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1. Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study

Author

Tini, G, Milani, P, Zampieri, M, Caponetti, A, Fabris, F, Foli, A, Argiro, A, Mazzoni, C, Gagliardi, C, Longhi, S, Saturi, G, Vergaro, G, Aimo, A, Russo, D, Varra, G, Serenelli, M, Fabbri, G, De Michieli, L, Palmiero, G, Ciliberti, G, Carigi, S, Sessarego, E, Mandoli, G, Ricci Lucchi, G, Rella, V, Monti, E, Gardini, E, Bartolotti, M, Crotti, L, Merli, E, Mussinelli, R, Vianello, P, Cameli, M, Marzo, F, Guerra, F, Limongelli, G, Cipriani, A, Perlini, S, Obici, L, Perfetto, F, Autore, C, Porto, I, Rapezzi, C, Sinagra, G, Merlo, M, Musumeci, B, Emdin, M, Biagini, E, Cappelli, F, Palladini, G, Canepa, M, Tini G., Milani P., Zampieri M., Caponetti A. G., Fabris F., Foli A., Argiro A., Mazzoni C., Gagliardi C., Longhi S., Saturi G., Vergaro G., Aimo A., Russo D., Varra G. G., Serenelli M., Fabbri G., De Michieli L., Palmiero G., Ciliberti G., Carigi S., Sessarego E., Mandoli G. E., Ricci Lucchi G., Rella V., Monti E., Gardini E., Bartolotti M., Crotti L., Merli E., Mussinelli R., Vianello P. F., Cameli M., Marzo F., Guerra F., Limongelli G., Cipriani A., Perlini S., Obici L., Perfetto F., Autore C., Porto I., Rapezzi C., Sinagra G., Merlo M., Musumeci B., Emdin M., Biagini E., Cappelli F., Palladini G., Canepa M., Tini, G, Milani, P, Zampieri, M, Caponetti, A, Fabris, F, Foli, A, Argiro, A, Mazzoni, C, Gagliardi, C, Longhi, S, Saturi, G, Vergaro, G, Aimo, A, Russo, D, Varra, G, Serenelli, M, Fabbri, G, De Michieli, L, Palmiero, G, Ciliberti, G, Carigi, S, Sessarego, E, Mandoli, G, Ricci Lucchi, G, Rella, V, Monti, E, Gardini, E, Bartolotti, M, Crotti, L, Merli, E, Mussinelli, R, Vianello, P, Cameli, M, Marzo, F, Guerra, F, Limongelli, G, Cipriani, A, Perlini, S, Obici, L, Perfetto, F, Autore, C, Porto, I, Rapezzi, C, Sinagra, G, Merlo, M, Musumeci, B, Emdin, M, Biagini, E, Cappelli, F, Palladini, G, Canepa, M, Tini G., Milani P., Zampieri M., Caponetti A. G., Fabris F., Foli A., Argiro A., Mazzoni C., Gagliardi C., Longhi S., Saturi G., Vergaro G., Aimo A., Russo D., Varra G. G., Serenelli M., Fabbri G., De Michieli L., Palmiero G., Ciliberti G., Carigi S., Sessarego E., Mandoli G. E., Ricci Lucchi G., Rella V., Monti E., Gardini E., Bartolotti M., Crotti L., Merli E., Mussinelli R., Vianello P. F., Cameli M., Marzo F., Guerra F., Limongelli G., Cipriani A., Perlini S., Obici L., Perfetto F., Autore C., Porto I., Rapezzi C., Sinagra G., Merlo M., Musumeci B., Emdin M., Biagini E., Cappelli F., Palladini G., and Canepa M.

Abstract

Aim: Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival. Methods and results: This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different ‘pathways’ according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III–IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III–IV and some comorbidities but not the HF pathway were independently associated with worse survival. Conclusions: Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself.

