title: The use of the Berlin Heart EXCOR in patients with functional single ventricle.
abstract: BACKGROUND
  The frequency and successful use of pediatric ventricular assist devices (VADs) as a bridge to cardiac transplantation have been steadily increasing since 2003, but the experience in patients with complex congenital heart disease has not been well described. Using a large prospectively collected dataset of children supported with the Berlin Heart EXCOR VAD, we have reviewed the experience in children with single ventricular anatomy or physiology (SV), and compared the results with those supported with biventricular circulation (BV) over the same time period.
  
  
  METHODS
  The EXCOR Investigational Device Exemption study database was retrospectively reviewed. VAD implants under the primary cohort and compassionate use cohort between May 2007 and December 2011 were included in this review.
  
  
  RESULTS
  Twenty-six of 281 patients supported with a VAD were SV. The most common diagnosis was hypoplastic left heart syndrome (15 of 26). Nine patients were supported after neonatal palliative surgery (Blalock-Taussig shunt or Sano), 12 after a superior cavopulmonary connection (SCPC), and 5 after total cavopulmonary connection (TCPC). Two patients received biventricular assist devices, 1 after stage I surgery and 1 after stage II. SV patients were supported for a median time of 10.5 days (range, 1-363 days) versus 39 days (range, 0-435 days) for BV (P = .01). The ability to be bridged to transplant or recovery in SV patients is lower than for BV patients (11 of 26 [42.3%] vs 185 of 255 [72.5%]; P = .001). Three of 5 patients with TCPC were successfully bridged to transplant and were supported with 1 VAD. Seven of 12 patients with SCPC were bridged to transplant, and only 1 of 9 patients supported after a stage I procedure survived.
  
  
  CONCLUSIONS
  The EXCOR Pediatric VAD can provide a bridge to transplant for children with SV anatomy or physiology, albeit less successfully than in children with BV. In this small series, results are better in patients with SCPC and TCPC. VAD support for patients with shunted sources of pulmonary blood flow should be applied with caution.
authors.affiliation: Cardiothoracic and Vascular Surgery, Montefiore Medical Center, New York, NY. Electronic address: sweinste@montefiore.org.
authors.forename: Samuel
authors.initials: S
authors.lastname: Weinstein
authors.affiliation: Cardiothoracic and Vascular Surgery, Montefiore Medical Center, New York, NY.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Bello
authors.affiliation: Nemours Cardiac Center, Alfred I DuPont Hospital for Children, Wilmington, Del.
authors.forename: Christian
authors.initials: C
authors.lastname: Pizarro
authors.affiliation: Cardiac Surgery, Boston Childrens Hospital, Boston, Mass.
authors.forename: Francis
authors.initials: F
authors.lastname: Fynn-Thompson
authors.affiliation: Cardiothoracic Surgery, University of Alabama in Birmingham, Birmingham, Ala.
authors.forename: James
authors.initials: J
authors.lastname: Kirklin
authors.affiliation: Cardiac Surgery, Childrens Medical Center Dallas, Dallas, Tex.
authors.forename: Kristine
authors.initials: K
authors.lastname: Guleserian
authors.affiliation: Cardiothoracic Surgery, Childrens Hospital of Wisconsin, Milwaukee, Wis.
authors.forename: Ronald
authors.initials: R
authors.lastname: Woods
authors.affiliation: Berlin Heart Inc, The Woodlands, Tex.
authors.forename: Christine
authors.initials: C
authors.lastname: Tjossem
authors.affiliation: Berlin Heart Inc, The Woodlands, Tex.
authors.forename: Robert
authors.initials: R
authors.lastname: Kroslowitz
authors.affiliation: Cardiothoracic and Vascular Surgery, Montefiore Medical Center, New York, NY.
authors.forename: Patricia
authors.initials: P
authors.lastname: Friedmann
authors.affiliation: Cardiac Surgery, Duke University Medical Center, Durham, NC.
authors.forename: Robert
authors.initials: R
authors.lastname: Jaquiss
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D016009:Chi-Square Distribution; D057176:Compassionate Use Trials; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology; D006353:Heart-Assist Devices; D006801:Humans; D011474:Prosthesis Design; D011652:Pulmonary Circulation; D020127:Recovery of Function; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D016276:Ventricular Function
keywords: 1-ventricle anatomy and physiology; 2-ventricle anatomy and physiology; 20; 27; 34; BSA; BT; BV; BiVAD; Blalock-Taussig; CHD; ECMO; FDA; HLHS; IDE; Investigational Device Exemption; RVAD; SV; US Food and Drug Administration; VAD; biventricular assist device; body surface area; congenital heart disease; extracorporeal membrane oxygenation; hypoplastic left heart syndrome; right ventricular assist device; ventricular assist device