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Stents in the Successful Management of Protein-Losing
Enteropathy after Fontan
Protein-losing enteropathy (PLE) is a serious, and if not treated, fatal complication of the Fontan-cavopulmonary anastomosis procedure.1 It has been suggested that creation of a fenestration may prevent2 and treat3,4 PLE in this setup. Other therapeutic modalities have included the use of heparin,5 spironolactone,6 steroids and angiotensin-converting enzyme inhibitors with variable success.6 Transcatheter interventional procedures have been thought to be beneficial in the treatment of PLE, but experience in this modality is limited.4
We report rapid reversal of PLE following stents therapy to relieve recurrent coarctation of the aorta and left pulmonary artery stenosis in a child who underwent the Fontan procedure for hypoplastic left heart syndrome.
Case Presentation. A female patient with hypoplastic left heart syndrome underwent a modified Norwood procedure at 1 week of age (July 2000). At the age of 4 months, she presented with heart failure, and on catheterization, she was found to have a severe coarctation of the aorta and stenosis of the innominate, left carotid and left subclavian arteries. She received a bidirectional Glenn shunt and homograft patch repair of the coarctation of the aorta. She had bilateral chylothorax postoperation, which was managed with a low-fat diet using Tolerex® (Novartis Nutrition Corp., Minneapolis, Minnesota) and diuretics. Later, a recurrence of the coarctation of the aorta, stenosis of all three branches of the brachiocephalic vessels and left pulmonary artery stenosis prompted the following procedures, with transient relief:
• June 2000: Cardiac catheterization; immediate good results after balloon angioplasty of the transverse aortic arch, left subclavian artery and left pulmonary artery stenoses.
• August 2003: Transient relief after balloon angioplasty of recurrent coarctation of the aorta.
• September 2003: Cardiac catheterization with coil embolization of collaterals and temporary successful balloon dilatation of recoarctation of the aorta.
• December 2003: Homograft patch angioplasty for recurrent left pulmonary artery stenosis.
• November 2004: Cardiac catheterization with transient relief after balloon angioplasty of recoarctation of the aorta.
In April 2005 the patient underwent an extracardiac Fontan procedure using a 20 mm Gore-Tex tube with a 6 mm fenestration. She developed chylothorax requiring treatment with chest tube drainage, total parental nutrition, heparin, oxygen, furosemide, spironolactone, steroids and albumin transfusions. When her drainage subsided, she was sent home on a low-fat diet. Five months later, she presented with anasarca and hepatomegaly due to PLE, as evidenced by the following lab results: albumin 1.7 gm/dL; total protein 3.6 gm/dL. She had normal BUN, creatine and electrolytes, and no evidence of protein loss in the urine. Her alpha-1 antitrypsin stool concentration was significantly elevated (15.2 mg/g; normal 0–1.9 mg/g). Despite continued treatment with digoxin, spironolactone, furosemide and captopril, there was no change in her serum protein levels. Her echocardiogram and computerized tomography scan with angiography suggested a recurrence of the left pulmonary artery stenosis and coarctation of the aorta. Cardiac catheterization (December 2005, age 5 years) showed a patent fenestration and the presence of severe, long-segment aortic coarctation (Figure 1A) and a long-segment left pulmonary artery stenosis (Figure 2A). There was a 36 mmHg pressure gradient across the coarctation and a 5 mmHg mean pressure difference across the left pulmonary artery stenosis with an elevated mean caval pressure (18 mmHg). We elected to enlarge the coarcted segment so that its inner diameter would be similar to that of the aorta at the level of the diaphragm. After heparinization with 100 U/kg, a Palmaz-Genesis stent (Cordis Corp., Miami Lakes, Florida), a 27 mm stent was successfully deployed over a BIB (Numed, Inc., Hopkins, New York) with an outer diameter of 10 mm. Angiography in the aortic arch demonstrated disappearance of the coarctation (Figure 1B). Pullback pressure across the stent demonstrated a 6 mmHg residual gradient. The stent in the left pulmonary artery was chosen to cover the length of the stenotic area and to be expanded to thewidest portion of the vessel at its attachment to the conduit. A 17 mm Palmaz-Genesis stent was deployed successfully using a BIB catheter (outer balloon 8 mm). Repeat angiography demonstrated a much wider-caliber left pulmonary artery and no residual narrowing. There was no pressure gradient between the distal left pulmonary artery and the superior vena cava on pullback. The mean pressure in the cavopulmonary connections dropped from 18 to 14 mmHg, despite significant contrast load. There was no change in the caval saturation (51%).
After discharge from the hospital, the patient began to show obvious clinical improvement over a period of 5 months. The anasarca resolved totally, and her liver size became normal. Her serial lab results showed gradual but complete recovery from PLE. Her most recent lab readings are as follows: total protein 7.9 gm/dL; albumin 5.1 gm/dL; repeat alpha– 1– antitrypsin stool concentration 2 mg/g. One year after the stent intervention, she remained free of edema and was off all diuretics. Her echocardiogram is consistent with sustained relief of her coarctation and left pulmonary artery stenosis. The fenestration remained patent, resulting in an oxygen saturation of 85%.
Discussion. This report, to our knowledge, describes the first successful management of PLE complicating a Fontan procedure with the use of stent therapy to relieve residual obstructive lesions. Correction of the abnormal hemodynamics resulted in a reduction of the patient’s caval pressure, which was paramount in resolving her PLE. It has been reported that fenestration of the Fontan will prevent and treat PLE.2–4 In our patient, the fenestration alone did not prevent PLE because of the residual obstructions in the aorta and left pulmonary artery. Likewise, aggressive management with many therapeutic agents which have been reported to help in the management of PLE5,6 was unsuccessful. However, after successful transcatheter intervention and relief of these obstructions, the patient’s PLE was resolved. The combination of the fenestration and medical therapy after relief of the obstruction was most likely key in reversing our patient’s PLE.
Our patient had recurrent coarctation and left pulmonary artery stenosis in addition to multiple stenoses in her brachiocephalic arteries. This may represent an arteriopathy associated with hypoplastic left heart syndrome.
We recommend transcatheter intervention with stenting to relieve any significant residual obstructions after the Fontan procedure, which may prevent or treat PLE.
References
1. Kwok LSK, Cheung YF, Yung TC, et al. Protein Losing Enteropathy after Fontan procedure. Hong Kong J Pediatr 2002;7:85–91.
2. Rychik J, Rome JJ, Jacobs ML. Late surgical fenestration for complication after the Fontan operation. Circulation 1997;96:33–36.
3. Jacobs ML, Rychik J, Byrum CJ, Norwood WI Jr. Protein losing enteropathy after Fontan operation: Resolution after baffle fenestration. Ann Thor Surg 1996;61:206–208.
4. Warnes CA, Feldt RH, Hagler DJ. Protein losing enteropathy after the Fontan operation: Successful treatment by percutaneous fenestration of the atrial septum. Mayo Clin Proc 1996;71:378–379.
5. Donnelly JP, Rosenthal A, Castle VP, Holme RD. Reversal of protein losing enteropathy with heparin therapy in three patients with Univentricular hearts and Fontan palliation. J Pediatr 1997;130:474–478.
6. Ringel RE, Peddy SB. Effects of high dose spironalactone on protein losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol 2003;91:1031–1032.
