{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,8,28]],"date-time":"2022-08-28T23:11:37Z","timestamp":1661728297251},"reference-count":23,"publisher":"Georg Thieme Verlag KG","issue":"17","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dtsch Med Wochenschr"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Was ist neu?<\/jats:title><jats:p>\n          Hypertrophe obstruktive Kardiomyopathie\u2002Mavacamten, der erste Myosin-Aktivator, konnte erfolgreich in dem EXPLORER-HCM-Studienprogramm getestet werden. Lebensqualit\u00e4t und linksventrikul\u00e4re Druckgradienten-Abnahme wurden optimiert. Ob damit jedoch auch die \u00dcberlebensrate und Prognose der betroffenen Patienten verbessert wird, kann bis heute noch nicht gesagt werden. Die europ\u00e4ischen Zulassungsbeh\u00f6rden sehen allerdings bisher die Ergebnisse als so bedeutsam an, dass mit einer F\u00fchrung der Substanzklasse vielleicht schon in den n\u00e4chsten 6\u20138 Monaten zu rechnen ist. In den USA ist die Substanz bereits seit April 2022 zugelassen. Der Einsatz der Substanz bei HCM-Patienten ohne Obstruktion wird ebenfalls zurzeit untersucht.<\/jats:p><jats:p>\n          Kardiale Transthyretin-Amyloidosen\u2002Kardiale Amyloidosen haben meist eine gute Ejektionsfraktion, jedoch sind auch EF mit 40 oder &lt;\u200a30\u200a% m\u00f6glich. Das Auftreten einer kardialen Amyloidose bei Patienten mit der Symptomatik einer Herzinsuffizienz mit erhaltener Ejektionsfraktion (HFpEF) ist in bis zu 15\u200a% der F\u00e4lle nachzuweisen. Tafamidis ist die einzige zurzeit zugelassene erfolgreiche Therapiem\u00f6glichkeit bei Patienten mit kardialer ATTR-Amyloidose. Dies wurde auch durch weitere Beobachtungsregister der ATTR-ACT-Studie gezeigt. F\u00fcr die Klinik ist zus\u00e4tzlich wichtig, dass die Amyloidose auch die Aortenklappe bef\u00e4llt. Zahlreiche Patienten entwickeln somit auch eine Aortenstenose, die erkannt und meist interventionell zu behandeln ist.<\/jats:p>","DOI":"10.1055\/a-1744-3126","type":"journal-article","created":{"date-parts":[[2022,8,28]],"date-time":"2022-08-28T22:30:26Z","timestamp":1661725826000},"page":"1127-1134","source":"Crossref","is-referenced-by-count":0,"title":["Hypertrophe Kardiomyopathien und die kardiale ATTR-Amyloidose \u2013 eine aktuelle \u00dcbersicht f\u00fcr\u00a0den\u00a0klinischen Alltag"],"prefix":"10.1055","volume":"147","author":[{"given":"Carsten","family":"Tsch\u00f6pe","sequence":"additional","affiliation":[{"name":"Berlin Institute of Health at Charit\u00e9 (BIH), Universit\u00e4tsmedizin Berlin, BIH Center for Regenerative Therapies (BCRT), Berlin, Germany"},{"name":"Department of Internal Medicine and Cardiology, Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany."},{"name":"DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmed","family":"Elsanhoury","sequence":"additional","affiliation":[{"name":"Berlin Institute of Health at Charit\u00e9 (BIH), Universit\u00e4tsmedizin Berlin, BIH Center for Regenerative Therapies (BCRT), Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonja","family":"Diekmann","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Cardiology, Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Uwe","family":"K\u00fchl","sequence":"additional","affiliation":[{"name":"Berlin Institute of Health at Charit\u00e9 (BIH), Universit\u00e4tsmedizin Berlin, BIH Center for Regenerative Therapies (BCRT), Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2022,8,28]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1002\/ejhf.1461","article-title":"Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology","volume":"21","author":"P M Seferovic","year":"2019","journal-title":"Eur J Heart Fail"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1161\/CIRCRESAHA.117.311059","article-title":"Hypertrophic Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy","volume":"121","author":"A J Marian","year":"2017","journal-title":"Circ Res"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1148\/rg.2016150137","article-title":"Hypertrophic Cardiomyopathy from A to Z: Genetics, Pathophysiology, Imaging, and Management","volume":"36","author":"A J Baxi","year":"2016","journal-title":"Radiographics"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1016\/S0140-6736(16)31321-6","article-title":"Hypertrophic obstructive cardiomyopathy","volume":"389","author":"J Veselka","year":"2017","journal-title":"Lancet"},{"key":"ref5","first-page":"21","article-title":"Small Molecules acting on Myofilaments as Treatments for Heart and Skeletal Muscle Diseases","author":"K Alsulami","year":"2020","journal-title":"Int J Mol Sci"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1016\/S0140-6736(20)31792-X","article-title":"Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial","volume":"396","author":"I Olivotto","year":"2020","journal-title":"Lancet"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"606","DOI":"10.1161\/CIRCULATIONAHA.120.052359","article-title":"Mavacamten Favorably Impacts Cardiac Structure in Obstructive Hypertrophic Cardiomyopathy: EXPLORER-HCM CMR Substudy Analysis","volume":"143","author":"S Saberi","year":"2021","journal-title":"Circulation"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"e212","DOI":"10.1016\/j.jacc.2011.06.011","article-title":"2011 ACCF\/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation\/American Heart Association Task Force on Practice Guidelines. 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