Portal hypertension is defined by the hepatic venous pressure gradient and is measured
via a percutaneous transhepatic route [1]. We describe a novel technique for direct portal pressure measurement using endoscopic
ultrasound (EUS).
A 27-year-old man with Noonan syndrome and congenital heart disease presented with
recurrent gastrointestinal bleeding. EUS revealed an extensive network of periduodenal
vessels that were attributed to congestive hepatopathy. However, bleeding continued
despite cardiac surgery. Angiography failed to visualize these vessels, but the gastroduodenal
artery and adjacent aberrant vessel were embolized prophylactically ([Fig. 1]). Clinically significant bleeding continued leading to EUS-guided insertion of 20
coils into the periduodenal vessels. EUS Doppler demonstrated both arterial and venous
components ([Fig. 2]). Follow-up imaging revealed a marked improvement of the vascular malformations
([Fig. 3 a, b]) with only occult, but transfusion-requiring bleeding remaining. EUS was repeated
with only one vessel large enough to allow coiling.
Fig. 1 A 27-year-old man with Noonan syndrome and congenital heart disease presented with
recurrent gastrointestinal bleeding, which continued despite cardiac surgery. a Mesenteric angiography demonstrating the gastroduodenal artery (orange arrow), a
replaced right hepatic artery (yellow arrow), and a branch communicating the replaced
right hepatic artery to the gastroduodenal artery (green arrow). b Given that the periduodenal vessels could not be visualized, coils were prophylactically
placed in the gastroduodenal artery (orange arrow) and communicating branch (green
arrow).
Fig. 2 a, b Endoscopic ultrasound (EUS)-guided coiling with insertion of a total of 20 coils
into multiple vascular networks in the 2nd and 3rd portion of the duodenum. c EUS reveals the periduodenal vessels with both an arterial and venous component and
a low resistance, high flow waveform.
Fig. 3 a Routine endoscopy revealed multiple duodenal serpiginous folds before therapy. b Their appearance following EUS-guided coil insertion therapy.
Given continued uncertainty with regard to the prior hepatic venous pressure gradient
and diagnosis, EUS was used to re-measure the portal pressure. The portal vein was
accessed using a 22-gauge fine needle aspiration (FNA) needle (Wilson-Cook Medical
Inc., Winston-Salem, North Carolina, United States). Portal blood was aspirated through
the needle, which was connected to an arterial pressure catheter ([Fig. 4 a, b], [Fig. 5 a]). After calibration, the portal pressure measured 11 mmHg, thereby excluding significant
portal hypertension. The middle hepatic vein pressure was then measured at 10 mmHg
([Fig. 4 c, d], [Fig. 5 b]), confirming a 1 mmHg pressure gradient as recorded by interventional radiology.
There was no evidence of bleeding and the hemoglobin was stable 4 days later.
Fig. 4 a EUS demonstrates the portal vein with power Doppler imaging. b Fine needle aspiration (FNA) needle position during pressure monitoring.
Fig. 5 a Fluoroscopy demonstrating the echoendoscope position during pressure monitoring of
the portal vein. b Fluoroscopy demonstrating the echoendoscope position during pressure monitoring of
the middle hepatic vein. c EUS demonstrates the middle hepatic vein with power Doppler imaging. d FNA needle position during pressure monitoring.
Prior EUS-guided portal pressure measurements in porcine models correlated with percutaneous
measurements [2]
[3]
[4]. This is the first clinical report demonstrating the feasibility and apparent safety
of portal vein and hepatic vein pressure measurements by EUS, thereby allowing diagnosis
of arteriovenous malformations as have been reported in Noonan syndrome [5].
Endoscopy_UCTN_Code_TTT_1AS_2AC
