Impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia

Objectives: To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia. Methods: Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). The 3D steady-state free precession acquisition was used to measure fetal brain volume. Surgical outcome, brain MRI, and neurodevelopmental follow-up were reviewed. Results: Twenty-six fetuses were live born (76%) and survival (65%) at a mean follow-up of 4 years. Nine fetuses had a brain MRI before discharge, and all had clinically silent injuries and volume loss. At 18 months, five single-ventricle patients had a neurodevelopmental delay in cognition and language (mean percentile: 11th), with gross-motor skills more affected than fine-motor skills (mean percentiles: 4th and 34th). Fetuses with Ebstein anomaly/tricuspid valve dysplasia had smaller brains, lower combined ventricular output, ascending aorta, superior caval vien and umbilical vein flows, lower oxygen saturation in ascending aorta and superior caval vien, lower cDO2 and cVO2 (p < 0.05). Superior caval vien/combined ventricular output and descending aorta/combined ventricular output ratios were lower in fetuses with circular shunt (p < 0.05). Fetuses requiring the Starnes procedure tended to have smaller brains, lower combined ventricular output, superior caval vien, descending aorta, and umbilical vein flows. Conclusions: All patients with Ebstein anomaly/tricuspid valve dysplasia are at high risk of neurodevelopmental delay and warrant follow-up. Fetal cardiovascular magnetic resonance revealed impaired brain growth with diminished cerebral blood flow and cDO2, the extenting dependent on the severity of the haemodynamic compromise.

Outcomes of Anti-Spike Monoclonal Antibody Therapy in Pregnant Women with Mild to Moderate COVID-19

Objective: To evaluate the efficacy and safety of anti-spike monoclonal antibodies (MAb) in the treatment of mild to moderate COVID-19 in high-risk patients who are pregnant. Methods: The database of patients treated with monoclonal antibodies in the Mayo Clinic Midwest region was reviewed for patients who were pregnant at the time of infusion. Manual chart review was performed to collect demographic details as well as COVID course for both the mother and the infant if delivered. The data are presented using descriptive methods. Results: We identified fifty-one pregnant patients with mild to moderate COVID-19 who were treated with MAb (4 with bamlanivimab monotherapy, 3 with bamlanivimab-etesevimab combination, and 44 with the casirivimab-imdevimab combination). No adverse effects were reported, and no patient required COVID-19 related hospitalization. Twenty-nine patients delivered healthy babies, there was one case of intrauterine fetal demise secondary to a congenital Ebstein anomaly (not related to MAb treatment), and twenty-one were uncomplicated pregnancies. Conclusion: MAb infusions were well tolerated in pregnant patients considered at high risk for COVID-19 complications, with no observed adverse effects to mother or fetus. Although preliminary data suggest MAb therapy in pregnancy is safe, further research is recommended to fully assess safety and efficacy in pregnancy.

Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome

Ebstein anomaly is a rare congenital heart disease characterized by a varying degree of anatomical and functional abnormalities of tricuspid valve and right ventricle. It often coexists with other congenital cardiac malformations. Up to 79–89% of patients with Ebstein anomaly have interatrial communication in the form of patent oval foramen or atrial septal defect and more than one-third has other types of cardiac malformations. Association between Ebstein anomaly and right aortic arch is extremely rare and only few cases have been described in the literature so far. Much rarer than with other cardiac malformations, Ebstein anomaly is associated with non-cardiac malformations or genetic syndromes. Several cases of association between Ebstein anomaly and Charge syndrome have been reported, nevertheless, Ebstein anomaly accounts for less than 1% of cardiac defects seen in patients with Charge syndrome. In this case report, we present a unique case of a patient with Charge syndrome where both Ebstein anomaly and right aortic arch are present. The diagnosis of Ebstein anomaly and right aortic arch was established prenatally. In the first years of life, the patient did not exhibit any remarkable symptoms. However, over time, deterioration of right ventricle function and increased tricuspid regurgitation were observed, requiring consideration of surgical treatment at the age of five. In addition, delay in physical, motor, and mental development was observed and thus, at the age of five, the patient was consulted by a medical geneticist and a gene panel to test for structural heart defects was ordered. The test showed a mutation in chromodomain helicase DNA binding protein 7 (CHD7) gene, which, along with clinical features, allowed to establish a diagnosis of Charge syndrome. To the best of the authors’ knowledge, this is the first case report of a patient with Charge syndrome, Ebstein anomaly, and right aortic arch that has been described in the literature.

Permanent pacemaker implantation in Ebstein anomaly with metallic tricuspid valve

Right ventricular endocardial pacing is partially contraindicated in the presence of mechanical tricuspid valve. Occurrences of atrioventricular block are commonly associated in postoperative period in Ebstein anomaly repaired with mechanical tricuspid valve. Coronary sinus (CS) pacing is the preferred site in this scenario. However, the anatomical variations in Ebstein anomaly leads to difficulties in hooking the CS. With the help of real time left coronary injection enabled in understanding the anatomical orientation of CS ostium take off, leading to successful CS lead implantation.

