Abstract 16983: Clinical Outcomes in Patients With Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome
A number of patients develop ventricular dysfunction after stage I palliation for hypoplastic left heart syndrome. We sought to describe their clinical course including the development of heart failure (HF) and outcomes.
All 138 children undergoing stage I palliation at our institution from 2004-2011 were reviewed. Twenty-two (16%) patients (6 Hybrid, 16 Norwood) with 2 sequential echocardiograms reporting at least moderate dysfunction were included. Clinical outcomes were shown with a cumulative incidence function and pre-stage II transplant (HTx)-free survival was compared to a matched control group using Kaplan-Meier analysis.
Of the 22 patients, 3 patients died prior to discharge following stage I and 2 patients remained in hospital, 1 until stage II and 1 until HTx. Seventeen (77%) patients were discharged after stage I with 13 (76%) requiring readmission for HF. The median duration of the first HF admission was 29 days (IQR 8-48). HF management included diuretics (73%), ACE inhibitors (59%), beta blockers (9%), inotropes (55%), positive-pressure ventilation (50%), and ECMO/VAD (23%). Fifteen (68%) patients were assessed for HTx with 8 (36%) listed. In total, 6 (27%) patients underwent HTx, 12 (55%) reached stage II, and 4 (18%) died (1A). HTx free survival at 7 months was lower compared to matched controls (50.6% vs. 90.9%, p=0.040; 1B). Of the 12 patients who reached stage II, 9 recovered to normal or mildly reduced function, of those only 2 had moderate tricuspid regurgitation. Following stage II, 2 (17%) patients died pre-Fontan and 2 underwent HTx (17%).
The majority of patients with ventricular dysfunction following stage 1 palliation required HF management and related admissions. However, a number of patients did recover function allowing for progression to stage II. Further large scale analysis is required to determine which patients are most likely to recover.
- © 2013 by American Heart Association, Inc.