How to treat recurrent idiopathic pulmonary embolism?

Question

Hello doctor,

I am a physician currently pursuing a postgraduate degree in histopathology. I am a 29-year-old female, and I have a medical history spanning four years. I experienced two episodes of syncope within two months, followed by unilateral (right) tinnitus that resolved upon pressing the right carotid artery, which I initially ignored. A year later, I was diagnosed with a pulmonary embolism in the right segmental branch, presenting with symptoms of hemoptysis, dyspnea, and another episode of syncope.

Initial tests included a thrombophilia profile and various other investigations. My homocysteine level was elevated at 22, and the thrombophilia profile revealed a decrease in protein S and factor V. I was placed on Rivaroxaban after the acute treatment. I continued taking Rivaroxaban for a year before discontinuing it.

After one and a half years from the first episode, I developed symptoms again. This time, a CT angiogram showed a decrease in the right segmental pulmonary embolism and multiple new left subsegmental thrombi. I repeated the investigations, and the thrombophilia profile returned to normal, with all other examinations also showing normal results. I was again placed on Rivaroxaban 20 mg once daily after the acute treatment. I did not have any deep vein thrombosis (DVT), and both my echocardiogram and carotid Doppler were normal.

I have no history of surgery, and I am neither married nor sexually active. Last week, I experienced chest pain accompanied by dyspnea. The CT angiogram revealed a left subsegmental thrombus with basal consolidation and pleural effusion. I have now been switched to Warfarin.

I kindly request your assistance in reaching a diagnosis. Given my recurrent idiopathic pulmonary embolism, what steps should I take next?

Please help.

Answer 1

Hello,

Welcome to icliniq.com.

I have read your query and can understand your concern.

You are experiencing recurrent, unprovoked idiopathic pulmonary embolism (PE). The optimal duration of anticoagulation therapy following a pulmonary embolism depends on the risk of recurrent venous thromboembolism after the anticoagulation is discontinued. Although the best method for assessing this risk remains uncertain, patients with idiopathic pulmonary embolism are at a higher risk of recurrence than those with PE associated with transient risk factors.

The incidence of recurrence is approximately 12 to 15 percent in patients with idiopathic pulmonary embolism, compared to 7 to 8 percent in those with PE linked to transient risk factors. Among patients assigned to continue therapy, the annual recurrence rate for venous thromboembolism is 3.1 percent per patient, whereas it is 4.1 percent per patient annually among those assigned to discontinue therapy.

Some causes of recurrent PE include active cancer, antiphospholipid syndrome, inherited thrombophilic disorders, pregnancy and the postpartum period, hormonal therapies, obesity, polycythemia vera, and essential thrombocythemia, particularly in the presence of the JAK2 V617F mutation. Obesity and reduced physical activity are significant risk factors for PE.

I hope this helps you.

Thank you.