobserver agreement between pathologists of different European centers when moving from the 1990 ISHLT classification to the "new" 2005 ISHLT classification. Morphologic suspicion of AMR in the 2004 system on hematoxylin-eosin-stained slides only was poor, highlighting the need for better standardization of morphologic criteria for AMR. Ongoing educational programs are needed to ensure standardization of diagnosis of both acute cellular and antibody-mediated rejection.
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 08/2011; 30(11):1214-20. · 3.54 Impact Factor
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Claudio Rapezzi,
Candida C Quarta,
Pier Luigi Guidalotti,
Cinzia Pettinato,
Stefano Fanti, Ornella Leone,
Alessandra Ferlini,
Simone Longhi,
Massimiliano Lorenzini,
Letizia Bacchi Reggiani,
Christian Gagliardi,
Pamela Gallo,
Caterina Villani,
Fabrizio Salvi
[show abstract]
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ABSTRACT: In a cohort of patients with hereditary transthyretin-related amyloidosis (ATTR), we aimed to assess the role of (99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid ((99m)Tc-DPD) in detecting myocardial amyloid infiltration across a wide spectrum of cardiac involvement and in predicting major adverse cardiac events (MACE).
Hereditary transthyretin-related amyloidosis is a challenging and underdiagnosed condition where both early diagnosis and prognosis remain problematic.
We evaluated 63 patients with ATTR: 40 with and 23 without echocardiographically diagnosed amyloidotic cardiomyopathy (AC). Myocardial uptake of (99m)Tc-DPD scintigraphy was semiquantitatively and visually assessed at 5 min and 3 h.
All patients with AC showed moderate-to-severe myocardial tracer uptake (i.e., visual score ≥2). Within the subgroup without AC, only 4 patients (with Ala36Pro, Gly47Ala, Thr49Ala, and Glu89Gln transthyretin mutations) showed myocardial tracer uptake and abnormal heart/whole body retention (H/WB) values: in all these cases endomyocardial biopsies showed amyloidotic infiltration. The H/WB was positively correlated with left ventricular (LV) mean wall thickness (Pearson's r=0.695, p<0.001) and negatively with LV ejection fraction (r=-0.368, p=0.004). The H/WB was an unfavorable predictor of MACE-free survival at Cox univariate analysis and contributed to the multivariate model. Notably, LV wall thickness >12 mm in combination with H/WB >7.5 was associated with the highest event rate.
In ATTR, (99m)Tc-DPD scintigraphy can identify myocardial infiltration across a wide spectrum of morphologic/functional cardiac involvement, allowing an early diagnosis of the disease (even before the appearance of echocardiographic abnormalities). The (99m)Tc-DPD myocardial uptake is a prognostic determinant of "cardiac" outcome in ATTR, either alone or in combination with LV wall thickness.
JACC. Cardiovascular imaging 06/2011; 4(6):659-70. · 14.29 Impact Factor
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Claudio Rapezzi,
Candida Cristina Quarta,
Pier Luigi Guidalotti,
Simone Longhi,
Cinzia Pettinato, Ornella Leone,
Alessandra Ferlini,
Fabrizio Salvi,
Pamela Gallo,
Christian Gagliardi,
Angelo Branzi
[show abstract]
[hide abstract]
ABSTRACT: We previously reported in a small series of patients that (99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid ((99m)Tc-DPD) scintigraphy tested positive in transthyretin-related (TTR) (both mutant and wild-type) but not in primary (AL) amyloidotic cardiomyopathy (AC). We extended our study to a larger cohort of patients with AC.
We evaluated (1) 45 patients with TTR-related AC (28 mutant and 17 wild-type), (2) 34 with AL-related AC and (3) 15 non-affected controls. Myocardial uptake of (99m)Tc-DPD (740 MBq i.v.) was semiquantitatively and visually assessed at 5 min and at 3 h.
Heart retention (HR) and heart to whole-body retention ratio (H/WB) of late (99m)Tc-DPD uptake were higher among TTR-related AC (HR 7.8%; H/WB 10.4) compared with both unaffected controls (HR 3.5%; H/WB 5.7; p < 0.0001) and AL-related AC (HR 4.0%; H/WB 6.1; p < 0.0001). For the diagnosis of TTR-related AC, positive and negative predictive accuracy of visual scoring of cardiac retention were: 80 and 100% (visual score ≥1); 88 and 100% (visual score ≥2); and 100 and 68% (visual score = 3). At adjusted linear regression analysis, TTR aetiology turned out to be the only positive predictor of increasing (99m)Tc-DPD uptake in terms of both HR [β 2.5, 95% confidence interval (CI) 1.5-3.5; p < 0.0001] and H/WB (β 3.5, 95% CI 2.1-4.9; p < 0.0001).
While (99m)Tc-DPD scintigraphy was confirmed to be useful for differentiating TTR from AL-related AC, diagnostic accuracy was lower than previously reported due to a mild degree of tracer uptake in about one third of AL patients. (99m)Tc-DPD scintigraphy can provide an accurate differential diagnosis in cases of absent or intense uptake evaluated by visual score.
European Journal of Nuclear Medicine 11/2010; 38(3):470-8. · 4.53 Impact Factor
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Giornale italiano di cardiologia (2006) 05/2010; 11(5):438-41.
