case_images: PMC10504831_01
case_images: Reddish-purple rash with hyperpigmentation on bilateral cheeks, nose, nasal bridge, and perioral regions.
case_images: 10.1177_2050313X231200965-fig1.jpg
case_images: PMC10504831_01_10.1177_2050313X231200965-fig1.jpg
case_images: fig1-2050313X231200965
case_images: We noted reddish-purple rash and hyperpigmentation on bilateral cheeks, nose, nasal bridge, and perioral region (Figure 1).
case_images: ECG showed atrial fibrillation with incomplete RBBB and RVH.
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case_images: PMC10504831_01_10.1177_2050313X231200965-fig2.jpg
case_images: fig2-2050313X231200965
case_images: Electrocardiogram (ECG) showed atrial fibrillation with rapid ventricular response, right axis deviation, incomplete right bundle branch block (RBBB), and right ventricular hypertrophy (RVH) (Figure 2).
case_images: Massive cardiomegaly with prominent pulmonary artery segment and cardiac waist.
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case_images: Chest X-ray showed marked cardiomegaly, widened right heart border, enlarged segment of main pulmonary artery and prominent cardiac waist (Figure 3).
case_images: (a) Parasternal long axis view showed typical rheumatic mitral disease such as chordal and leaflet thickening of MV, hockey stick deformity of anterior mitral leaflet, and an enlarged LA. (b) Doppler evaluation showed a mean mitral gradient of 10-12 mmHg and a MV area of 0.8 cm2 by pressure half-time measurement, indicating severe MS. (c) Two-chamber view showed severe MR indicated by a vena contracta width of 0.7 cm with eccentric regurgitation jet. (d) Four-chamber view showed severe TR. (e) Zoomed parasternal long axis view showed moderate AR jet.
case_images: 10.1177_2050313X231200965-fig4.jpg
case_images: PMC10504831_01_10.1177_2050313X231200965-fig4.jpg
case_images: fig4-2050313X231200965
case_images: Transthoracic echocardiogram demonstrated severe MS with concomitant severe mitral regurgitation (MR), moderate aortic regurgitation (AR), severe tricuspid regurgitation (TR) with significant tricuspid stenosis, and mild pulmonic regurgitation (Figure 4). There were typical characteristics of rheumatic valvular disease such as chordal and leaflet thickening, hockey stick deformities of mitral valves (MV), restricted leaflet motions and coaptation defect (Figure 4). Doppler measurement showed a mean MV gradient of 11-12 mmHg and a MV area of 0.8-0.9 cm2 by pressure half-time measurement, indicating severe stenosis (Figure 4).
case_images: (a) Continuous wave Doppler measurement at TR jet showed a TR maximal velocity of 3.4 m/s. (b) Pulsed wave Doppler measurement at LV outflow tract showed LVOT VTI of 14 cm.
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case_images: fig5-2050313X231200965
case_images: Hemodynamic measurement by Doppler echocardiography showed left ventricular outflow tract velocity time integral (LVOT VTI) of 14 cm, stroke volume of 44 ml and cardiac output of 3.4 lpm (Figure 5).