Systematic review of electrophysiology procedures in patients with obstruction of the inferior vena cava.

AIMS: The objective of the study was to conduct a systematic review to describe and compare the different approaches for performing cardiac electrophysiology (EP) procedures in patients with interrupted inferior vena cava (IVC) or equivalent entities causing IVC obstruction. METHODS: We conducted a structured search to identify manuscripts reporting EP procedures with interrupted IVC or IVC obstruction of any aetiology published up until August 2020. No restrictions were applied in the search strategy. We also included seven local cases that met inclusion criteria. RESULTS: The analysis included 142 patients (mean age 48.9 years; 48% female) undergoing 143 procedures. Obstruction of the IVC was not known before the index procedure in 54% of patients. Congenital interruption of IVC was the most frequent cause (80%); and, associated congenital heart disease (CHD) was observed in 43% of patients in this setting. The superior approach for ablation was the most frequently used strategy (52%), followed by inferior approach via the azygos or hemiazygos vein (24%), transhepatic approach (14%), and retroaortic approach (10%). Electroanatomical mapping (58%), use of long sheaths (41%), intracardiac echocardiography (19%), transesophageal echocardiography (15%) and remote controlled magnetic navigation (13%) were used as adjuncts to aid performance. Ablation was successful in 135 of 140 procedures in which outcomes were reported. Major complications were only reported in patients undergoing AF ablation, including two patients with pericardial effusion, one of whom required surgical repair, and another patient who died after inadvertent entry into an undiagnosed atrioesophageal fistula from a previous procedure. CONCLUSION: The superior approach is most frequent approach for performing EP procedures in the setting of obstructed IVC. Transhepatic approach is a feasible alternative, and may provide a "familiar approach" for transseptal access when it is required. Adjunctive use of long sheaths, intravascular echocardiography, electro-anatomical mapping and remote magnetic navigation may be helpful, especially if there is associated complex CHD. With careful planning, EP procedures can usually be successfully performed with a low risk of complications.

Fluoroscopy-free ablation in congenital heart disease of moderate or great complexity.

BACKGROUND: Fluoroscopy-free (FF) ablation has been demonstrated to be safe and successful in patients with structurally normal hearts, but has not been systematically evaluated in patients with congenital heart disease (CHD) of moderate or great (M/G) complexity. This study aimed to evaluate and compare feasibility, safety, and outcomes of FF ablation in patients with or without M/G-CHD. METHODS: Consecutive patients undergoing electrophysiologic study and intended catheter ablation over a 24-month period were included. Subgroups were created based on presence and complexity of CHD-M/G-CHD or simple complexity/no CHD (S/N-CHD). Cases with total radiation dose of zero qualified as FF. Demographic and peri-procedural variables and outcome data were analyzed. RESULTS: A total of 89 procedures were included with 62 comprising the S/N-CHD group and 27 comprising the M/G-CHD group. Of the M/G-CHD patients, 13 had CHD of great complexity (including 6 single ventricle/Fontan and 2 atrial switch patients). Patients with M/G-CHD were older, had higher BMI, had higher incidence of ventricular dysfunction, and greater incidence of complex arrhythmias. Fluoroscopy-free ablation was achieved in 59% of M/G-CHD and 69% of S/N-CHD patients. Both groups had similar rates of acute procedural success, recurrence, and complications. Fluoroscopy was primarily used to visualize pre-existing transvenous leads and peripheral venous anomalies or to guide transbaffle/transseptal puncture. CONCLUSIONS: A fluoroscopy-free ablation approach is feasible, safe, and successful even in patients with M/G-CHD with comparable outcomes to those with S/N-CHD.

A 'holistic' sonographic view on congenital heart disease - How automatic reconstruction using fetal intelligent navigation echocardiography (FINE) eases the unveiling of abnormal cardiac anatomy part I: Right heart anomalies.