Published
2023

2. ECG/echo indexes in the diagnostic approach to amyloid cardiomyopathy: A head-to-head comparison from the AC-TIVE study

Author

Pagura, L, Porcari, A, Cameli, M, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Pavasini, R, Limongelli, G, Perlini, S, Metra, M, Boriani, G, Emdin, M, Sinagra, G, Merlo, M, Longo, F, Rossi, M, Varrà, G, Saro, R, Dore, F, Girardi, F, Vergaro, G, Musumeci, B, Autore, C, Cappelli, F, Perfetto, F, Olivotto, I, Favale, S, Carella, M, Guaricci, A, Ciccone, M, Di Bella, G, Tomasoni, D, Rella, V, Branzi, G, Badano, L, Parati, G, Palmiero, G, Caiazza, M, Caponetti, A, Saturi, G, Labate, M, Andreis, A, Paneva, E, De Ferrari, G, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Pagura L., Porcari A., Cameli M., Biagini E., Canepa M., Crotti L., Imazio M., Forleo C., Pavasini R., Limongelli G., Perlini S., Metra M., Boriani G., Emdin M., Sinagra G., Merlo M., Longo F., Rossi M., Varrà G. G., Saro R., Dore F., Girardi F., Vergaro G., Musumeci B., Autore C., Cappelli F., Perfetto F., Olivotto I., Favale S., Carella M. C., Guaricci A. I., Ciccone M. M., Di Bella G., Tomasoni D., Rella V., Branzi G., Badano L., Parati G., Palmiero G., Caiazza M., Caponetti A. G., Saturi G., Labate M. E., Andreis A., Paneva E., De Ferrari G. M., Di Ienno L., De Carli G., Giacomin E., Arzilli C., Pagura, L, Porcari, A, Cameli, M, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Pavasini, R, Limongelli, G, Perlini, S, Metra, M, Boriani, G, Emdin, M, Sinagra, G, Merlo, M, Longo, F, Rossi, M, Varrà, G, Saro, R, Dore, F, Girardi, F, Vergaro, G, Musumeci, B, Autore, C, Cappelli, F, Perfetto, F, Olivotto, I, Favale, S, Carella, M, Guaricci, A, Ciccone, M, Di Bella, G, Tomasoni, D, Rella, V, Branzi, G, Badano, L, Parati, G, Palmiero, G, Caiazza, M, Caponetti, A, Saturi, G, Labate, M, Andreis, A, Paneva, E, De Ferrari, G, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Pagura L., Porcari A., Cameli M., Biagini E., Canepa M., Crotti L., Imazio M., Forleo C., Pavasini R., Limongelli G., Perlini S., Metra M., Boriani G., Emdin M., Sinagra G., Merlo M., Longo F., Rossi M., Varrà G. G., Saro R., Dore F., Girardi F., Vergaro G., Musumeci B., Autore C., Cappelli F., Perfetto F., Olivotto I., Favale S., Carella M. C., Guaricci A. I., Ciccone M. M., Di Bella G., Tomasoni D., Rella V., Branzi G., Badano L., Parati G., Palmiero G., Caiazza M., Caponetti A. G., Saturi G., Labate M. E., Andreis A., Paneva E., De Ferrari G. M., Di Ienno L., De Carli G., Giacomin E., and Arzilli C.

Abstract

Background and aims: The discordance between QRS voltages on electrocardiogram (ECG) and left ventricle (LV) wall thickness (LVWT) on echocardiogram (echo) is a recognized red flag (RF) of amyloid cardiomyopathy (AC) and can be measured by specific indexes. No head-to-head comparison of different ECG/echo indexes among subjects with echocardiographic suspicion of AC has yet been undertaken. The study aimed at evaluating the performance and the incremental diagnostic value of different ECG/echo indexes in this subset of patients. Methods: Electrocardiograms of subjects with LV hypertrophy, preserved ejection fraction and ≥ 1 echocardiographic RF of AC participating in the AC-TIVE study, an Italian prospective multicenter study, were independently analyzed by two cardiologists. Low QRS voltages and 8 different ECG/echo indexes were evaluated. Cohort specific cut-offs were computed. Results: Among 170 patients, 55 (32 %) were diagnosed with AC. Combination of low QRS voltages with interventricular septum ≥ 1,6 cm was the most specific (specificity 100 %, positive predictive value 100 %) ECG/echo index, while the ratio between the sum of all QRS voltages and LVWT <7,8 was the most sensitive and accurate (sensitivity 94 %, negative predictive value 97 %, accuracy 82 %). When the latter index was added to a model using easily-accessible clinical variables, the diagnostic accuracy for AC greatly increased (AUC from 0,84 to 0,95; p = 0,007). Conclusions: Among patients with non-dilated hypertrophic ventricles with normal ejection fraction and echocardiographic RF of AC, easily-measurable ECG/echo indexes, mainly when added to few clinical variables, can help the physician orient second level investigations. External validation of the results is warranted.

