Department of Cardiology, Fujian Provincial Hospital, Fujian Medical University, Fujian Cardiovascular Institute, Fuzhou, 350001, China.
BMC Cardiovasc Disord. 2019 Jun 13;19(1):141. doi: 10.1186/s12872-019-1124-1.
Congenital extrahepatic portocaval shunt (CEPS), also known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension.
We report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient's condition improved in the first year after discharge. However, one year later, the patient's conditions start to deteriorate.
This article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography. This malformation had limited treatment and poor prognosis.
先天性肝外门腔静脉分流（CEPS），又称 Abernethy 畸形，是一种极为罕见的内脏静脉系统异常，特别是当伴有肺动脉高压时。
我们报告了一例 15 岁女性，被诊断为伴有肺动脉高压的 CEPS（Abernethy 型 Ib）。该病例是在腹部超声检查中偶然发现的，并通过肠系膜和脾动脉造影得到证实。在超过 4 年的随访中，患者在出院后第一年接受西地那非（80mg/天）治疗后病情有所改善。然而，一年后，患者的病情开始恶化。
本文报告了一例罕见的伴有肺动脉高压的 Abernethy 畸形病例，可通过腹部超声、门静脉 CT 血管造影或肠系膜和脾动脉造影进行诊断。这种畸形的治疗方法有限，预后不良。
