Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome

Abstract

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.

The Optimal Timing of Stage-2-Palliation After the Norwood Operation

“…Jean-St-Michel et al . demonstrated that developing RV dysfunction post-Norwood is often associated with failing to complete SVP (25). Although it appears that later S2P would maximize survival in high-risk patients, this likely reflects that some infants survived long enough to develop RV dysfunction at an older age.…”

Impact of Lactate Clearance on Early Outcomes in Pediatric ECMO Patients

“…High lactate levels over a brief time period after ECMO start can result from a "washout effect" of underperfused tissues. Furthermore, some previous studies suggested that hyperglycemia might stimulate tissue glucose uptake and glycolysis that contribute to hyperlactatemia [18,19]. These cognitions suggest that lactate levels might be indicative for patients' global and cardiac status only at the time of cardiogenic shock or a short time after ECMO start.…”

Periscopic technique in Norwood operation is associated with better preservation of early ventricular function

“…This may help patients avoid complications of RV dysfunction such as pseudoaneurysm formation, heart failure requiring increased inotropic support, use of extracorporeal membrane oxygenation or ventricular assist device, heart transplants, and death. 21 , 22 , 23 , 24 Although we routinely perform left-sided RV-PA-C, the PT could potentially also be used for right-sided RV-PA-C placement.…”