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

Autor: Hubert Seggewiss, Franco Cecchi, Paolo Ferrazzi, Giuseppe Limongelli, Martin S. Maron, Iacopo Olivotto, Magdi H. Yacoub, Juan Pablo Kaski, Claudio Rapezzi, Paolo Calabrò, Francesco Pelliccia, Felice Gragnano, Ottavio Alfieri
Přispěvatelé: Pelliccia, F., Alfieri, O., Calabro, P., Cecchi, F., Ferrazzi, P., Gragnano, F., Kaski, J. P., Limongelli, G., Maron, M., Rapezzi, C., Seggewiss, H., Yacoub, M. H., Olivotto, I.
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Adult
Heart Defects
Congenital

medicine.medical_specialty
Alcohol septal ablation
030204 cardiovascular system & hematology
Ventricular Outflow Obstruction
NO
03 medical and health sciences
0302 clinical medicine
Ventricular hypertrophy
Obstruction
medicine
Humans
cardiovascular diseases
030212 general & internal medicine
Myectomy
Cardiac Surgical Procedures
Intensive care medicine
Gradient
Hypertrophic cardiomyopathy
Left ventricular outflow tract
Genetic testing
Heart Failure
medicine.diagnostic_test
Interventional cardiology
business.industry
Evidence-based medicine
Cardiomyopathy
Hypertrophic

medicine.disease
alcohol septal ablation
cradient
hypertrophic cardiomyopathy
left ventricular outflow tract
myectomy
obstruction
Cardiac surgery
Heart failure
cardiovascular system
Cardiology and Cardiovascular Medicine
business
Popis: 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.
Databáze: OpenAIRE