Published
2023

3. Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy.

Author

Norrish, G, Cleary, A, Field, E, Cervi, E, Boleti, O, Ziółkowska, L, Olivotto, I, Khraiche, D, Limongelli, G, Anastasakis, A, Weintraub, R, Biagini, E, Ragni, L, Prendiville, T, Duignan, S, McLeod, K, Ilina, M, Fernandez, A, Marrone, C, Bökenkamp, R, Baban, A, Kubus, P, Daubeney, PEF, Sarquella-Brugada, G, Cesar, S, Klaassen, S, Ojala, TH, Bhole, V, Medrano, C, Uzun, O, Brown, E, Gran, F, Sinagra, G, Castro, FJ, Stuart, G, Yamazawa, H, Barriales-Villa, R, Garcia-Guereta, L, Adwani, S, Linter, K, Bharucha, T, Gonzales-Lopez, E, Siles, A, Rasmussen, TB, Calcagnino, M, Jones, CB, De Wilde, H, Kubo, T, Felice, T, Popoiu, A, Mogensen, J, Mathur, S, Centeno, F, Reinhardt, Z, Schouvey, S, Elliott, PM, Kaski, JP, Norrish, G, Cleary, A, Field, E, Cervi, E, Boleti, O, Ziółkowska, L, Olivotto, I, Khraiche, D, Limongelli, G, Anastasakis, A, Weintraub, R, Biagini, E, Ragni, L, Prendiville, T, Duignan, S, McLeod, K, Ilina, M, Fernandez, A, Marrone, C, Bökenkamp, R, Baban, A, Kubus, P, Daubeney, PEF, Sarquella-Brugada, G, Cesar, S, Klaassen, S, Ojala, TH, Bhole, V, Medrano, C, Uzun, O, Brown, E, Gran, F, Sinagra, G, Castro, FJ, Stuart, G, Yamazawa, H, Barriales-Villa, R, Garcia-Guereta, L, Adwani, S, Linter, K, Bharucha, T, Gonzales-Lopez, E, Siles, A, Rasmussen, TB, Calcagnino, M, Jones, CB, De Wilde, H, Kubo, T, Felice, T, Popoiu, A, Mogensen, J, Mathur, S, Centeno, F, Reinhardt, Z, Schouvey, S, Elliott, PM, and Kaski, JP

Abstract

BACKGROUND: Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. OBJECTIVES: The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. METHODS: Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. RESULTS: At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. CONCLUSIONS: Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages.

Published
2022

4. Prospective follow-up in various subtypes of cardiomyopathies: Insights from the EORP Cardiomyopathy Registry of the ESC

Author

Gimeno, J., primary, Elliott, P.M., additional, Tavazzi, L., additional, Tendera, M., additional, Kaski, J.P., additional, Laroche, C., additional, Barriales, R., additional, Seferovic, P., additional, Biagini, E., additional, Arbustini, E., additional, Rochas Lopes, L., additional, Linhart, A., additional, Mogensen, J., additional, Hagège, A., additional, Espinosa, M.A., additional, Saad, A., additional, Maggioni, A.P., additional, Caforio, A.L.P., additional, and Charron, P., additional

Published
2021
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5. Long-Term Implications of Atrial Fibrillation in Patients With Degenerative Mitral Regurgitation

Author

Grigioni, Francesco, primary, Benfari, Giovanni, additional, Vanoverschelde, Jean-Louis, additional, Tribouilloy, Christophe, additional, Avierinos, Jean-Francois, additional, Bursi, Francesca, additional, Suri, Rakesh M., additional, Guerra, Federico, additional, Pasquet, Agnés, additional, Rusinaru, Dan, additional, Marcelli, Emanuela, additional, Théron, Alexis, additional, Barbieri, Andrea, additional, Michelena, Hector, additional, Lazam, Siham, additional, Szymanski, Catherine, additional, Nkomo, Vuyisile T., additional, Capucci, Alessandro, additional, Thapa, Prabin, additional, Enriquez-Sarano, Maurice, additional, Suri, R., additional, Clavel, M.A, additional, Maalouf, J., additional, Michelena, H., additional, Enriquez-Sarano, M., additional, Tribouilloy, C., additional, Trojette, F., additional, Szymanski, C., additional, Rusinaru, D., additional, Touati, G., additional, Remadi, J.P., additional, Guerra, F., additional, Capucci, A., additional, Grigioni, F., additional, Russo, A., additional, Biagini, E., additional, Pasquale, F., additional, Ferlito, M., additional, Rapezzi, C., additional, Savini, C., additional, Marinelli, G., additional, Pacini, D., additional, Gargiulo, G.D., additional, Di Bartolomeo, R., additional, Boulif, J., additional, de Meester, C., additional, El Khoury, G., additional, Gerber, B., additional, Lazam, S., additional, Pasquet, A., additional, Noirhomme, P., additional, Vancraeynest, D., additional, Vanoverschelde, J-L., additional, Avierinos, J.F., additional, Collard, F., additional, Théron, A., additional, Habib, G., additional, Barbieri, A., additional, Bursi, F., additional, Mantovani, F., additional, Lugli, R., additional, Modena, M.G., additional, Boriani, G., additional, and Bacchi-Reggiani, L., additional

