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+A 69-year-old man became aware of the onset of exertional dyspnea in July 2010.
+He had a history of smoking for more than 40 years, 20 cigarettes per day.
+His parents and 1 brother had lung cancer.
+His past medical history was unremarkable.
+He had no occupational exposure.
+Due to the exertional dyspnea, he was admitted to the People's Hospital of Shunde District for echocardiography and computed tomography pulmonary angiography (CTPA).
+Echocardiography indicated that he had severe PAH, because his pulmonary arterial pressure (PAP) was 104 mmHg, and his right heart was enlarged (the right ventricle diameter: 57 mm).
+CTPA showed that he had not only PAH, but also a thrombus in the upper lobe artery of the right lung.
+However, the region in the lung affected by this PE was considered too small to induce such a severe PAH.
+In addition, he did not have connective tissue disease, drug use, history of chemotherapy, or HIV test (+), so he was diagnosed as IPAH combined with PE.
+He was treated with warfarin (2.5 mg per day with a target of the international normalized ratio [INR] between 1.5 and 2.5) and slidenafil targeted PAH therapy (20 mg twice per day).
+The patient's dyspnea was relieved slightly after the use of these medications for 1 month and the retested PAP by echocardiography was decreased to 72 mmHg, but all the other symptoms and signs failed to further improve after taking these medications for 6 months.
+In March 2011, he went to the Kyorin University affiliated hospital in Japan for further treatment.
+Right heart catheterization revealed PAP 72/34/47 mmHg, pulmonary capillary wedge pressure (PCWP) 17/5/10 mmHg, cardiac output 4.3 L/min, cardiac ejection index 2.36 L/min/m2, and pulmonary vascular resistance 7.3 Wood units.
+He was retested for CTPA that showed that the thrombus in the upper lobe artery of the right lung disappeared.
+He was still diagnosed as having IPAH, so treated with sildenafil (20 mg 3 times a day) and sorafenib (400 mg once a day) targeted PAH therapy and oral warfarin anticoagulation therapy (2.5–3.75 mg per day, but the daily dose was adjusted by INR that was between 2 and 3).
+Disappointingly, his dyspnea was not relieved and the PAP tested by the echocardiography was around 95 mmHg.
+In September 2011, he visited the Shanghai Pulmonary hospital in China because of no relief of dyspnea.
+Arterial blood gas analysis showed that PaO2 was 63 mmHg with oxygen saturation 93%; lung function test revealed a mild obstructive dysfunction of the pulmonary ventilation (FEV1/FVC 62.06%, FEV1% Prediction 80.9%) and a severe reduction in the diffusing capacity of the lungs for carbon monoxide (DLCO 42%); 6-minute walk test was 385 m.
+Chest high-resolution computed tomography (HRCT) (Fig.1A and B) showed smooth thickening of interlobular septa, diffuse centrilobular nodules, and the enlargement of mediastinal lymph nodes.
+The right heart catheterization revealed that PAP was 83/34/53 mmHg and PCWP was 14/6/11 mmHg.
+Acute vasodilator testing was negative.
+Coronary angiography and CTPA were both normal; pulmonary ventilation/perfusion (V’/Q’) lung scan showed a matched ventilation and perfusion defects.
+Bronchoalveolar lavage showed an elevated percentage (65%) of hemosiderin-laden macrophages with the Golde score 80 (the normal range: 0–20).
+He was still diagnosed as having IPAH, but he was suspected as actually having PVOD.
+He was continuously treated with the targeted PAH therapy, and he was asked to continue his anticoagulation treatment, but he stopped warfarin anticoagulation treatment by himself sometime in 2014 (he failed to recall the exact date.) because he felt that it was too cumbersome to frequently test the INR for blood coagulation monitoring.
+Furthermore, after he discontinued his warfarin therapy, he also failed to monitor D-Dimer to prevent PE recurrence.
+His dyspnea was relatively stable, but he developed many, but not fatal, episodes of pulmonary edema during the therapy.
+In January 2015, he was rushed to hospital because his dyspnea was suddenly exacerbated.
+CTPA showed a thrombus in the right main pulmonary artery (Fig.1C).
+Rivaroxaban was administered for anticoagulation treatment, but this medication did not relieve his dyspnea, therefore, he was too ill to walk.
+Instead, he was bound to his wheelchair for half a year.
+The severe PAH (suspected as PVOD) combined with a life-threatening PE mandates a lung transplantation for his survival.
+Six months later, he was undergone a bilateral sequential allogenic lung transplantation assisted by veno-arterial extra-corporeal membrane oxygenation (VA-ECMO) after he signed the written consent on July 29, 2015.
+Histology of the explanted lung specimen showed pulmonary vein occlusion (Fig.1D–F) and the pulmonary embolism in the right pulmonary artery.
+Until then, he was confirmed as having PVOD combined with PE, instead of IPAH.
+He has been living well without dyspnea for more than 1 year after the lung transplantation and the echocardiography showed the normalizations of PAP and the once enlarged right ventricle of his heart.
+This study was approved by the Ethics Committee of The People's Hospital of Zhongshan City.
+The written informed consent was obtained from the patient.