Prenatal Diagnosis of Ebstein Anomaly in a Centre with Limited Resources: A Potential Pitfall

BACKGROUND: Ebstein anomaly is a rare congenital cardiac malformation characterized by varying degrees of downward displacement of the tricuspid valve leaflets into the cavity of the right ventricle. The pathophysiology varies with the degree of valvular malfunction, the size, and contractility of the right ventricle, and the presence of obstruction within the right ventricular outflow tract. An early diagnosis makes worse the often prognosis. Fetal echocardiography is a useful tool for the diagnosis of congenital heart disease including the Ebstein anomaly which shows a wide spectrum of ultrasonographic manifestations and, sometimes it can be difficult to make a true prenatal diagnosis. CASE REPORT: Here, we describe a unique case of an Ebstein anomaly in 26 weeks of gestation fetus through prenatal echocardiographic evaluation. Fetus was suspected as a severe form of the Ebstein anomaly, with a severe form of right atrium enlargement, huge tricuspid insufficiency, and hypoplastic pulmonary artery. The atrial septum, dimensions of cardiac structures, left and right cardiac output, and Doppler interrogation of cardiac flows were examined. CONCLUSION: This case study presents a well-documented case of Ebstein’s anomaly type D that was diagnosed prenatally using fetal echocardiography.

Left ventricular hypertrabeculation in Ebstein anomaly – a clinically significant or irrelevant problem?

Background Ebstein anomaly (EA) is characterized by anomalous tricuspid valve resulting in the whole right heart's distorted anatomy. Heart failure is the most frequent complication in adults with EA. Recently more attention is also paid to the left ventricle. Some limited echocardiographic data suggest a markedly increased occurrence of the left ventricular hypertrabeculation, by some authors called a non-compacted myocardium. Currently, cardiac magnetic resonance (CMR) is a gold standard in the evaluation of hypertrabeculation. However, different diagnostic criteria and discrepancies in the study outcomes make the clinical interpretation of this phenomenon questionable and challenging. Purpose To determine the frequency of the left ventricular hypertrabeculation among adults with EA; to assess its association with the ventricular size and function and the markers of heart failure. Methods Study group consisted of 35 unoperated adults (mean age 41.9±14.8 years) with Ebstein anomaly. The study protocol included: a) cardiac magnetic resonance (CMR) with the assessment of the left ventricle (LV) and functional right ventricle (fRV), including ejection fraction (EF), end-diastolic, end-systolic, and stroke volumes indexed by body surface area (respectively: EDVind, ESVind, SVind), and presence of hypertrabeculation defined according to Jacquier (1) as trabeculation mass &gt;20% of the LV mass; b) cardiopulmonary tests with the assessment of peak oxygen consumption (peak VO2, % of predicted peak VO2), ventilation/carbon dioxide slope (VE/VCO2 slope) c) brain natriuretic peptide (BNP). Results Left ventricular hypertrabeculation was present in 13 (37.1%) patients. We demonstrated no difference between patients with and without LV hypertrabeculation regarding: s Conclusions Left ventricular hypertrabeculation demonstrated by cardiac magnetic resonance is a phenomenon frequently occurring among adults with Ebstein anomaly. However, its presence is not associated with both ventricles' worse function nor with reduced exercise capacity. Therefore, the clinical relevance of left ventricular hypertrabeculation in this population remains questionable and requires further prospective studies. FUNDunding Acknowledgement Type of funding sources: None.

Left ventricular global longitudinal strain is superior to ejection fraction for prognostication in ebstein anomaly

Background Certain factors such as left ventricular (LV) geometry and loading conditions affect the validity and reliability of LV ejection fraction (LVEF) as a true measure of LV contractility. LV global longitudinal strain (LVGLS) is less sensitive to these factors, and it has superior prognostic performance in patients with acquired heart disease. The purpose of this study was to determine the clinical implications of using LVGLS (instead of LVEF) as the measure of LV systolic function in adults with Ebstein anomaly given the inherent abnormalities of LV geometry and preload in this population. Methods Retrospective cohort study of 673 adults with Ebstein anomaly (2003–2018). We hypothesized that LVGLS had a stronger correlation with heart failure indices and transplant-free survival compared to LVEF. Results Compared to LVEF, LVGLS had stronger correlations with cardiac index (r=0.46 vs r=0.21, p=0.007), glomerular filtration rate (r=0.57 vs r=0.19, p&lt;0.001), and NT-proBNP (r=−0.64 vs r=−0.41, p=0.01). Of 673 patients, 514 (76%) had normal LV systolic function (LVGLSNormal-LVEFNormal), 87 (13%) had subclinical LV dysfunction (LVGLSLow-LVEFNormal) and 66 (10%) had overt LV dysfunction (LVGLSLow-LVEFLow). Compared to the overt LV dysfunction group, the subclinical LV dysfunction group had similar 10-year transplant-free survival (64% vs 63%, p=0.6), but were less likely to be on heart failure therapy (12% vs 82%, p&lt;0.001). LVGLS (but not LVEF) was the independent predictor of transplant-free survival Conclusions LVGLS provided more robust risk stratification and prognostication than LVEF in patients with Ebstein anomaly, and patients with low LVGLS had reduced transplant-free survival regardless of LVEF. The use of LVGLS (rather than LVEF) as the measure of LV systolic function has important clinical implications with regards to initiation of medical and surgical therapies. FUNDunding Acknowledgement Type of funding sources: None.