Published
2019
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18. Diagnosis and Management of Cardiovascular Involvement in Fabry Disease

Author

Adelaide Fusco, Eduardo Bossone, Emanuele Monda, Marta Rubino, Laura Capodicasa, Elena Biagini, Paolo Orabona, Francesca Dongiglio, Giuseppe Limongelli, Marialuisa Mazzella, Maurizio Pieroni, Dominique P. Germain, Martina Caiazza, Annapaola Cirillo, Giuseppe Palmiero, Antonio Pisani, Flavia Chiosi, Michele Lioncino, Paolo Calabrò, Arturo Cesaro, Rubino, M., Monda, E., Lioncino, M., Caiazza, M., Palmiero, G., Dongiglio, F., Fusco, A., Cirillo, A., Cesaro, A., Capodicasa, L., Mazzella, M., Chiosi, F., Orabona, P., Bossone, E., Calabro, P., Pisani, A., Germain, D. P., Biagini, E., Pieroni, M., and Limongelli, G.

Subjects
medicine.medical_specialty, medicine.medical_treatment, Left ventricular hypertrophy, Targeted therapy, Quality of life, Fibrosis, Internal medicine, medicine, Lysosomal storage disease, Humans, Enzyme Replacement Therapy, business.industry, Hypertrophic cardiomyopathy, General Medicine, Cardiomyopathy, Hypertrophic, medicine.disease, Fabry disease, Heart failure, Quality of Life, Cardiology, Fabry Disease, Hypertrophy, Left Ventricular, Therapy, Cardiology and Cardiovascular Medicine, business, Diagnosi
Abstract

Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disease caused by pathogenic variants in the GLA gene. Cardiac involvement is common in FD and is responsible for impaired quality of life and premature death. The classic cardiac involvement is a nonobstructive form of hypertrophic cardiomyopathy, usually manifesting as concentric left ventricular hypertrophy, with subsequent arrhythmogenic intramural fibrosis. Treatment of patients with FD should be directed to prevent the disease progression to irreversible organ damage and organ failure. The aim of this review is to describe the current state of knowledge regarding cardiovascular involvement in FD, focusing on clinical and instrumental features, cardiovascular management, and targeted therapy.

Published
2022

19. A Pathogenic Galactosidase A Mutation Coexisting With an MYBPC3 Mutation in a Female Patient With Hypertrophic Cardiomyopathy

Author

Lorenzo Monserrat, Maddalena Graziosi, Irene Capelli, Ornella Leone, Mario Torrado, Giovanna Cenacchi, Emilia Maneiro, Raffaello Ditaranto, Ferdinando Pasquale, Claudio Rapezzi, Fabio Niro, Giovanni Vitale, Elena Biagini, Vitale G., Pasquale F., Leone O., Cenacchi G., Niro F., Torrado M., Maneiro E., Graziosi M., Ditaranto R., Capelli I., Monserrat L., Rapezzi C., and Biagini E.

Subjects
Pathology, medicine.medical_specialty, MYBPC3 Mutation- Fabry disease- cardiomyopathy, Cardiomyopathy, Hypertrophic Cardiomyopathy, 030204 cardiovascular system & hematology, medicine.disease_cause, NO, 03 medical and health sciences, 0302 clinical medicine, Biopsy, Medicine, 030212 general & internal medicine, Phenocopy, Mutation, medicine.diagnostic_test, MYBPC3 Mutation, business.industry, Hypertrophic cardiomyopathy, Histology, medicine.disease, Phenotype, Immunohistochemistry, Hypertrophic Cardiomyopathy, MYBPC3 Mutation, Cardiology and Cardiovascular Medicine, business
Abstract

The coexistence of GLA (Pro259Ser, c.775C>T) and MYBPC3 (c.1351+2T>C) mutations was found in a female patient with hypertrophic cardiomyopathy. Histology documented abundant vacuolisation with osmiophilic lamellar bodies and positive Gb3 immunohistochemistry. In the presence of a hypertrophic cardiomyopathy phenotype, the systematic search for unusual findings is mandatory to rule out a phenocopy.

