Myocardial Infarction and Pulmonary Arterial Hypertension in a Young Patient with Systemic Lupus Erythematosus

Abstract

We report a-27-year old girl with 20 years of systemic lupus erythematosus (SLE) who had not only pulmonary arterial hypertension but also acute myocardial infarction. The girl was admitted to the emergency room due to acute but persistent chest pain for 34 h. ECG showed ST-segment depression in leads II, III, avF, T wave inversion in lead V1- V4, and small Q wave in leads V7, V8, V9. Increased levels of creatinine kinase isoenzyme MB 170 ng/mL (N < 5) and cardiac-specific troponin I 11.76 ng/mL (N = 0-0.5) were also detected. Echocardiogram revealed hypokinesia at posterior part of her left heart. Laboratory tests showed she had significant proteinuria, hyperlipidemia, hypoalbuminemia with normal kidney and liver function. She has elevated erythrocyte sedimentation rate and C-reactive protein,positive Both ANA and anti-dsDNA were positive, but complements were within normal range. Other prothrombotic parameters such as homocysteine, anti-cardiolipin antibody, lupus anticoagulant were all normal. Her chest radiography showed significant bulging in pulmonary artery segment. Computed tomography (CT) scan of the chest showed a markedly enlarged main PA with a diameter of 4.4 cm. No signs of pulmonary embolism were detected by CTPA. She was diagnosed as active SLE and received pulse methylprednisolone (1 g for 3 times) followed by oral prednisolone 1 mg/kg, in combination with cyclophosphamide 1 g per month, and low molecular heparin, clopidogrel, provastatin, isosorbide dinitrate were used. After disappearance of her chest pain, coronary angiography and right heart catheterization were undertaken. Occlusion in LCX and 80% of stenosis in LAD were observed. The right heart catheterization showed elevated pulmonary artery pressure (93/32/57 mmHg) and pulmonary arteriolar resistance (11 Woods units), however with normal pulmonary capillary wedge pressure. Acute vasoreactivity test was negative to adenosine. We didn't perform percutaneous coronary intervention for the patient due to her active lupus. The girl was prescribed warfarin and bosentan targeting her pulmonary arterial pressures. Prednisolone was tapered to 10 mg/day within six months. ECG and cardiac enzyme levels returned to normal ranges. Coronary angiography showed recanalization in LCX, and still 80% stenosis in LAD. A decision was made to proceed with PCI, and three stents were placed in the coronary artery. She remained stable and asymptomatic without complications during the follow-up years. The mean pulmonary pressure was around 50 mmHg evaluated by UCG.