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Percutaneous vascular plug closure of persistent ductus venosus presenting with elevated liver enzyme in a 6-year-old infant: a case report

Published online by Cambridge University Press:  25 March 2026

Özlem BayramOpen the ORCID record for Özlem Bayram[Opens in a new window],
Mehmet Gökhan RamoğluOpen the ORCID record for Mehmet Gökhan Ramoğlu[Opens in a new window],
Emre Can Çelebioğlu,
Jeyhun Bakhtiyarzada,
Alperen Aydın,
Begum MurtOpen the ORCID record for Begum Murt[Opens in a new window],
Mehmet Mustafa Yilmaz,
Mehmet Sadık Bilgiç,
Suat Fitoz and
Tayfun Ucar
Özlem Bayram*
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
Mehmet Gökhan Ramoğlu
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
Emre Can Çelebioğlu
Affiliation:
Department of Radiology, Ankara University School of Medicine, Türkiye
Jeyhun Bakhtiyarzada
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
Alperen Aydın
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
Begum Murt
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
Mehmet Mustafa Yilmaz
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
Mehmet Sadık Bilgiç
Affiliation:
Department of Radiology, Ankara University School of Medicine, Türkiye
Suat Fitoz
Affiliation:
Department of Pediatric Radiology, Ankara University School of Medicine, Türkiye
Tayfun Ucar
Affiliation:
Department of Pediatric Cardiology, Ankara University School of Medicine, Türkiye
*
Corresponding author: Özlem Bayram; Email: drbayram.ozlem@gmail.com
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Abstract

The ductus venosus plays a critical role in fetal circulation. Postnatal persistence of the ductus venosus (PDV) is associated with secondary portal vein hypoplasia and results in a porto-systemic shunt. In this extremely rare case, we present a 6-year-old male diagnosed with PDV, who had a history of elevated liver enzymes and mild hepatic dysfunction first detected at 11 months of age. Due to the presence of PDV, endovascular closure was planned. Portal venous pressure was measured as 10 mmHg both before and after balloon occlusion. Percutaneous occlusion was successfully performed using a vascular plug. Post-intervention ultrasonography confirmed complete occlusion of the ductus venosus and increased portal vein flow. During follow-up, liver function tests returned to normal; a slight elevation in transaminases persists. PDV is a rare congenital vascular anomaly that may present with a wide spectrum of clinical symptoms. While some patients may be diagnosed during early infancy due to cholestatic jaundice, hepatic dysfunction, or hyperammonaemia, others may remain asymptomatic and undiagnosed for years. Endovascular closure represents a minimally invasive treatment option. A balloon occlusion test to measure portal venous pressure is recommended before and after the procedure to evaluate the safety of shunt closure.

Keywords

Ductus venosusporto-systemic shuntportal venous hypertensionpercutaneous occlusion

Information

Type
Case Report
Information
Cardiology in the Young , Volume 36 , Issue 3 , March 2026, pp. 648 - 652
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Copyright
© The Author(s), 2026. Published by Cambridge University Press

Introduction

During the fetal stage, the ductus venosus connects the umbilical vein to the inferior vena cava), and it is critical for fetal circulation. The postnatal persistence of the ductus venosus (PDV) in the postnatal period can lead to secondary portal vein hypoplasia and result in a porto-systemic shunt. Portal vein hypoplasia may be explained by reduced portal inflow, as blood is preferentially diverted through the low-resistance ductus venosus, thereby limiting the vascular stimulus required for normal portal vein development. Reference Papamichail, Pizanias and Heaton1

PDV has been associated with a wide range of clinical manifestations, including elevated liver enzymes, hyperammonemia, pulmonary arterial hypertension, and multi-organ failure during the postnatal period. Reference Jacob, Farr, De Vun, Takiff and Mason2,Reference Uchino, Endo, Ikeda, Shiraki, Sera and Matsuda3 Measuring portal venous pressure before and after temporary occlusion is essential in deciding whether definitive closure of the ductus venosus is safe and appropriate. Reference Ikeda, Yamaguchi, Sera, Ohshiro, Uchino and Ogawa4,Reference Chacko, Kock, Joshi, Mitchell and Ahmad5

In this extremely rare case, we present a 6-year-old male diagnosed with PDV and severe portal vein hypoplasia, who was successfully treated via percutaneous closure.

