case_images: PMC5985094_01
case_images: Clinical images on admission leading to diagnosis of pulmonary artery hypertension. (a) Chest X-ray on admission showed cardiac enlargement and severe bilateral plural effusion. (b) ECG suggested mild right ventricular overload and clockwise rotation. (c) Short axis view of the TTE showed flattening of the interventricular septum. (d) Apical four-chamber view of the TTE showed decreased left ventricular chamber size pressured by enlarged right ventricle. TTE: transthoracic echocardiography; LV: left ventricular; LA: left atrium; RV: right ventricular; RA: right atrium.
case_images: CRIC2018-3895197.001.jpg
case_images: PMC5985094_01_CRIC2018-3895197.001.jpg
case_images: fig1
case_images: An X-ray examination found significant bilateral pleural effusion and heart enlargement (Figure 1(a)). An electrocardiogram showed low voltage in the limb lead, left axis deviation, clockwise rotation, and negative T waves in the V1-V2 lead indicating pleural effusion and right ventricular enlargement (Figure 1(b)). A transthoracic echocardiography revealed an enlarged right ventricle (D1 59 mm, D2 48 mm) with flattening of the interventricular septum during systolic and diastolic phases and severe tricuspid regurgitation with the elevated tricuspid regurgitation pressure gradient (TRPG) of 66 mmHg, indicating increased PAP and volume overload (Figures 1(c) and 1(d)).
case_images: Clinical course of the case. BNP: B-type natriuretic peptide; PAP: pulmonary artery pressure; PVR: pulmonary vascular resistance; TRPG: tricuspid regurgitation peak gradient; WHO-Fc: World Health Organization functional class; F: furosemide 20 mg/day i.v. for three days; ND: not detected.
case_images: CRIC2018-3895197.002.jpg
case_images: PMC5985094_01_CRIC2018-3895197.002.jpg
case_images: fig2
case_images: II, and flattening of the interventricular septum and elevated TRPG (44 mmHg) was observed by the echocardiography. In right heart catheterization (RHC) on day 15, PAP was 55 (systolic)/26 (diastolic)/37 (mean) mmHg, pulmonary capillary wedge pressure was 8 mmHg, cardiac output was 6.55 l/min, cardiac index was 3.72 l/min/m2, and pulmonary vascular resistance was 353 dyne s cm-5, respectively (Figure 2). Six months later, mean PAP measured by RHC was decreased to 31 mmHg (Figure 2).
case_images: Follow-up echocardiography, 6 months after the upfront combination therapy. (a) Short axis view of the TTE showed no compression of the interventricular septum. (b) Apical four chambers view the TTE showed improvement of the right ventricular size. TTE: transthoracic echocardiography; LV: left ventricular; LA: left atrium; RV: right ventricular; RA: right atrium.
case_images: CRIC2018-3895197.003.jpg
case_images: PMC5985094_01_CRIC2018-3895197.003.jpg
case_images: fig3
case_images: Figure 3. Finally, 12 months after the upfront combination therapy and dasatinib discontinuation, TRPG evaluated by the echocardiography was decreased to 24 mmHg.