title: Outcomes of infants undergoing superior cavopulmonary connection in the presence of ventricular dysfunction.
abstract: Outcomes in patients with ventricular dysfunction undergoing superior cavopulmonary connection (SCPC) are not well known. We reviewed records of patients undergoing SCPC at our center from December 2005 to October 2009 and studied those whose pre-SCPC echocardiograms demonstrated at least moderate systemic ventricular dysfunction. Of the 213 patients undergoing SCPC, 19 (9%) met inclusion criteria. Diagnoses were hypoplastic left heart syndrome (n = 18) and rightward unbalanced atrioventricular canal with pulmonary stenosis (n = 1). In those surviving >2 months after SCPC, ventricular function was assessed by echocardiography 4.9 (range 3.5 to 9.7) months after SCPC and was improved in ten of 17 (59%), unchanged in six of 17 (35%), and worsened in one of 17 (16%) patients. After SCPC, three patients died, and one underwent heart transplant 21.9 months after SCPC. Transplant-free survival was attained by 15 of 19 (79%) patients during follow-up of 33.0 months (range 10.8 to 51.4). Fontan completion was performed on six survivors on reaching an appropriate age. Ventricular dysfunction before SCPC is not uncommon and occurs primarily in patients with a morphologic right ventricle. Ventricular performance improves in the majority of patients after SCPC, and midterm outcomes are comparable with previous reports, suggesting that these patients remain candidates for staged palliation.
authors.affiliation: Division of Cardiology, Department of Pediatrics, University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104-4399, USA. mjoconnor@uams.edu
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: O'Connor
authors.forename: Matthew D
authors.initials: MD
authors.lastname: Elias
authors.forename: Meryl S
authors.initials: MS
authors.lastname: Cohen
authors.forename: Michael D
authors.initials: MD
authors.lastname: Quartermain
mesh_terms: D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods* / Q000401:mortality; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D014683:Vena Cava, Superior / Q000503:physiopathology / Q000601:surgery*; D018754:Ventricular Dysfunction / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016278:Ventricular Function, Right
references.citation: J Am Soc Echocardiogr. 2007 Dec;20(12):1364-73
references.pmid: 17604955
references.citation: J Thorac Cardiovasc Surg. 2007 Jul;134(1):82-9, 89.e1-2
references.pmid: 17599490
references.citation: J Cardiol. 1995 Dec;26(6):357-66
references.pmid: 8558414
references.citation: Am J Cardiol. 2000 Nov 15;86(10):1106-10
references.pmid: 11074208
references.citation: J Thorac Cardiovasc Surg. 2010 Sep;140(3):545-9
references.pmid: 20723724
references.citation: Ann Thorac Surg. 1998 Feb;65(2):503-8
references.pmid: 9485254
references.citation: Ann Thorac Surg. 1997 Dec;64(6):1795-802
references.pmid: 9436575
references.citation: J Am Soc Echocardiogr. 1998 Sep;11(9):849-56
references.pmid: 9758376
references.citation: Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2010;13(1):91-5
references.pmid: 20307869
references.citation: Curr Opin Cardiol. 2009 Mar;24(2):113-8
references.pmid: 19225294
references.citation: J Thorac Cardiovasc Surg. 2008 Nov;136(5):1237-42
references.pmid: 19026809
references.citation: Ann Thorac Surg. 2008 Sep;86(3):869-74; discussion 869-74
references.pmid: 18721575
references.citation: Eur J Cardiothorac Surg. 2010 Jul;38(1):59-65
references.pmid: 20206539
references.citation: J Am Coll Cardiol. 2009 Jul 7;54(2):160-5
references.pmid: 19573734
references.citation: Eur J Cardiothorac Surg. 2003 Oct;24(4):502-10; discussion 510
references.pmid: 14500066
references.citation: Circulation. 2004 Nov 16;110(20):3229-33
references.pmid: 15533860
references.citation: Eur J Cardiothorac Surg. 2007 Mar;31(3):344-52; discussion 353
references.pmid: 17236782
references.citation: Nat Rev Cardiol. 2010 Oct;7(10):551-63
references.pmid: 20697412
references.citation: Cardiol Young. 2010 Dec;20 Suppl 3:107-12
references.pmid: 21087566
references.citation: JACC Cardiovasc Imaging. 2011 Feb;4(2):128-37
references.pmid: 21329896
references.citation: J Am Coll Cardiol. 1997 May;29(6):1365-70
references.pmid: 9137237
references.citation: Am J Cardiol. 2009 Aug 1;104(3):419-28
references.pmid: 19616678
references.citation: Am J Cardiol. 2000 Nov 1;86(9):964-8
references.pmid: 11053708
references.citation: J Am Soc Echocardiogr. 2010 Jul;23(7):685-713; quiz 786-8
references.pmid: 20620859
references.citation: Cardiol Young. 2006 Dec;16(6):556-62
references.pmid: 17116269
references.citation: Echocardiography. 2010 Nov;27(10):1247-55
references.pmid: 20584069