Published
2020

20. The Residual Risk of Cerebral Embolism after Carotid Stenting: The Complex Interplay between Stent Coverage and Aortic Arch Atherosclerosis

Author

Elena Biagini, Andrea Stella, Raffaele Lodi, Carla Serra, Claudio Rapezzi, Gianluca Faggioli, Lamberto Manzoli, Monica Ferri, Faggioli GL, Ferri M, Serra C, Biagini E, Manzoli L, Lodi R, Rapezzi C, and Stella A

Subjects
Male, Aortic arch, Middle Cerebral Artery, Duplex ultrasonography, ultrasonography, Ultrasonography, Ultrasonography, Doppler, Transcranial, medicine.medical_treatment, Aorta, Thoracic, Aged, Aorta, Risk Factors, Carotid artery disease, Cerebral embolism, Prospective Studies, Ultrasonography, epidemiology/etiology/ultrasonography, Italy, Medicine(all), Ultrasonography, Doppler, Duplex, Incidence, Doppler, Prognosis, Prosthesis Failure, Stroke, Intracranial Embolism, Italy, Middle cerebral artery, Disease Progression, Cardiology, Female, Stents, Radiology, diagnosis/surgery, Atherosclerosi, Cardiology and Cardiovascular Medicine, medicine.medical_specialty, methods, Three-Dimensional, Incidence, Intracranial Embolism, Aortic Diseases, Socio-culturale, ultrasonography, Prognosis, Prospective Studies, Prosthesis Failure, Risk Factors, Stent, Transcranial, Duplex scanning, Imaging, Three-Dimensional, epidemiology, Male, Middle Cerebral Artery, medicine.artery, Internal medicine, medicine, Humans, cardiovascular diseases, Aged, Aorta, business.industry, Thoracic, Aortic Disease, diagnosis/surgery, Disease Progression, Echocardiography, Stent, adverse effects, Tunica Intima, Atherosclerosis, medicine.disease, Carotid stent, Duplex, Ultrasonography, Surgery, Carotid stenting, Tunica Intima, Transesophageal, Female, Follow-Up Studies, Humans, Imaging, business, Echocardiography, Transesophageal, Follow-Up Studies
Abstract

This study investigated the fate of the stent inner surface in carotid artery stenting (CAS). In addition, the occurrence of late cerebral micro-embolism after CAS has been studied in order to identify predictors and correlate it with a possible neo-intimal layer. METHODS: A series of patients were evaluated before CAS through aortic arch trans-oesophageal echocardiography. Six months after CAS, the stent coverage by neo-intima and the possible presence of uncovered plaques were determined by high-resolution duplex scanning (5-17-Hz probe and 3D reconstruction). Possible micro-embolic signals (MESs) were evaluated through transcranial duplex scanning (30-min analysis of ipsilateral middle cerebral artery with a 1-4-Hz probe) and correlated with patients' characteristics, intimal media thickness (IMT) (>0.9mm vs. 4mm). Fisher's and Wilcoxon tests were used to evaluate differences across groups for categorical and continuous variables, respectively. RESULTS: In the 68 CASs examined (40 closed cells and 28 open cells), the stent was completely covered by neo-intima in 52 cases (76.4%). Complete coverage was significantly correlated with IMT

Published
2009

21. Paradoxical Contributions of Non-Compacted and Compacted Segments to Global Left Ventricular Dysfunction in Isolated Left Ventricular Noncompaction

Author

Ferdinando Pasquale, Folkert J. ten Cate, Guido Rocchi, Marinella Ferlito, Giovanni Bracchetti, Enrica Perugini, Angelo Branzi, Elena Biagini, Carla Lofiego, Kadir Caliskan, Ornella Leone, Claudio Rapezzi, Lofiego C, Biagini E, Ferlito M, Pasquale F, Rocchi G, Perugini E, Leone O, Bracchetti G, Caliskan K, Branzi A, ten Cate FJ, Rapezzi C., and Cardiology

