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肝移植术后球囊闭塞逆行经静脉栓塞术治疗门肺高压的持续有效性:一项5年随访病例报告

Sustained effectiveness of balloon-occluded retrograde transvenous obliteration for portopulmonary hypertension after liver transplantation: a 5-year follow-up case report.

作者信息

Fukushima Masanori, Ueno Yuki, Ayano Yurika, Takahashi Kosuke, Miuma Satoshi, Soyama Akihiko, Ishimaru Hideki, Eguchi Susumu, Ikeda Satoshi, Miyaaki Hisamitsu

机构信息

Department of Gastroenterology and Hepatology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.

Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

出版信息

Clin J Gastroenterol. 2025 Sep 9. doi: 10.1007/s12328-025-02222-y.

Abstract

Portopulmonary hypertension (POPH), a subtype of pulmonary arterial hypertension (PAH), develops with portal hypertension and may persist after liver transplantation. While there have been successes using balloon-occluded retrograde transvenous obliteration (BRTO) for POPH, no reports exist on long-term follow-up. A 60-year-old man with hepatitis C cirrhosis developed POPH. At the time POPH was diagnosed, the mean pulmonary artery pressure (mPAP) was 31 mmHg. After medical management, mPAP improved to 20 mmHg, and he underwent living-donor liver transplantation. Three years post-transplant, he presented with severe dyspnea and elevated mPAP of 67 mmHg. Despite pharmacological treatment for PAH, his symptoms and pulmonary pressures remained uncontrolled. After transplantation, the pre-existing splenorenal shunt expanded, suggesting that increased shunt blood flow contributed to worsening POPH. BRTO was performed after confirming that shunt occlusion did not elevate portal pressure. Post-procedure, his pulmonary pressures improved (mPAP, 25 mmHg), and symptoms resolved. Over 5 years, no recurrence of POPH, portal hypertension, or liver dysfunction occurred. This case demonstrates the long-term effectiveness of BRTO in treating POPH exacerbation post-liver transplantation. In cases of POPH following liver transplantation, portosystemic shunt occlusion via BRTO may be an effective strategy to improve POPH.

摘要

门肺高压(POPH)是肺动脉高压(PAH)的一种亚型,与门静脉高压相关,且在肝移植后可能持续存在。虽然使用球囊闭塞逆行静脉闭塞术(BRTO)治疗POPH已取得成功,但尚无长期随访报告。一名60岁丙型肝炎肝硬化男性发生了POPH。在诊断出POPH时,平均肺动脉压(mPAP)为31mmHg。经过药物治疗,mPAP改善至20mmHg,随后他接受了活体供肝肝移植。移植后三年,他出现严重呼吸困难,mPAP升高至67mmHg。尽管接受了PAH的药物治疗,但其症状和肺压力仍未得到控制。移植后,原有的脾肾分流扩大,提示分流血流量增加导致POPH恶化。在确认分流闭塞不会升高门静脉压力后,进行了BRTO。术后,他的肺压力得到改善(mPAP,25mmHg),症状缓解。在超过5年的时间里,未出现POPH、门静脉高压或肝功能障碍复发。该病例证明了BRTO在治疗肝移植后POPH加重方面的长期有效性。在肝移植后发生POPH的病例中,通过BRTO闭塞门体分流可能是改善POPH的有效策略。

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