Case presentation

A six-year-old boy presented with a history of elevated liver enzymes and mild hepatic dysfunction first detected at 11 months of age during hospitalisation for bronchiolitis. On physical examination, the patient’s growth parameters were within normal limits for his age. Vital signs were stable, and cardiovascular and respiratory system examinations were unremarkable. No hepatomegaly or splenomegaly was noted. Laboratory parameters at the time of admission revealed mild direct hyperbilirubinemia (total bilirubin: 1.64 mg/dl; direct bilirubin: 0.87 mg/dl) and a marked elevation in liver enzymes (alanine aminotransferase: 426 U/L, aspartate aminotransferase [AST]: 834 U/L), while alkaline phosphatase and gamma-glutamyl transferase levels remained within normal limits. Additionally, serum albumin was low (2.9 g/dL), and prothrombin time/international normalised ratio was prolonged (1,47 sec), suggesting mild hepatic dysfunction (Figure 1). Viral hepatitis serologies, as well as cytomegalovirus and Epstein–Barr virus PCR tests, were negative. Alpha-fetoprotein and metabolic screenings (including blood and urine amino acid analysis and tandem mass spectrometry) were within normal limits. Abdominal ultrasonography and echocardiography were performed, and no abnormalities were identified.

Figure 1.

Liver enzymes and hepatic function parameters.

The patient was asymptomatic during the follow-up. Although liver enzyme levels gradually decreased, mild direct hyperbilirubinemia and prolonged PT persisted. Due to ongoing mild hepatic dysfunction and enzyme elevation, an abdominal ultrasonography was performed at 4.5 years of age, revealing a vascular structure measuring 4 mm in diameter and 25 mm in length, extending from the level of the portal vein bifurcation to the inferior vena cava, suggestive of a PDV. The patient was lost to follow-up for approximately 1.5 years. At 6 years of age, he was readmitted to our hospital with a diagnosis of PDV. On presentation, the patient had no symptoms, and the physical examination was unremarkable. Laboratory evaluation at our centre revealed elevated AST (54 U/L) and alkaline phosphatase (388 U/L), normal alanine aminotransferase (38 U/L) and gamma-glutamyl transferase (10 U/L), mild direct hyperbilirubinemia (total bilirubin: 1.23 mg/dl; direct bilirubin: 0.47 mg/dl), normal serum albumin (3.7 g/dL), and prolonged PT (14.5 sec). Hepatitis serologies were negative, and there was a small increase in ammonia (79 µmol/L). Due to ongoing suspicion of PDV, abdominal ultrasonography and computed tomography angiography were performed. Imaging revealed the following: the main portal vein was visible at the hepatic hilum, measuring 8 mm in diameter; the right and left portal branches were not opacified; and a portosystemic collateral vessel was identified, extending from the main portal vein to the middle hepatic vein, with a maximal diameter of 10 mm (Figure 2).

Figure 2.

( a ) Computed tomography angiography of a PDV in the axial and coronal planes, demonstrating its patency, and the intrahepatic portal vein branches are not opacified. ( b ) The white arrow is the DV in greyscale and Doppler ultrasonography. The black and white arrows indicate the DV.

