Onoe Takashi, Tanaka Asuka, Ishiyama Kohei, Ide Kentaro, Tashiro Hirotaka, Ohdan Hideki
Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-0037, Japan.
Institute for Clinical Research, National Hospital Organization Kure Medical Center/Chugoku Cancer Center, Kure, Japan.
Surg Case Rep. 2018 Feb 7;4(1):15. doi: 10.1186/s40792-018-0423-6.
Portopulmonary hypertension (PPH) is a relatively rare but well-recognized complication of end-stage liver disease. Moderate or severe PPH (mean pulmonary artery pressure [mPAP] ≥ 35 mmHg) is usually a contraindication for liver transplantation due to high operation-related mortality. Here, we report on a patient with moderate PPH whose condition was successfully managed with a phosphodiesterase type 5 (PDE5) inhibitor (tadalafil) and prostaglandin E1, who experienced rapid improvement of PPH after living-donor liver transplantation (LDLT).
A 63-year-old woman with alcoholic decompensated cirrhosis was referred to our hospital for LDLT. She had mild dyspnea on exertion as well as fatigue. Echocardiography and subsequent cardiac catheterization revealed a high mPAP (35 mmHg), and she was diagnosed with moderate PPH. We commenced treatment with oral tadalafil for the PPH. A second preoperative echocardiography demonstrated improved PPH, and she underwent LDLT. An intravenous infusion of prostaglandin E1 was introduced instead of tadalafil during and after the operation. The mPAP value showed a rapid decrease in mPAP value to 22 mmHg in 2 days. After discontinuation of the prostaglandin E1, the mPAP value remained 23 mmHg. Postoperative catheterization 2 months after LDLT showed no exacerbation of PPH. She was discharged on foot 70 days after LDLT in good condition and has shown a good clinical condition more than 2 years after LDLT.
LDLT could be a radical treatment for PPH with careful management and adequate patient selection. PDE5 inhibitor and PGE1 is effective and feasible for perioperative management of the patient with moderate portopulmonary hypertension in LDLT.
门肺高压（PPH）是终末期肝病一种相对罕见但已被充分认识的并发症。中度或重度PPH（平均肺动脉压[mPAP]≥35mmHg）通常因手术相关死亡率高而成为肝移植的禁忌证。在此，我们报告一例中度PPH患者，其病情通过5型磷酸二酯酶（PDE5）抑制剂（他达拉非）和前列腺素E1成功得到控制，该患者在活体肝移植（LDLT）后PPH迅速改善。
一名63岁酒精性失代偿性肝硬化女性因LDLT转诊至我院。她有劳力性轻度呼吸困难及疲劳症状。超声心动图及随后的心导管检查显示mPAP较高（35mmHg），她被诊断为中度PPH。我们开始用口服他达拉非治疗PPH。第二次术前超声心动图显示PPH有所改善，随后她接受了LDLT。术中及术后用前列腺素E1静脉输注替代他达拉非。mPAP值在2天内迅速降至22mmHg。停用前列腺素E1后，mPAP值维持在23mmHg。LDLT术后2个月的术后导管检查显示PPH未加重。她在LDLT术后70天步行出院，状况良好，且在LDLT术后2年多来临床状况一直良好。
通过谨慎管理和适当的患者选择，LDLT可能是治疗PPH的一种根治性方法。PDE5抑制剂和PGE1对LDLT中中度门肺高压患者的围手术期管理有效且可行。
