Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report

• Published in: BMC cardiovascular disorders 2019-06, Vol.19 (1), p.141-5, Article 141
• Main Authors: Lin, Kai-Yang, Chen, Hui, Yu, Ling
• Format: Article
• Language: English
• Subjects: Abdominal ultrasonography; Abernethy malformation; Adolescent; Angiography; Antihypertensive Agents - therapeutic use; Arterial Pressure - drug effects; Arteriography; Case Report; Case reports; CAT scans; Catheters; Computed tomography; Congenital extrahepatic portocaval shunt; Diagnosis; Diagnostic imaging; Disease Progression; Electrocardiography; Female; Females; Genetic disorders; Humans; Hypertension; Incidental Findings; Liver; Medical imaging; Portal vein; Portal Vein - abnormalities; Portal Vein - diagnostic imaging; Portal Vein - physiopathology; Pulmonary arterial hypertension; Pulmonary Arterial Hypertension - diagnosis; Pulmonary Arterial Hypertension - drug therapy; Pulmonary Arterial Hypertension - etiology; Pulmonary Arterial Hypertension - physiopathology; Pulmonary arteries; Pulmonary Artery - drug effects; Pulmonary Artery - physiopathology; Pulmonary hypertension; Risk factors; Sildenafil; Sildenafil Citrate - therapeutic use; Spleen; Tomography; Transplants & implants; Treatment Outcome; Ultrasonic imaging; Ultrasound; Ultrasound imaging; Vascular Malformations - complications; Vascular Malformations - diagnostic imaging; Vascular Malformations - physiopathology; Vasodilator Agents - therapeutic use; Veins & arteries; Vena Cava, Inferior - abnormalities; Vena Cava, Inferior - diagnostic imaging; Vena Cava, Inferior - physiopathology
• ISSN: 1471-2261; 1471-2261
• 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.