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The Natural And Unnatural History Of 231 Patients With Heterotaxy: A Single Center 5-decade Experience
Elizabeth Stephens, MD, Ph.D., Gabriel graham, BS, Joseph A. Dearani, MD, Talha Niaz, MD, Frank Cetta, MD.
Mayo Clinic, Rochester, MN, USA.

Objective(s): To compare long-term outcomes between patients with heterotaxy syndrome (HS) with single ventricle (SV) palliation versus biventricular (BiV) physiology.
Methods: Institutional echo and Fontan databases were queried for patients with polysplenia or asplenia. Comparisons were made between patients managed with SV ( with/without Fontan) versus BiV physiology.
Results: HS was identified in 231 patients [polysplenia (46%), asplenia (54%)]. 200 patients had SV physiology, 181 (78.4%) had a Fontan. 31 patients had BiV, 20 (8.7%) had surgical intervention, and 11 (4.8%) had no surgical intervention. Details of anatomy and surgical history are in Table. Median age at Fontan was 7.5 (IQR 8.8) years, 50.2% were male. Survival for those with SV physiology (p=0.002) was decreased. Overall 8 (3.5%) patients had cardiac transplant. Risk factors associated with mortality included: Glenn (OR 7.14, 95% CI 2.72-18.9), pacemaker (PPM) (OR 2.8, 95% CI 1.17-6.70), and asplenia (OR 2.3, 95% CI 1.07-4.91). Lower survival among asplenia patients is reflected in the Kaplan Meier curve (P<0.001). Oldest survivor in each group was >60 y/o, except for asplenia without Fontan (38y/o).
Conclusions: Patients with asplenia have lower survival compared to polysplenia. SV physiology had decreased survival compared to BiV. SV Fontan appear to have lower mortality compared to without Fontan. Need for PPM was associated with decreased survival. Overall BiV physiology with the polysplenia had improved survival compared to SV and asplenia.


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