<p>Rhabdomyomas are relatively rare, cardiac tumors that are predominantly seen in infancy. Involvement of the mitral valve is considered exceptionally uncommon. We retrospectively reviewed three cases of mitral valve rhabdomyomas in patients with tuberous sclerosis complex (TSC) at a single institution, focusing on clinical presentation, imaging, and management. All patients presented with low cardiac output and mitral inflow obstruction, resulting in compromised circulatory dynamics. Echocardiography revealed mobile, hyperechoic masses on the mitral leaflets. Despite moments of hemodynamic concerns and fluctuating clinical status, all patients were managed medically with antiarrhythmics, supportive care, and one patient received sirolimus. Tumor regression and symptom improvement were observed with time, indicating a positive long-term prognosis. Neonatal mitral valve rhabdomyomas, though rare, have improved with medical therapy in these cases and surgery was avoided. In select cases, mTOR inhibitors, medical management, and echocardiographic surveillance may offer a safe alternative to surgery.</p>

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Rhabdomyomas of the Mitral Valve: Case Series and Conservative Management Approach

  • Diego Solis,
  • Mario O’Connor,
  • Olivia Roy,
  • Daniel Shmourhun,
  • Gregory Johnson,
  • Keren Hasbani,
  • Kenneth Shaffer,
  • Hugo R. Martinez

摘要

Rhabdomyomas are relatively rare, cardiac tumors that are predominantly seen in infancy. Involvement of the mitral valve is considered exceptionally uncommon. We retrospectively reviewed three cases of mitral valve rhabdomyomas in patients with tuberous sclerosis complex (TSC) at a single institution, focusing on clinical presentation, imaging, and management. All patients presented with low cardiac output and mitral inflow obstruction, resulting in compromised circulatory dynamics. Echocardiography revealed mobile, hyperechoic masses on the mitral leaflets. Despite moments of hemodynamic concerns and fluctuating clinical status, all patients were managed medically with antiarrhythmics, supportive care, and one patient received sirolimus. Tumor regression and symptom improvement were observed with time, indicating a positive long-term prognosis. Neonatal mitral valve rhabdomyomas, though rare, have improved with medical therapy in these cases and surgery was avoided. In select cases, mTOR inhibitors, medical management, and echocardiographic surveillance may offer a safe alternative to surgery.