Abstract

Background: Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease in the United States which can infrequently present with acute hemodynamic instability. Our study aimed to identify the precipitants of acute cardiovascular decompensation resulting in Cardiac Intensive Care Unit (CICU) admission in patients with HCM. Methods: All patients admitted to Cleveland Clinic Cardiac Intensive Care Unit (CICU) between Jan 1 st , 2010, to Dec 31 st , 2021, with a diagnosis of CS and co-existing HCM were identified through electronic medical charts. Only those with previously uncorrected HCM in the form of no prior myectomy/alcohol septal ablation and not on mavacamten were included in our study. Chart review was performed by physician investigators to identify the etiology precipitating hemodynamic instability in these patients. Results: Overall, 2754 patients were admitted to our CICU with CS during the study period, of which 21 patients (1%) had coexisting uncorrected HCM. The median age of patients with HCM admitted to our CICU was 61 years, 52% (n=11) were females, and 86% (n=18) were White Americans. The in-hospital mortality of patients with HCM-CS was around 24% (n=5). Retrospective chart review to identify the precipitating agent revealed atrial fibrillation with rapid ventricular response (Afib-RVR) to be the most common precipitant (33%, n=7), followed by severe MR in the setting of dynamic outflow obstruction (19%, n=4) and non-obstructive heart failure (19%, n=4). Conclusion: Identification of the precipitating mechanisms is critical to manage HCM patients presenting with hemodynamic instability. Rapid atrial fibrillation appears to be the most common precipitant leading to hemodynamic instability in this population.

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