Chronic myeloproliferative disorder and splenectomy leading to chronic thromboembolic pulmonary hypertension: case report

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severely underdiagnosed form of pulmonary hypertension. Early diagnosis and treatment of CTEPH is essential for the outcome of the patients. The purpose of this study is to raise awareness to this rare disease by presenting a case report, where CTEPH is mimicking acute pulmonary embolism and discuss clinical symptoms and various manipulation data. Invasive diagnostic criteria for CTEPH: Mean pulmonary arterial pressuren (mPAP) > 20 mmHg,Pulmonary arterial wedge pressure (PCWP) < 15 mmHg, Elevated pulmonary vascular resistance (PVR) ≥ 3 Woods units,Evidence of chronic pulmonary embolism on CT or V/Q scan. A 68-year-old woman was admitted to the Cardiology unit of PSCUH due to progressive dyspnoea for last 2 weeks. Transthoracic echocardiography (TTEho) showed enlargement of right chambers with a right ventricular systolic pressure 60-70mmHg. Laboratory results showed thrombocytosis, hyperchromic macrocytic anaemia, slightly elevated inflammatory markers and high BNP. Anamnesis relieved a splenectomy procedure 27 years ago due to diagnosis of polycythemia vera. During USG imaging was found old deep vein thrombosis (DVT). Chest CT angiography results showed acute bilateral pulmonary embolism. Anticoagulation therapy of rivaroxaban was started. Patient had various risk factors for CTEPH (splenectomy, old DVT, polycythemia vera), thereby after 3 month patient was checked with TTEho and elevated RVSP was still found. Thereby patient underwent right heart catheterisation where mPAP was 37 mmHg with PCWP 5 mmHg and PVR 8,91 Wood units, thus confirming CTEPH diagnosis. Scrupulous patient anamnesis is crucial in early CTEPH diagnosis revealing important risk factors for CTEPH manifestation. Keeping these risk factors in mind can help to make the right decisions in treatment earlier. Untreated CTEPH patient prognosis is poor, however, there are various treatment methods (medication, interventional, surgery) for these patients to improve patient prognosis.