Tailored Therapies for Cardiogenic Shock in Hypertrophic Cardiomyopathy: Navigating Emerging Strategies

Abstract

Hypertrophic cardiomyopathy (HCM) is a complex and heterogeneous cardiac disorder, often complicated by cardiogenic shock, a life-threatening condition marked by severe cardiac output failure. Managing cardiogenic shock in HCM patients presents unique challenges due to the distinct pathophysiology of the disease, which includes dynamic left ventricular outflow tract obstruction, diastolic dysfunction, and myocardial ischemia. This review discusses current and emerging therapeutic strategies tailored to address the complexities of HCM-associated cardiogenic shock and other diseases with similar pathophysiology that provoke left ventricular outflow tract obstruction. We explore the role of pharmacological interventions, including the use of vasopressors and inotropes, which are crucial in stabilizing hemodynamics but require careful selection to avoid exacerbating the outflow obstruction. Additionally, the review highlights advancements in mechanical circulatory support devices such as extracorporeal membrane oxygenation (ECMO) and left ventricular assist devices (LVADs), which have become vital in the acute management of cardiogenic shock. These devices provide temporary support and bridge patients to recovery, definitive therapy, or heart transplantation, which remains a critical option for those with end-stage disease. Furthermore, the review delves into the latest research and clinical trials that are refining these therapeutic approaches, ensuring they are optimized for HCM patients. The impact of these treatments on patient outcomes, including survival rates and quality of life, is also critically assessed. In conclusion, this review underscores the importance of a tailored therapeutic approach in managing cardiogenic shock in HCM patients, integrating pharmacological and mechanical support strategies to improve outcomes in this high-risk population.

Keywords: beta-blockers; cardiogenic shock; extracorporeal membrane oxygenation; hypertrophic cardiomyopathy; left ventricular outflow tract obstruction; mechanical circulatory support.

Figure 1

The figure illustrates the key physiological changes in hypertrophic cardiomyopathy (HCM). It highlights increased left ventricular (LV) wall thickness, often due to genetic mutations affecting sarcomere proteins. The resulting myocyte disarray contributes to arrhythmias, while thickened myocardium leads to reduced LV cavity size and diastolic dysfunction. Dynamic LV outflow tract (LVOT) obstruction and systolic anterior motion (SAM) of the mitral valve further impair hemodynamics. Chronic stress promotes interstitial fibrosis, exacerbating diastolic dysfunction, with some patients progressing to systolic dysfunction. Parts of the figure were drawn by using pictures from Servier Medical Art. Servier Medical Art by Servier is licensed under a Creative Commons Attribution 4.0 Unported License (https://creativecommons.org/licenses/by/4.0/, accessed on 9 October 2024).

Figure 2

Based on the data of the table outcomes of intra-aortic balloon pump (IABP) in patients with left ventricular outflow tract obstruction (LVOTO), this flow chart presents the outcomes of patients treated with intra-aortic balloon pumps (IABPs) in the context of left ventricular outflow tract obstruction (LVOTO), differentiating between those with hypertrophic cardiomyopathy (HCM) and those without. Out of 17 patients, 6 had HCM, with 3 showing improvement and 3 experiencing treatment failure. Among the 11 patients without HCM, 4 had Takotsubo cardiomyopathy, where 3 improved and 1 failed treatment. The remaining 7 patients had LVOTO due to various medical conditions, with all experiencing failure. This highlights the variable efficacy of IABP based on the underlying condition. Abbreviations: IABP—intra-aortic balloon pump, LVOTO—left ventricular outflow tract obstruction, HCM—hypertrophic cardiomyopathy.

Figure 3

Suggested protocol for the management of cardiogenic shock due to LVOTO in HCM patients. Abbreviations: calcium channel blockers (CCBs), extracorporeal membrane oxygenation (ECMO), hypertrophic cardiomyopathy (HCM), left ventricular assist devices (LVADs), left ventricular outflow tract obstruction (LVOTO). Parts of the figure were drawn by using pictures from Servier Medical Art. Servier Medical Art by Servier is licensed under a Creative Commons Attribution 4.0 Unported License (https://creativecommons.org/licenses/by/4.0/, accessed on 9 October 2024).