Ethical Considerations in Fetal Cardiology.

Fetal cardiology has evolved over the last 40 years and changed the timing of diagnosis and counseling of congenital heart disease, decision-making, planning for treatment at birth, and predicting future surgery from the postnatal to the prenatal period. Ethical issues in fetal cardiology transect multiple aspects of biomedical ethics including improvement in prenatal detection and diagnostic capabilities, access to equitable comprehensive care that preserves a pregnant person's right to make decisions, access to all reproductive options, informed consent, complexity in shared decision-making, and appropriate use of fetal cardiac interventions. This paper first reviews the literature and then provides an ethical analysis of accurate and timely diagnosis, equitable delivery of care, prenatal counseling and shared decision-making, and innovation through in utero intervention.

Perspectives of Challenges in Counseling for Congenital Heart Defects.

Prior work regarding counseling patients about congenital heart defects (CHD) has focused on their perceptions about accurate communication of cardiac anatomy, and the emotional support received from the provider. The objectives of this study were to identify the additional CHD counseling-specific challenges and areas for future intervention, using a practical communication framework. This is a secondary analysis of qualitative data provided by caretakers of infants who received congenital heart surgery from 2019 to 2020 in the Chicagoland area. While the survey in the primary study pertained to barriers in obtaining prenatal diagnosis, respondents with both prenatal and postnatal diagnosis reported challenges to effective counseling. Qualitative data measuring counseling challenges were collected from semi-structured phone interviews. Thematic analysis was performed using an inductive approach. Themes were organized into five domains using SPIKES (Setting, Perception, Invitation, Knowledge, Empathy, and Summarize/Strategy), a previously validated framework to help clinicians effectively break bad news. Among 160 survey respondents, 35 (21.9%) reported a challenge during CHD counseling that they received. In total, 12 challenges were identified and spanned all six SPIKES domains. The three most common challenges were as follows: perception of repeated imaging studies for accurate diagnosis or management (n = 19, Knowledge), the lack of cardiologist presence at the time of initial CHD detection (n = 8, Setting), and insufficient information provided about the CHD diagnosis (n = 7, Knowledge). Patients perceive counseling as a key component of prenatal diagnosis of CHD and identify the challenges that exist at all stages of the counseling process. These findings suggest that effective counseling extends beyond conveying information about anatomy and prognosis.

Incidence of Fetal Arrhythmia Before and During the COVID-19 Pandemic: A Single-Center Experience.

Fetal arrhythmias are rare and carry significant morbidity and mortality without appropriate treatment. Initial reports exist of fetal arrhythmia in the setting of maternal COVID-19 infection. Our study sought to evaluate incidence of fetal arrhythmia before and during the COVID-19 pandemic at our institution. This retrospective cohort study from a tertiary care fetal cardiac center utilized the institutional REDCap database to search fetal arrhythmia diagnostic codes. Medical records of mother-fetus dyads were reviewed and data were collected on diagnoses, gestational age, treatment regimen, and postnatal outcomes. Patients were divided into pre-COVID and peri-COVID segments. 8368 total pregnancies were evaluated during the 7.3 years of study period. Forty-five patients (0.5%) had a significant fetal arrhythmia and were included in this study: 19 (42%) in the pre-COVID-19 group and 26 (58%) in the peri-COVID-19 group. No patients had associated congenital heart disease. There was a notable increase in the incidence of fetal supraventricular tachycardia (SVT) (1.82 per 1000 vs 2.65 per 1000 pregnancies) and complete heart block (1.04 per 1000 vs 1.77 per 1000 pregnancies) but no apparent change in other tachyarrhythmias during the COVID era. The proportion of antibody-mediated complete heart block increased from 50 to 87.5%. There was also an increase in the percentage of SVT patients requiring postnatal treatment during COVID-19 (53.8% vs 62.5%). Our experience shows an increased incidence of some fetal arrhythmia diagnoses during the COVID-19 pandemic. Additional multi-center studies will be necessary to fully evaluate the increased burden of fetal arrhythmias during the COVID-19 era as well as to elucidate etiology.

Impact of pulmonary stenosis on right ventricular global longitudinal strain in repaired tetralogy of Fallot patients post transcatheter pulmonary valve replacement.

BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Fetal Congenital Complete Heart Block: A Rare Case with an Extremely Low Ventricular Rate and Review of Current Management Strategies.

Congenital complete heart block (CCHB) is associated with high intrauterine and post-natal mortality. Prenatal detection and management, as well as appropriate delivery planning, may improve the outcomes in CCHB. We describe a rare case of CCHB that initially presented with fetal ascites and high-grade second-degree heart block noted on fetal echocardiography. The mother was noted to be positive for anti-SSA antibodies, and treatment with maternal steroids was started in an effort to reverse the fetal cardiac conduction abnormality. However, the fetal cardiac rhythm progressed to complete heart block by the follow up evaluation and the fetus had a continual declination of heart rate throughout the pregnancy to a low fetal heart rate of 25 beats per minute (bpm). This case demonstrates the lowest fetal ventricular rate documented in the literature and illustrates a severe presentation of a rare disease process. An overview of the existing knowledge related to etiology, prenatal evaluation with fetal echocardiography and fetal magnetocardiography, prenatal management, and delivery planning in fetuses with prenatally detected CCHB is included.

Congenital Solitary Left Atrial Mass.

Rhabdomyoma in the absence of tuberous sclerosis presenting as a large solitary atrial mass is an atypical finding. We hereby present images from an infant with a fetally diagnosed large left atrial mass with no evidence of mitral valve dysfunction who subsequently developed persistent arrhythmias requiring surgical resection of the mass in the neonatal period.

Intrapericardial NUT Midline Carcinoma: Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations.

Rearrangements in the nuclear protein in testis (NUT) gene cause carcinomas that represent a rare but aggressive tumor type that often present at advanced stages in midline structures. Survival rarely exceeds 12 months from the time of diagnosis. There have been no reports of a primary cardiac presentation, and few studies have reported on the numerous treatment strategies. Given their aggressive and invasive nature, NUT midline carcinomas present a therapeutic dilemma. Treatment may include surgical resection, chemotherapy, or radiotherapy, but no consistently successful treatment has been established. Surgical resection is indicated to reduce symptomatic mass effect whenever present. Novel therapies with bromodomain extra-terminal inhibitors may be associated with potential survival benefit. Here, we describe an unusual presentation of this tumor. Literature review with management considerations is underlying.