Congenital Portosystemic Shunts (CPSS), a rare malformation which can be treated

Congenital Portosystemic Shunts (CPSS) represent a rare vascular malformation that can have serious, wide-ranging health implications but is treatable with the right interventions. This informative video follows the journey of Charlotte, diagnosed at the age of six with CPSS, and explores the medical insights and solutions that transformed her life.

Congenital Portosystemic Shunts (CPSS), a rare malformation which can be treated

Congenital Portosystemic Shunts (CPSS) represent a rare vascular malformation that can have serious, wide-ranging health implications but is treatable with the right interventions. This informative video follows the journey of Charlotte, diagnosed at the age of six with CPSS, and explores the medical insights and solutions that transformed her life.

What Is CPSS?

In a healthy individual, blood from the intestines is filtered by the liver before continuing to the heart. However, in patients with CPSS, this filtration process is bypassed due to an abnormal connection between the portal and systemic blood vessels. There are two types of CPSS:

Extrahepatic Shunts: Blood flows directly from the intestines to the systemic circulation, bypassing the liver entirely.
Intrahepatic Shunts: A partial bypass occurs, with blood circumventing parts of the liver.

This condition can lead to a wide range of health complications at any age, including:

Liver tumors (benign or malignant)
Pulmonary vascular disorders like pulmonary hypertension
Neuropsychological and cognitive issues
Renal and endocrine disorders

Diagnosis and Treatment

Early diagnosis of CPSS is key. A simple abdominal ultrasound with Doppler can confirm the presence of a shunt. Charlotte’s diagnosis was confirmed through imaging, triggered by her declining health and the recognition of an unusual link between her lungs and liver by her cardiologist.

The video delves into the two main approaches to treating CPSS:

Surgical Closure – Closing the abnormal vessel through surgery.
Interventional Radiology – Using minimally invasive techniques to place a device that corrects the shunt.

Charlotte underwent a successful shunt closure procedure, resulting in a dramatic improvement in her oxygen saturation levels and overall health. Today, she enjoys a normal, active life, even participating in sports.

Why Watch This Video?

This video not only explains the science behind CPSS but also shares an inspiring story of hope and recovery. It serves as a critical reminder for medical professionals and families to consider CPSS when faced with puzzling symptoms such as liver nodules, neurocognitive decline, or unexplained pulmonary issues.

Take a moment to learn more about this rare condition and the life-changing treatments that can restore health and quality of life. Watch the full video to witness the impact of advanced medical care and the remarkable resilience of patients like Charlotte.