Impact of High-Risk Characteristics in Hypoplastic Left Heart Syndrome

Abstract

Background: Management of hypoplastic left heart syndrome (HLHS) presents many challenges. We describe our institutional outcomes for management of patients with HLHS over the past 12 years and highlight our strategy for those with highly restrictive/intact interatrial septum (R/I-IAS). Methods: Eighty-eight neonates with HLHS underwent surgical treatment, divided equally into Era-I (n = 44, April 2006 to February 2013) and Era-II (n = 44, March 2013 to June 2018). Up to 2013, all patients with R/I-IAS were delivered at an adjacent adult hospital and then moved to our hospital for intensive care and management. From 2014, these patients were delivered at a co-located theatre in our hospital with immediate atrial septectomy. The hybrid approach was occasionally used with preference for the Norwood procedure for suitable candidates. Results: One-year survival after Norwood procedure was 62.5% and 80% for Era-I and Era-II ( P = not significant (ns)), respectively, and 41% of patients were categorized as high risk using conventional criteria. Survival at 1 year differed significantly between high-risk and standard-risk patients ( P = 0.01). For high-risk patients, survival increased from 42% to 65% between eras ( P = ns). In the R/I-IAS subgroup (n = 15), 11 underwent Norwood procedure after emergency atrial septectomy. Of these, seven born at the adjacent adult hospital had 40% survival to stage II versus 60% for the four born at the colocated theatre. Delivery in a colocated theatre reduced the birth-to-cardiopulmonary bypass median time from 445 (150-660) to 62 (52-71) minutes. Conclusion: Reported surgical outcomes are comparable to multicenter reports and international databases. Proactive management for risk factors such as R/I-IAS may contribute to improved overall outcomes.

Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum

“…No interstage mortality among the 8 survivors of LAD was recorded, nor hospital mortality for the 6 infants undergoing bidirectional Glenn and the 5 completing TCPC (Table 3). Our cumulative 5-year survival is comparable to those reported literature, 5,6,20 given 4 early deaths pre-Norwood, 1 death after Glenn completion and one late death related to graft failure following transplantation. Although not statistically significant in the face of small cohort numbers, mortality for HLHS IS/HRIC was considerably higher than for HLSH (Figures3-4).…”

“…This finding confirms previous reports. 6,20 In our experience, ECMO support following Norwood procedure was not associated with increased hospital mortality, as opposed to other series. [3][4][5][6]9 Our cannulations were performed electively in the operating room in the perioperative period in patients n.3 and 8 due to hemodynamic instability and semielectively on postoperative day 11 patient n.4 due to worsening hypoxemia and hypercarbia unresponsive to maximal ventilatory support.…”

“…It is widely reported that concomitant lung disease is associated with HLHS/IS. 5,6,20 We withdrew care due to established lung disease in patient n.12. This was a scenario where the clinical picture and the CT scan findings together influenced our decision making.…”

Invited Commentary: What Do We Achieve With Expedited Left Atrial Decompression?

“…Diagnosis of a highly restrictive or intact atrial septum (HR/IAS) in the context of a fetus with hypoplastic left heart syndrome 1 is managed differently across the world, with the divide not defined by resource availability alone. There are many case series 2–7 like this study by Generali and colleagues, 8 published in this issue of the World Journal. Small numbers of patients accrued over several decades, handled in different ways and likely by different key individuals in interventional cardiology and surgical roles.…”

Disturbed regional right heart mechanics assessed by strain echocardiography in genetically diverse hypoplastic left heart syndrome

“…Treatment of HLHS is challenging and relies on a 3 stage surgical palliation procedure (Norwood procedure) to rebuild parts of the heart and to redirect the way of the blood flow [1]. The Norwood procedure includes stage-1 palliation by immediate atrial septectomy (Blalock-Taussig shunt) or RV-to pulmonary artery conduit, stage-2 palliation using the bidirectional Glenn shunt, and the stage-3 palliation using a tube graft connecting the inferior vena cava to the central pulmonary artery (Fontan procedure) [7,8]. A second approach for reshaping cardiac morphology and repairing cardiac functions in HLHS is the "hybrid procedure" which consists of postnatal bi-lateral pulmonary banding (bPAB), percutaneous ductal stenting, and in case of need, manipulation of the atrial septum [8].…”