An endovascular closure was planned due to the presence of a PDV. The procedure was performed under sedation, with systemic heparinisation and antibacterial prophylaxis administered. A 4F and a 5F sheath were inserted into the right jugular vein. A 5F MPA catheter was advanced through the jugular vein into the superior vena cava, right atrium, inferior vena cava, and ductus venosus. The ductus venosus and portal vein were selectively catheterised using a Cobra catheter. Angiographic evaluation revealed minimal flow within the intrahepatic portal branches, while the splenic and superior mesenteric veins showed normal flow and contrast filling. Pressure measurements were obtained from these sites. The portal vein pressure was measured at 10 mmHg using a reverse Berman catheter. No change in pressure was observed following balloon occlusion of the ductus venosus. Angiographic measurements showed a minimum diameter of 12 mm and a maximum diameter of 15 mm at the narrowest and widest portions of the ductus venosus, respectively. Using a superstiff exchange guidewire and a guiding catheter, the 5F sheath positioned in the right jugular vein was replaced with a steerable 9F introducer sheath. Considering that complete occlusion with the reverse Berman balloon may not have been achieved due to its maximum inflated diameter of 10 mm, a 3F microcatheter was advanced into the portal vein via the ductus venosus to reassess portal venous pressure after deployment of a 20 mm Cera™ Vascular Plug for definitive occlusion. During placement of the plug device at the origin of the portosystemic shunt, portal venous pressure was measured by microcatheter as 10 mmHg. The device was finally deployed after confirming the portal venous pressure and withdrawing the microcatheter (Figure 3). A control angiogram showed a marked decrease in shunt flow, accompanied by improved opacification of the portal veins. Aspirin was initiated as anticoagulant therapy and continued for six months. Follow-up ultrasonography demonstrated complete occlusion of the shunt with increased portal vein flow (Figure 4). Liver function tests returned to within normal limits; however, a mild elevation in transaminase levels persisted one month after percutaneous occlusion. Regrettably, the patient did not attend for the scheduled follow-up; therefore, subsequent laboratory values could not be evaluated.

Figure 3.

Portal venous pressure measurement using a microcatheter after ductus venosus occlusion.

Figure 4.

Angiography in the right jugular vein in posterior–anterior view, demonstrating the ductus venosus ( a ). Angiography after vascular plug occlusion; the black and red arrows indicate the DV ( b ). Greyscale and Doppler ultrasonography showing the PDV following endovascular closure, with the vascular plug. The white arrow indicates the vascular plug in the DV ( c ). Improved perfusion of the intrahepatic portal veins is observed, with no residual flow through the DV ( d ).

Discussion

The ductus venosus is crucial for fetal circulation; however, its persistence in the postnatal period may lead to serious complications. Affected individuals may present with a wide spectrum of clinical manifestations. Symptoms often develop during early infancy due to portal vein hypoplasia and the presence of a porto-systemic shunt. However, in some cases, the condition may remain undiagnosed for a long time due to the absence of clinical symptoms.

We reported a case of a six-year-old boy with PDV, who presented with a history of elevated liver enzymes and mild hepatic dysfunction, initially indicated at 11 months. Due to the rarity of PDV, the available literature primarily consists of case reports. Most reported patients are diagnosed in the neonatal period or early infancy, often presenting with hyperbilirubinemia, cholestasis, or hyperammonemia. Reference Kardos, Kitzmueller, Olejnik and Behnke6,Reference Kamali, Moradi, Ebrahimian, Esfahani and Jafarpishe7 Children and adults who remain undiagnosed during their asymptomatic period may later present with complications related to porto-systemic shunt, such as hepatic encephalopathy, pulmonary hypertension, or hepatopulmonary syndrome. Reference Kamali, Moradi, Ebrahimian, Esfahani and Jafarpishe7,Reference Subramanian, Krishnamoorthy, Sivasubramonian and Sasidharan8

There are two types of congenital portosystemic venous shunts: intrahepatic and extrahepatic. The pathogenesis and treatment of the two types are different. Since PDV originates in the left portal vein, it is classified as an intrahepatic shunt, specifically type 5. Reference Papamichail, Pizanias and Heaton1 The treatment approach for congenital portosystemic venous shunts depends largely on the developmental status of the portal venous system. In cases where the portal venous system is absent, liver transplantation remains the only treatment option. However, in patients with portal vein hypoplasia, shunt closure may be feasible and beneficial. According to recent data, measurement of the portal venous pressure during angiography with balloon occlusion testing is critical for treatment planning. Reference Kardos, Kitzmueller, Olejnik and Behnke6,Reference Subramanian, Krishnamoorthy, Sivasubramonian and Sasidharan8 The normal range of portal venous pressure is between 5 and 10 mmHg and should not exceed 20 mmHg during the shunt occlusion. Reference Franchi-Abella, Branchereau, Lambert, Fabre, Steimberg and Losay9

