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Intravenous leiomyomatosis with involvement of the right heart: surgeries performed highest in PUMCH
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A multidisciplinary team (MDT), spearheaded by the Department of Cardiovascular Surgery, in Peking Union Medical College Hospital (PUMCH) has developed a relatively standardized multidisciplinary diagnosis and treatment mode for the rarely seen intravenous leiomyomatosis (IVL) with involvement of the right heart after 17 years of explorations and efforts. IVL with involvement of the right heart is an extremely rare condition and only several hundreds of cases have been reported globally. PUMCH has diagnosed and treated nearly 70 patients, making it the largest single center in the surgical treatment of this disease. Due to this achievement, the team won the 2018 PUMCH Annual Medical Research Achievement Award (third grade).

IVL, a special variant of uterine leiomyoma, is a rare disease that seriously harms the health of childbearing-age women. Since the clinical and pathological features of IVL are similar to those of uterine leiomyoma, early diagnosis is particularly challenging. As a national center guiding the diagnosis and treatment of difficult and serious diseases, PUMCH has treated more than 200 IVL patients. Similar to the uterine leiomyoma, IVL is also a pelvic space-occupying lesion; however, the biggest difference is that IVL will grow along the venous blood flow (from the parauterine vein to the iliac vein or ovarian vein) and further grows upwards the vena cava, right atrium, right ventricle, and eventually the pulmonary artery. Sudden death occurs once the tumor thrombus blocks blood flow.

Surgery remains the preferred treatment for IVL. Since the tumor is estrogen-dependent, it recurs shortly after simple resection. A second operation is difficult, along with severe trauma injuries. “A crucial step in the surgical treatment of the disease is to remove the estrogen-secreting ovary.” said a gynecologist in the MDT, “the estrogen level decreases after the ovary is resected, thus inhibiting the growth or relapse of the tumor.” IVL can spread along the bloodstream, affecting multiple organs including the heart, large blood vessels, liver, and reproductive and urinary systems. Therefore, a successful surgery requires the close cooperation of MDT members. In particular, surgical safety remains a major concern.

For IVL with involvement of the right heart, an MDT with its members from the departments of cardiac surgery, vascular surgery, obstetrics and gynecology, liver surgery, urology, anesthesiology, radiology, and ultrasound has been established in PUMCH to offer a standardized diagnosis and treatment mode to the patients. Under the leadership of Miao Qi, director of the Division of Cardiac Surgery, the MDT has continuously optimized and innovated the surgical protocol, which remarkably shortens the operative time, reduces the amount of bleeding and blood transfusion, and shortens the ICU stay time and the average hospital stay. The team also found that these intravenous tumors typically have a smooth surface and do not adhere to the wall of blood vessels. Thus, a surgical procedure that does not open the chest without the use of extracorporeal circulation was designed: for patients whose tumor is not thick and large and does not fill blood vessels, an open abdominal surgery can be performed with real-time transesophageal ultrasound monitoring to remove the tumor from blood vessel, which further reduces the surgical trauma. The PUMCH MDT has so far treated about 70 patients with IVL with right heart involvement. There was no death or serious complication after surgery. More than 10 patients underwent the surgery without the use of extracorporeal circulation, achieving the individualization of the surgical protocol. For this purpose, the Division of Cardiac Surgery timely summarized their surgical experience and proposed new clinical staging and classification of the disease. The relevant results have been published in top journals including Annals of Thoracic Surgery and European Journal of Cardio-Thoracic Surgery. The team also for the first found the “intravenous skip metastasis” in some of IVL cases.

PUMCH diagnosed and treated its first case of IVL with right heart involvement in 2002. Since then, the MDT, led by the Department of Cardiovascular Surgery, has successfully simplified and standardized the IVL management based on their rich clinical experience. Meanwhile, the team has constantly explored the innovative, individualized, and evidence-based surgical care to benefit the patients. “As a large number of cases (of IVL with right heart involvement) have been accumulated, more basic research will be carried out to explore the hidden genetic codes behind this disease,” stated Ma Guotao, associate professor at the Division of Cardiac Surgery.

CT image (A) and surgical sample (B) of IVL with right heart involvement (photo courtesy of the Division of Cardiac Surgery)