دورية أكاديمية
Diagnostic and prognostic contribution of DPD scintigraphy in transthyretin V30M cardiac amyloidosis.
| العنوان: | Diagnostic and prognostic contribution of DPD scintigraphy in transthyretin V30M cardiac amyloidosis. |
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| المؤلفون: | Azevedo Coutinho MC; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal.; Translational Clinical Physiology Unit, Institute of Molecular Medicine, University of Lisbon, Portugal., Cortez-Dias N; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal., Cantinho G; Institute of Nuclear Medicine, Lisbon Academic Medical Centre, University of Lisbon, Lisbon, Portugal., Gonçalves S; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal., Cunha N; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal., Rodrigues T; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal., Santos L; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal., Conceição I; Translational Clinical Physiology Unit, Institute of Molecular Medicine, University of Lisbon, Portugal.; Department of Neurosciences, Santa Maria University Hospital, Lisbon Academic Medical Centre, Portugal., Agostinho J; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal., Pinto FJ; Department of Cardiology, Santa Maria University Hospital, Lisbon Academic Medical Centre and Cardiovascular Centre of the University of Lisbon, Faculty of Medicine, Lisbon, Portugal. |
| المصدر: | Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis [Amyloid] 2024 Mar; Vol. 31 (1), pp. 32-41. Date of Electronic Publication: 2023 Jul 26. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Taylor & Francis Country of Publication: England NLM ID: 9433802 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1744-2818 (Electronic) Linking ISSN: 13506129 NLM ISO Abbreviation: Amyloid Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: 2015- : Abingdon, Oxford : Taylor & Francis Original Publication: Pearl River, NY : Parthenon Pub., c1994- |
| مواضيع طبية MeSH: | Amyloid Neuropathies, Familial*/diagnostic imaging , Amyloid Neuropathies, Familial*/genetics , Cardiomyopathies*/diagnostic imaging , Cardiomyopathies*/genetics, Male ; Humans ; Middle Aged ; Female ; Prognosis ; 3-Iodobenzylguanidine ; Prealbumin/genetics ; Radionuclide Imaging |
| مستخلص: | Background: Early diagnosis and prognostic stratification of cardiac transthyretin amyloidosis are crucial. Although 99m Tc 3,3-diphosphono-1,2-propanedicarboxylic acid (DPD) scintigraphy is the preferred method for the non-invasive diagnosis, its accuracy appears to be limited in transthyretin amyloidosis protein (ATTR) V30M mutation. Furthermore, its prognostic value in this mutation is unknown. This study investigated the diagnostic value of DPD scintigraphy to detect ATTR cardiomyopathy in V30M mutation and explored its prognostic value regarding mortality. Methods: A total of 288 ATTR V30M mutation carriers (median age: 46 years; 49% males) without myocardial thickening (defined as septal thickness ≥13mm) attributable to other causes and who underwent DPD scintigraphy were enrolled. ATTR cardiomyopathy was defined by septal thickness ≥13mm and at least one of the criteria: late heart-to-mediastinum (H/M) 123 I-metaiodobenzylguanidine (MIBG) uptake ratio <1.60; electrical heart disease or biopsy-documented amyloidosis. Results: ATTR cardiomyopathy was identified in 41 (14.2%) patients and cardiac DPD uptake in 34 (11.8%). During a mean follow-up of 33.6 ± 1.2 months, 16 patients died (5.6%). Mortality was 14 times higher in patients with ATTR cardiomyopathy, 13 times higher in those with DPD uptake and 10 times higher in those with late H/M MIBG <1.60. The combined assessment of septal thickness and cardiac DPD uptake improved risk stratification: patients without septal thickening and without DPD retention had an excellent prognosis while those who presented either or both of them had a significantly worse prognosis, with 5-year mortality rates ranging from 39.9 to 53.3%. Conclusions: DPD scintigraphy is useful for prognostic stratification of ATTR V30M mutation carriers. Patients without septal thickening and no DPD uptake present the best prognosis compared to those with any signs of cardiac involvement. |
| فهرسة مساهمة: | Keywords: Amyloidosis; DPD-scintigraphy; prognosis; scintigraphy; transthyretin |
| المشرفين على المادة: | 35MRW7B4AD (3-Iodobenzylguanidine) 0 (Prealbumin) |
| SCR Disease Name: | Amyloidosis, Hereditary, Transthyretin-Related |
| تواريخ الأحداث: | Date Created: 20230726 Date Completed: 20240226 Latest Revision: 20240226 |
| رمز التحديث: | 20240226 |
| DOI: | 10.1080/13506129.2023.2239987 |
| PMID: | 37493395 |
| قاعدة البيانات: | MEDLINE |
PDF full text from Publisher | تدمد: | 1744-2818 |
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| DOI: | 10.1080/13506129.2023.2239987 |