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Ornella Leone,
Claudio Rapezzi,
Gianfranco Sinagra,
Annalisa Angelini,
Eloisa Arbustini,
Giovanni Bartoloni,
Cristina Basso,
Alida L P Caforio,
Fiorella Calabrese,
Fabio Coccolo, [......],
Ornella Milanesi,
Savina Nodari,
Fabrizio Oliva,
Andrea Perkan,
Daniela Prandstraller,
Angela Pucci,
Angelo Ramondo,
Furio Silvestri,
Marialuisa Valente,
Gaetano Thiene
[show abstract]
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ABSTRACT: The Italian Scientific Associations of Cardiologists and Cardiovascular Pathologists have produced this consensus document on the diagnostic role of endomyocardial biopsy (EMB) in terms of techniques, analysis and reporting. The document is intended for clinical cardiologists, hemodynamic experts, electrophysiologists, surgical pathologists, and cardiac surgeons. It has three main aims: a) to collocate EMB in the context of currently available tools for diagnosis of heart diseases; b) to provide recommendations for rational implementation; c) to outline key characteristics (standards) for Italian cardiology and surgical pathology centers that perform and analyze EMB. A general lack of prospective, controlled studies addressing EMB prohibited the use of traditional evidence-based recommendations that rely on classes of available evidence. Thus, it was agreed that three key points should be taken into account: a) the specific pathology to be diagnosed (or excluded); b) the existence of any alternative, non-invasive diagnostic techniques; c) the overall consequences of reaching a definite diagnosis on patients' clinical management. Accordingly, we propose recommendations for EMB based on the following levels of diagnostic value: level 1: no alternative method exists to reach a definite diagnosis that can have obvious consequences for clinical management; level 2a: no alternative method exists to reach a definite diagnosis; however, the implications for clinical management are uncertain; level 2b: no alternative method exists to reach a definite diagnosis; however, the diagnosis would not influence clinical management; level 3: an alternative method exists to reach a definite diagnosis. The second part of the document proposes current protocols for the preparation, analysis and reporting of EMB in the context of each main pathologic entity. Particular attention is given to tissue characterization and implementation of molecular tests.
Giornale italiano di cardiologia (2006) 09/2009; 10(9 Suppl 1):3S-50S.
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Claudio Rapezzi,
Giampaolo Merlini,
Candida C Quarta,
Letizia Riva,
Simone Longhi, Ornella Leone,
Fabrizio Salvi,
Paolo Ciliberti,
Francesca Pastorelli,
Elena Biagini, [......],
Alessandra Ferlini,
Michele Cavo,
Elena Zamagni,
Maria Luisa Fonte,
Giovanni Palladini,
Francesco Salinaro,
Francesco Musca,
Laura Obici,
Angelo Branzi,
Stefano Perlini
[show abstract]
[hide abstract]
ABSTRACT: 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.
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.
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.
Circulation 09/2009; 120(13):1203-12. · 14.74 Impact Factor
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ABSTRACT: Owing to the shortage of donor hearts, the criteria for acceptance have been considerably expanded. Pharmacologic stress echocardiography is highly accurate in identifying prognostically significant coronary artery disease, but brain death and catecholamine storm in potential heart donors may substantially alter the cardiovascular response to stress. This study assessed correlates of an abnormal resting/stress echocardiography results in potential donors.
From April 2005 to December 2007, 18 marginal candidate donors (9 men) aged 58 +/- 5 years were initially enrolled. After legal declaration of brain death, all marginal donors underwent bedside echocardiography, with baseline and (when resting echocardiography was normal) dipyridamole (0.84 mg/kg in 6 min) or dobutamine (up to 40 microg/kg/min) stress echo. Non-eligible hearts (with abnormal rest or stress echo findings) were excluded and underwent cardioautoptic verification.
Resting echocardiography showed wall motion abnormalities in 5 patients (excluded from donation). Stress echocardiography was performed in the remaining 13 (dipyridamole in 11; dobutamine in 2). Results were normal in 7, of which 6 were uneventfully transplanted in marginal recipients. Results were abnormal in 6, and autoptic verification performed showed coronary artery disease in 5, and initial cardiomyopathy in 1.
Bedside pharmacologic stress echocardiography can safely be performed in candidate heart donors, is able to unmask occult coronary artery disease or cardiomyopathy, and shows potential to extend donor criteria in heart transplantation. Further experience with using marginal donors is needed before exact guidelines can be established.
The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 09/2009; 28(11):1141-9. · 3.54 Impact Factor
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The Annals of thoracic surgery 06/2009; 87(5):1649-50. · 3.74 Impact Factor
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[show abstract]
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ABSTRACT: The aims of this study were to detect the incidence of thoracic histologically proven aortitis in a large series of 788 patients operated on for thoracic aortic disease, to describe the surgical and histologic features of inflammatory thoracic aortopathies, and to evaluate the frequency of postsurgical complications and mortality.
Thirty-nine patients (4.9%) were affected by aortitis (mean age, 72.6 +/- 9.6). There were 24 women (61.5%). Thirty-four (87.2%) were operated on because of aneurysms and 5 because of dissection. In all cases the diagnosis of aortitis was incidental and was made on the basis of histopathologic findings.
Histologically, there were 30 cases of giant cell aortitis (76.9%), 3 inflammatory aneurysms (7.7%), 2 cases of aspecific lymphoplasmacellular aortitis (5.1%), 1 of Takayasu aortitis, 1 of systemic erythematosus lupus-associated aortitis, and 1 of Behçet's disease-associated aortitis. The only case of infectious aortitis was a syphilitic aortitis. In 79.5% of cases, inflammatory infiltrates were moderate to severe in degree; the most widespread inflammation was seen in Takayasu aortitis, systemic erythematosus lupus-associated aortitis, and in Behçet's disease. The overall in-hospital mortality was 10.3% (4 of 39 patients). Neurologic complications occurred in 4 patients (10.3%).
During surgery of the thoracic aorta, an inflammatory etiology of aneurysms is found in almost 5% of cases. The inflammatory process is in a histologically advanced phase, often with systemic development. Surgery can be associated with high morbidity and mortality.
The Annals of thoracic surgery 12/2008; 86(5):1518-23. · 3.74 Impact Factor
