Chronic thromboembolic pulmonary hypertension in a pt with A
The following case has been reported in the journal Annals of Clinical Case Reports.

A 29-year-old man was admitted to this hospital because of chest pain, decrease of exercise capacity, and hemoptysis. The patient had been well until September 2015, when chest pain, dull pain on the left shoulder and back, and paroxysmal cough developed. Computed Tomography Pulmonary Angiography (CTPA) revealed a left inferior lobe pulmonary embolism. Rivaroxaban 20 mg Qd was prescribed and the patient got a pain remission.

One month later, the patient felt symptoms repeated and shortness of breath after some activities developed. He went to hospital and the result of lupus anticoagulant (LA), anticardiolipin antibody (ACL), and anti-beta-glycoprotein (anti-β2GPI) are above normal, which showed the diagnosis of primary antiphospholipid syndrome (PAPS).

A transthoracic echocardiogram (ECG) showed the size of the right ventricle and right atrium was large; the pulmonary artery systolic pressure was 98 mmHg, with an ejection fraction of 63%. He was considered as chronic thromboembolic pulmonary hypertension (CTEPH) and hydroxychloroquine 200 mg Qd as well as sildenafil 20 mg Tid was added. Then the patient felt relief of symptoms.

Three months later, the patient went to the hospital again because of hemoptysis. The erythrocyte sedimentation rate was 87 mm/h. A computed tomographic (CT) scan of the chest showed a 4.5 x 5.4 cm high density, clear boundary shadow in the apex of the left lung. The lobes of right lung showed multiple ill-defined patchy ground glass opacities and nodules.

While hospitalized, anticoagulant therapy was suspended, and pituitrin was used for hemostasis. The symptom of hemoptysis was getting better. 2 weeks later, the ECG reexamination showed that the pulmonary artery systolic pressure was 101 mmHg. And this time the CTPA showed multiple pulmonary embolism. Complete obstruction of left pulmonary artery and fresh thrombosis of right lung could be seen.

Approach to this situation, the patient was given low molecular weight heparin 4000 iu IH Qd. But next day he presented hemoptysis again; and the amount of the blood was about 200-300ml. Bronchial arteriography (BAG) showed pulmonobronchial shunt of the left lung. Embolism of bronchial artery was used to treat hemoptysis and it worked out. Anticoagulation was continued under close surveillance.
1 month later, PTE was operated on the patient. After the surgery, the patient felt an obvious increase of exercise capacity. ECG reexamination revealed that the pulmonary artery systolic pressure was down into the normal range; with triple antiphospholipid antibodies (aPLs) positive.

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