[Tricuspid valve stenosis--an serious complication to Port-a-cath]

Area of Science:

• Cardiology
• Pediatric Rheumatology
• Medical Devices

Background:

• Port-a-cath (central venous access device) is crucial for long-term therapies, including in pediatric patients with autoimmune diseases like Systemic Lupus Erythematosus (SLE).
• Cyclophosphamide is an immunosuppressive agent often used in managing SLE.
• Complications associated with central venous catheters can be severe and impact multiple organ systems.

Observation:

• A pediatric patient with SLE developed symptoms 5-7 years post-Port-a-cath insertion and cyclophosphamide treatment.
• Symptoms included hepatomegaly, abdominal distension, and venous distension, indicative of significant circulatory compromise.
• Intraoperative findings revealed the Port-a-cath positioned adjacent to and through the tricuspid valve, causing severe stenosis and thrombus formation.

Findings:

• The Port-a-cath was directly implicated in severe tricuspid valve stenosis and superior vena cava (SVC) occlusion.
• Surgical intervention involved catheter and thrombus removal, tricuspid valve repair (commissurotomy, bicuspidization), chordeal replacement, and SVC repair with a pericardial patch.
• Retrospective imaging confirmed the catheter's proximity to the tricuspid valve and its tip within the right ventricle.

Implications:

• This case highlights the potential for delayed, severe cardiac complications from long-term Port-a-cath use, even years after insertion.
• Early detection and removal of malpositioned or problematic catheters are critical.
• Limiting the indwelling duration of central venous catheters, particularly in immunocompromised patients with autoimmune conditions, is advised to mitigate risks.