Introduction: T he presence of multiple pulmonary lesions in the young poses a difficult differential diagnosis between congenital causes, autoimune diseases and chronic embolism (CPTE). A multimodal imaging approach with careful clinical examination can aid in choosing the correct treatment.
Case presentation: A 32 years old man referred to our clinic for the first detailed cardiac workup. Notable as-pects from his early medical history include an inva-sive study at age 17, documenting the absence of the left inferior lobar artery (LILA), hypoplasia of the right inferior lobar artery (RILA) and a stenosis of the left superior lobar artery (LSLA) with a 20 mmHg gradient. More recently, in the past 3 years, he had two acute episodes consisting of hemoptysis, severe hypoxia and cough, occurring while on oral anticoagulation.
At presentation he was mildly hypoxic with moderate dyspnea. Inflammatory markers were normal. The echocardiography showed a small left pulmonary artery, normal RV systolic function and indirect signs of pulmonary hypertension. The invasive hemodynamic study confirmed the elevated pulmonary artery pressures (63 mmHg) and vascular resistances (11 uW); the LSLA and RILA lesions appeared to be more seve-re than at 17 years. Additionally there were numerous anomalous collaterals. The available CT scans descri-bed no intraluminal thrombi. In lack of a clear CTPE diagnosis the anticoagulation was discontinued. At one month, he is hospitalized with his first episode of deep vein thrombosis and anticoagulation was started. He is reevaluated in our clinic, having worsened exertio-nal dyspnea. A venous and arterial Doppler scan was performed, showing a fresh occlusive thrombus in the right deep femoral vein and abnormal arterial resistance indexes. A repeat pulmonary angio-CT showed no signs of acute thrombembolism. The review of the high resolution images confirmed no antegrade flow in the RILA and LILA, a focal, short severe stenosis of the LSLA and evidence of retrograde flow through the collaterals.
Conclusions: In a young patient the differential diagnosis of multiple pulmonary artery lesions is difficult. The progressive nature of the disease, the asymmetrical wall thickening, irregular intraluminal contours, without signs of chronic inflammation, post-stenotic collaterals supplying the occluded branches, all with a recent thrombotic event suggest the most likely dia-gnosis is CTPE. More unusual is the punctiform steno-sis on the LSLA, rarely seen in CTPE. He is scheduled to undergo dilation of this stenosis, will be evaluated for thrombectomy. He is treated with oral anticoagu-lation, bosentan and diuretics. Patients with multiple pulmonary arterial lesions require detailed multimodal evaluation to aid in the correct management pathway.