PMC5730424_01
A: Electrocardiography shows a normal sinus rhythm with right axis deviation and SIQIIITIII. B: A chest X-ray documents cardiac dilation and dilated right pulmonary artery. C: The echocardiographic findings reveal compression of the left ventricular. D: Reduced trace uptake in left parotid gland ( ). E: Lymphocyte infiltration around acinus were detected in subcutaneous tissue of lip.
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Electrocardiography revealed right axis deviation with SIQIIITIII (Fig. 1A). A chest X-ray showed cardiomegaly with a dilated right pulmonary artery (Fig. 1B). Echocardiography detected 56 mmHg of a tricuspid regurgitation pressure gradient, hypertrophy (RV free wall thickness: 7.3 mm) and low systolic function of right ventricle (TAPSE: 12 mm), and a compressed left ventricle forming a D-shape (Fig. 1C). On the basis of the complaint of a dry mouth, the patient underwent salivary gland scintigraphy showing reduced uptake in the left parotid gland (Fig. 1D). Shilmer's test and lip biopsy (Fig. 1E) were positive.
A: Hemodynamic improvement after upfront combination therapy in the first 3 days. B: Fluid retention was also treated by diuretics from day 4-8. C: Low oxygen saturation sustained until day 14 under noninvasive positive pressure ventilation (NPPV: day 5-). Pulmonary edema was dramatically improved steroid pulse therapy (day 14-16). CI: Cardiac index, BW: Body weight, UV: Urine volume.
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Due to the severity of the patient's hemodynamics and resting dyspnea with a WHO functional class IV, upfront combination therapy using intravenous epoprostenol, macitentan, (10 mg/day), and sildenafil (60 mg/day) was initiated from day 0 together with dobutamine (Fig. 2A). To improve the congestion, we introduced continuous intravenous furosemide and a NPPV with 6 cmH2O positive end-expiratory pressure (PEEP) (Fig. 2B) and stopped the dose escalation of epoprostenol. Regardless, the patient experienced intensive dehydration (2.2-5.7 L of urine volume/day), weight loss (64-54 kg), and a reduced PE during days 5-10, and low oxygen saturation (SpO2 93%) (Fig. 2C) and GGO exacerbated (Fig. 3B) during days 10-14. After steroid administration, pulmonary edema dramatically improved (Fig. 2, Fig. 3B) and finally returned to baseline (Fig. 3B) on day 32.
A: In chest X-ray, pulmonary edema worsening from day 4 to day 14 in spite of reduced cardiomegaly, which improved by steroid at day 15. B: A chest CT-scan at day 0 is within normal limit. Multiple ground glass opacities (GGO) and pleural effusion (PE: ) emerged from day 4. Regardless PE improvement, GGO without inter-lobular septal thickening became worse from day 10-14. Intravenous prednisolone pulse (1000 mg/day for 3 days) improved GGO at day 18 to normal limit (day 32).
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Chest contrast-enhanced computed tomography (CT) revealed no lung diseases, pulmonary embolism, pleural effusion, or lymphadenopathy (Fig. 3B). However, multiple slight ground glass opacities (GGO) and bilateral pleural effusion (PE) emerged as well (Fig. 3B). After steroid administration, pulmonary edema dramatically improved (Fig. 2, Fig. 3B) and finally returned to baseline (Fig. 3B) on day 32.