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Endovascular Closure Resolves Trimethylaminuria Caused by Congenital Portosystemic Shunts
María Dolores Ponce-Dorrego1, Gonzalo Garzón-Moll1
1Interventional Radiologist, Hospital General Universitario La Paz, Madrid, Spain.
Pediatric Gastroenterology, Hepatology & Nutrition
|November 29, 2019
Summary
Congenital portosystemic shunts (CPSS) can be associated with trimethylaminuria (TMAU). Endovascular closure of CPSS effectively resolved TMAU symptoms in most patients, offering a potential treatment for this rare metabolic disorder.
Area of Science:
- Hepatology
- Metabolic Disorders
- Vascular Surgery
Background:
- Congenital portosystemic shunts (CPSS) are rare vascular anomalies.
- Trimethylaminuria (TMAU) is a metabolic disorder characterized by the accumulation of trimethylamine.
- The association between CPSS and TMAU is infrequently reported.
Observation:
- Three patients with CPSS presented with clinical symptoms of TMAU.
- Urine analysis confirmed elevated trimethylamine (TMA) and TMA n-oxide levels in these patients.
- Percutaneous endovascular shunt closure was performed for CPSS management.
Findings:
- Two out of three patients experienced complete resolution of TMAU symptoms and normalization of urine parameters one year post-procedure.
- One patient showed no improvement, developing new portosystemic communications after treatment.
- Endovascular closure demonstrated efficacy in resolving TMAU linked to CPSS.
Implications:
- Endovascular closure of CPSS may be an effective therapeutic strategy for associated TMAU.
- This study highlights the importance of screening for TMAU in patients with CPSS.
- Further research is warranted to understand the pathophysiology and long-term outcomes.
