Frequency of and Prognostic Significance of Cardiac Involvement at Presentation in Hereditary Transthyretin-Derived Amyloidosis and the Value of N-Terminal Pro-B-Type Natriuretic Peptide
| Autor: | Sebastiaan H C Klaassen, Bouke P. C. Hazenberg, Hans Blokzijl, Peter van der Meer, Maarten P. van den Berg, Hans L A Nienhuis, Jasper Tromp, Dirk J. van Veldhuisen |
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| Přispěvatelé: | Cardiovascular Centre (CVC), Translational Immunology Groningen (TRIGR), Restoring Organ Function by Means of Regenerative Medicine (REGENERATE) |
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
Adult
Male medicine.medical_specialty Heart Diseases medicine.drug_class 030204 cardiovascular system & hematology DIAGNOSIS Sensitivity and Specificity DISEASE Muscle hypertrophy Cohort Studies 03 medical and health sciences 0302 clinical medicine Internal medicine Natriuretic Peptide Brain Biopsy medicine Natriuretic peptide Journal Article Humans Aged Amyloid Neuropathies Familial medicine.diagnostic_test biology business.industry Amyloidosis Hazard ratio Middle Aged Prognosis medicine.disease Peptide Fragments Transthyretin Heart failure Cardiology biology.protein HEART-FAILURE Female Cardiology and Cardiovascular Medicine business ECHOCARDIOGRAPHY Biomarkers 030217 neurology & neurosurgery SYSTEM Cohort study |
| Zdroj: | American Journal of Cardiology, 121(1), 107-112. EXCERPTA MEDICA INC-ELSEVIER SCIENCE INC |
| ISSN: | 0002-9149 |
| Popis: | The aim of this study is to assess the prevalence of cardiac involvement in hereditary transthyretin-derived (ATTRm) amyloidosis at the time of diagnosis and to determine the diagnostic and clinical value of N-terminal pro-B-type natriuretic peptide (NT-proBNP). The University Medical Center Groningen is the national center of expertise for amyloidosis. All consecutive patients between 1994 and 2016 with ATTRm amyloidosis were followed prospectively. Baseline was set at the time of the first positive biopsy. All patients underwent a standard cardiac and neurologic work-up. Cardiac involvement was defined by otherwise unexplained left and/or right ventricular wall hypertrophy on cardiac ultrasound and/or advanced conduction disturbances. Seventy-seven patients had ATTRm amyloidosis and were included in the study. The TTR V30M mutation was present in 30 patients (39%). In both the V30M and the non-V30M groups, the neurologic presentation dominated (77% vs 51%), whereas cardiac presentation was infrequent (7% vs 15%). Clinical work-up showed that cardiac involvement was present at baseline in 51% of all patients irrespective of genotype and was associated with increased overall mortality (hazard ratio 5.95, 95% confidence interval 2.12 to 16.7), independent from clinical confounders. At a cutoff level of 125 ng/L, NT-proBNP had a sensitivity of 92% for establishing cardiac involvement. In conclusion, irrespective of the frequent noncardiac presentation of ATTRm amyloidosis, cardiac involvement is already present at diagnosis in half of the patients and is associated with increased mortality. NT-proBNP is a useful marker to determine cardiac involvement in this disease. |
| Databáze: | OpenAIRE |
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