“Denervation” of autonomous nervous system in idiopathic pulmonary arterial hypertension by low-dose radiation: a case report with an unexpected outcome

Figures & data

Figure 1 Standard chest X-ray on admission.

Figure 2 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.

Figure 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.

Figure 4 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.

Figure 5 Lung biopsy showing increased interstitial collagen (⇦ blue), elastic fibers (a gray), activated pneumocytes (➞), and pathological vessels with thickened walls. ☆ 100×.

Figure 6 Lung biopsy with increased interstitial collagen (red) and elastic fibers (blue), Activated pneumocytes➞, Pathologic vessels with thick walls. ☆ evaluation of vessel invasion, 100×.

Figure 7 Lung biopsy with interstitial fibrosis ★ and activated pneumocytes ➞. Hematoxylin and eosin, 100×.

Figure 8 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.

Figure 9 Right sided pulmonary artery angiography performed at Giessen indicating chronic thromboembolic pulmonary hypertension which was also demonstrated by two ventilation/perfusion (V/Q) mismatches.

Figure 10 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).