Subjects
Adult, Male, medicine.medical_specialty, Heart disease, FEATURES, Cardiomyopathy, Ventricular Dysfunction, Left, Internal medicine, medicine, Humans, Wall motion, Myopathy, Observer Variation, Lv function, CARDIOMYOPATHY, Ejection fraction, business.industry, Stroke Volume, ADULTS, Middle Aged, medicine.disease, Echocardiography, Doppler, Color, Circulatory system, Cardiology, Left ventricular noncompaction, Female, medicine.symptom, Cardiology and Cardiovascular Medicine, business, MYOCARDIUM
Abstract

The pathophysiologic mechanisms of left ventricular (LV) dysfunction in isolated ventricular noncompaction (IVNC) remain unclear. Evaluating global and segmental systolic LV function in 65 patients with IVNC, this study found that normal wall motion was more common in noncompacted than in compacted segments. The number of noncompacted segments per patient correlated positively with the LV ejection fraction and negatively with LV end-diastolic volume index. These paradoxical findings support the concept that noncompaction represents a marker of a more generalized (cardio)myopathy rather than the direct pathophysiologic substrate of this still little-understood disease.

Published
2006

22. Prognostic Significance of Left Anterior Hemiblock in Patients With Suspected Coronary Artery Disease

Author

Stefan P. Nelwan, Jeroen J. Bax, Elena Biagini, Maarten L. Simoons, Ron T. van Domburg, Abdou Elhendy, Guido Rocchi, Don Poldermans, Arend F.L. Schinkel, Vittoria Rizzello, Claudio Rapezzi, Biagini E, Elhendy A, Schinkel AF, Nelwan S, Rizzello V, van Domburg RT, Rapezzi C, Rocchi G, Simoons ML, Bax JJ, Poldermans D., and Cardiology

Subjects
Male, medicine.medical_specialty, Bundle-Branch Block, Stress testing, Ischemia, Coronary Artery Disease, Risk Assessment, Coronary artery disease, Ventricular Dysfunction, Left, Risk Factors, Internal medicine, medicine, Humans, In patient, Prospective Studies, cardiovascular diseases, Myocardial infarction, Prospective cohort study, Aged, business.industry, Mortality rate, Middle Aged, Prognosis, medicine.disease, Cardiology, Left anterior hemiblock, Female, Radiology, Cardiology and Cardiovascular Medicine, business, Echocardiography, Stress
Abstract

This study was designed to assess the functional and prognostic significance of left anterior hemiblock (LAHB) in patients with no history of myocardial infarction referred for dobutamine stress echocardiography (DSE). BACKGROUND: The significance of isolated LAHB in patients with suspected coronary artery disease (CAD) is unclear. METHODS: We studied 1,187 patients with suspected CAD and no history of myocardial infarction who underwent DSE and were followed for occurrence of cardiac death. RESULTS: Left anterior hemiblock was detected on baseline electrocardiogram in 159 patients (13%). Ischemia occurred more frequently in patients with LAHB (43% vs. 33%, p = 0.02). During a mean follow-up of 5.0 +/- 2.5 years, 125 patients (11%) died of cardiac causes. The annual cardiac death rate was 4.9% in patients with LAHB and 1.9% for patients without (p < 0.0001). Patients with both LAHB and an abnormal DSE had the highest annual cardiac death rate (6.3%). In a Cox multivariable analysis, independent predictors of cardiac death were age, smoking, history of heart failure, diabetes, and ischemia. Left anterior hemiblock was independently associated with increased risk of cardiac death among patients with normal DSE (hazard ratio 1.8, 95% confidence interval 1.1 to 3.8) and in patients with abnormal DSE (hazard ratio 1.7, 95% confidence interval 1.1 to 2.7). CONCLUSIONS: In patients with suspected CAD referred for stress testing, LAHB is associated with increased risk of cardiac death. This risk is persistent after adjustment for major clinical data and abnormalities on the stress echocardiogram. Therefore, isolated LAHB should not be considered a benign electrocardiographic abnormality in these patients.

