Long-term Impact Of Anatomic Subtype In Hypoplastic Left Heart Syndrome After Fontan Completion
Jiyong Moon, MD1, Timothy Lancaster, MD1, Vikram Sood, MD1, Ming-Sing Si, MD2, Richard G. Ohye, MD1, Jennifer C. Romano, MD, MS1.
1University of Michigan Health System, ANN ARBOR, MI, USA, 2University of California Los Angeles Medical Center, Los Angeles, CA, USA.
Objective(s): Aortic atresia (AA)/mitral stenosis (MS) is a risk factor for survival after Norwood, however the effect of anatomical subtypes in those who survive surgical palliation is unknown. This study aims to determine the long-term impact of anatomic subtype in hypoplastic left heart syndrome (HLHS) after Fontan.
Methods: We included classic HLHS who underwent Fontan at our center between 8/1989 - 7/2017 as a retrospective single-center cohort study. Patient data were linked to the National Death Index and the Scientific Registry of Transplant Recipients. HLHS variants, those who underwent in-hospital Fontan takedown or death, and unknown anatomic subtypes were excluded. The effects of HLHS anatomy on transplant-free survival as well as ventricular and atrioventricular valve (AVV) function were determined.
Results: There were 418 HLHS (AA/mitral atresia [MA] 153, AA/MS 100, aortic stenosis [AS]/MS 154, and AS/MA/ventricular septal defect [VSD] 11). Overall transplant-free survival was 75% at 17 years. There were no significant outcome differences between AA and AS. The sub-analysis between AA/MA and AA/MS revealed AA/MS was associated with higher mortality and tended to have higher rate of ventricular dysfunction (Table).
Conclusions: The survival risk of the anatomic subtype AA/MS shown to be present after Norwood, persisted long-term after Fontan, and was likely due to a higher rate of ventricular dysfunction rather than AVV failure. These findings may suggest an unfavorable ventricular interaction with AA/MS
| Transplant-free survival | AVV regurgitation>moderate or AVV replacement | Ventricular dysfunction > moderate | |
| AA vs AS | AA 71% vs AS 80% at 17 years (P=0.26) | AA 37% vs AS 25% at 17 years (P=0.065) | AA 15% vs AS 20% At 17 years (P=0.7) |
| AA/MA vs AA/MS | AA/MA 75% vs AA/MS 56% at 17 years (P=0.038) | AA/MA 36% vs AA/MS 34 % at 15 years (P=0.52) | AA/MA 11% vs AA/MS 26% at 17 years (P=0.053) |
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