CASE REPORT Novel Transatrial Septoplasty Technique for Neonates with Hypoplastic Left Heart Syndrome and an Intact or Highly Restrictive Atrial Septum Kevin Hill • J. Curt Fudge • Piers Barker • James Jaggers • John Rhodes Received: 30 September 2009 / Accepted: 15 December 2009 / Published online: 5 January 2010 Ó Springer Science+Business Media, LLC 2010 Abstract Two patients born with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum requiring emergent intervention are presented. For both patients, rapid septoplasty was performed using a hybrid approach involving direct atrial puncture and transatrial cutting balloon dilation of the atrial septum. This technique facilitates safe and rapid procedural completion and mini- mizes the risk of procedural complications in this very- high-risk patient population. Keywords Hypoplastic left heart syndrome Á Intact atrial septum Á Restrictive atrial septum Á Septoplasty In children born with hypoplastic left heart syndrome (HLHS), the presence of an intact or highly restrictive atrial septum is poorly tolerated in the immediate postnatal period. They experience hemodynamic instability, acidosis, pulmonary edema, and severe hypoxia. Several emergent management strategies have been used to improve the outcomes for these children. Early surgical intervention with Norwood completion predicts a very poor outcome, with early mortality rates of 50–90% [2, 11, 13]. Fetal intervention offers the potential for improved outcomes but requires prenatal diagnosis. Furthermore, fetal balloon septoplasty often does not completely relieve obstruction, so an emergent neonatal procedure still is required [9]. Perhaps the most effective postnatal strategy is imme- diate catheter intervention followed by a period of recovery to allow for resolution of acidosis and improvement in pulmonary vascular resistance before Norwood interven- tion. With this strategy, several institutions have reported improved outcomes [1, 13]. A variety of transcatheter techniques have been descri- bed including traditional Rashkind balloon septostomy, transseptal puncture with static balloon dilation, blade septostomy, and stent placement [1, 3, 6, 10, 13]. However, acute procedural failure is reported in up to 30% of the patients, and in this subset, mortality remains very high [7]. Furthermore, the procedure is technically difficult, with high complication rates and long procedure times. Frequently a transseptal puncture is required, but it is associated with a significant risk of cardiac perforation [1, 7]. In this unstable patient population, rapid procedural efficacy is crucial and conflicts with the prolonged trans- portation and setup times that may be required if the pro- cedure is performed in a catheterization laboratory. We report a novel hybrid septoplasty technique using a direct transatrial approach and cutting balloon dilation. The procedure can be performed rapidly in the intensive care unit (ICU) using transesophageal echo (TEE) guidance. Overall, the procedure is technically less complex, thus decreasing the risk of adverse events and allowing quicker procedural completion times. We successfully used this technique for two neonates presenting with HLHS and severely restrictive atrial communications. Electronic supplementary material The online version of this article (doi:10.1007/s00246-009-9627-7) contains supplementary material, which is available to authorized users. K. Hill (&) Á J. C. Fudge Á P. Barker Á J. Rhodes Division of Pediatric Cardiology, Duke University Medical Center, DUMC 3090, Durham, NC 27710, USA e-mail: kevin.hill@duke.edu J. Jaggers Division of Cardiovascular and Thoracic Surgery, Duke University Medical Center, DUMC 3090, Durham, NC 27710, USA 123 Pediatr Cardiol (2010) 31:545–549 DOI 10.1007/s00246-009-9627-7