Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease with marked clinical variation requiring individualized treatment. Diastolic dysfunction due to impaired relaxation and small left ventricular (LV) chamber size are ubiquitous findings in HCM. A dichotomy determining medication selection is whether patients are obstructed or non-obstructed. Obstruction is more common, occurring in two-thirds of patients at rest or after exercise. Obstruction, when present, is most often due to systolic anterior motion of the mitral valve (SAM) and mitral-septal contact. Many patients can be managed conservatively with few or even no medications. In this chapter, we discuss the medical management of symptoms that has proved effective for many patients over the last six decades, albeit with failure to control symptoms in about a third of patients. In obstructed patients, septal reduction therapy should generally only be considered once a patient has failed maximal medical treatment. This chapter also discusses selective management issues: hypertension in obstructive HCM, apical HCM, mid-ventricular obstruction, apical aneurysms due to mid-LV obstruction, transformation into LV systolic dysfunction, and LV ballooning due to obstruction. The advent of new agents, cardiac myosin inhibitors (CMI), is likely to transform pharmacotherapy of HCM.

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Pharmacologic Therapy of Hypertrophic Cardiomyopathy 1964–2024: From Beta-Blockers to Disopyramide

  • Mark V. Sherrid,
  • Daniele Massera

摘要

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease with marked clinical variation requiring individualized treatment. Diastolic dysfunction due to impaired relaxation and small left ventricular (LV) chamber size are ubiquitous findings in HCM. A dichotomy determining medication selection is whether patients are obstructed or non-obstructed. Obstruction is more common, occurring in two-thirds of patients at rest or after exercise. Obstruction, when present, is most often due to systolic anterior motion of the mitral valve (SAM) and mitral-septal contact. Many patients can be managed conservatively with few or even no medications. In this chapter, we discuss the medical management of symptoms that has proved effective for many patients over the last six decades, albeit with failure to control symptoms in about a third of patients. In obstructed patients, septal reduction therapy should generally only be considered once a patient has failed maximal medical treatment. This chapter also discusses selective management issues: hypertension in obstructive HCM, apical HCM, mid-ventricular obstruction, apical aneurysms due to mid-LV obstruction, transformation into LV systolic dysfunction, and LV ballooning due to obstruction. The advent of new agents, cardiac myosin inhibitors (CMI), is likely to transform pharmacotherapy of HCM.