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Introduction:The impact of center volume on the procedural outcomes of fetal aortic valvuloplasty (FAV) is unknown. The relationship between volume and outcome is crucial to understand if regionalization should be implemented worldwide.Hypothesis:We hypothesized that higher center volume is associated with greater technical success in FAV.Methods:Data were included from institutions that performed ?3 procedures (2001-2018) and reported technical success. Technical success was defined as ?1 balloon inflation across the aortic valve, with clear evidence of increased flow across the valve and/or new aortic regurgitation by color Doppler. Secondary endpoint measures were fetal periprocedural complications (pericardial/pleural effusion or bradycardia requiring treatment, balloon rupture, or death within 48 hours of the procedure) and fetal periprocedural death.Results:A total of 162 FAVs from 11 centers were included, with FAV center volumes ranging from 6-31. Overall technical success rate was 80%. Higher center volume was not associated with technical success or fetal complications (p=0.948 and p=0.953 respectively), but was associated with fewer periprocedural fetal deaths (p=0.02, Figure). Periprocedural fetal death rate appeared to plateau after 15 FAVs. Intervention in larger fetuses was associated with greater technical success (p<0.001), fewer complications (p=0.01), and fewer periprocedural deaths (p<0.01). Intervention in older fetuses trended towards greater technical success (p=0.08), with significantly fewer complications (p<0.01) and periprocedural deaths (p<0.01). There were 3 significant maternal complications (1.9%) with no association with center volume.Conclusions:In this registry cohort, higher volume centers did not have more FAV technical success or fewer complications, but they did have fewer fetal deaths. Improved FAV outcomes are associated with larger and older fetuses.

Introduction:The etiology of hypoplastic left heart syndrome (HLHS) is unclear. Supravalvar aortic banding in fetal lambs produced conflicting results as to whether LV hypoplasia or hypertrophy occurred. We hypothesized low flow in the fetal left heart results in hypoplasiaMethods:LV inflow obstruction created in 75day fetal lambs by percutaneous implantation of LA coils and harvest at 125 days.Results:19 coiled fetuses and 1 control died prior to harvest. 15 coiled fetuses reached 125 days, 1 had anasarca (10.6kg) due to severe mitral regurgitation. Effective coiling resulted in tissue growth into the coil scaffold leading to severe acquired mitral stenosis and a slit-like LV. LV inflow and antegrade aortic valve (AoV) flow was so low in 9 that there was retrograde flow in the ascending aorta from the duct. 4 of 9 had no antegrade AoV flow, with the brain and coronaries wholly perfused from the duct. These low-flow LV had the most severe left heart hypoplasia and the LV was no longer apex forming. Due to the variation in fetal size (non-hydropic weight 1.74-4.45kg), each fetus?s left heart measurements were normalized to the corresponding right-heart structure and these were all smaller in the low-flow LV hearts as compared to controls (Table 1).Conclusion:Long after cardiogenesis, a severe, prolonged (1/3 gestation) decrease in left heart flow is sufficient to produce LV and ascending aortic hypoplasia and retrograde perfusion of the brain and coronaries, similar to human HLHS. This model will allow investigation of HLHS development and therapies.graphic_{3CF29FE0-3EAF-40EA-AAA1-AE98366853C0}

Background:Ebstein?s anomaly and tricuspid valve dysplasia (EA/TVD) carry high risks of fetal (F) and neonatal (N) death. Factors previously shown to be associated with mortality include increased cardio-thoracic ratio (CTR), right atrial area index (RAAI), absent pulmonary flow, pulmonary regurgitation (PR), and prognostic scores (SAS score; SickKids (SK) score). Scores combine the results of 5 echocardiographic findings according to severity (value 0-2 per variable; score range 0-10) with higher scores connoting higher odds of mortality. For SAS, the variables include CTR, RAAI, right/left ventricular ratio, pulmonary and ductal flows. The SK score includes CTR, RAAI, pulmonary flow, tricuspid regurgitation (TR) severity ? TR peak gradient (TRPG), and PR. We sought to validate factors associated with F/N outcomes in this ancillary multicenter study.Methods:Echocardiograms of fetuses with EA/TVD from 18 institutions were reviewed without prior knowledge of patient outcomes. Excluded were cases with incomplete exams and without intended active care. Area under receiver operating curves were used to compare the SAS and SK scores.Results:Of 129 subjects, 53 (41%) died in-utero or as neonates, while 76 survived to neonatal hospital discharge. Factors at the initial exam (27.6?5.5 weeks gestation) associated with mortality (p<0.001) included hydrops (OR 28.58; [95%CI: 1.61-507.3]), moderate/severe TR with lower TRPG (per mmHg decrease: OR 1.22 [1.14-1.30]), PR (OR 11.81 [4.9-28.2]), CTR (OR per % increase: 1.20 [1.13-1.28], RAAI (OR 97.1 [16.0-589]), and absent pulmonary flow (OR 31.5 [7.12-138]). Baseline SK and SAS scores were 5.0?3.0 and 4.8?2.7, respectively. Areas under the curve were 0.93 (SK) vs 0.83 (SAS) for F/N death (p<0.001). Survival to hospital discharge with a score <6 was 93.5% (SK: 58/62) vs 87.3% (SAS: 55/63) and for scores >7 2.9% (SK: 1/34) vs 15.8% (SAS: 3/19). A prenatal TRPG ?30 mmHg was significantly associated with biventricular survival to hospital discharge (OR 110.5 [20.90-584.35]; p<0.001).Conclusion:The SK score system is superior to SAS in predicting perinatal outcomes. F/N death was associated with significant hemodynamic alterations already present on the baseline exam, including significant TR with a low TRPG.