title: Intermediate-Term Results After Extracardiac Conduit Fontan Palliation in Children and Young Adults with Single Ventricle Physiology-A Single-center Experience.
abstract: The Fontan operation and its modifications are currently the standard palliation in patients with single ventricle physiology. The advantages of extracardiac conduit Fontan operation (ECF) have previously been described. We evaluated the outcome of patients who underwent ECF operation at our center since the year 2000 to assess morbidity, mortality and New York Heart Association (NYHA) functional class in the current era. Medical records of 97 patients who underwent ECF operation from November 2000 till October 2013 were reviewed to determine the diagnosis, age at surgery, surgical technique, postoperative complications, NYHA class at last follow-up, echocardiographic parameters, and most recent Holter monitor results. The median age at surgery was 3.4 years with 4 total deaths until last follow-up (hospital mortality was 1 %, and mortality after hospital discharge was 3 %). Seventy-eight of the 97 patients (80 %) underwent fenestrated Fontan surgery. Median duration of follow-up was 3.9 years (IQR 0.2-12.2 years). At the latest follow-up, 62 patients (67 %) were determined to be in NYHA class I, 25 patients (27 %) in NYHA class II, and 6 (6 %) patients in NYHA class III, and the median oxygen saturation was 92 % (IQR 86-96). There were 15 patients with history of preoperative arrhythmias and 6 of them required pacemaker placement at the time of surgery. One patient developed ectopic atrial tachycardia, and 1 patient had clinical thromboembolism on follow-up. Systolic function of the single ventricle by transthoracic echocardiogram at the latest follow-up was described as normal in 77 patients (83 %), fair in 15 patients (16 %), and poor in 1 patient (1 %). The results of our study show that patients who undergo extracardiac conduit Fontan operation in the modern era may have significantly less morbidity and mortality when compared to earlier years.
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Heart Center, Holtz Children's Hospital at the University of Miami, Miller School of Medicine/Jackson Memorial Medical Center, 1611 NW 12th Avenue, P.O Box 016960 (R-76), Miami, FL, 33101, USA. shashi-raj@uiowa.edu.
authors.forename: Shashi
authors.initials: S
authors.lastname: Raj
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Children's Heart Center, Holtz Children's Hospital at the University of Miami, Miller School of Medicine/Jackson Memorial Medical Center, Miami, FL, USA.
authors.forename: Eliot
authors.initials: E
authors.lastname: Rosenkranz
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Children's Heart Center, Holtz Children's Hospital at the University of Miami, Miller School of Medicine/Jackson Memorial Medical Center, Miami, FL, USA.
authors.forename: Barbara
authors.initials: B
authors.lastname: Sears
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Heart Center, Holtz Children's Hospital at the University of Miami, Miller School of Medicine/Jackson Memorial Medical Center, 1611 NW 12th Avenue, P.O Box 016960 (R-76), Miami, FL, 33101, USA.
authors.forename: Sethuraman
authors.initials: S
authors.lastname: Swaminathan
mesh_terms: D000293:Adolescent; D001145:Arrhythmias, Cardiac; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital; D006352:Heart Ventricles; D006801:Humans; D016896:Treatment Outcome; D055815:Young Adult
keywords: Extracardiac conduit; Fontan; Protein-losing enteropathy; Single ventricle