Patent Ductus Venosus Research Articles

Canine intrahepatic portosystemic shunts: Interlobar and intralobar classifications.

This two-part study design showed that a canine congenital intrahepatic portosystemic shunt (IPSS) may be classified by its location within a liver fissure (interlobar) or lobe (intralobar). A prospective anatomic study reviewed normal canine liver morphology and showed the CT angiography (CTA) appearance of the normal canine ductus venosus (DV), which was confirmed via dissection and literature review to be between the papillary process and left-lateral liver lobe (in the fissure for ligamentum venosum). A retrospective multi-institutional case series documented the frequency of imaging findings in 56 dogs with a single IPSS that underwent portal CTA at Cornell University or the Schwarzman Animal Medical Center between June 2008 and August 2022. An interlobar IPSS was seen in 24 of 56 (43%) dogs, all arose from the left portal branch except one. These shunts were typically near the median plane, remained interlobar throughout the course, and were nearly always (96%) craniodorsal to the porta hepatis. Four types were distinguished: patent DV (11 dogs), left interlobar (11 dogs), right interlobar (1 dog), and ventral interlobar (1 dog). Only about half (46%) were in the fissure for ligamentum venosum and therefore classified as a patent DV. An intralobar IPSS was seen in 32 of 56 (57%) dogs, most (88%) originated from the right portal branch and were in the right-lateral liver lobe (21 dogs) or caudate process (7 dogs). During canine portal CTA, documenting the interlobar or intralobar location of an IPSS might increase the consistency and validity of IPSS description.

Patent Ductus Venosus in an early term infant with mild respiratory distress – Case report

Patent ductus venosus is an extremely rare form of congenital portosystemic shunt that results in the diversion of portal blood into the systemic circulation, decreasing hepatic blood flow and increasing blood volume and toxic substances in the systemic circulation. We present a clinical case of a male infant born at 37 5/7 weeks gestational age with patent ductus venosus. Because very few cases of patent ductus venosus have been reported, the prudent approach it would be to perform serial Doppler studies and laboratory tests to follow the course of the ductus venosus until closure and to intervene if the infant becomes unstable and symptomatic. The medical team decided that the conservative management in this case was a better choice, since the infant remained stable with good growth.

Clinical findings, diagnosis and therapy of patent ductus venosus in children: a case series.

Patent ductus venosus (PDV) is a rare form of congenital portosystemic shunt. Because of the diversity of clinical symptoms and insufficient knowledge of this condition, clinicians often fail to perform targeted examinations, resulting in missed diagnoses and misdiagnoses. This study summarized the clinical and radiological findings, as well as surgical methods, of PDV with the aim of improving early diagnosis and guiding treatment. Clinical, laboratory, and radiologic data of patients with PDV were analyzed retrospectively. In all, 9 patients with PDV were included in the study (7 male, 2 female; median age 1.6 years, age range 16 days to 16.5 years). Data for all 9 patients with PDV were reviewed. The most common initial clinical presentations were jaundice and respiratory symptoms. Laboratory data revealed hypoxemia in 5 patients, hyperammonemia in 2, hyperbilirubinemia in 7, abnormal coagulation function in 6, abnormal myocardial enzymes in 4, hepatic dysfunction in 8, and renal dysfunction in 3. The direct imaging sign of PDV was a vascular structure connecting the left branch of the portal vein (LPV) to the inferior vena cava. Secondary imaging findings observed in all 9 patients were dilated right heart, pulmonary artery, and LPV, and an atrophic right branch of the portal vein. The main portal vein was dilated in 8 patients and shrunk in 1. Moreover, 8 patients had enlarged livers, and 3 presented with hypoperfusion in the right lobe of the liver. The spleen was enlarged in 8 patients but shrunk in 1. Renal imaging was abnormal in 2 patients. Hepatic encephalopathy was found in 4 patients; 7 patients had PDV combined with other malformations, with congenital heart disease and vascular abnormalities being the most common; 3 patients successfully underwent surgical ligation of PDV. PDV can lead to multisystem damage. Secondary radiological signs of PDV play an important role in early diagnosis and preoperative evaluation. Complications and coexisting malformations were common and should not be missed during preoperative evaluation. Early surgical closure for PDV is recommended.

