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• W4310376498 abstract "HomeCirculation: Cardiovascular ImagingVol. 15, No. 12Identification of Transthyretin Cardiac Amyloidosis Among Patients Previously Diagnosed With Hypertrophic Cardiomyopathy Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBIdentification of Transthyretin Cardiac Amyloidosis Among Patients Previously Diagnosed With Hypertrophic Cardiomyopathy Ethan J. Rowin, Frederick L. Ruberg, Gaurav Das, Daniel Higgins, Willard C. Lipe, Nadia Bokhari, Monica Dehn, Barry J. Maron and Martin S. Maron Ethan J. RowinEthan J. Rowin Correspondence to: Ethan J. Rowin, MD, HCM Center Lahey Hospital and Medical Center, 67 South Bedford St, Suite 302W, Burlington, MA. Email E-mail Address: [email protected] https://orcid.org/0000-0002-3953-0143 Hypertrophic Cardiomyopathy Center at Lahey Hospital and Medical Center, Burlington MA (E.J.R., B.J.M., M.S.M.). Search for more papers by this author , Frederick L. RubergFrederick L. Ruberg https://orcid.org/0000-0002-6424-4413 Section of Cardiovascular Medicine, Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA (F.L.R.). Search for more papers by this author , Gaurav DasGaurav Das CardioVascular Center, Tufts Medical Center, Boston MA (G.D., D.H., W.C.L., N.B., M.D.). Search for more papers by this author , Daniel HigginsDaniel Higgins CardioVascular Center, Tufts Medical Center, Boston MA (G.D., D.H., W.C.L., N.B., M.D.). Search for more papers by this author , Willard C. LipeWillard C. Lipe https://orcid.org/0000-0003-0978-668X CardioVascular Center, Tufts Medical Center, Boston MA (G.D., D.H., W.C.L., N.B., M.D.). Search for more papers by this author , Nadia BokhariNadia Bokhari CardioVascular Center, Tufts Medical Center, Boston MA (G.D., D.H., W.C.L., N.B., M.D.). Search for more papers by this author , Monica DehnMonica Dehn CardioVascular Center, Tufts Medical Center, Boston MA (G.D., D.H., W.C.L., N.B., M.D.). Search for more papers by this author , Barry J. MaronBarry J. Maron https://orcid.org/0000-0002-1710-8670 Hypertrophic Cardiomyopathy Center at Lahey Hospital and Medical Center, Burlington MA (E.J.R., B.J.M., M.S.M.). CardioVascular Center, Tufts Medical Center, Boston MA (G.D., D.H., W.C.L., N.B., M.D.). Search for more papers by this author and Martin S. MaronMartin S. Maron https://orcid.org/0000-0002-9923-3853 Hypertrophic Cardiomyopathy Center at Lahey Hospital and Medical Center, Burlington MA (E.J.R., B.J.M., M.S.M.). Search for more papers by this author Originally published30 Nov 2022https://doi.org/10.1161/CIRCIMAGING.122.014938Circulation: Cardiovascular Imaging. 2022;15Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: November 30, 2022: Ahead of Print Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a cardiac phenotype of increased LV wall thickness that may appear morphologically similar to hypertrophic cardiomyopathy (HCM).1,2 Differentiation is critical because prognosis and treatment strategies differ, even more relevant now in the era of novel therapies that can alter natural history of ATTR-CM.2 However, the prevalence and clinical profile of patients with ATTR-CM in a population of patients referred with suspected HCM is unresolved.Utilization of bone scintigraphy has resulted in the reliable noninvasive diagnosis of ATTR-CM. Therefore, we sought to determine the prevalence of ATTR-CM in a prospective consecutive cohort of 150 patients ≥60 years referred to an HCM center with presumed HCM by utilizing technetium-99m pyrophosphate (99mTc-PYP) cardiac scintigraphy. ATTR-CM was diagnosed by myocardial 99mTc-PYP uptake, with visual score ≥2 and heart to contralateral (H/CL) ratio ≥1.5 in absence of monoclonal plasma cell disorder.2 This study was approved by the IRB and informed consent for participation in registry was obtained. Study data are available from the corresponding author upon reasonable request.The 150 study patients were 68±6 years at initial evaluation (Table 1). Maximum LV wall thickness was 17±3 mm; 87 (61%) had LV outflow obstruction at rest or with provocation (≥30 mm Hg), while 63 were nonobstructive.Table 1. Demographics, Characteristics, and Clinical Features in the 5 Patients With TTR Cardiac Amyloidosis Compared With the 145 HCM PatientsTTR cardiac amyloidosis initially diagnosed with HCM (n=5)HCM patients ≥60 years of age (n=145)PMen5 (100%)72 (50%)0.06Age at clinical evaluation68±468±60.98 60–69 y3 (60%)97 (67%) 70–79 y2 (40%)43 (30%) 80+ y05 (3%)Time from diagnosis to PYP3.3±1.73.2±50.98 <1 y1 (20%)82 (57%) 1–4 y3 (60%)48 (33%) ≥5 y1 (20%)15 (10%)Imaging Maximum LV wall thickness18±317±30.58 Lateral wall thickness15±111±20.01 LVOT gradient ≥30 mm Hg rest055 (38%)0.007 LVOT gradient <30 mm Hg at rest and ≥30 mm Hg with exercise032 (22%) Ejection fraction51±1066±7<0.001 <50%2 (40%)2 (1%) 50–59%2 (40%)11 (8%) >60%1 (20%)132 (91%) LA dimension, mm43±842±70.70 RVSP > 30mmHg by echo1 (20%)27 (19%)1.00 LV end-diastolic dimension48±741±60.02 Global longitudinal strain−10±4%−18±3%<0.001CMR performed3124 LGE present3 (100%)63 (50%)Pyrophosphate scan Grade 0052 (36%) Grade 1093 (64%) Grade 200 Grade 35 (100%)0 Ratio2.2±0.241.1±0.24<0.001ECG findings Conduction disease*3 (60%)46 (32%)0.33 Low voltage†1 (20%)5 (3%)0.19 ≥1 LV hypertrophy criteria‡072 (50%)0.06Atrial fibrillation2 (40%)32 (22%)0.32Polyneuropathy1 (20%)3 (2%)0.13Orthostatic hypotension1 (20%)10 (7%)0.32Carpal tunnel syndrome Bilateral4 (80%)15 (10%)<0.001 Unilateral09 (7%)1.00Comorbidities Body mass index28.4±330.6±60.43 Hypertension1 (20%)92 (63%)0.07 Coronary artery disease1 (20%)28 (19%)1.00 Cerebrovascular event1 (20%)8 (6%)0.27 Chronic kidney disease2 (40%)13 (9%)0.08 Diabetes028 (19%)0.58 Obstructive sleep apnea1 (20%)27 (19%)1.00Family history of HCM022 (15%)1.00Unexplained syncope1 (20%)16 (11%)0.46Family history of sudden death01 (1%)1.00NSVT on ambulatory monitor2 (40%)37 (26%)0.61Symptoms Dyspnea3 (60%)102 (70%)0.64 Chest pain038 (26%)0.33 Fatigue1 (20%)29 (20%)1.00 Presyncope1 (20%)3 (5%)0.13 Palpitations1 (20%)36 (25%)1.00NYHA functional class I2 (40%)50 (34%)0.80 II3 (60%)49 (34%) III/IV046 (32%)Medications Betablockers5 (100%)104 (72%)0.32 Calcium channel blockers048 (33%)0.18 Disopyramide04 (3%)1.00 Diuretics035 (24%)0.59 Anticoagulation2 (40%)30 (20%)0.29 Antiarrhythmic09 (5%)1.00CMR indicates cardiac magnetic resonance imaging; HCM, hypertrophic cardiomyopathy; LA, left atrium; LGE, late gadolinium enhancement; LV, left ventricle; LVOT, left ventricular outflow tract; NSVT, nonsustained ventricular tachycardia; NYHA, New York Heart Failure Association; RVSP, right ventricular systolic pressure; and TTR, transthyretin.* Conduction disease: 1st degree atrioventricular block, left anterior fascicular block, left posterior fascicular block, right bundle branch block, or left bundle branch block.† Low voltage in limb or precordial leads.‡ LVH criteria Solow Lyon, R in aVL ≥ 11 mm, or Cornell criteria.With 99mTc-PYP testing, 5 of the 150 patients (3%) met diagnostic criteria for ATTR-CM. Each of these 5 patients had Grade 3 uptake confirmed by SPECT with H/CL ratio ranging 1.5 to 2.8. Genetic testing did not identify a TTR gene variant, and serum analysis was negative for pathologic light-chains, confirming wild-type ATTR-CM. ATTR-CM represented 8% of nonobstructive patients (5 of 63), and none with obstruction (0 of 87).The 5 patients were 68±4 years at ATTR-CM diagnosis; 0.5 to 5 years after the initial HCM diagnosis. At HCM referral, 2 patients were asymptomatic and 3 had class II symptoms.Among the 5 ATTR-CM patients, findings considered typical of cardiac amyloid were uncommon2: 1 patient had low QRS voltages on ECG, and 2 patients had symmetric hypertrophy pattern. Global longitudinal strain was abnormal in each patient (range –6% to –15%), but none had apical sparing pattern, considered characteristic of amyloid. Cardiac magnetic resonance was performed on 3 patients with ATTR but only 1 had a pattern most consistent with amyloidosis (ie, diffuse subendocardial LGE).After a confirmed diagnosis of ATTR-CM, each of the 5 patients were treated with tafamidis. One died suddenly 1.1 years later, whereas 4 have survived with stable course 1.0 to 1.7 years.When compared with the 145 patients with HCM, the 5 ATTR-CM patients were similar in age, NYHA class, maximum LV thickness, and LA size (Table 1). Although patients with ATTR-CM were more likely to have carpal tunnel syndrome (80% versus 10%, P≤0.001), there were no differences between other systemic abnormalities (Table 1).By systematically performing 99mTc-PYP in this prospectively enrolled cohort of patients previously diagnosed with HCM, we identified 3% of patients >60 years of age with ATTR-CM. Although prior studies have examined the prevalence of the inherited form of amyloidosis in HCM cohorts by testing for TTR gene mutations, they were unable to assess for the more common wild-type disease.3 Other investigators relied on “classical” clinical and imaging findings (without PYP) to diagnose cardiac amyloidosis in HCM; however, we found those markers unreliable for identifying