PMC6083429_01
a. Flattening of inspiratory flow volume loop; b. CXR with interstitial changes in lung bases; c,d. CT Chest with ground-glass opacities (c) and air-trapping and enlarged pulmonary artery (d).
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Baseline spirometry was normal with normal diffusion capacity of carbon monoxide (DLCO) of 105% predicted and flattening of the inspiratory flow volume loops, which was consistent with upper airway obstruction seen in VCD (Fig. 1A). CXR showed mild interstitial changes within the lung bases (Fig. 1B). Computed tomography (CT) of the chest, while negative for pulmonary embolism, demonstrated diffuse nodular ground-glass opacities and soft centrilobular nodules bilaterally (Fig. 1C). Additionally, there were areas of air trapping and the main pulmonary artery was enlarged (Fig. 1D).
a-d. Explanted lungs showing intimal fibrosis of veins and venules, diffuse alveolar septal thickening, alveolar hemosiderosis and intimal fibrosis of pulmonary arteries and hypertensive arterial changes.
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The explanted lungs demonstrated veins and venules with marked intimal fibrosis (Fig. 2A) and diffuse alveolar septal thickening (Fig. 2B). Additionally, there was congestion and edema noted with the presence of alveolar hemosiderosis indicating with chronic congestion. (Fig. 2C). Furthermore, the pulmonary arteries showed intimal fibrosis and hypertensive arterial changes (Fig. 2D).
PMC6083429_02
a. Normal CXR; b. Flow volume loop with flattening of inspiratory limb; c-d. CT chest showing bronchiectasis, ascending aorta aneurysm and mild pulmonary artery enlargement.
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Chest x-ray was normal with the exception of mild mid-thoracic scoliosis (Fig. 3A). Repeat spirometry showed flattening of the inspiratory limb of the flow-volume loop and restrictive pattern that were not consistent with asthma (Fig. 3B). However, as her exertional dyspnea persisted, it was further evaluated with a high resolution chest CT scan which showed a focal area of bronchiectasis in the right upper lobe and otherwise normal lung parenchyma (Fig. 3C). There was mild aneurysmal dilatation of the ascending aorta at 3.7cm and mild enlargement of the main pulmonary artery at 3.4cm (Fig. 3D).
a-b. Chest CT with volume rendered images showing a large patent ductus arteriosis.
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These were remarkable for a large patent ductus arteriosus (PDA) with a diameter of 5mm (Fig. 4A &B).