Published
2005

23. Comparison of All-Cause Mortality in Women With Known or Suspected Coronary Artery Disease Referred for Dobutamine Stress Echocardiography With Normal Versus Abnormal Test Results

Author

Vittoria Rizzello, Jeroen J. Bax, Maarten L. Simoons, Olaf Schouten, Ron T. van Domburg, Guido Rocchi, Fabiola B. Sozzi, Boudewijn J. Krenning, Angelo Branzi, Arend F.L. Schinkel, Elena Biagini, Abdou Elhendy, Don Poldermans, Biagini E, Elhendy A, Schinkel AF, Rizzello V, van Domburg RT, Krenning BJ, Schouten O, Sozzi FB, Branzi A, Rocchi G, Simoons ML, Bax JJ, Poldermans D., Cardiology, and Public Health

Subjects
medicine.medical_specialty, Time Factors, Myocardial ischemia, Dobutamine stress echocardiography, Myocardial Ischemia, Coronary Artery Disease, Risk Assessment, Coronary artery disease, Risk Factors, Cause of Death, Internal medicine, Epidemiology, medicine, Humans, In patient, business.industry, Middle Aged, Prognosis, medicine.disease, Survival Analysis, Increased risk, Cardiology, Female, Dobutamine, Cardiology and Cardiovascular Medicine, business, All cause mortality, Echocardiography, Stress, medicine.drug
Abstract

The presence of myocardial ischemia during dobutamine stress echocardiography is independently associated with an increased risk of all-cause mortality in women after adjustment for clinical data. This association is observed in patients who have proved coronary artery disease and in patients who have no history of coronary artery disease.

Published
2005

24. Prognostic Implications of the Doppler Restrictive Filling Pattern in Hypertrophic Cardiomyopathy

Author

Giuseppe Boriani, Luca Boni, Elena Biagini, Angelo Branzi, Claudio Rapezzi, Paolo Spirito, Stefania Rosmini, Massimiliano Lorenzini, Marinella Ferlito, Francesca Terzi, Letizia Bacchi-Reggiani, Francesco Lai, Guido Rocchi, Biagini E, Spirito P, Rocchi G, Ferlito M, Rosmini S, Lai F, Lorenzini M, Terzi F, Bacchi-Reggiani L, Boriani G, Branzi A, Boni L, and Rapezzi C.

Subjects
Adult, Cardiomyopathy, Dilated, Male, medicine.medical_specialty, Heart disease, Cardiomyopathy, Doppler echocardiography, Risk Assessment, Sudden death, Ventricular Dysfunction, Left, symbols.namesake, Predictive Value of Tests, Risk Factors, Cardiomyopathy, Hypertrophic, Death, Sudden, Cardiac, Female, Humans, Middle Aged, Prognosis, Echocardiography, Doppler, Internal medicine, Ventricular Function, Medicine, Ventricular function, medicine.diagnostic_test, business.industry, Hazard ratio, Hypertrophic cardiomyopathy, Reproducibility of Results, Dilated cardiomyopathy, medicine.disease, Myocardial Contraction, Confidence interval, symbols, Cardiology, Cardiology and Cardiovascular Medicine, business, Doppler effect
Abstract

The Doppler echocardiographic pattern of restrictive left ventricular (LV) filling has proved to be an important predictor of clinical course and prognosis in dilated cardiomyopathy. However, the relation between restrictive filling pattern and clinical course has not been systematically investigated in hypertrophic cardiomyopathy (HC). We assessed the prognostic implications of the Doppler restrictive filling pattern in 239 consecutive patients with HC in whom Doppler measurements of LV filling had been systematically recorded at initial evaluation and during follow-up. Restrictive LV filling was identified in 14 patients (5.9%) at initial evaluation and developed in 22 (9.2%) during follow-up. A close relation was identified between restrictive filling pattern and end-stage HC, with patients with restrictive filling showing a sixfold increase in risk of developing end-stage HC (hazard ratio 6.25, 95% confidence interval 1.90 to 20.57, p = 0.003). Over a median follow-up of 9.7 years, 22 patients (9.2%) died suddenly or received appropriate cardioverter-defibrillator interventions, and 54 (22.6%) had HC-related death or underwent heart transplantation. In a set of univariate and multivariate analyses including each of the generally accepted risk factors for cardiac death in HC, the restrictive filling pattern was a strong and independent marker of increased risk (hazard ratio for sudden cardiac events 3.51, 95% confidence interval 1.37 to 8.95, p = 0.009; hazard ratio for HC-related death or heart transplantation 3.54, 95% confidence interval 1.91 to 6.57, p

Published
2010

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