Isolated coarctation of the aorta in the fetus: A diagnostic challenge.

Isolated coarctation of the aorta (CoA) is estimated by the Centers for Disease Control and Prevention to account for 4%-6% of all congenital heart disease (CHD) in the United States, with a reported prevalence of ~4 per 10 000 live births. Prenatal recognition of coarctation is important as it may improve neonatal survival and reduce morbidity. However, despite advances in imaging and the trend toward detailed aortic arch assessment as part of a comprehensive fetal echocardiogram, isolated CoA may still elude prenatal detection, with potentially lethal consequences if the diagnosis is not suspected and the patent ductus arteriosus (PDA) closes spontaneously in postnatal life. The purpose of this review is to outline the methods of antenatal aortic arch evaluation in the current era, discuss "red flags" that raise the suspicion for CoA, including associated anomalies and serve as a repository of the most up to date information regarding its diagnosis in utero and its perinatal management. Other aortic arch abnormalities, such as interrupted aortic arch, or CoA associated with complex single ventricles, are not included in this review.

Catheter-Based Fetal Cardiac Interventions.

Fetal cardiac intervention (FCI) is an emerging and rapidly advancing group of interventions designed to improve outcomes for fetuses with cardiovascular disease. Currently, FCI is comprised of pharmacologic therapies (e.g., trans-placental antiarrhythmics for fetal arrhythmia), open surgical procedures (e.g., surgical resection of pericardial teratoma), and catheter-based procedures (e.g., fetal aortic valvuloplasty for aortic stenosis). This review focuses on the rationale, criteria for inclusion, technical details, and current outcomes of the three most frequently performed catheter-based FCI procedures: (1) aortic valvuloplasty for critical aortic stenosis (AS) associated with evolving hypoplastic left heart syndrome (HLHS), (2) atrial septal intervention for HLHS with severely restrictive or intact atrial septum (R/IAS), and (3) pulmonary valvuloplasty for pulmonary atresia with intact ventricular septum (PA/IVS).

Atrial septal defect closure is associated with improved clinical status in patients ≤ 10 kg with bronchopulmonary dysplasia.

Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post-ASD closure improvement is secondary to the expected course in BPD patients or related to the closure itself. A novel BPD-ASD score was created to assess patients' clinical status (higher score = worse disease) and applied to 10 BPD-ASD inpatients weighing ≤ 10 kg who underwent ASD closure. The score and its subcomponents were retrospectively calculated serially ranging from 8 weeks pre- to 8 weeks post-intervention, and pre- and post-intervention score slopes were created. These slopes were compared using mixed regression modeling with an interaction term. There was a significant difference in pre- versus post-intervention slope with the most score drop the first week post-intervention (-2.1 + /- 0.8, p = 0.014). The mean score also dropped through weeks 2 (slope -0.8 + /- 0.8, p = 0.013) and 4 (slope -1.0 + /- 0.5, p = 0.001) post-intervention. There was a significant difference in pre- and post-intervention slopes for diuretics (p = 0.018) and the combined score of respiratory support, FiO2 need, and respiratory symptoms (p = 0.018). This study demonstrated significant improvement in BPD-ASD score, diuretic need, and respiratory status after ASD closure in BPD-ASD patients ≤ 10 kg that was outside of the natural course of BPD. Our study was limited by its small, single-center, retrospective nature. Future studies should be performed in a larger multicenter population to both validate the scoring system and compare to non-intervention infants.

Inter-twin differences in fetal echocardiographic findings are associated with decreased dual twin survival in twin-twin transfusion syndrome.

OBJECTIVE: To determine if inter-twin differences in fetal echocardiographic findings are associated with fetal survival in monochorionic pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). METHODS: This study included women who underwent laser surgery for TTTS between 2012 and 2018 at a single institution. Echocardiographic cardiac parameters in the donor and recipient twins were compared using Z-scores and regression analyses (adjusted for confounding variables) to determine whether any measurable inter-twin differences were associated with neonatal survival at birth. Results are expressed as (Odds ratio [95% confidence interval], p-value). RESULTS: Fetal echocardiography and delivery information was available in 124 TTTS cases. Dual live-birth occurred in 72% and at least one live-birth was seen in 89% of cases.Sixty-four percent (51/79) of recipient twins had evidence of cardiac dysfunction compared to 10% (8/79) of the donor twins (p < .01). In the logistic regression, inter-twin differences in left ventricle short axis dimension (0.62[0.44-0.87], p < .01), aortic valve diameter (0.67[0.45-0.99], p = .047), peak systolic velocity across the pulmonary artery (PA-PSV) (0.09[0.01-0.53], p < .01) and mitral valve diameter (0.56[0.38-0.84], p < .01) were associated with lower dual twin survival at birth. CONCLUSION: Inter-twin differences in left cardiac geometry and function are associated with decreased survival at delivery in TTTS.