PMC4356039_01
Chest roentgenogram and computed tomography (a). Chest roentgenogram and computed tomography at the initial visit Honeycombing along with traction bronchiectasis in the subpleural and basal areas of both lungs were detected. (b). Chest roentgenogram and computed tomography at exacerbation of pulmonary hypertension Compared with Fig. 1a, the findings did not show marked progression of IPF, while congestion was prominent with an increased cardiothoracic ratio.
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Computed tomography (CT) showed honeycombing along with traction bronchiectasis in the subpleural and basal areas of both lungs (Fig. 1a). IPF was unlikely because the only significant finding from imaging examination and blood tests was elevated KL-6 that was attributed to influence of diuretic therapy given since before her admission and influence of congestion (Table 1 and Fig. 1b). In addition, after admission, transthoracic echocardiography and right heart catheterization were performed.
Images from transthoracic echocardiography. (a). Transthoracic echocardiography after admission in Oct. 2013 Upper panels: The right ventricle was enlarged and the interventricular septum was straightened or bowed into the left ventricle. Middle panels: Severe tricuspid regurgitation (TR) was detected. TR pressure gradient was 86.6 mmHg Lower panel: Mild pulmonary regurgitation (PR) was detected. PR pressure gradient was 16.1 mmHg Total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm (b). Transthoracic echocardiography at 1 year from start of bosentan therapy Upper panels: Findings improved compared with Fig. 2a. Middle panels: Severe TR was noted. TR pressure gradient was 48.2 mmHg Lower panel: Mild PR was noted. PR pressure gradient was too small to measure. Tei index was 0.40 and tricuspid annular plane systolic excursion (TAPSE) was 1.6 cm.
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The transthoracic echocardiographic finding suggested severe PH and a congestive condition (TR pressure gradient was 86.6 mmHg and PR pressure gradient was 16.1 mmHg total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm). (Fig. 2a). At the follow-up assessment one year later her pulmonary function showed no significant changes and no apparent worsening of arterial blood gases, with evident improvement of PH, WHO functional class, maximum exercise tolerance on treadmill exercise testing, right heart catheterization, and transthoracic echocardiography (Table 2 and Fig. 2b).