ATTR-CM in our HCM cohort.4 In contrast, using bone scintigraphy, we were able to reliably identify the prevalence of ATTR-CM in a consecutive cohort suspected and previously diagnosed with HCM.Prevalence of ATTR-CM was highest at 8% when confined to patients with nonobstructive HCM. This relatively high prevalence of ATTR-CM could justify routine inclusion of 99mTc-PYP imaging in patients >60 years old with a suspected nonobstructive HCM diagnosis. However, while we did not identify ATTR-CM in patients with obstructive HCM, outflow gradients have uncommonly been associated with cardiac amyloidosis, including 1% of patients undergoing surgical myectomy.5Identification of ATTR-CM in patients with HCM presents the opportunity to introduce novel therapies for amyloidosis that may reduce mortality and morbidity,2 a possibility that is more likely with the utilization of 99mTc-PYP screening. Given the higher than expected prevalence of ATTR-CM in nonobstructive HCM, it is likely that the ATTR-CM patients in our cohort were initially misdiagnosed with HCM. However, we cannot exclude the possibility that these patients had pre-existent underlying HCM and developed ATTR-CM subsequently.In conclusion, using 99mTc-PYP imaging, we identified an important but previously unrecognized subset of patients with presumed nonobstructive HCM who in actuality have ATTR-CM. These findings support future multicenter studies in larger patient cohorts to more definitively determine yield of incorporating 99mTc-PYP into routine evaluation of older patients diagnosed with nonobstructive HCM and its impact on management and outcome.Article InformationSources of FundingThis study was supported by an investigator sponsored research grant from Pfizer.Disclosures Dr. Rowin has a research grant from Pfizer. Dr. Ruberg has research support from Pfizer, Alnylam Pharmaceuticals, and Akcea Therapeutics, and consulting income from Alexion Therapeutics and Attralus. Dr. Martin Maron is a consultant for cytokinetics and Imbria pharmaceuticals and has a research grant from iRhythm. The remaining authors have no conflicts of interest to declare.FootnotesThis article was sent to Brian D. Hoit, MD, Guest Editor, for review by expert referees, editorial decision, and final disposition.For Sources of Funding and Disclosures, see page 915.Correspondence to: Ethan J. Rowin, MD, HCM Center Lahey Hospital and Medical Center, 67 South Bedford St, Suite 302W, Burlington, MA. Email ethan.[email protected]orgReferences1. Maron BJ, Desai MY, Nishimura RA, Spirito P, Rakowski H, Towbin JA, Dearani JA, Rowin EJ, Maron MS, Sherrid MV. Management of hypertrophic cardiomyopathy: JACC state-of-the-art review.J Am Coll Cardiol. 2022; 79:390–414. doi: 10.1016/j.jacc.2021.11.021CrossrefMedlineGoogle Scholar2. Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS. Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review.J Am Coll Cardiol. 2019; 73:2872–2891. doi: 10.1016/j.jacc.2019.04.003CrossrefMedlineGoogle Scholar3. Damy T, Costes B, Hagege AA, Donal E, Eicher JC, Slama M, Guellich A, Rappeneau S, Gueffet JP, Logeart D, et al. Prevalence and clinical phenotype of hereditary transthyretic amyloid cardiomyopathy in patients with increased left ventricular wall thickness.Eur Heart J. 2016; 37:1826–1834. doi: 10.1093/eurheartj/ehv583CrossrefMedlineGoogle Scholar4. Maurizi N, Rella V, Fumagalli C, Salerno S, Castelletti S, Dagradi F, Torchio M, Marceca A, Meda M, Gasparini M, et al. Prevalence of cardiac amyloidosis among adult patients referred to tertiary centres with an initial diagnosis of hypertrophic cardiomyopathy.Int J Cardiol. 2019: 300; 191–195. doi: 10.1016/j.ijcard.2019.07.051CrossrefMedlineGoogle Scholar5. Helder MRK, Schaff HV, Nishimura RA, Gersh BJ, Dearani JA, Ommen SR, Mereuta OM, Theis JD, Dogan A, Edwards WD. Impact of incidental amyloidosis on the prognosis of patients with hypertrophic cardiomyopathy undergoing septal myectomy for left ventricular outflow tract obstruction.Am J Cardiol. 2014; 114:1396–1399. doi: 10.1016/j.amjcard.2014.07.058CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails December 2022Vol 15, Issue 12 Advertisement Article InformationMetrics © 2022 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.122.014938PMID: 36448453 Originally publishedNovember 30, 2022 Keywordstechnetium pyrophosphate scantransthyretin cardiac amyloidosishypertrophic cardiomyopathyPDF download Advertisement SubjectsCardiomyopathyNuclear Cardiology and PET" @default.
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