Surreal illustration of infant surgery complicated by preduodenal portal vein.

Hidden Danger: How a Rare Vein Anomaly Complicates Infant Surgery

Nico Varela in Health & Wellness November 2025 • 4 min read.

"Discover how preduodenal portal vein (PDPV) impacts heterotaxy syndrome treatment and what surgeons need to know."

Heterotaxy syndrome (HS) is a complex condition characterized by a wide array of cardiovascular and other malformations. These can include pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and a preduodenal portal vein (PDPV). The combination of these issues can create significant challenges for infants requiring surgical intervention.

One particularly rare complication involves a preduodenal portal vein (PDPV), where the portal vein passes in front of the duodenum instead of behind it. This unusual positioning can lead to duodenal obstruction and, as highlighted in a recent case report, can cause life-threatening complications during surgery, especially when combined with infracardiac TAPVC.

This article explores a case report detailing the intricate perioperative management of an infant with heterotaxy syndrome, an infracardiac TAPVC, and a preduodenal portal vein. The case underscores the critical need for surgeons and anesthesiologists to be aware of PDPV's presence and potential impact to avoid catastrophic pulmonary venous obstruction (PVO) during surgery.

What is Preduodenal Portal Vein (PDPV) and Why Does It Matter?

Surreal illustration of infant surgery complicated by preduodenal portal vein.

A preduodenal portal vein (PDPV) is a rare congenital anomaly where the portal vein, which carries blood from the intestines to the liver, is located in front of the duodenum (the first part of the small intestine) rather than behind it. This abnormal positioning can cause several issues:

PDPV is often associated with other congenital anomalies, particularly heterotaxy syndrome and situs inversus (a condition where organs are mirrored from their normal position). The presence of PDPV can complicate surgical procedures, especially in infants with other complex conditions.

Duodenal Obstruction: The PDPV can compress the duodenum, leading to partial or complete obstruction, causing feeding problems and requiring surgical intervention.
Surgical Challenges: During surgery for other conditions (like intestinal malrotation), the PDPV can be inadvertently injured or compressed, leading to severe complications.
Pulmonary Venous Obstruction (PVO): In cases where the TAPVC drains into the PDPV, surgical manipulation can cause PVO, a life-threatening condition where blood flow from the lungs is blocked.

The case report highlights an instance where an infant with heterotaxy syndrome, infracardiac TAPVC, and PDPV experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction. The surgical team discovered that the dilated PDPV was compressing the duodenum. Manipulation of the PDPV during the procedure led to pulmonary venous obstruction, causing severe drops in blood pressure and oxygen levels.

Why Awareness Matters for Future Pediatric Care

This case underscores the critical importance of awareness and careful preoperative planning when dealing with infants with heterotaxy syndrome. Surgeons and anesthesiologists must consider the possibility of PDPV, especially when intestinal malrotation and duodenal obstruction are present. Detailed imaging and a thorough understanding of the patient's unique anatomy are essential to avoid catastrophic complications during surgery.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

This article is based on research published under:

DOI-LINK: 10.1186/s40981-018-0154-5, Alternate LINK

Title: Atypical Perioperative Management For Duodenal Obstruction In An Infant With Heterotaxy Syndrome: A Case Report

Subject: Anesthesiology and Pain Medicine

Journal: JA Clinical Reports

Publisher: Springer Science and Business Media LLC

Authors: Hirofumi Obinata, Shinichi Nishibe, Yoko Ishihara

Published: 2018-02-12

Everything You Need To Know

What is a preduodenal portal vein (PDPV) and why is it considered a rare congenital anomaly?

A preduodenal portal vein (PDPV) is a rare congenital anomaly where the portal vein, which normally carries blood from the intestines to the liver, is located in front of the duodenum instead of behind it. Its rarity stems from the typical anatomical arrangement where the portal vein passes behind the duodenum. This abnormal positioning can cause duodenal obstruction and significantly complicates surgical procedures, especially in infants with conditions like heterotaxy syndrome. The atypical positioning presents potential risks during surgical interventions, making awareness and careful preoperative planning crucial.

How does a preduodenal portal vein (PDPV) complicate the treatment of heterotaxy syndrome?

A preduodenal portal vein (PDPV) complicates the treatment of heterotaxy syndrome because it can lead to duodenal obstruction, causing feeding problems and necessitating surgical intervention. During surgery for related conditions like intestinal malrotation or total anomalous pulmonary venous connection (TAPVC), the PDPV can be inadvertently injured or compressed, leading to severe complications such as pulmonary venous obstruction (PVO). The combination of heterotaxy syndrome's complex cardiovascular and other malformations with a PDPV requires careful preoperative planning and detailed imaging to avoid life-threatening complications.

What is heterotaxy syndrome and what other conditions are commonly associated with it?

Heterotaxy syndrome is a complex condition characterized by a wide array of cardiovascular and other malformations, indicating that the body's organs are abnormally arranged across the left-right axis. Commonly associated conditions include pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and a preduodenal portal vein (PDPV). The combination of these issues presents significant challenges for infants requiring surgical intervention, necessitating a comprehensive understanding of the patient's unique anatomy.

In the context of infant surgery, how can a preduodenal portal vein (PDPV) lead to pulmonary venous obstruction (PVO) and what are the implications?

A preduodenal portal vein (PDPV) can lead to pulmonary venous obstruction (PVO) during infant surgery, particularly when a total anomalous pulmonary venous connection (TAPVC) drains into the PDPV. Surgical manipulation in the area can compress the PDPV, obstructing blood flow from the lungs and causing PVO. This is a life-threatening condition characterized by severe drops in blood pressure and oxygen levels. The implications are dire, requiring immediate recognition and correction to prevent irreversible damage or death. Detailed preoperative imaging and awareness of the PDPV's presence are critical to avoid this catastrophic complication.

Why is awareness of preduodenal portal vein (PDPV) critical for surgeons and anesthesiologists treating infants with heterotaxy syndrome, intestinal malrotation, and duodenal obstruction?

Awareness of preduodenal portal vein (PDPV) is critical because its presence can significantly complicate surgical procedures and lead to life-threatening complications such as pulmonary venous obstruction (PVO). Surgeons and anesthesiologists must consider the possibility of PDPV, especially when intestinal malrotation and duodenal obstruction are present in infants with heterotaxy syndrome. Detailed imaging and a thorough understanding of the patient's unique anatomy are essential to avoid inadvertent injury or compression of the PDPV during surgery, preventing catastrophic drops in blood pressure and oxygen levels. The case highlights that careful preoperative planning and vigilance can improve outcomes.