title: Percutaneous valvulation of failing Fontan: rationale, acute effects and follow-up.
abstract: BACKGROUND
  Fontan circulation is fragile and unfavourable evolution is frequent. Fontan physiology largely depends on respiration and gravity. The hypothesis for valvulation is that valvulation of the circuit reduces the effects of respiration and the proclive position, and increases anterograde flow towards the systemic circulation, with increasing exercise capacity and benefits for enteropathy. Because it originates from the bovine jugular vein, the Melody(®) valve (Medtronic, Minneapolis, MN, USA) is naturally designed to work in a low-pressure environment.
  
  
  OBJECTIVE
  To report our experience of percutaneous valvulation of refractory failing Fontan circulation.
  
  
  METHODS
  We reviewed all patients who received a Melody valve in Fontan circulation in our unit.
  
  
  RESULTS
  Four patients were included: two had severe and refractory protein-losing enteropathy; one had severe oedema and ascites; and one had very severe lower limb venous insufficiency. The Melody valve was successfully implanted in all patients. Central venous pressure and inferior vena cava pressure did not change after valvulation. There were no early complications. At follow-up, no acute or mid-term thrombosis was noted. Two patients had intracardiac echocardiography 6 and 24 months after valvulation: the Melody valve was found not to be functioning in both cases. One patient died 3 months after valvulation; the cause was unrelated to the procedure.
  
  
  CONCLUSIONS
  Percutaneous valvulation of Fontan circulation is technically feasible. More clinical studies are needed before considering this treatment as an option.
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker-Enfants-Malades, Cardiologie Pédiatrique, 149, rue de Sèvres, 75015 Paris cedex, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Malekzadeh-Milani
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker-Enfants-Malades, Cardiologie Pédiatrique, 149, rue de Sèvres, 75015 Paris cedex, France.
authors.forename: Magalie
authors.initials: M
authors.lastname: Ladouceur
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker-Enfants-Malades, Cardiologie Pédiatrique, 149, rue de Sèvres, 75015 Paris cedex, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Iserin
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker-Enfants-Malades, Cardiologie Pédiatrique, 149, rue de Sèvres, 75015 Paris cedex, France; Hôpital Européen Georges-Pompidou, Assistance Publique des Hopitaux de Paris, Paris, France. Electronic address: younes.boudjemline@nck.aphp.fr.
authors.forename: Younes
authors.initials: Y
authors.lastname: Boudjemline
mesh_terms: D000293:Adolescent; D000328:Adult; D001705:Bioprosthesis; D006328:Cardiac Catheterization / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods; D006351:Heart Valves / Q000503:physiopathology; D006439:Hemodynamics; D006801:Humans; D008297:Male; D011474:Prosthesis Design; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Cardiopathies congénitales; Congenital heart diseases; Défaillance du Fontan; Failing Fontan; Fontan; Fontan circulation; Melody valve; Percutaneous valve implantation; Valve Mélody; Valvulation percutanée