Key result
Individualized perioperative RV protection and VA-ECMO prove critical for managing coexisting HOCM and pulmonary hypertension.
Why the study?
When right ventricle failure develops in hypertrophic obstructive cardiomyopathy with pulmonary hypertension, management becomes complex due to opposing hemodynamic goals for right ventricle failure and left ventricular outflow tract obstruction.
Case Report (n=1)
This case report highlights the complex perioperative management of patients with coexisting HOCM and severe pulmonary hypertension complicated by acute right heart failure, emphasizing the role of mechanical circulatory support.
Emphasizes RV-focused perioperative care in HOCM-PH; case-level evidence leaves open need for prospective validation.
Hypertrophic obstructive cardiomyopathy (HOCM) is a prevalent genetic cardiac condition, often complicated by pulmonary hypertension (PH) that affects up to 40 % of patients. PH in HOCM is associated with increased morbidity, including atrial fibrillation, heart failure, and thromboembolic events. When right ventricle (RV) failure develops in this context, management becomes complex due to opposing hemodynamic goals for RV failure and left ventricular outflow tract (LVOT) obstruction. Here, we present a case that emphasizes the importance of thorough preoperative evaluation, including assessment of PH type (pre- vs. post-capillary), right heart function, optimization of PH, and volume status. Intraoperative goals must balance reduction in pulmonary vascular resistance (PVR) to support RV function, while avoiding exacerbation of LVOT obstruction. Inotrope selection is challenging since it is essential in supporting a failing RV, but at the same time, it might worsen LVOT obstruction. In patients progressing to advanced heart failure, mechanical circulatory support (MCS) such as venous-arterial extracorporeal membrane oxygenation (VA-ECMO) can provide temporary stabilization, although the role of isolated RV support remains unclear. This case illustrates the critical need for individualized perioperative strategies, prioritizing RV protection and PVR reduction, and highlights the growing role of MCS in managing patients with coexisting HOCM and PH.
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An et al. (2025) conducted a case report in Hypertrophic obstructive cardiomyopathy with severe pulmonary hypertension and acute right heart failure (n=1). Perioperative management and mechanical circulatory support (VA-ECMO) was evaluated. Individualized perioperative strategies prioritizing right ventricular protection and mechanical circulatory support are critical for managing coexisting HOCM and pulmonary hypertension.
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