Stenting of ductus venosus as a palliation for portal hypertension because of neonatal cirrhosis.

Portal hypertension because of liver cirrhosis is a significant cause of morbidity and mortality. Treatment options in these patients include liver transplant, symptomatic treatment of oesophageal varices via endoscopic treatment and symptomatic management of ascites. Portosystemic shunt creation can be challenging in newborns and infants. We present a newborn with Trisomy 21, severe portal hypertension secondary to neonatal cirrhosis, oesophageal varices and upper GI bleeding, severe ascites refractory to medical management who underwent ductus venosus stenting as a palliative procedure. He demonstrated remarkable clinical improvement with no subsequent upper GI bleed and resolution of ascites till his last follow-up at 12months of age. To the best of our knowledge, stenting of the patent ductus venosus to create a portosystemic shunt to relieve portal hypertension has not been reported in English literature.

Hepatosplenomegaly and Periventricular Cyst in a Neonate with Direct Hyperbilirubinemia

Hepatosplenomegaly and Periventricular Cyst in a Neonate with Direct Hyperbilirubinemia

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

We present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses towards biliary cirrhosis, end-stage liver failure and death by age 3. Differentiation of biliary atresia from other nonsurgical causes of neonatal cholestasis is challenging because there is no single method for diagnosing biliary atresia, and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. Concerning imaging, our systematic literature review shows that ultrasonography is the main tool for pre- and neonatal diagnosis. Key prenatal features, when present, are non-visualisation of the gallbladder, cyst in the liver hilum, heterotaxy syndrome and irregular gallbladder walls. Postnatal imaging features have a very high specificity when present, but a variable sensitivity. Triangular cord sign and abnormal gallbladder have the highest sensitivity and specificity. The presence of macro- or microcyst or polysplenia syndrome is highly specific but less sensitive. The diameter of the hepatic artery and hepatic subcapsular flow are less reliable. When present in the context of acholic stools, dilated intrahepatic bile ducts rule out biliary atresia. Importantly, a normal US exam does not rule out biliary atresia. Signs of chronic hepatopathy and portal hypertension (portosystemic derivations such as patent ductus venosus, recanalised umbilical vein, splenomegaly and ascites) should be actively identified for - but are not specific for - biliary atresia.

Ligation of patent ductus venosus in a child with pulmonary arterial hypertension and hypersplenism

Introduction:Patent ductus venosus (PDV) is a rare and critical disease, and the majority of patients present with pulmonary arterial hypertension (PAH) or hepatopulmonary syndrome due to congenital portosystemic shunt. We reported that both PAH and hypersplenism were major complications of PDV in this case. This case report can assist the treatment and recovery of the patients with similar symptoms.Patient concerns:A 4-year-old male patient presented to our institution with a history of recurrent respiratory infections accompanied by leukocytopenia, thrombocytopenia and presented with tachypnoea. upon mild exertion.Diagnosis:A wide communication, 10 mm in diameter, between the portal vein and inferior vena cava was identified in the subcostal echocardiogram and computed tomography images. Echocardiography showed an estimated systolic pulmonary artery pressure of 106 mm Hg. Right-sided cardiac catheterization indicated a mean pulmonary arterial pressure of 30 mm Hg and a pulmonary vascular resistance of 3 Wood units. Chest X-ray revealed cardiomegaly with a prominent pulmonary segment.Interventions:The patient was treated with combination pharmacotherapy of bosentan and tadalafil and PDV ligation.Outcomes:A year later, the boy showed normal exercise tolerance and weight gain. Liver and spleen parameters, liver function, blood cells and the general condition of the boy improved.Conclusion:Initial combination therapy of bosentan and tadalafil is safe and effective in children with PAH associated with PDV. When PDV banding test shows normal portal pressure, PDV ligation is considered acceptable in children with PAH and hypersplenism associated with PDV.

