Surgical treatment of intravenous leiomyomatosis involving the right heart: a case series

Abstract

Objective This study was performed to investigate the surgical treatment of intravenous leiomyomatosis involving the right heart. Methods The clinical data of five patients with intracardiac leiomyomatosis treated from April 2002 to October 2017 at a single center were retrospectively analyzed. Results All five patients underwent successful intravenous and right atrial tumor removal via abdominal and inferior vena cava incisions. In three patients, these incisions were combined with thoracotomy and a right atrial incision, and in two patients, they were combined with uterine and bilateral fallopian tube and ovarian resection. One patient with advanced disease underwent a one-stage procedure and died thereafter. Of the remaining four patients who underwent follow-up for 1.5 to 12.0 years, one developed recurrence at 1 year postoperatively. The recurrent tumor, which was pathologically confirmed to be an intravenous leiomyoma, was removed via inferior vena cava and internal iliac vein incisions without subsequent recurrence. Conclusions The main treatment goal for inferior vena cava leiomyomas involving the right heart is to first address the severe obstruction of cardiac blood flow and then pursue second-stage surgery. Concurrent thoracotomy appears unnecessary because moderately sized right heart tumors can be gently removed via the inferior vena cava.