title: Current outcomes of hypoplastic left heart syndrome with restrictive atrial septum: a single-center experience.
abstract: Advances in the management of hypoplastic left heart syndrome (HLHS) have resulted in improved survival. However, short and long-term mortality in patients with a restrictive atrial septum remains high. All neonates diagnosed with HLHS from 2003 to 2010 at our institution were evaluated. Patients who underwent atrial septostomy within the first 72 h conformed the restrictive atrial septum group (HLHS-RS). Patients with a non-restrictive communication (HLHS-NRS) formed the control group. Outcomes and survival status were determined from review of medical records. Of the 141 newborns diagnosed with HLHS, 20 (14 %) required intervention for a restrictive atrial septum. Procedural success was achieved in 17/20 (85 %) patients. Complications occurred in ten procedures, two of which were life threatening. No procedural deaths occurred. Overall median follow up was 35.5 months (0.4-104). Initial hospitalization survival was 16/20 (80 %) for the HLHS-RS group and 114/121(94 %) for the HLHS-NRS (p = 0.028). Twenty (14 %) patients were lost to follow up and 9 (6 %) underwent heart transplant. Overall survival was 10/16 (62 %) for HLHSRS patients and 77/95 (81 %) for HLHS-NRS (p = 0.1). Survival after initial discharge was 10/12 (83 %) for the HLHS-RS group and 77/88 (87 %) for the HLHS-NRS (p = 0.67). No predictors for HLHS-RS outcome were identified. Mortality at first-stage palliation in HLHS neonates with a restrictive atrial septum remains higher than in those with an unrestrictive communication. However, survival after initial hospital discharge is similar.
authors: Division of Pediatric Cardiology, Morgan Stanley Children's Hospital of New York-Presbyterian, 3959 Broadway, 2North, New York, NY, USA.
authors: Tasneem
authors: T
authors: Hoque
authors: Marc
authors: M
authors: Richmond
authors: Julie Ann
authors: JA
authors: Vincent
authors: Emile
authors: E
authors: Bacha
authors: Alejandro
authors: A
authors: Torres
mesh_terms: D006348:Cardiac Surgical Procedures; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
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