Effects of patent ductus venosus on bile acid homeostasis in aryl hydrocarbon receptor (AhR)-null mice

The Aryl hydrocarbon receptor (AhR) is primarily known as one of the xenosensors and regulators of drug-metabolizing genes. Bile acids (BAs) are synthesized in the liver, and undergo several enterohepatic recirculations in which the liver removes BAs from the portal blood, minimizing the BAs that spill over into the systemic circulation. Previous studies revealed a lifelong patent ductus venosus (PDV) in AhR-null mice. Increased concentration of total BAs (Σ-BAs) in AhR-null mice is known; however, the impact of PDV on BA homeostasis in liver and bile remains unclear. This work investigated the consequences of PDV on BA homeostasis by comparing AhR-null and wild-type (WT) mice of both genders.In serum, Σ-BAs were markedly higher (64–85-fold) in AhR-null mice than in WT mice, especially due to the increase of tri-OH primary BAs (86–142-fold). Despite the extremely high concentration of serum BAs, the concentration of BAs in livers of AhR-null mice remained similar to WT mice. AhR-null livers were protected against increased BA influx by downregulation of uptake transporters and BA synthetic enzymes in the alternative pathway. Although livers of AhR-null mice are 20–25% smaller than WT mice, biliary excretion of BAs was maintained in the AhR-null mice, and even tended to increase. Surprisingly, intestinal Fgf15 expression was not increased, even though there was a marked increase in serum BA concentrations. Although PDV resulted in extremely high BA concentrations in serum of AhR-null mice, they maintained a concentration of BAs in liver and biliary excretion of BAs similar to control mice.

Patent ductus venosus and exercise related pulmonary hypertension: a case of a young adult with successful surgery closure

The patent ductus venosus is an embryological portosystemic shunt that connects the umbilical vein to the inferior vena cava and it can be diagnosed incidentally or in subjects suffering from hepatic encephalopathy, hypoxemia or hypoglycaemia. Sometimes it can be found in patients with cardiac defects or hypoxia caused by pulmonary arteriovenous shunting.Case presentationA 34-year-old male patient was referred to our medical centre for further evaluation of abdominal pain and moderate exertional dyspnoea. An exercise stress echocardiogram was performed in order to understand the mechanism of the exertional dyspnoea. The test was interrupted due to dyspnoea and desaturation and an estimated pulmonary pressure value of 65 mmHg was detected. Exercise pulmonary hypertension (PH) seems to represent the hemodynamic manifestation of early pulmonary vascular disease, acting as a possible transitional phase anticipating resting PH.An MRI of the abdomen showed the presence of a portosystemic shunt from a patent ductus venosus, associated with stenosis of the celiac tripod artery. A CT scan, of the pulmonary circulation, showed a normal pulmonary venous return,mediastinal vessel a normal pulmonary artery. Subsequently, taking into consideration the large size (> 25 mm) of the duct, treatment (closure) of the patent ductus venosus with the help of a detachable vascular plug device was not feasible and open surgery rather than a percutaneous invasive approach would be advisable.ConclusionPersistent ductus venous can lead to pulmonary arteriovenous shunt fistula and exercise related pulmonary hypertension. Percutaneous or surgical closure requires detailed planning and an anatomical and physiological evaluations.

