title: Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome.
abstract: We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.
authors.affiliation: Division of Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. emilie.jean-st-michel@sickkids.ca.|Department of Paediatrics, University of Toronto, Toronto, ON, Canada. emilie.jean-st-michel@sickkids.ca.
authors.forename: Emilie
authors.identifier: http://orcid.org/0000-0003-1725-2215
authors.initials: E
authors.lastname: Jean-St-Michel
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.|Department of Surgery, University of Toronto, Toronto, ON, Canada.
authors.forename: Devin
authors.initials: D
authors.lastname: Chetan
authors.affiliation: Department of Paediatrics, University of Toronto, Toronto, ON, Canada.|Department of Critical Care Medicine, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Schwartz
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.|Department of Surgery, University of Toronto, Toronto, ON, Canada.
authors.forename: Glen S
authors.initials: GS
authors.lastname: Van Arsdell
authors.affiliation: Department of Paediatrics, University of Toronto, Toronto, ON, Canada.|Department of Critical Care Medicine, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Alejandro A
authors.initials: AA
authors.lastname: Floh
authors.affiliation: Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.|Department of Surgery, University of Toronto, Toronto, ON, Canada.
authors.forename: Osami
authors.initials: O
authors.lastname: Honjo
authors.affiliation: Division of Pediatric Cardiology, Stollery Children's Hospital, Edmonton, AB, Canada.|Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Conway
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006085:Graft Survival; D006333:Heart Failure / Q000209:etiology / Q000628:therapy*; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications* / Q000628:therapy*; D008297:Male; D058327:Norwood Procedures; D009864:Ontario; D010166:Palliative Care; D063868:Patient Outcome Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D018754:Ventricular Dysfunction / Q000209:etiology / Q000628:therapy*
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors
keywords: Heart failure; Hybrid procedure; Hypoplastic left heart syndrome; Interstage outcomes; Norwood procedure