Thrombocytopenia is a common complication in patients with portal hypertension due to chronic liver disease and is often life-threatening. Partial splenic embolization (PSE) was developed by Spigos as a treatment with fewer complications than splenic artery embolization. In recent years, the number of PSE procedures has decreased in Japan due to the introduction of lusutrombopag, an orally active, small-molecule human thrombopoietin (TPO) receptor agonist that induces platelet production. Since the report by Spigos, PSE has been found to not only improve thrombocytopenia, but also reduce portal venous pressure and improve liver function. Therefore, PSE can play a significant role as one of the multidisciplinary treatments for portal hypertension. We believe that PSE is a procedure that will never go away and deserves to be performed more aggressively.

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Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension in patients with or without cirrhosis, and it results from complex pathophysiological interactions between the portal and pulmonary circulations. PoPH is a relatively rare complication of portal hypertension, and female sex, autoimmune hepatitis as the underlying etiology, and portosystemic shunt development have been reported as clinical risk factors for PoPH. The prognosis of this disease is quite poor, which could be related to the delayed diagnosis and treatment of PoPH compared with PAH caused by other etiologies. In clinical practice, echocardiography, with which we can noninvasively estimate pulmonary arterial pressure, is used as a gatekeeper for the diagnosis of PAH including PoPH prior to right heart catheterization, which is the gold standard for the establishment of a definite diagnosis of pulmonary hypertension. Recently, multidrug therapies including endothelin receptor antagonists have come into commom use for patients with PoPH, providing good outcomes, and pharmacotherapies followed by liver transplantation are thought to be particularly appropriate for those with moderate to severe PoPH. In this review article, we summarize the current topics in the diagnosis and treatment of PoPH. In addition, we present our novel clinical research on this rare disease with a poor prognosis, along with an illustrative case.

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Ethanolamine oleate (EO; Oldamine^®) is the approved sclerosing agent for BRTO in Japan, but the drawback of EO is that its upper limit of use is 0.4 mL/kg or 30 mL as 5% ethanolamine oleate iopamidol (EOI). With large varices, the upper limit of sclerosing agent permitted may be insufficient for obliteration. Even with small varices, if there are many drainage veins, embolization with coils is difficult, and there is leakage throughout the systemic circulation, it is expected that the upper limit of sclerosing agent will be insufficient. To solve these problems, stepwise injections have been used to inject small amounts of sclerosing agent, coil embolization of the drainage veins is performed, and additional glucose solution is injected. However, there are still some difficult cases. We have been treating patients with a mixture of a small amount of gelatin sponge particles and 5% EOI for retrograde transvenous obliteration (GERTO). This is a very good treatment that reduces the systemic release of the sclerosing agent while also shortening the treatment time.

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Balloon-occluded retrograde transvenous obliteration (BRTO) is effective in the treatment of gastric varices and hepatic encephalopathy. However, in cases of severe liver dysfunction (Child-Pugh grade C), shunt occlusion leads to an increased risk of liver failure due to portal hypertension. Recent advances in devices such as double-balloon catheters, microcatheters, and embolic agents including n-butyl-2-cyanoacrylate (NBCA) and metallic coils have enabled partial BRTO and multiple-stage BRTO, leading to improved outcome. Strategic treatment planning with preoperative analysis of collateral vascular anatomy by computed tomography (CT) and pressure measurement before occlusion may provide safer and more effective BRTO. For cases of severe liver dysfunction, medical management is the priority of treatment. BRTO is part of a multidisciplinary treatment approach. When performing treatment, a strategy taking into account the long-term prognosis of the patient should be considered.

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Plug-assisted retrograde transvenous obliteration (PARTO) is a modified balloon-occluded retrograde transvenous obliteration (BRTO) procedure, devised in South Korea, in which a shunt vessel is embolized with gelatin sponge under vessel blockage by a vascular plug instead of a balloon catheter. PARTO has the advantages of resolving several problems of BRTO: PARTO can simplify a procedure with no need to downgrade, and it can shorten the patient’s resting time with no need for overnight retention of a balloon catheter. PARTO can be the first interventional treatment option for gastric varices and hepatic encephalopathy with a comparable clinical outcome to BRTO in cases in which the vascular plug can be appropriately placed.

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