Investigation of Clinical Safety of Human iPS Cell-Derived Liver Organoid Transplantation to Infantile Patients in Porcine Model

Transplantation of liver organoids has been investigated as a treatment alternative to liver transplantation for chronic liver disease. Transportal approach can be considered as a method of delivering organoids to the liver. It is important to set the allowable organoid amount and verify translocation by intraportal transplantation. We first examined the transplantation tolerance and translocation of porcine fetal liver-derived allogeneic organoids using piglets. Fetal liver-derived organoids generated from the Kusabira Orange-transduced pig were transplanted to the 10-day-old piglet liver through the left branch of the portal vein. All recipients survived without any observable adverse events. In contrast, both local and main portal pressures increased transiently during transplantation. In necropsy samples, Kusabira Orange-positive donor cells were detected primarily in the target lobe of the liver and partly in other areas, including the lungs and brain. As we confirmed the transplantation allowance by porcine fetal liver-derived organoids, we performed intraportal transplantation of human-induced pluripotent stem cell (iPSC)-derived liver organoid, which we plan to use in clinical trials, and portal pressure and translocation were investigated. Human iPSC-derived liver organoids were transplanted into the same 10-day-old piglet. Portal hypertension and translocation of human iPSC-derived liver organoids to the lungs were observed in one of two transplanted animals. Translocation occurred in the piglet in which patent ductus venosus (PDV) was observed. Therefore, a 28-day-old piglet capable of surgically ligating PDV was used, and after the PDV was ligated, human iPSC-derived liver organoids with the amount of which is scheduled in clinical trials were transplanted. This procedure inhibited the translocation of human iPSC-derived liver organoids to extrahepatic sites without no portal hypertension. In conclusion, human iPSC-derived liver organoids can be safely transplanted through the portal vein. Ligation of the ductus venosus prior to transplantation was effective in inhibiting extrahepatic translocation in newborns and infants.

Laparoscopic ligation of a congenital extrahepatic portosystemic shunt for children with hyperammonemia: a single-institution experience.

A congenital extrahepatic portosystemic shunt (CEPS) associated with hyperammonemia requires occlusion of the shunt vessels. We evaluated the effectiveness and safety of laparoscopic ligation of a CEPS in children with hyperammonemia. The subjects of this retrospective study were seven children with hyperammonemia who underwent laparoscopic ligation of a CEPS. Their median age was 5.2years (range 1-16years). Before the laparoscopic procedure, a catheter was inserted through the femoral vein and placed in the portal vein via the shunt vessel. The shunt vessel was dissected and taped laparoscopically. After measuring the portal vein pressure under temporal occlusion, the shunt vessels were ligated. The types of shunts according to location were patent ductus venosus (n = 2), splenorenal shunt (n = 2), gastrorenal shunt (n = 2), and superior mesenteric vein-inferior vena cava shunt (n = 1). Laparoscopic ligation of the shunt vessel was completed uneventfully in all patients. The median portal vein pressure was 19mmHg after ligation. The median preoperative blood ammonia level was 94µg/dL (range 71-259µg/dL), which decreased after ligation in all patients. There was no incidence of postoperative liver failure. Laparoscopic ligation of a CEPS is safe and effective for children with hyperammonemia.

An adult case of primary biliary cholangitis accompanied by a patent ductus venosus showing type-I congenital absence of the portal vein

An adult case of primary biliary cholangitis accompanied by a patent ductus venosus showing type-I congenital absence of the portal vein

Endovascular Treatment of Congenital Arterioportal Fistulas.

To characterize anatomy of congenital arterioportal fistulas (CAPF) and correlate this with technique and outcomes of transcatheter embolization (TCE). Retrospective review was conducted of children with CAPF that underwent TCE in a 10-year period. Medical records, imaging and procedure details were reviewed. TCE was performed via transarterial (n = 5), portal (n = 5) or patent ductus venosus (n = 1) approach. Embolic agents used were coils (n = 10), Onyx (n = 1) and Amplatzer septal occluder (n = 1). A total of 7 patients were included (4 female). Median age at treatment was 4months (2month-3year). Most common symptoms were GI bleeding (n = 4), anemia (n = 4) and ascites (n = 3). Hepatopetal flow was seen in all on pre-procedure sonography. On angiography, a simple lesion, with direct fistulous connections, from hepatic arterial (HA) branches to portal venous (PV) system was seen in 3. A complex lesion with multiple connections was identified in 4. In simple lesions, cessation of shunting was achieved in 1 procedure, with embolization of afferent vessels. In complex lesions, multiple procedures were required in 3 of 4 patients. All 4 with complex connections required embolization of the aneurysmal PV segment. There were no major complications. Two minor complications were noted: localized biliary leak in 1 and femoral pseudoaneurysm in 1. There was resolution of symptoms in 6. TCE is effective as primary treatment of CAPF. Patients with simple arteriovenous connections can be treated with embolization of afferent vessels, but children with multiple complex connections usually require embolization of the dilated PV segment.

