Chronic Thromboembolic Pulmonary Hypertension Presented with Dyspnea on Exertion: A Case Report

Abstract

<p>慢性血栓栓塞性肺高壓是一種罕見、嚴重且持續惡化的肺部血管性疾病，隨病程進展，致死率極高，儘早鑑別診斷，方能提供準確治療計劃。本文是一位67歲女性案例，因活動呼吸喘就醫， 初步臆斷為心臟衰竭合併急性惡化，入院後藉由病史詢問、檢驗及理學檢查結果顯示BNP持續上升且心電圖呈現S1Q3T3，初步懷疑可能有肺栓塞併心衰竭，故安排心臟超音波檢查，結果發現有肺動脈高壓（肺動脈壓：62.3 mmHg）與右心衰竭，安排胸部電腦斷層攝影，顯示肺動脈栓塞，隨即召開家庭會議解釋病情後，接受右心導管合併肺動脈血管攝影檢查，最終確診為遠端型慢性血栓栓塞性肺高壓併右心衰竭。經由醫療團隊評估後，治療計劃即以藥物為主，故協助申請標靶藥物Riociguat。針對此類病人，即便臨床表徵不具特異性，仍可依病史詢問、檢驗及理學檢查預測肺栓塞的可能性，之後進入影像學鑑別診斷。換言之，雖然慢性血栓栓塞性肺高壓有其高致命的危險性，倘若能及早做出診斷與治療，便能降低死亡風險。</p> <p> </p><p>Chronic thromboembolic pulmonary hypertension is a rare, severe and continuous deterioration of pulmonary vascular disease which has high mortality rate along with disease progression. Earlier di­agnosis could contribute to a more accurate treatment plan. A case of 67-year-old female was admitted to hospital because of exertional dyspnea. She was initially suffering from heart failure with acute deterioration. After admission, the results of medical history inquiry, laboratory data and physical ex­amination revealed that BNP level kept raising and the electrocardiogram showed S1Q3T3, which pul­monary embolism with heart failure was suspected. Therefore, echocardiography revealed pulmonary hypertension (pulmonary artery pressure: 62.3 mmHg) and right heart failure. Pulmonary embolism was noticed on chest computed tomography. Soon after the family meeting of medical condition dis­cussion, the patient received right heart catheterization combined with pulmonary artery angiography, and the final diagnosed is distal chronic thromboembolic pulmonary hypertension with right heart failure. After medical team’s assessment, the treatment plan was based on armaceutical control. Thus, the target therapy of Riociguat was performed. For such type of patients, even with non-specific symptoms, the possibility of pulmonary embolism can still be predicted based on medical history, lab­oratory data, physical examination, and the imaging differential diagnosis. If diagnosis and treatment can be conducted as early as possible, the high mortality rate of chronic hromboembolic pulmonary hypertension can be reduced.</p> <p> </p>