Abstract
Diagnostic delay of cardiac amyloidosis (CAm) continues to challenge clinicians. We investigated features associated with delay and ascertained if a diagnostic delay had negative implications for the patient. We performed a retrospective chart review identifying 82 subjects with biopsy-proven and mass-spectrometry-identified CAm with clinical and epidemiologic data including first potential symptom of amyloidosis. Pathology slides were scored for extent of amyloid. Robust statistical analyses including generalized linear and ordered logistic regression analysis were performed. There was a 22 month (median) delay in diagnosis, more pronounced (34 months) in subjects with transthyretin (ATTR) amyloidosis. Seven factors predict a delayed diagnosis including ATTR amyloid type (ratio =2.17, 95% CI 1.31-3.59), having carpal tunnel syndrome (2.13, CI 1.49-3.03) and age <70 at first symptom (1.85, CI 1.30-2.61). Individuals with delays of 1+ years had higher levels of NT proBNP (4451 vs. 2559 pg/mL, p = .016) and longer PR intervals (225 vs. 162 ms, p < .001) at the time of diagnosis. Diagnostic delays negatively affect cardiac function. Of the predictive clinical features, carpal tunnel syndrome was frequent and its presence should le...Continue Reading
References
Jul 1, 1997·Heart·S W DubreyR H Falk
Jul 20, 1999·JAMA : the Journal of the American Medical Association·I AtroshiI Rosén
Jun 5, 2003·Lancet·Angela DispenzieriAllan S Jaffe
Jun 10, 2004·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Lawreen Heller ConnorsMerrill D Benson
Jun 29, 2005·Archives of Internal Medicine·Belinda NgRodney H Falk
Feb 15, 2012·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Shaji KumarMorie A Gertz
May 18, 2012·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Marco LuigettiMario Sabatelli
Jun 30, 2012·European Heart Journal·Claudio RapezziStefano Perlini
Aug 11, 2012·American Heart Journal·Frederick L RubergDonna Roy Grogan
Oct 30, 2012·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Joseph J MaleszewskiMarc K Halushka
Dec 15, 2012·Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine·Gunnar AntoniJens Sörensen
Jan 1, 2015·The New England Journal of Medicine·C Cristina QuartaScott D Solomon
Apr 16, 2015·Circulation. Heart Failure·Mathew S MaurerRodney H Falk
Aug 1, 2015·European Heart Journal·Esther González-LópezPablo Garcia-Pavia
Sep 13, 2015·Circulation·Marianna FontanaJames C Moon
Oct 27, 2015·Advances in Therapy·Isabelle LousadaGiampaolo Merlini
May 5, 2016·Circulation·Julian D GillmorePhilip N Hawkins
Oct 21, 2016·Current Heart Failure Reports·Rajshekhar ChakrabortyMorie A Gertz
May 1, 2017·Heart·Martha GroganMorie A Gertz
Jan 7, 2018·Journal of Cardiac Failure·Nikolaos PapoutsidakisDaniel Jacoby
Citations
Sep 26, 2019·ESC Heart Failure·Hiroyuki Yamamoto, Tomoki Yokochi
Jun 27, 2020·ESC Heart Failure·Daniel DangOlivier Lairez
Nov 30, 2019·Current Heart Failure Reports·Sandra IhneStefan Störk
Sep 19, 2020·Cardiac Failure Review·Sebastiaan Hc KlaassenPeter van der Meer
Jul 2, 2020·Journal of Medical Economics·Trine PilgaardSteen Hvitfeldt Poulsen
Jan 22, 2020·Cardiology and Therapy·Dennis Mircsof
Jan 10, 2021·BMC Musculoskeletal Disorders·Austin E WiningerShari R Liberman
Dec 5, 2020·Journal of Internal Medicine·P EldhagenP Westermark
Jan 12, 2021·Circulation. Genomic and Precision Medicine·Gita A PathakRenato Polimanti
Dec 23, 2020·American Heart Journal·Joban VaishnavNisha A Gilotra
Nov 20, 2020·American Heart Journal·Kathleen W ZhangDaniel J Lenihan
Jan 15, 2021·The American Journal of Medicine·Kathleen W ZhangDaniel J Lenihan
Jan 13, 2021·Orphanet Journal of Rare Diseases·Montserrat Vera-LlonchMichael Pollock
Apr 4, 2021·Healthcare·Manuel A Soto-MurilloHamurabi Gamboa-Rosales
Apr 21, 2021·Cardiology and Therapy·Mark H RozenbaumJose Nativi-Nicolau
Nov 23, 2020·JACC. Cardiovascular Imaging·Timothy J PoteruchaMathew S Maurer
Jun 19, 2021·Journal of Comparative Effectiveness Research·Mark H RozenbaumJose Nativi-Nicolau
Jul 13, 2021·Cardiology Clinics·Steven LawJulian D Gillmore
Aug 7, 2021·Mayo Clinic Proceedings·Carlo FumagalliFrancesco Cappelli
Oct 30, 2021·ESC Heart Failure·Mounira KharoubiThibaud Damy
Feb 1, 2022·Future Cardiology·Connor WillisThomas Kannampallil