Congenital Vascular Malformations of the Liver: an Association With Trisomy 21.

A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2). Group (ii) (n = 4): Involvement of all 3 vascular systems. Two infants had arterioportal hypertension caused by hepatic arteries feeding into a left portal vein aneurysm within the umbilical fissure. Two infants had more complex hepatic artery to hepatic vein shunts developing early cardiorespiratory failure with progressive jaundice. Our series shows a clear association between cardiac anomalies and CVM in children with trisomy 21.

Congenital Vascular Malformations of the Liver: An Association With Trisomy 21.

A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2). Group (ii) (n = 4): Involvement of all 3 vascular systems. Two infants had arterioportal hypertension caused by hepatic arteries feeding into a left portal vein aneurysm within the umbilical fissure. Two infants had more complex hepatic artery to hepatic vein shunts developing early cardiorespiratory failure with progressive jaundice. Our series shows a clear association between cardiac anomalies and CVM in children with trisomy 21.

Fetal Intra-abdominal Umbilical Vein Varix Complicated with Patent Ductus Venosus and Atrial Septal Defect

Umbilical vein varix has diverse clinical features and an unpredictable course during the pregnancy and/or perinatal period. We report a rare case of isolated fetal varix of the intra-abdominal umbilical vein, which was associated with fetal cardiomegaly. After birth, the umbilical vein varix remained with continuous blood flow through the patent ductus venosus. In addition, persistent cardiomegaly was complicated with an atrial septal defect.

Shunts porto-systémiques congénitaux : expérience du Grand-Ouest sur cinq ans

Congenital portosystemic shunts (CPS) are rare vascular malformations that lead to liver metabolic modifications. The aim of this study was to describe the management and outcome of CPS in children who were followed in the western region of France and to compare our experience to a literature review. This retrospective study reports nine children with CPS who were followed in the western region of France between 2008 and 2013. Among these nine children, six were detected by prenatal ultrasonography, one had cholestatic jaundice during the postoperative period, one had cholestatic jaundice during the first days of life, and one had dyspnea and fatigue. Two children had liver tumors. The CPS was intrahepatic for four children and extrahepatic for two children. Three others had a patent ductus venosus. Among the intrahepatic shunts that were discovered before the age of 2 years, three closed spontaneously. The five other shunts were closed surgically or by interventional radiology techniques, after angiography for four children. After closure of eight shunts, the outcome was favorable for six children. Two children presented complications after surgery: portal hypertension and portal thrombus. When CPS is suspected, the diagnosis should be confirmed and complications should be investigated. Shunt closure restores portal flow and prevents complications.

Loss of Nrf2 in mice evokes a congenital intrahepatic shunt that alters hepatic oxygen and protein expression gradients and toxicity.

