PMC3972028_01
Standard chest X-ray on admission.
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At that time, only a standard chest X-ray taken on admission was available, and showed a prominent bihilar region with a suspicious mass in the left apex of the lung (Figure 1).
Esophageal ultrasound.. Notes: (A) The mass in the left upper lobe was diagnosed histologically as fibrosis. (B) The main branch of the pulmonary artery is larger than the main stem of the aorta, which is typical for pulmonary arterial hypertension. Initially, this was thought to be chronic thromboembolic pulmonary hypertension but there was no ventilation/perfusion (V/Q) mismatch. The thickened and irregular left pulmonary artery walls and thrombi-like formations in the endobronchial ultrasound images are not shown. (C) Fibrosis in the left upper lobe. (D) Some compression of the left upper lobe is evident but this seems to be adherent thrombus formation with wall thickening, as seen on magnetic resonance imaging.
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After the weekend, we observed a mediastinal mass, abnormally enlarged central pulmonary arteries with tortuous peripheral branches, and a consolidation in the left upper lobe on the patient's computed tomography scan (Figures 2 and 3).
Maximum intensity projection view.. Notes: (A) Maximum intensity projection view showing a segmental pulmonary vein compressed by fibrosis in the left upper lobe. (B) Several tortuous vessels, mainly branches of the pulmonary artery. Note that the pale whitish vessels are pulmonary veins and the intense whitish vessels are branches of the pulmonary arteries. Left atrial tortuosity seen mainly in the branches of the pulmonary artery indicates high precapillary pressure.
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After the weekend, we observed a mediastinal mass, abnormally enlarged central pulmonary arteries with tortuous peripheral branches, and a consolidation in the left upper lobe on the patient's computed tomography scan (Figures 2 and 3). Small tortuous peripheral pulmonary arteries were seen on cardiac MRI and computed tomography (Figure 3).
Magnetic resonance imaging.. Notes: (A) Magnetic resonance imaging on February 21, 2013 showing pulmonary artery thrombi, enlargement of the walls of the right pulmonary artery main and segmental left pulmonary artery (yellow arrow), and fibrosis in the mediastinum (blue arrow) in the low subcarinal position. (B) Fibrosis in the left upper lobe (blue arrow). (C) Subcarinal/mediastinal fibrosis anterior to the main branch of the right pulmonary artery and thrombi (green arrow), and thickening of the wall of the left pulmonary artery (the right bronchus is indicated by the yellow arrow and the left bronchus by the red arrow). (D) Compression of segmental pulmonary artery in the left upper lobe (yellow arrow) by wall thickening and the mass of the incorporated thrombus. (E and F) Paradoxical shift of septum (bulging) demonstrating clearly typical signs of precapillary pulmonary hypertension.
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Histologically, there was no suggestion of malignancy, but a strong suspicion for fibrosis was raised on evaluation of the magnetic resonance imaging (MRI) scans (Figure 4) but could not be proven. Cardiac MRI showed reduced right ventricular function, dilatation of the right ventricle and a hypertrophic wall, a flattened septum with paradoxical movement (Figure 4), and dilated pulmonary artery trunks.
Lung biopsy showing increased interstitial collagen (  blue), elastic fibers (a gray), activated pneumocytes ( ), and pathological vessels with thickened walls.   100x.
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A transthoracic lung puncture of the mass in the left upper lobe revealed thickened pulmonary arterial walls, activated pneumocytes, and fibrosis (Figures 5-7).
Lung biopsy with increased interstitial collagen (red) and elastic fibers (blue), Activated pneumocytes , Pathologic vessels with thick walls.   evaluation of vessel invasion, 100x.
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A transthoracic lung puncture of the mass in the left upper lobe revealed thickened pulmonary arterial walls, activated pneumocytes, and fibrosis (Figures 5-7).
Lung biopsy with interstitial fibrosis   and activated pneumocytes  . Hematoxylin and eosin, 100x.
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A transthoracic lung puncture of the mass in the left upper lobe revealed thickened pulmonary arterial walls, activated pneumocytes, and fibrosis (Figures 5-7).
Coronary angiography.. Notes: (A) Although an MRI stress test was negative, the patient showed marked endothelial dysfunction on coronary angiography (synthesis of nitric oxide was decreased, as occurs in pulmonary arterial hypertension). A small dilatation coronary pathia with minor coronary artery disease is observed. PA0/caudal 25 degrees: left main stem and circumflex (left anterior descending artery with branches). (B) RAO30/0 dextro-angiography with pigtail during end diastolic phase (left) and end systolic phase (right) on February 20, 2013, there was no severe tricuspid regurgitation. Moreover, she had no right heart decompensation. This could be colored and overlaid for assumption of right ventricular ejection fraction in live pictures the right ventricular ejection fraction was reduced to 45%. Note right ventricle anterior wall-thickness of around 10 mm (n<=3 mm, arrow).. Abbreviations: PA, pulmonary artery; RV, right ventricular.
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Precapillary PAH with a PAR of 1,360 dyn   s   cm-5 and completely normal wedge pressure was seen on right heart Swan-Ganz catheterization, coronary angiography, and dextrography (Figure 8). The right ventricle was hypokinetic especially in its anterior portion (Figure 8C and D) with an enlarged wall which, on echocardiography, was 8 mm thick (ie, thickness of right ventricular anterior wall in end diastole). The left marginal artery and diagonal branch showed an angiographic stenosis of 70% (Figure 8A and B), and left ventricular ejection fraction was 70%. There was endothelial dysfunction with low flow phenomenon in different coronary arteries, besides the partly abnormal coronary anatomy with dilated cardiomyopathy (Figure 8A and B).
Right sided pulmonary artery angiography performed at Giessen indicating chronic thromboembolic pulmonary hypertension which was also demonstrated by two ventilation/perfusion (V/Q) mismatches.
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At the end of April, the patient was presented for the first time at the National Pulmonary Hypertension Center of Excellence in Bad Nauheim/Giessen and a second time in May 2013 (Figure 9) with a differential diagnosis of idiopathic PAH. Repeat pulmonary angiography showed no thrombus (Figure 9), and chronic thromboembolic pulmonary hypertension was excluded on ventilation/perfusion (V/Q) scintigraphy.
Isodoses clearly showing the main branch of the pulmonary artery with the first segmentation included.. Note: These images were taken on the first weekend of admission during which the patient received a total radiation dose of 4 Gy delivered over 2 days (February 2, and February 3, 2013).
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Meanwhile, her spontaneous pO2 at rest had increased to 76 mmHg and her 6-minute walking distance was 360 m (Figure 10).