Unmasking the Prevalence of AC in an Unselected Echocardiographic Population

NCT04738266 | Unknown

• 1 other identifier: 199_2019
• Study Type: observational
• Target: 381
• Locations: 1 country
• Sites: 16

Brief Summary

This study will investigate the prevalence of echocardiographic red-flags of amyloid cardiomyopathy (AC) in patients undergoing clinically-indicated echocardiography (observational phase) and the prevalence of AC among AC-suggestive echocardiograms (interventional phase).

Conditions

Amyloid Cardiomyopathy

Keywords

Amyloid Cardiomyopathy; Echocardiography; Red flags; Epidemiology; Bone tracer cardiac scintigraphy

Outcome Measures

Primary Outcomes (3)

• The prevalence of Amyloid Cardiomyopathy

  The prevalence of Amyloid Cardiomyopathy in unselected echocardiographic population of patients aged \> 55 years

  3 months

• The prevalence of echocardiographic red flags of Amyloid Cardiomyopathy

  The prevalence of echocardiographic red flags of Amyloid Cardiomyopathy in unselected echocardiographic population of patients aged \> 55 years

  6 months

• The diagnostic accuracy of echocardiographic red flags of Amyloid Cardiomyopathy

  At the end of phase 2 of this study, patients with Amyloid Cardiomyopathy will be diagnosed, thus allowing to measure the diagnostic accuracy of the following echocardiographic red flags of Amyloid Cardiomyopathy: * Restrictive filling pattern (E wave deceleration time \<120 ms or ≤150 ms in presence of E/A ≥2) and/or E/E'≥15; * "Granular sparkling" appearance of the myocardium; * Pericardial effusion of any entity; * Interatrial septum thickness \>0.5 cm measured in subcostal or four-chamber view; * Thickening of the atrio-ventricular (AV) valves (leaflets thickness \>0.5 cm); * Left ventricular "apical sparing" pattern at speckle-tracking echocardiography.

  6 months

Secondary Outcomes (1)

• Multiparametric echocardiographic score

  6 months

Interventions

Amyloid Cardiomyopathy DIAGNOSTIC_TEST

Echocardiographic red flags of Amyloid Cardiomyopathy to guide the suspicion of disease and to proceed with second level diagnostic work up including cardiac scintigraphy with bone tracers and search of monoclonal component in serum and urine

Eligibility Criteria

• Age: 55 Years - 100 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Consecutive inpatients and outpatients subjects aged ≥55 years undergoing routine, clinically-indicated echocardiography at the laboratories of participating centers.

You may qualify if:

• All the following:
• Interventricular septum thickness ≥ 13 mm in men and ≥ 12 mm in women;
• Left ventricular ejection fraction ≥ 50%;
• Indexed end-diastolic left ventricular volume ≤ 85 mL/m2.
• AND
• At least one of the following criteria:
• "Granular sparkling" appearance of the myocardium defined granular texture with uniform increased brightness of echo-reflections;
• Pericardial effusion regardless of severity;
• Increased interatrial septum thickness (\> 5 mm);
• Restrictive filling pattern (Dec. Time E wave \<120 ms or Dec. Time E wave ≤150 ms e E/A ratio ≥2) or increased ventricular filling pressures (E/E'15);
• Speckle tracking derived global longitudinal strain with apical sparing pattern;
• Increased thickness (\> 5 mm) of mitral and tricuspid valve leaflets.

You may not qualify if:

• Patients aged \< 55 years or \> 100 years
• Echocardiography performed due to known or suspected amyloid cardiomyopathy;
• Echocardiography performed due to known hypertrophic cardiomyopathy or phenocopies;
• Refuse to sign the informed consent to the study

Sponsors & Collaborators

• University of Trieste: lead
• Pfizer: collaborator

Study Sites (16)

Emergency Department and Amyloid Research and Treatment Center, IRCCS Policlinico San Matteo Foundation, Department of Internal Medicine, University of Pavia

Pavia, Milano, 27100, Italy

Location

Cardiology Unit, Department of Emergency and Organ Transplantation, University of Bari Aldo Moro

Bari, 70121, Italy

Location

Cardiovascular Department, Policlinico Sant'Orsola

Bologna, 40138, Italy

Location

Cardiology, Department of Medical and Surgical Specialities, Radiological Sciences and Public Health - University of Brescia

Brescia, 25100, Italy

Location

Cardiology Unit, Azienda Ospedaliero Universitaria di Ferrara

Ferrara, 44121, Italy

Location

Cardiomyopathy Unit, Careggi University Hospital

Florence, 50134, Italy

Location

Cardiovascular Unit, Department of Internal Medicine, University of Genova

Genova, 16126, Italy

Location

Department of Cardiology, University of Messina

Messina, 98122, Italy

Location

Department of medicine and surgery, University Milano-Bicocca

Milan, 20126, Italy

Location

Department of Cardiovascular, Neural and Metabolic Sciences - Istituto Auxologico Italiano

Milan, 20149, Italy

Location

Department of Translational Medical Sciences, Inherited and Rare Heart Disease, Vanvitelli Cardiology, University of Campania Luigi Vanvitelli

Napoli, 80131, Italy

Location

Istituto di Scienze della Vita, Scuola Superiore Sant'Anna

Pisa, 56127, Italy

Location

Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology, Sapienza University

Roma, 00155, Italy

Location

Division of Cardiology, University of Siena

Siena, 53100, Italy

Location

University Cardiology A.O.U., Città della Salute e della Scienza di Torino

Torino, 10126, Italy

Location

Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI)

Trieste, 34149, Italy

Location

Related Publications (7)

• Maurer MS, Bokhari S, Damy T, Dorbala S, Drachman BM, Fontana M, Grogan M, Kristen AV, Lousada I, Nativi-Nicolau J, Cristina Quarta C, Rapezzi C, Ruberg FL, Witteles R, Merlini G. Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis. Circ Heart Fail. 2019 Sep;12(9):e006075. doi: 10.1161/CIRCHEARTFAILURE.119.006075. Epub 2019 Sep 4.

