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Percutaneous Stenting Of Fontan Conduit To Treat Protein Losing Enteropathy In An Adolescent With Complex Congenital Heart Disease

A 15-year-old boy who underwent a fenestrated Fontan operation for complex single ventricle congenital heart disease 2 years ago presented with body edema and right ankle joint pain. Fontan operation is a procedure where deoxygenated blood from vena cavae is ‘passively’ directed to lungs by means of a conduit/prosthetic tube (instead of the right ventricle ‘pumping’ blood to lungs as occurs in a normal heart). He has past history of intermittent body edema with hypoalbuminemia for the past one year. A diagnosis of protein-losing enteropathy (PLE) was made which was being managed medically.

Current evaluation showed massive right pleural effusion, reactive arthritis of ankle joint and albumin level of 0.8 mg/dL. A right chest tube drained 1500 ml of non-chylous fluid which was left in situ. Despite intense medical management for PLE with high protein diet, diuretics, ACE inhibitors, albumin infusions, steroids, warfarin and octreotide injections, his albumin levels remained low and his chest tube drainage continued.

Hemodynamic assessment in the cardiac catheterization lab revealed normal Fontan conduit (FC) / pulmonary artery pressures and no angiographic abnormalities. The fenestration in the FC was very tiny without any significant right to left shunting. A coronary wire was passed with difficulty across this tiny opening and dilated using coronary balloons (3mm diameter followed by 5 mm). Following this, a 6 mm x 15 mm stent (Rx Herculink Elite) was deployed across the fenestration. Angiography showed good right to left shunt. Procedure was uncomplicated. One week after the stent procedure, chest tube drainage began to decrease, his albumin increased to 1.8 gm/dl. The tube was removed 2 weeks after stenting and patient discharged home successfully.

Protein Losing Enteropathy

PLE is a complex disorder characterized by enteric protein loss due to primary gastrointestinal abnormality or secondary to cardiac disease. The reported prevalence of PLE in Fontan patients is 3.7% to 24% and is associated with significant morbidity and mortality. Because the etiology of PLE is largely unknown, the methods of management are arbitrary and medical treatment is often empiric, difficult and temporary. Creation of fenestration in the FC or enlarging an existing one rarely improves PLE, likely due to decrease in venous presure. Otherwise, cardiac transplantation is the last resort.

Dr. Kavitha Chintala, MD, FACC (Consultant Pediatric Cardiologist)
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