Table 3

Medical and Surgical Treatment Strategies for PLE After Fontan Operation

Alive
(n = 31)
Deceased (n = 11)Total Cohort (N = 42)
Medical therapies
 Heparin14 (45)7 (63)21 (50)
 Loop diuretics25 (81)10 (91)35 (83)
 Low-fat diet, MCT oil17 (55)11 (100)28 (67)
 ACE inhibitors, ARB23 (74)9 (81)32 (76)
 Spironolactone21 (67)5 (45)26 (62)
 Antiarrhythmic medications10 (32)5 (45)15 (36)
 Octreotide7 (23)1 (9)8 (19)
 Iron ± transfusion4 (13)6 (55)10 (24)
 Sildenafil6 (19)1 (9)7 (17)
 Steroids9 (29)3 (27)12 (29)
Surgical/interventional therapies
 Fontan fenestration16 (52)5 (45)21 (50)
 Fontan conversion7 (23)2 (18)9 (21)
 Fontan obstruction relief7 (23)2 (18)9 (21)
 Ablation therapy15 (48)8 (73)23 (55)
 Valve replacement, repair, or closure3 (10)1 (9)4 (10)

Values are n (%).

ACE = angiotensin-converting enzyme; ARB = angiotensin receptor blocker; MCT = medium chain triglycerides; other abbreviation as in Table 1.

  • Performed for atrial arrhythmias (7 cases) and obstruction (2 cases).