The transcription factor Nrf2 (Nfe2l2 nuclear factor, erythroid 2-like 2) regulates gene expression directly, controlling pharmacological and toxicological responses. These processes may also be influenced by the structure of the hepatic vasculature, which distributes blood flow through compartmentalized microenvironments to maintain organismal stability. Castings of the hepatic portal vasculature of albino C57BL/6J but not ICR Nrf2(-/-) mice revealed a congenital intrahepatic shunt that was present in two thirds of Nrf2-disrupted mice. This shunt directly connected the portal vein to the inferior vena cava and displayed characteristics of a patent ductus venosus. Immunohistochemistry revealed that Nrf2(-/-) mice with an intrahepatic shunt manifest changes to hepatic oxygen and protein expression gradients when compared with wild-type (WT) and non-shunted Nrf2(-/-) mice. Centrilobular hypoxia found in WT and Nrf2(-/-) mice without shunts was reduced in Nrf2(-/-) livers with a shunt. Hepatic protein expression of phosphoenolpyruvate carboxykinase (Pepck), normally confined to the periportal zone, exhibited both periportal and centrilobular zonal expression in livers from Nrf2(-/-) mice with an intrahepatic shunt. Centrilobular expression of Cytochrome P450 2E1 (Cyp2e1) was diminished in shunted Nrf2(-/-) livers compared with WT and Nrf2(-/-) livers without shunts. The intrahepatic shunt in Nrf2(-/-) mice was further found to diminish acetaminophen hepatoxicity compared with WT and Nrf2(-/-) non-shunted mice following a 6 h challenge with 250 mg/kg acetaminophen. The presence of an intrahepatic shunt influences several physiological and pathophysiological properties of Nrf2(-/-) mice through changes in blood flow, hepatic oxygenation, and protein expression that extent beyond loss of canonical transactivation of Nrf2 target genes.

Congenital Portosystemic Shunts in Children

To propose an anatomical classification of congenital portosystemic shunts (CPSs) correlating with conservative surgery. CPSs entail a risk of life-threatening complications because of poor portal inflow, which may be prevented or cured by their closure. Current classifications based on portal origin of the shunt are not helpful for planning conservative surgery. Twenty-three patients who underwent at least 1 surgical procedure to close the CPSs were included in this retrospective study (1997-2012). We designed a classification according to the ending of the shunt in the caval system. We analyzed the results and outcomes of surgery according to this classification. Two patients had an extrahepatic portosystemic shunt, 17 had a portacaval shunt [subdivided in 5 end-to-side-like portal-caval, 7 side-to-side-like portal-caval, and 5 H-shaped (H-type portal-caval)], 2 had portal-to-hepatic vein shunts (portohepatic), and 2 had a persistent ductus venosus. All extrahepatic portosystemic shunts, H-type portal-caval, portohepatic, and patent ductus venosus patients had a successful 1-stage ligation. All 5 end-to-side-like portal-caval patients had a threadlike intrahepatic portal venous system; a 2-stage complete closure was successfully achieved for 4 and a partial closure for 1. The first 2 side-to-side-like portal-caval patients had a successful 2-stage closure whereas the 5 others had a 1-stage longitudinal caval partition. All patients are alive and none needed a liver transplantation. Our classification correlates the anatomy of CPSs and the surgical strategy: outcomes are good provided end-to-side-like portal-caval shunts patients have a 2-stage closure, side-to-side portal-caval shunts patients have a 1-stage caval partition, and the others have a 1-stage ligation.

Umbilical Vein Catheterization

* Abbreviations: CDC, : Centers for Disease Control and Prevention CVC, : central venous catheter DV, : ductus venosus UVC, : umbilical venous catheter Please watch the video below depicting resistance to the passage of an umbilical venous catheter. Think first, then answer the question below.⇓ Figure. Click here to view the video. (Reproduced with permission of Akshaya Vachharajani, MD, and The Saigh Foundation Pediatric Simulation Center, St. Louis Children's Hospital & Washington University School of Medicine. Copyright 2012.) How long can an umbilical venous catheter be used without risk of blood stream infection? 1. 1 week 2. 5 days 3. 4 weeks 4. 14 days 5. 10 days Time out is not recorded on the video to protect the identity of the neonate. The video depicts the resistance to the passage of the catheter. A momentary resistance was frequently but not always felt as the catheter tip entered the ductus venosus (DV) in a study by Dunn. (1) A study revealed that all 50 (100%) of the neonates examined by ultrasound had a patent DV on day 1. (2) Only 33 (66%) of these neonates had a patent DV at 5 days of age. The resistance felt is most probably due to the anatomy and not DV closure. This is because the DV connects the …