PMC4821363_01
Chest X-ray Film. Chest X-ray images showing cardiac dilatation with a cardiothoracic ratio of 55%, enlarged pulmonary shadow and enlargement of the left second and fourth aortic arches.
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Chest X-ray revealed an enlarged pulmonary artery shadow with a cardiothoracic ratio of 55% and protrusion of the left second and fourth aortic arches (Fig. 1).
12-Lead Electrocardiogram. Electrocardiogram showing right axis deviation, a pulmonary P wave in leads II, III, and aVF, and a negative T wave in leads III, aVF, and V1-4.
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Electrocardiogram revealed right axis deviation; a pulmonary P wave in leads II, III, and aVF; and a negative T wave in leads III, aVF, and V1-4 (Fig. 2).
Echocardiography. The left and right images show the short-axis and four-chamber views, respectively. Enlargement of the right atrium and ventricle and marked left-ventricular displacement, are observed.
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Echocardiogram revealed enlargement of the right atrium and right ventricle with left-ventricular displacement (Fig. 3).
Pulmonary Angiography. The left and right images show the right and left pulmonary arteries, respectively. Pulmonary arteriography shows webbing, bands, abrupt narrowing, and disruption but no centrally operable lesion.
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Pulmonary arteriography revealed webbing, bands, abrupt narrowing, and disruption (Fig. 4), and pulmonary ventilation/perfusion scintigraphy revealed a mismatch (Fig. 5).
Balloon Pulmonary Angioplasty. The right and left images are pre-BPA and post-BPA, respectively. Contrast-enhanced images from before and after additional treatment showing the lateral basal segmental artery of right lung (Rt.A9b). On DSA images, many peripheral vessels are seen post-BPA. Abbreviations: BPA, balloon pulmonary angioplasty; DSA, digital subtraction angiography.
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Fig. 6 shows contrast-enhanced images from before and after additional treatment on the right lower lobe branch.
Clinical Course. This figure shows changes in the pulmonary arterial pressure with TRPG measured on cardiac echocardiography as an index. After PEA, the TRPG dropped to 24 mmHg but it later reached 95 mmHg during an exacerbation. After hospitalization, gradual exacerbation was observed despite the use of targeted medical therapy, and TRPG eventually reached 120 mmHg. After that, TRPG decreased with each BPA procedure. After five BPA procedures, TRPG decreased to 30 mmHg at 4 months and 22 mmHg at 15 months. Abbreviations: Apr, April; BPA, balloon pulmonary angioplasty; Dec, December; Jan, January; PEA, pulmonary endarterectomy; TRPG, tricuspid regurgitation pressure gradient; Nov, November; Oct, October.
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Table 2 and Fig. 7 show the changes in clinical data.
Changes over Time in Chest X-ray Film. Left image is post-PEA, center image is during an exacerbation after hospitalization, and right image is 15 months after BPA. During exacerbation, enlargement of the second and fourth aortic arches is seen together with blood congestion, but these improved after BPA. Abbreviations: BPA, balloon pulmonary angioplasty; PEA, pulmonary endarterectomy.
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The changes observed in chest X-ray images, 12-lead electrocardiograms, echocardiograms, and ventilation scintigraphy in Fig. 8, Fig. 9, Fig. 10, Fig. 11 are consistent with pulmonary hypertension pressure dynamics.
Changes over Time in Echocardiography. Left image is post-PEA, center image is during an exacerbation after hospitalization, and right image is 15 months after BPA. The D-shape showing left-ventricular displacement disappeared after BPA. Abbreviations: BPA, balloon pulmonary angioplasty; PEA, pulmonary endarterectomy.
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The changes observed in chest X-ray images, 12-lead electrocardiograms, echocardiograms, and ventilation scintigraphy in Fig. 8, Fig. 9, Fig. 10, Fig. 11 are consistent with pulmonary hypertension pressure dynamics.