Medical Mystery: Difficulty breathing, light-headedness in walking

A 49-year-old woman arrived at the Temple University Hospital emergency room complaining of difficulty breathing, light-headedness when walking, and worsening swelling in her legs. Her breathing was so labored that she had to be examined in a wheelchair.

Her medical history included a prior stroke and a history of deep vein thrombosis (DVT) with pulmonary embolism (PE). Deep vein thrombosis occurs when a blood clot forms in one or multiple veins deep in the body, most commonly in the legs. The clots can break loose and travel to, get lodged in, and block blood flow in, the lungs - a condition called pulmonary embolism. In this case, there were no clear reasons for why she had prior DVT and PE.

The patient was admitted to Temple University Hospital Pulmonary Hypertension Service for further workup and treatment for right-sided heart failure. Additional testing revealed chronic thromboembolic pulmonary hypertension (CTEPH), a form of elevated blood pressure in the lungs that results from unresolved blood clots from a prior pulmonary embolism. Those clots block blood flow through the lungs, and cause right-sided heart failure, shortness of breath, and other issues.

In addition, the patient had an enlarged uterus with multiple fibroids.

Was it possible that these two conditions were somehow related?

There is little to no prior evidence in the medical literature to suggest that uterine fibroids and chronic thromboembolic pulmonary hypertension could be linked. But in this patient, we wondered whether her enlarged uterus could be compressing the veins in her pelvis, leading to her history of repeated clots in her legs that broke off and lodged in her lungs.

Imaging and tests revealed that her enlarged uterus and fibroids were, indeed, compressing the veins of her pelvis and a large vein leading up into her chest called the vena cava.

The patient underwent pulmonary thromboendarterectomy surgery to remove all the blood clots from her lungs and her pulmonary hypertension resolved.

The procedure was a success, and improved her health markedly. She then could have a hysterectomy to remove her uterus and fibroids. Once that happened, all the veins of her pelvis and vena cava reopened. The patient's health and her overall quality of life have dramatically improved.

Paul Forfia, M.D., is director of the Pulmonary Hypertension/Right Heart Failure and Pulmonary Thromboendarterectomy Program at Temple University Hospital, and also is associate professor of medicine at Lewis Katz School of Medicine at Temple.