  PMID: 31480867 BACKGROUND

• Boldrini M, Cappelli F, Chacko L, Restrepo-Cordoba MA, Lopez-Sainz A, Giannoni A, Aimo A, Baggiano A, Martinez-Naharro A, Whelan C, Quarta C, Passino C, Castiglione V, Chubuchnyi V, Spini V, Taddei C, Vergaro G, Petrie A, Ruiz-Guerrero L, Monivas V, Mingo-Santos S, Mirelis JG, Dominguez F, Gonzalez-Lopez E, Perlini S, Pontone G, Gillmore J, Hawkins PN, Garcia-Pavia P, Emdin M, Fontana M. Multiparametric Echocardiography Scores for the Diagnosis of Cardiac Amyloidosis. JACC Cardiovasc Imaging. 2020 Apr;13(4):909-920. doi: 10.1016/j.jcmg.2019.10.011. Epub 2019 Dec 18.

  PMID: 31864973 BACKGROUND

• Gillmore JD, Maurer MS, Falk RH, Merlini G, Damy T, Dispenzieri A, Wechalekar AD, Berk JL, Quarta CC, Grogan M, Lachmann HJ, Bokhari S, Castano A, Dorbala S, Johnson GB, Glaudemans AW, Rezk T, Fontana M, Palladini G, Milani P, Guidalotti PL, Flatman K, Lane T, Vonberg FW, Whelan CJ, Moon JC, Ruberg FL, Miller EJ, Hutt DF, Hazenberg BP, Rapezzi C, Hawkins PN. Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis. Circulation. 2016 Jun 14;133(24):2404-12. doi: 10.1161/CIRCULATIONAHA.116.021612. Epub 2016 Apr 22.

  PMID: 27143678 BACKGROUND

• Maurer MS, Elliott P, Comenzo R, Semigran M, Rapezzi C. Addressing Common Questions Encountered in the Diagnosis and Management of Cardiac Amyloidosis. Circulation. 2017 Apr 4;135(14):1357-1377. doi: 10.1161/CIRCULATIONAHA.116.024438.

  PMID: 28373528 BACKGROUND

• Porcari A, Merlo M, Rapezzi C, Sinagra G. Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery. Eur J Intern Med. 2020 Dec;82:7-15. doi: 10.1016/j.ejim.2020.09.025. Epub 2020 Oct 5.

  PMID: 33032855 BACKGROUND

• Maurer MS, Hanna M, Grogan M, Dispenzieri A, Witteles R, Drachman B, Judge DP, Lenihan DJ, Gottlieb SS, Shah SJ, Steidley DE, Ventura H, Murali S, Silver MA, Jacoby D, Fedson S, Hummel SL, Kristen AV, Damy T, Plante-Bordeneuve V, Coelho T, Mundayat R, Suhr OB, Waddington Cruz M, Rapezzi C; THAOS Investigators. Genotype and Phenotype of Transthyretin Cardiac Amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey). J Am Coll Cardiol. 2016 Jul 12;68(2):161-72. doi: 10.1016/j.jacc.2016.03.596.

  PMID: 27386769 BACKGROUND

• Merlo M, Pagura L, Porcari A, Cameli M, Vergaro G, Musumeci B, Biagini E, Canepa M, Crotti L, Imazio M, Forleo C, Cappelli F, Perfetto F, Favale S, Di Bella G, Dore F, Girardi F, Tomasoni D, Pavasini R, Rella V, Palmiero G, Caiazza M, Carella MC, Igoren Guaricci A, Branzi G, Caponetti AG, Saturi G, La Malfa G, Merlo AC, Andreis A, Bruno F, Longo F, Rossi M, Varra GG, Saro R, Di Ienno L, De Carli G, Giacomin E, Arzilli C, Limongelli G, Autore C, Olivotto I, Badano L, Parati G, Perlini S, Metra M, Emdin M, Rapezzi C, Sinagra G. Unmasking the prevalence of amyloid cardiomyopathy in the real world: results from Phase 2 of the AC-TIVE study, an Italian nationwide survey. Eur J Heart Fail. 2022 Aug;24(8):1377-1386. doi: 10.1002/ejhf.2504. Epub 2022 May 10.

  PMID: 35417089 DERIVED

MeSH Terms

Conditions

Amyloid Neuropathies, Familial

Condition Hierarchy (Ancestors)

Heredodegenerative Disorders, Nervous System; Neurodegenerative Diseases; Nervous System Diseases; Amyloid Neuropathies; Peripheral Nervous System Diseases; Neuromuscular Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Amyloidosis, Familial; Metabolism, Inborn Errors; Metabolic Diseases; Nutritional and Metabolic Diseases; Amyloidosis; Proteostasis Deficiencies

Study Officials

• Gianfranco Sinagra, MD, Full Professor

  Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste

  STUDY CHAIR

• Claudio Rapezzi, MD, Full Professor

  Cardiology Unit, Azienda Ospedaliero Universitaria di Ferrara - Ferrara

  STUDY CHAIR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Target Duration: 4 Months
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: MD, Associate Professor

Study Record Dates

• First Submitted: January 28, 2021
• First Posted: February 4, 2021
• Study Start: November 20, 2020
• Primary Completion: January 20, 2021
• Study Completion: July 25, 2021
• Last Updated: February 4, 2021

Record last verified: 2021-02

Data Sharing

• IPD Sharing: Will not share

Locations

IT (16)