{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T21:18:01Z","timestamp":1774473481518,"version":"3.50.1"},"reference-count":110,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"20","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2021,10,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p xml:lang=\"en\">It is important for both the patient and physician communities to have timely access to information recognizing rapid progress in the diagnosis and treatment of familiar but relatively uncommon cardiovascular diseases. Patients with 3 cardiovascular diseases (ie, hypertrophic cardiomyopathy, pulmonary arterial hypertension, and transthyretin (TTR) cardiac amyloidosis (ATTR)]), once considered rare without effective management options and associated with malignant prognosis, have now benefited substantially from the development of a variety of innovative therapeutic strategies. In addition, in each case, enhanced diagnostic testing has expanded the patient population and allowed for more widespread administration of contemporary treatments. In hypertrophic cardiomyopathy, introduction of implantable defibrillators to prevent sudden death as well as high\u2010benefit:low\u2010risk septal reduction therapies to reverse heart failure have substantially reduced morbidity and disease\u2010related mortality (to 0.5% per year). For pulmonary arterial hypertension, a disease once characterized by a particularly grim prognosis, prospective randomized drug trials with aggressive single (or combined) pharmacotherapy have measurably improved survival and quality of life for many patients. In cardiac amyloidosis, development of disease\u2010specific drugs can for the first time reduce morbidity and mortality, prominently with breakthrough ATTR\u2010protein\u2013stabilizing tafamidis. In conclusion, in less common and visible cardiovascular diseases, it is crucial to recognize substantial progress and achievement, given that penetration of such information into clinical practice and the patient community can be inconsistent. Diseases such as hypertrophic cardiomyopathy, pulmonary arterial hypertension, and ATTR cardiac amyloidosis, once linked to a uniformly adverse prognosis, are now associated with the opportunity for patients to experience satisfactory quality of life and extended longevity.<\/jats:p>","DOI":"10.1161\/jaha.121.021095","type":"journal-article","created":{"date-parts":[[2021,10,8]],"date-time":"2021-10-08T10:55:07Z","timestamp":1633690507000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["Cardiovascular Diseases That Have Emerged From the Darkness"],"prefix":"10.1161","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1710-8670","authenticated-orcid":false,"given":"Barry J.","family":"Maron","sequence":"first","affiliation":[{"name":"Division of Cardiology HCM Institute Tufts Medical Center Boston MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9923-3853","authenticated-orcid":false,"given":"Martin S.","family":"Maron","sequence":"additional","affiliation":[{"name":"Division of Cardiology HCM Institute Tufts Medical Center Boston MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1867-0526","authenticated-orcid":false,"given":"Mathew S.","family":"Maurer","sequence":"additional","affiliation":[{"name":"Cardiac Amyloidosis Center Columbia University Irving Medical CenterNew York\u2013Presbyterian Hospital New York NY"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3953-0143","authenticated-orcid":false,"given":"Ethan J.","family":"Rowin","sequence":"additional","affiliation":[{"name":"Division of Cardiology HCM Institute Tufts Medical Center Boston MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6784-764X","authenticated-orcid":false,"given":"Bradley A.","family":"Maron","sequence":"additional","affiliation":[{"name":"Division of Cardiovascular Medicine Brigham &amp; Women's Hospital and Harvard Medical School Boston MA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nazzareno","family":"Gali\u00e8","sequence":"additional","affiliation":[{"name":"Alma Mater Studiorum University of Bologna Bologna Italy"},{"name":"S. Orsola University Hospital Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1710575"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2015.0354"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200002103420601"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.298.4.405"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2019.1391"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.117.027738"},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"e_1_3_1_9_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_3_1_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehq152"},{"key":"e_1_3_1_11_2","first-page":"195","article-title":"Recent studies in primary pulmonary hypertension, including pharmacodynamic observations on pulmonary vascular resistance","volume":"30","author":"Dresdale DT","year":"1954","unstructured":"Dresdale DT, Michtom RJ, Schultz M. Recent studies in primary pulmonary hypertension, including pharmacodynamic observations on pulmonary vascular resistance. Bull N Y Acad Med. 1954;30:195\u2013207.","journal-title":"Bull N Y Acad Med"},{"key":"e_1_3_1_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"e_1_3_1_13_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.020207"},{"key":"e_1_3_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2019.04.003"},{"key":"e_1_3_1_15_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1805689"},{"key":"e_1_3_1_16_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.20.1.1"},{"key":"e_1_3_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2018.03.004"},{"issue":"4","key":"e_1_3_1_18_2","first-page":"3","article-title":"Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based on an analysis of 64 patients","volume":"30","author":"Braunwald E","year":"1964","unstructured":"Braunwald E, Lambrew CT, Rockoff SD, Ross J, Morrow AG. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based on an analysis of 64 patients. Circulation. 1964;30(suppl 4):3\u2013119.","journal-title":"Circulation"},{"key":"e_1_3_1_19_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehu284"},{"key":"e_1_3_1_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2015.01.019"},{"key":"e_1_3_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2016.05.003"},{"key":"e_1_3_1_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2019.09.020"},{"key":"e_1_3_1_23_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.118.037264"},{"key":"e_1_3_1_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.08.079"},{"key":"e_1_3_1_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2017.08.048"},{"key":"e_1_3_1_26_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.119.315159"},{"key":"e_1_3_1_27_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.017633"},{"key":"e_1_3_1_28_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCEP.117.005820"},{"key":"e_1_3_1_29_2","doi-asserted-by":"publisher","DOI":"10.21037\/acs.2017.07.03"},{"key":"e_1_3_1_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2018.11.148"},{"key":"e_1_3_1_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.02.090"},{"key":"e_1_3_1_32_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2011.08.040"},{"key":"e_1_3_1_33_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.112.969626"},{"key":"e_1_3_1_34_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2013.06.009"},{"key":"e_1_3_1_35_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.117.029267"},{"key":"e_1_3_1_36_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2017.12.044"},{"key":"e_1_3_1_37_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2012.04.030"},{"key":"e_1_3_1_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2020.04.045"},{"key":"e_1_3_1_39_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2017.06.011"},{"key":"e_1_3_1_40_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2005.068577"},{"key":"e_1_3_1_41_2","volume-title":"Primary Pulmonary Hypertension Report on a WHO Meeting","author":"World Health Organization","year":"1975","unstructured":"World Health Organization . Primary Pulmonary Hypertension Report on a WHO Meeting. World Health Organization; 1975."