PMC8764447_01
Echocardiography shows both RA and RV are significantly dilated, and the RV wall thickness is increased (A,B). An enlargement of the PA and (C) its branches. PA, pulmonary artery; RPA, right pulmonary artery; LPA, left pulmonary artery; AO, aorta; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.
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F1
Echocardiography further confirmed that both the right atrium (RA) and right ventricle (RV) were significantly dilated (the inner diameters of the RA and RV were 4.24 and 4.31 cm, respectively, measured in the four-chamber view), and the RV wall was thickened (1.02 cm) (Figures 1A,B). Notably, there was an enlargement of the pulmonary artery and its branches (Figure 1C), and the estimated pulmonary arterial pressure (PAP) was 98 mmHg, suggesting pulmonary arterial hypertension (PAH).
Abdominal US shows that the intrahepatic PV system is absent (arrows) (A), and the GDV is tortuous and dilated (B). US, ultrasonography; PV, portal vein; GDV, gastric fundus vein; STO: stomach.
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F2
At that moment, an amazing discovery was found by abdominal ultrasonography (US): his intrahepatic portal vein (PV) was absent, which seemed to be occluded (Figure 2A).
PV-CTA shows that one branch of SV flows into IVC via LRV (A). The other branch of SV merges with SMV into PV, the intrahepatic branch of PV is slim, and the proximal trunk of PV is short and calcified (B). The esophageal and gastric fundus vein (A), and the SV are dilated and tortuous (B). Dilatation of intrahepatic bile duct (B). PV-CTA, portal vein-computed tomographic angiography; SV, splenic vein; IVC, Inferior vena cava; LRV, left renal vein; SMV, superior mesenteric vein.
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There seemed to be two pathways for blood to flow in the main splenic vein (SV): one was in the portal vein that merged with the superior mesenteric vein and sent out a small branch into the liver (Figures 3A,B), whilst the other entered the inferior vena cava (IVC) via left renal vein.
Hepatic MR shows the existence of liver nodule. MR, magnetic resonance.
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F4
Liver magnetic resonance imaging (MRI) confirmed their existence (Figure 4).