Technical Success and Serious Adverse Events for Fetal Aortic Valvuloplasty in a Large 20-Year Cohort.

Background: FAV is offered to fetuses with severe aortic valve stenosis and evolving hypoplastic left heart syndrome. An inferential analysis of TS and SAE in a large series has not been reported. Objectives: The purpose of this study was to determine factors associated with fetal aortic valvuloplasty (FAV) technical success (TS) and serious adverse events (SAEs). Methods: Retrospective, single-center, cohort analysis of attempted FAV from March 1, 2000, to December 31, 2020. The primary outcome was the TS of FAV, and the secondary outcome was the presence of an SAE. Results: A total of 165 FAVs were attempted in 163 patients with a median gestational age of 24.6 weeks (IQR: 22.9-27.1 weeks). FAV TS was 85% (141/165) and was higher in the 2010 to 2020 era (94% [85/90] vs 75% [56/75]; P < 0.001). Pre-FAV echocardiographic left ventricle (LV) long axis dimension z-score >-0.10 (P < 0.001) and higher LV ejection fraction (P = 0.037) were independently associated with a higher odds of TS. There were 117 SAEs in 67 attempted FAVs (41%), 13 of which were fetal deaths (7.9%). By classification and regression tree analysis, gestational age <21 weeks or in older fetuses, a procedure time of ≥39.6 minutes was associated with higher SAE rate. In the multivariable logistic regression model correcting for gestational age, fetuses with an LV end-diastolic volume <4.09 mL had an age-adjusted OR of 4.71 (95% CI: 1.67-13.29; P = 0.004) for experiencing an SAE. Conclusions: TS of FAV has improved over time, and failure is associated with smaller fetal left heart sizes. SAEs are common and are associated with smaller left hearts and longer procedure times.

Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly).

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

Prenatal Counseling and Care for Single-Ventricle Heart Disease: One Center's Model for Care.

Advances in prenatal imaging allow early detection of single-ventricle congenital heart disease, which may enhance prenatal care and maximize care options and decision making. Boston Children's Hospital's Advanced Fetal Care Center and fetal cardiology program provide prenatal counseling and care for single-ventricle congenital heart disease. Key points for optimal prenatal counseling and education include explanation of the diagnosis, delivery, the first surgery, cardiac neurodevelopmental issues, feeding and growth issues, quality of life and long-term care, family stressors, and fetal cardiac intervention. Such counseling and education help families make the difficult decisions required in this situation.

Biventricular conversion after single ventricle palliation in patients with small left heart structures: short-term outcomes.

BACKGROUND: Patients with borderline small left heart (LH) structures who initially undergo single ventricle palliation (SVP) may eventually become candidates for biventricular conversion (BC). The purpose of this study was to describe our surgical experience with BC in patients with small LH. METHODS: We reviewed our institution's records for patients who underwent BC after an initial SVP between 1995 and 2012. Patients underwent an aortopulmonary amalgamation procedure as a part of their initial palliation. Data on imaging, BC operative details, and re-interventions after BC were collected. RESULTS: Twenty-eight patients underwent BC. Twenty patients had hypoplastic left heart syndrome (HLHS), 7 patients had unbalanced common atrioventricular canal (uCAVC), and 1 had interrupted aortic arch with VSD. Stage of palliation at BC was stage 1 in 6 patients (21.4%), bidirectional Glenn in 19 (67.9%), and Fontan in 3 (10.7%). Prior to BC, the median left ventricular end-diastolic volume (LVEDV) by echocardiography was 58.1 mL/m(2) in the HLHS group and 28.1 mL/m(2) in the uCAVC group. After BC, the LVEDV increased to 91.3 mL/m(2) in the HLHS group and 58.5 mL/m(2) in the uCAVC group (p < 0.05 compared with pre-BC in both groups). Right ventricular pressure was less than half systemic in 8 patients (53.3% of those measured). Seventeen patients (61%) have required either catheter-based or surgical re-intervention. Twenty-five patients (89.3%) were alive at a median follow-up of 2.6 years. CONCLUSIONS: Biventricular conversion can be applied to patients with HLHS and uCAVC and borderline LH with acceptable short-term results. Left heart size increases after BC, but follow-up for potential left atrial hypertension is warranted.