Multidisciplinary evaluation and management of obstructive hypertrophic cardiomyopathy in 2020: Towards the HCM Heart Team

INTERNATIONAL JOURNAL OF CARDIOLOGY

卷:304

Article

Pelliccia, Francesco¹  Alfieri, Ottavio²  Calabro, Paolo^3,4  Cecchi, Franco⁵  Ferrazzi, Paolo⁶  Gragnano, Felice⁴  Kaski, Juan Pablo⁷  Limongelli, Giuseppe^4,8  Maron, Martin⁹  Rapezzi, Claudio^10,11  Seggewiss, Hubert¹²  Yacoub, Magdi H.¹³  Olivotto, Iacopo¹⁴ 

[1] Univ Sapienza Rome, Dept Cardiovasc Sci, Via Policlin 155, I-00161 Rome, Italy

[2] San Raffaele Univ Hosp, Dept Cardiovasc & Thorac Surg, Milan, Italy

[3] AORN St Anna & San Sebastiano, Div Cardiol, Caserta, Italy

[4] Univ Campania Luigi Vanvitelli, Dept Translat Med Sci, Naples, Italy

[5] San Luca Hosp, IRCCS Ist Auxol Italiano, Dept Cardiovasc Neural & Metab Sci, Milan, Italy

[6] Policlin Monza, Hypertroph Cardiomyopathy Ctr, Monza, Italy

[7] UCL, Great Ormond St Hosp, Inst Cardiovasc Sci, Ctr Inherited Cardiovasc Dis, London, England

[8] UCL, St Bartholomews Hosp, Inst Cardiovasc Sci, London, England

[9] Tufts Med Ctr, Hypertroph Cardiomyopathy Ctr, Div Cardiol, Boston, MA 02111 USA

[10] Univ Ferrara, Cardiol, Cotignola, Italy

[11] Maria Cecilia Hosp, GVM Care & Res, Cotignola, Italy

[12] Standort Juliusspital, Klinikum Wurzburg Mitte, Wurzburg, Germany

[13] Imperial Coll London, Natl Heart & Lung Inst, Heart Sci Ctr, London, England

[14] Careggi Univ Hosp, Cardiomyopathy Unit, Florence, Italy

关键词: Alcohol septal ablation;    Hypertrophic cardiomyopathy;    Gradient;    Left ventricular outflow tract;    Myectomy;    Obstruction;   

DOI  :  10.1016/j.ijcard.2020.01.021

来源: Elsevier

【 摘 要 】

Patients with hypertrophic cardiomyopathy (HCM) exhibit a variable phenotype with ventricular hypertrophy as the cardinal manifestation and left ventricular (LV) outflow tract obstruction (LVOTO) as a key pathophysiologic determinant. Patients with severe LVOTO usually present with exertional dyspnea, exertional syncope, and heart failure symptoms, while successful relief of LVOTO by pharmacological or invasive interventions leads to a dramatic improvement in clinical status. Proper management of obstructive HCM remains challenging and poses numerous clinical dilemmas. Since the development of surgical myectomy over half a century ago, progress in the management of LVOTO in HCM has paralleled technological advances in genetic testing, cardiac imaging, arrhythmic prophylaxis, cardiac surgery and interventional cardiology. These changes have been incorporated in dedicated scientific guidelines on both sides of the Atlantic. However, either the 2011 American guidelines or the 2014 European guidelines remain largely based on expert consensus for lack of recommendations with level of evidence A regarding any of the treatment options commonly employed in HCM. Consequently, management of obstructive HCM patients remains largely subjective and dependent on clinical judgment, local expertise, and patient preference. Following the trend that has emerged for other cardiac diseases amenable to invasive interventions, adequate evaluation and management of obstruction in HCM today requires a multidisciplinary team capable of optimizing referral, choosing the best available options, minimizing complications and ensuring state-of-the-art results. The concept of an HCM Heart Team is coming of age. This review aims to provide an update of available pharmacologic and invasive options for the management of LVOTO in HCM, either in adulthood or in childhood, highlighting areas for multidisciplinary integration and future development. (C) 2020 Elsevier B.V. All rights reserved.

【 授权许可】

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