We planned endovascular closure for our case. A reverse Berman™ catheter was used to perform a test occlusion of the ductus venosus, and portal venous pressure was measured at 10 mmHg both before and after balloon occlusion. Also, a microcatheter placed into the portal vein was used for pressure measurement after occlusion of the ductus venosus by the device. Percutaneous occlusion was carried out using a 20 mm Cera vascular plug. Following the procedure, complete occlusion of the shunt was achieved. The procedure was completed without any intra- or post-procedural complications. Post-procedural ultrasonography revealed increased portal venous flow. This finding was considered to have resulted from the dilation of previously collapsed intrahepatic portal veins due to the increased flow. At the one-month follow-up, pulmonary artery pressure and right heart dimensions were within normal limits, and there were no signs of portal hypertension. Recent studies have shown that following interventional closure of PDV, patients typically experience normalisation of liver function and ammonia levels, increased portal venous flow on ultrasound and CT imaging, and no development of portal hypertension or pulmonary hypertension. Reference Kardos, Kitzmueller, Olejnik and Behnke6,Reference Kamali, Moradi, Ebrahimian, Esfahani and Jafarpishe7 Several case reports have described that, when portal hypertension is demonstrated during balloon occlusion testing in patients with porto-systemic shunt, shunt management may involve the use of fenestrated vascular plugs to allow partial decompression or a multistage interventional strategy to facilitate safe and definitive occlusion. Facas et al. reported a multistage closure approach in a patient with an extrahepatic porto-systemic shunt, which allowed gradual adaptation to elevated portal pressures. Reference Facas, Cruz, Costa, Agostinho and Donato10 Similarly, Rao et al. described the use of a modified vascular plug permitting partial shunt flow, thereby providing a safer option for shunt closure in the presence of portal hypertension. Reference Rao, Karuppasamy, Radhakrishnan and Fagan11

In conclusion, PDV is a rare congenital vascular anomaly with a broad spectrum of clinical presentations. If left undiagnosed, it may lead to serious portosystemic shunt-related complications, including hepatic encephalopathy, pulmonary hypertension, and hepatopulmonary syndrome. Awareness of PDV as a potential cause of unexplained liver dysfunction is crucial, and early diagnosis and timely intervention are essential to prevent long-term complications. Endovascular closure represents a minimally invasive and effective therapeutic option. However, prior to permanent closure, a balloon occlusion test should be performed to evaluate portal venous pressure and determine the safety of the procedure.

Acknowledgements

None.

Financial support

This research received no specific grant from any funding agency, commercial or not-for-profit sectors.

Competing interests

None.

References

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Figure 0

Figure 1.Liver enzymes and hepatic function parameters.

Figure 1

Figure 2.(a) Computed tomography angiography of a PDV in the axial and coronal planes, demonstrating its patency, and the intrahepatic portal vein branches are not opacified. (b) The white arrow is the DV in greyscale and Doppler ultrasonography. The black and white arrows indicate the DV.

Figure 2

Figure 3.Portal venous pressure measurement using a microcatheter after ductus venosus occlusion.

Figure 3

Figure 4.Angiography in the right jugular vein in posterior–anterior view, demonstrating the ductus venosus (a). Angiography after vascular plug occlusion; the black and red arrows indicate the DV (b). Greyscale and Doppler ultrasonography showing the PDV following endovascular closure, with the vascular plug. The white arrow indicates the vascular plug in the DV (c). Improved perfusion of the intrahepatic portal veins is observed, with no residual flow through the DV (d).