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Pulmonary artery intimal sarcoma misdiagnosed as chronic thromboembolic pulmonary hypertension and treated with balloon pulmonary angioplasty: A case report
摘要
Pulmonary artery intimal sarcoma (PAIS) is a rare disease with poor prognosis. PAIS is often misdiagnosed as chronic thromboembolic pulmonary hypertension (CTEPH) since both disorders share similar clinical presentations and image findings. A 47 years old female suffered from exertional dyspnea for 3 months. The transthoracic echocardiogram(TTE) and right heart catheterization revealed pulmonary hypertension. CTEPH was diagnosed according to the computed tomography pulmonary angiography (CTPA), the ventilation/perfusion scintigraphy and the selective pulmonary angiography. After the patient's symptoms progressed to WHO functional class III, we arranged balloon pulmonary angioplasty (BPA). After the procedure, the patient had significant clinical improvement to WHO functional class II. Hemodynamical improvement was documented by TTE. Unfortunately clinical deterioration occurred again 6 months after BPA. The CT scan revealed tumor invasion, metastatic lymph nodes and the pathology examination confirmed the diagnosis of PAIS. This is the first reported case about the impact of BPA on patient with PAIS.
延伸閱讀
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