Hypertrophic Cardiomyopathy: From Medical Treatment to Advanced Heart Failure Therapies

被引:1
作者
Mazur, Matylda [1 ]
Braksator, Wojciech [2 ]
Popjes, Eric [3 ]
机构
[1] Cleveland Clin Fdn, Heart & Vasc Inst, Kaufman Ctr Heart Failure, 9500 Euclid Ave, Cleveland, OH 44195 USA
[2] Med Univ Warsaw, Fac Med, Dept Cardiol & Noninvas Cardiovasc Imaging, Warsaw, Poland
[3] Penn State Hlth Milton S Hershey Med Ctr, Heart & Vasc Inst, Hershey, PA USA
关键词
Hypertrophic cardiomyopathy; Heart failure; Cardiac myosin inhibitors; Heart transplant; Left ventricular assist device; CARDIAC TRANSPLANTATION; INTERNATIONAL SOCIETY; CLINICAL PROFILE; DE-NOVO; OUTCOMES; MANAGEMENT; PREVALENCE; MAVACAMTEN; DISEASE; DIAGNOSIS;
D O I
10.1007/s11886-024-02095-6
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Purpose of ReviewThere has been much debate surrounding novel medical therapies and heart transplantation listing challenges in patients with hypertrophic cardiomyopathy (HCM).Recent FindingsRecent clinical trials led to FDA approval of mavacamten (a cardiac myosin inhibitor), offering symptom relief and potentially delaying/avoiding invasive septal reduction therapies for some patients with HCM and left ventricular outflow obstruction (LVOTO). For those with refractory symptoms and end-stage heart failure, heart transplantation remains the gold standard. However, the concern for the organ allocation system failing to prioritize those individuals persists.SummaryHCM is a heterogeneous genetic condition with variable penetration and clinical presentation. Even though a large portion of patients remain asymptomatic, an important minority develops debilitating symptoms refractory to medical therapy. Post-HT short- and long-term outcomes are favorable. However, HT waitlist mortality remains high. For highly selected patients with HCM, a left ventricular assist device is a viable option.
引用
收藏
页码:985 / 994
页数:10
相关论文
共 81 条
  • [1] Alfares AA, 2015, GENET MED, V17, P880, DOI [10.1038/gim.2014.205, 10.1038/gim.2015.16]
  • [2] 2023 ESC Guidelines for the management of cardiomyopathies Developed by the task force on the management of cardiomyopathies of the European Society of Cardiology (ESC)
    Arbelo, Elena
    Protonotarios, Alexandros
    Gimeno, Juan R.
    Arbustini, Eloisa
    Barriales-Villa, Roberto
    Basso, Cristina
    Bezzina, Connie R.
    Biagini, Elena
    Blom, Nico A.
    de Boer, Rudolf A.
    De Winter, Tim
    Elliott, Perry M.
    Flather, Marcus
    Garcia-Pavia, Pablo
    Haugaa, Kristina H.
    Ingles, Jodie
    Jurcut, Ruxandra Oana
    Klaassen, Sabine
    Limongelli, Giuseppe
    Loeys, Bart
    Mogensen, Jens
    Olivotto, Iacopo
    Pantazis, Antonis
    Sharma, Sanjay
    Van Tintelen, J. Peter
    Ware, James S.
    Kaski, Juan Pablo
    [J]. EUROPEAN HEART JOURNAL, 2023, 44 (37) : 3503 - 3626
  • [3] Heart transplantation in hypertrophic cardiomyopathy
    Biagini, Elena
    Spirito, Paolo
    Leone, Ornella
    Picchio, Fernando M.
    Coccolo, Fabio
    Ragni, Luca
    Lofiego, Carla
    Grigioni, Francesco
    Potena, Luciano
    Rocchi, Guido
    Bacchi-Reggiarn, Letizia
    Boriani, Giuseppe
    Prandstraller, Daniela
    Arbustini, Eloisa
    Branzi, Angelo
    Rapezzi, Claudio
    [J]. AMERICAN JOURNAL OF CARDIOLOGY, 2008, 101 (03) : 387 - 392
  • [4] Efficacy of Combined Cox-Maze IV and Ventricular Septal Myectomy for Treatment of Atrial Fibrillation in Patients With Obstructive Hypertrophic Cardiomyopathy
    Boll, Griffin
    Rowin, Ethan J.
    Maron, Barry J.
    Wang, Wendy
    Rastegar, Hassan
    Maron, Martin S.
    [J]. AMERICAN JOURNAL OF CARDIOLOGY, 2020, 125 (01) : 120 - 126
  • [5] BRAUNWALD E., 1964, Circulation, V29, pIV, DOI [10.1161/01.CIR.29.5S4.IV-3, 10.1161/01.cir.29.5s4.iv-3, DOI 10.1161/01.CIR.29.5S4.IV-3]
  • [6] Clinical and Mechanistic Insights Into the Genetics of Cardiomyopathy
    Burke, Michael A.
    Cook, Stuart A.
    Seidman, Jonathan G.
    Seidman, Christine E.
    [J]. JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2016, 68 (25) : 2871 - 2886
  • [7] Role of Genetic Testing in Inherited Cardiovascular Disease A Review
    Cirino, Allison L.
    Harris, Stephanie
    Lakdawala, Neal K.
    Michels, Michelle
    Olivotto, Iacopo
    Day, Sharlene M.
    Abrams, Dominic J.
    Charron, Philippe
    Caleshu, Colleen
    Semsarian, Christopher
    Ingles, Jodie
    Rakowski, Harry
    Judge, Daniel P.
    Ho, Carolyn Y.
    [J]. JAMA CARDIOLOGY, 2017, 2 (10) : 1153 - 1160
  • [8] Outcomes in Patients With Hypertrophic Cardiomyopathy Awaiting Heart Transplantation
    Cisneros, Julio Zuniga
    Stehlik, Josef
    Selzman, Craig H.
    Drakos, Stavros G.
    McKellar, Stephen H.
    Wever-Pinzon, Omar
    [J]. CIRCULATION-HEART FAILURE, 2018, 11 (03) : e004378
  • [9] Myosin Inhibition in Patients With Obstructive Hypertrophic Cardiomyopathy Referred for Septal Reduction Therapy
    Desai, Milind Y.
    Owens, Anjali
    Geske, Jeffrey B.
    Wolski, Kathy
    Naidu, Srihari S.
    Smedira, Nicholas G.
    Cremer, Paul C.
    Schaff, Hartzell
    McErlean, Ellen
    Sewell, Christina
    Li, Wanying
    Sterling, Lulu
    Lampl, Kathy
    Edelberg, Jay M.
    Sehnert, Amy J.
    Nissen, Steven E.
    [J]. JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2022, 80 (02) : 95 - 108
  • [10] 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)
    Elliott, Perry M.
    Anastasakis, Aris
    Borger, Michael A.
    Borggrefe, Martin
    Cecchi, Franco
    Charron, Philippe
    Hagege, Albert Alain
    Lafont, Antoine
    Limongelli, Giuseppe
    Mahrholdt, Heiko
    McKenna, William J.
    Mogensen, Jens
    Nihoyannopoulos, Petros
    Nistri, Stefano
    Pieper, Petronella G.
    Pieske, Burkert
    Rapezzi, Claudio
    Rutten, Frans H.
    Tillmanns, Christoph
    Watkins, Hugh
    [J]. EUROPEAN HEART JOURNAL, 2014, 35 (39) : 2733 - +