PMC9052963_01
Severe pulmonary hypertension due to congenital extrahepatic portocaval shunt. (a) CT upon admission shows mildly to moderately expressed centrilobular ground-glass opacities in all lobes (arrows) and in places interlobular septal thickening suggesting PCH/POVD. (b) CT 2 days after macitentan in combination with NO shows worsening of interlobular septal thickening (arrows). (c) Histological examination of explanted lungs shows complex lesion characteristic of pulmonary arterial hypertension showing plexiform changes with a network of vascular channels lined with endothelial cells, surrounded by dilated thin-walled vein-like vessels (thin arrows) and thick-walled small pulmonary artery (thick arrow) due to a concentric intimal proliferation (hematoxylin-eosin, original magnification x100). (d) CT venography, volume rendering technique (VRT):reconstruction shows anomalous drainage (marked with *) of a major part of superior mesenteric vein and splenic vein into inferior vena cava through the left renal vein and hypoplastic portal vein. (CT, computed tomography; IVC, inferior vena cava; LRV, left renal vein; PCH/POVD, pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis; PV, portal vein; RRV, right renal vein; SMV, superior mesenteric vein; SV, splenic vein)
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Radiological characteristics suggested a diagnosis of pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH) rather than PAH (Figure 1a). The clinical state did not improve, and a control computed tomography (CT) 2 days later showed significant deterioration of interlobular septal thickening (Figure 1b) further suggesting PVOD/PCH. Unexpectedly, histological examination of the explanted lungs was consistent with changes due to PAH and not PVOD/PCH (Figure 1c). It finally revealed CEPS Type II as the underlying reason for severe pulmonary hypertension (Figure 1d).