Longitudinal Cardiac Remodeling in High-Output Heart Failure From Intravascular Leiomyomatosis

Intravascular leiomyomatosis can extend into nearby pelvic vasculature and to sites as distant as the heart and form arteriovenous malformations, leading to shunting and eventual high-output heart failure. A 45-year-old woman with biopsy-proven intravascular leiomyomatosis presented with biventricular dysfunction. Right heart catheterization demonstrated significant left-to-right shunting with high cardiac output. Serial echocardiography over 12 years showed the development of both left- and right-sided cardiac dysfunction. With treatment, left ventricular function recovered; however, right ventricular dilation and functional tricuspid regurgitation from annular dilation established a self-sustaining cycle of right-sided failure. Prior reports capture a single point in time; this 12-year echocardiographic course documents how shunt-mediated volume overload can progress to structural, self-perpetuating right heart failure independent of the original precipitant. Shunt-mediated high-output heart failure can produce progressive cardiovascular deterioration. Early recognition and multidisciplinary care are essential to prevent irreversible structural remodeling.