title: Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome.
abstract: OBJECTIVE
  We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.
  
  
  METHODS
  From January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children's Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis-aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan-Meier method was used to assess survival.
  
  
  RESULTS
  Thirty-eight (23%) of 165 patients had mitral stenosis-aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis-aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle-subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis-aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate.
  
  
  CONCLUSIONS
  With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis-aortic atresia variant and left ventricle-subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.
authors.affiliation: Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115, USA.
authors.forename: Vladimiro L
authors.initials: VL
authors.lastname: Vida
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.forename: Alesandro
authors.initials: A
authors.lastname: Larrazabal
authors.forename: Kimberly
authors.initials: K
authors.lastname: Gauvreau
authors.forename: Adam L
authors.initials: AL
authors.lastname: Dorfman
authors.forename: Gerald
authors.initials: G
authors.lastname: Marx
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
authors.forename: Audrey C
authors.initials: AC
authors.lastname: Marshall
authors.forename: Frank A
authors.initials: FA
authors.lastname: Pigula
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: Francis
authors.initials: F
authors.lastname: Fynn-Thompson
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D001011:Aorta / Q000002:abnormalities*; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality*; D002423:Cause of Death; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality* / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D008946:Mitral Valve Stenosis / Q000601:surgery; D010166:Palliative Care / Q000379:methods; D011336:Probability; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis; D016896:Treatment Outcome