title: Lifelong management of patients with a single functional ventricle: a protocol.
abstract: Of the wide variety of congenital heart defects, the single functional ventricle continues to be one of the most enigmatic. Compared with patients who have complex congenital heart defects and 2 functional ventricles, patients with a single functional ventricle have greater surgical and long-term morbidity and mortality, and use more medical resources. Recent investigations have shown that patients with a single functional ventricle often have very satisfactory hemodynamics soon after a modified Fontan repair but then develop dilated cardiomyopathy during the subsequent decade. The cause and pathogenesis of the cardiomyopathy have not yet been determined. Many publications have examined individual aspects of the care of these patients based on retrospective reviews, but very few have provided comprehensive prospective methods for managing patients with a single functional ventricle. We hypothesized that a comprehensive management protocol with regular review of the results would provide a better understanding of the problems encountered in these patients and thus improve long-term outcome. Herein, we present our initial protocol for the lifelong management of patients with a single functional ventricle.
authors.affiliation: Lillie Frank Abercrombie Section of Pediatric Cardiology, Department of Pediatrics, Texas Children's Hospital, Houston 77030, USA.
authors.forename: D J
authors.initials: DJ
authors.lastname: Fisher
authors.forename: T
authors.initials: T
authors.lastname: Geva
authors.forename: T F
authors.initials: TF
authors.lastname: Feltes
authors.forename: F
authors.initials: F
authors.lastname: Cecchin
authors.forename: M R
authors.initials: MR
authors.lastname: Nihill
authors.forename: R
authors.initials: R
authors.lastname: Grifka
authors.forename: G J
authors.initials: GJ
authors.lastname: Reul
authors.forename: D A
authors.initials: DA
authors.lastname: Ott
mesh_terms: D002985:Clinical Protocols; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008134:Long-Term Care; D011182:Postoperative Care; D017410:Practice Guidelines as Topic; D011652:Pulmonary Circulation; D012307:Risk Factors; D014463:Ultrasonography