Abstract 16450: Left Ventricular Coronary-Cameral Fistulae in the Mitral Stenosis-Aortic Atresia Variant of Hypoplastic Left Heart Syndrome Are Associated With Poor Outcomes
Background: Neonates with the mitral stenosis aortic atresia (MSAA) subtype of hypoplastic left heart syndrome have increased hospital and interstage mortality after stage one palliation (S1P). In one retrospective report of these patients, left ventricular coronary-cameral fistulae diagnosed by echocardiography were associated with hospital mortality of 50%.
Methods: Since January of 2007, 21 of 22 consecutive patients with MSAA underwent diagnostic catheterization prior to palliation, to evaluate for the presence of left ventricular coronary-cameral fistulae. Those without angiographically-evident fistulae underwent standard S1P.Those with fistulae seen at catheterization were predominantly palliated with a ductal stent and branch pulmonary artery bands (hybrid palliation). For this study, all available angiography was interpreted by a single reviewer blinded to clinical outcomes. The angiographic findings and clinical outcomes through June 1, 2010 are presented.
Results: Of the 21 patients who had pre-palliative catheterization, diagnostic angiography was considered adequate in all but one case. Seven patients (35%) were found to have no fistulae. All 7 subsequently underwent S1P and are alive after BDG. Of the 13 (65%) patients with fistulae, 8 were managed with hybrid palliation; 6 of these have undergone a comprehensive stage II procedure, with 4 survivors and 2 deaths. One patient died following hybrid, and one was converted to a S1P. Of the 5 patients with fistulae who did not have a hybrid, two died and one was transplanted. Two others with fistulae underwent a S1P and died postoperatively. Two patients with fistulae were noted to have in situ thrombus in the aortic root with subsequent clinical thromboembolic events. Overall transplant-free survival for patients with fistulae at catheterization was 34% versus 100% for those without fistulae (p=0.02)
Conclusion: Coronary-cameral fistulae in the MSAA subtype of HLHS are associated with poor survival even when approached with a cardiopulmonary bypass-sparing palliative scheme in the neonatal period. Further efforts to elucidate higher risk fistulae and associated hemodynamic and anatomic patterns are warranted to allow for proper risk stratification and improvement in outcomes.
- © 2010 by American Heart Association, Inc.