},{"key":"e_1_3_1_42_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2019.3345"},{"key":"e_1_3_1_43_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.15-0300"},{"key":"e_1_3_1_44_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"e_1_3_1_45_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040291"},{"key":"e_1_3_1_46_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-112-7-485"},{"key":"e_1_3_1_47_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)60919-8"},{"key":"e_1_3_1_48_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp022"},{"key":"e_1_3_1_49_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.742510"},{"key":"e_1_3_1_50_2","doi-asserted-by":"publisher","DOI":"10.1161\/circulationaha.108.839274"},{"key":"e_1_3_1_51_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1503184"},{"key":"e_1_3_1_52_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213917"},{"key":"e_1_3_1_53_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa050010"},{"key":"e_1_3_1_54_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2020.05.051"},{"key":"e_1_3_1_55_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1209655"},{"key":"e_1_3_1_56_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199207093270203"},{"key":"e_1_3_1_57_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.76.1.135"},{"key":"e_1_3_1_58_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486"},{"key":"e_1_3_1_59_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2016.4471"},{"key":"e_1_3_1_60_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00101211"},{"key":"e_1_3_1_61_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01889-2018"},{"key":"e_1_3_1_62_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2011.11.002"},{"key":"e_1_3_1_63_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02044-2014"},{"key":"e_1_3_1_64_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1413687"},{"key":"e_1_3_1_65_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2019.07.028"},{"key":"e_1_3_1_66_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2020.10.007"},{"key":"e_1_3_1_67_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.112.000091"},{"key":"e_1_3_1_68_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2017.3882"},{"key":"e_1_3_1_69_2","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.118.009729"},{"key":"e_1_3_1_70_2","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(17)30498-8"},{"key":"e_1_3_1_71_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01913-2018"},{"key":"e_1_3_1_72_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/suz211"},{"key":"e_1_3_1_73_2","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(20)30317-9"},{"key":"e_1_3_1_74_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00962-2020"},{"key":"e_1_3_1_75_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2020.01.046"},{"key":"e_1_3_1_76_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.018852"},{"key":"e_1_3_1_77_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.843334"},{"key":"e_1_3_1_78_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.03.596"},{"key":"e_1_3_1_79_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.113.006242"},{"key":"e_1_3_1_80_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehz905"},{"key":"e_1_3_1_81_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.119.006075"},{"key":"e_1_3_1_82_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000792"},{"key":"e_1_3_1_83_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.118.038169"},{"key":"e_1_3_1_84_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-2836(78)90368-6"},{"key":"e_1_3_1_85_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)42128-5"},{"key":"e_1_3_1_86_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111390"},{"key":"e_1_3_1_87_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1991.tb03085.x"},{"key":"e_1_3_1_88_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.05.073"},{"key":"e_1_3_1_89_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2020.04.022"},{"key":"e_1_3_1_90_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.116.021612"},{"key":"e_1_3_1_91_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.112.000132"},{"key":"e_1_3_1_92_2","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2017.1301419"},{"key":"e_1_3_1_93_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.113.000890"},{"key":"e_1_3_1_94_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1121005109"},{"key":"e_1_3_1_95_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182661eb1"},{"key":"e_1_3_1_96_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2020.09.011"},{"key":"e_1_3_1_97_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2011.12.060"},{"key":"e_1_3_1_98_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2019.12.013"},{"key":"e_1_3_1_99_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.118.035831"},{"key":"e_1_3_1_100_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1716793"},{"key":"e_1_3_1_101_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2019.0849"},{"key":"e_1_3_1_102_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamacardio.2019.5233"},{"key":"e_1_3_1_103_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.119.045093"},{"key":"e_1_3_1_104_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.120.045966"},{"key":"e_1_3_1_105_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2019.17935"},{"key":"e_1_3_1_106_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2020.03.015"},{"key":"e_1_3_1_107_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.05.002"},{"key":"e_1_3_1_108_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2015.02.061"},{"key":"e_1_3_1_109_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)31792-X"},{"key":"e_1_3_1_110_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp297"},{"key":"e_1_3_1_111_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01654-2019"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.121.021095","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T10:59:40Z","timestamp":1715597980000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.121.021095"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,19]]},"references-count":110,"journal-issue":{"issue":"20","published-print":{"date-parts":[[2021,10,19]]}},"alternative-id":["10.1161\/JAHA.121.021095"],"URL":"https:\/\/doi.org\/10.1161\/jaha.121.021095","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,10,19]]},"assertion":[{"value":"2021-10-08","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e021095"}}