Incorporating Fetal Echo Metrics Into The Clinical Decision Algorithm For Pulmonary Atresia With Intact Ventricular Septum: A Paradigm Shift
Miza Salim Hammoud1, Chang Yoon Doh2, Caroline Pennacchio2, Kaliegh Cummins1, Rajeswaran Jeevanantham1, Chandrakant Patel3, Kristen Breedlove3, Hani K.Najm1, Eugene Blackstone1, Tara Karamlou1.
1Cleveland Clinic, Cleveland, OH, USA, 2Case Western Reserve University, Cleveland, OH, USA, 3Akron Children's Hospital, Cleveland, OH, USA.
Background: Pulmonary atresia with intact ventricular septum (PAIVS) includes a wide anatomic and physiologic spectrum enabling multiple repair pathways: biventricular (2V), univentricular (1V), or 1.5 ventricle (1.5 V) repair. Postnatal tricuspid valve Z score (TVZS), has been utilized to triage neonates into one of these pathways. We aim to couple fetal TVZS with the postnatal values and characterize their with right heart growth. Methods: From 07/2003- 05/2022, 139 infants with PAIVS were diagnosed and treated at Cleveland Clinic. Thirteen patients with available fetal echocardiographic data were included in the preliminary analysis. Median gestational age at birth was 38 weeks (15th,85th percentile: 32,39) and 7 (54%) were females. Longitudinal fetal and postnatal TVZS echocardiographic measurements (Figure 1A) were incorporated into a multilinear mixed regression model. Results: Five out of 13 patients had a 2V, two had a 1.5 V and 2 had a 1V. The remaining 4 underwent a primary heart transplant (HTx) (Figure1A). Death occurred in 2 patients who had a primary HTx and 1 undergoing a 1V repair. All 2V and 1.5 V patients showed an increase in the growth trajectory of the TVZS from the fetal till the postnatal period (Figure 1B&C). Steeper longitudinal growth trajectories were noted in larger fetal TVZS. Conclusion: PAIVS patients managed with 1.5 V and 2V demonstrate “catch-up growth”, which correlates with larger fetal TVZS. 
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