PMC9623250_01
A case of leflunomide-induced PAH in a young male patient with nephrotic syndrome. (A). Electrocardiograph showing signs of RV hypertrophy. (B) Echocardiogrph showing enlargement of the right side of the heart. (C). Enlarged right side of the heart pressing on the LV to form a D sign. (D). Computed tomography pulmonary angiography showing the ratio of the diameter of the dilated PA to the diameter of the aorta greater than (E). Cardiac magnetic resonance showing dilation of the right side of the heart, consistent with pulmonary hypertension-induced cardiac changes. AO, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle. (F,G) Comparison of PA diameter before suspension of leflunomide and after suspension of leflunomide.
fphar-13-992457-g001.jpg
PMC9623250_01_fphar-13-992457-g001.jpg
F1
An electrocardiogram revealed sinus rhythm with signs of right ventricular hypertrophy such as right axis deviation (>90 ), R/S in V1 > 1, and R in V1+S in V5 > 1.2 mv (Figure 1A). A preliminary diagnosis of pulmonary hypertension (PH) was made and PH of the left heart was excluded (Figure 1B). Chest X-ray and computed tomography pulmonary angiography suggested PA distension and dilatation of the right heart; there was no evidence of thromboembolic and restrictive pulmonary diseases (Figure 1D) and no signs of portopulmonary hypertension (PoPH) in the abdominal ultrasound. Cardiac magnetic resonance findings were consistent with PH-related cardiac changes (Figure 1E). The echocardiogram showed an RV diameter of 43 mm, RA diameter of 38 mm, PA diameter of 27 mm, tricuspid regurgitation velocity of 4 0.7 m/s, and estimated PASP of 97 mmHg (Figure 1C). Compared with the first X-ray, the PA diameter was obviously reduced (Figures 1F,G).