Attempting a comprehensive examination of the fetal heart remains challenging for unexperienced operators as it emphasizes the acquisition and documentation of sequential cross-sectional and sagittal views and inevitably results in diminished detection rates of fetuses affected by congenital heart disease. The introduction of four-dimensional spatio-temporal image correlation (4D STIC) technology facilitated a volumetric approach for thorough cardiac anatomic evaluation by the acquisition of cardiac 4D datasets. By analyzing and re-arranging of numerous frames according to their temporal event within the heart cycle, STIC allows visualization of cardiac structures as an endless cine loop sequence of a complete single cardiac cycle in motion. However, post-analysis with manipulation and repeated slicing of the volume usually requires experience and in-depth anatomic knowledge, which limits the widespread application of this advanced technique in clinical care and unfortunately leads to the underestimation of its diagnostic value to date. Fetal intelligent navigation echocardiography (FINE), a novel method that automatically generates and displays nine standard fetal echocardiographic views in normal hearts, has shown to be able to overcome these limitations. Very recent data on the detection of congenital heart defects (CHDs) using the FINE method revealed a high sensitivity and specificity of 98% and 93%, respectively. In this two-part manuscript, we focused on the performance of FINE in delineating abnormal anatomy of typical right and left heart lesions and thereby emphasized the educational potential of this technology for more than just teaching purposes. We further discussed recent findings in a pathophysiological and/or functional context.

Intracardiac Echocardiography to Guide Mapping and Ablation of Arrhythmias in Patients with Congenital Heart Disease.

Catheter ablation of arrhythmias in congenital heart disease can be a challenging undertaking with often complicated anatomic considerations. Understanding this anatomy and the prior surgical repairs is key to procedural planning and a successful outcome. Intracardiac echocardiography (ICE) adds complimentary real-time visualization of anatomy and catheter positioning along with other imaging modalities. In addition, ICE can visualize suture lines, baffles, and conduits from repaired congenital heart disease and forms a useful part of the toolkit required to deal with these complex arrhythmias.

Four-dimensional Multiphase Steady-State MRI with Ferumoxytol Enhancement: Early Multicenter Feasibility in Pediatric Congenital Heart Disease.

Background The value of MRI in pediatric congenital heart disease (CHD) is well recognized; however, the requirement for expert oversight impedes its widespread use. Four-dimensional (4D) multiphase steady-state imaging with contrast enhancement (MUSIC) is a cardiovascular MRI technique that uses ferumoxytol and captures all anatomic features dynamically. Purpose To evaluate multicenter feasibility of 4D MUSIC MRI in pediatric CHD. Materials and Methods In this prospective study, participants with CHD underwent 4D MUSIC MRI at 3.0 T or 1.5 T between 2014 and 2020. From a pool of 460 total studies, an equal number of MRI studies from three sites (n = 60) was chosen for detailed analysis. With use of a five-point scale, the feasibility of 4D MUSIC was scored on the basis of artifacts, image quality, and diagnostic confidence for intracardiac and vascular connections (n = 780). Respiratory motion suppression was assessed by using the signal intensity profile. Bias between 4D MUSIC and two-dimensional (2D) cine imaging was evaluated by using Bland-Altman analysis; 4D MUSIC examination duration was compared with that of the local standard for CHD. Results A total of 206 participants with CHD underwent MRI at 3.0 T, and 254 participants underwent MRI at 1.5 T. Of the 60 MRI examinations chosen for analysis (20 per site; median participant age, 14.4 months [interquartile range, 2.3-49 months]; 33 female participants), 56 (93%) had good or excellent image quality scores across a spectrum of disease complexity (mean score ± standard deviation: 4.3 ± 0.6 for site 1, 4.9 ± 0.3 for site 2, and 4.6 ± 0.7 for site 3; P < .001). Artifact scores were inversely related to image quality (r = -0.88, P < .001) and respiratory motion suppression (P < .001, r = -0.45). Diagnostic confidence was high or definite in 730 of 780 (94%) intracardiac and vascular connections. The correlation between 4D MUSIC and 2D cine ventricular volumes and ejection fraction was high (range of r = 0.72-0.85; P < .001 for all). Compared with local standard MRI, 4D MUSIC reduced the image acquisition time (44 minutes ± 20 vs 12 minutes ± 3, respectively; P < .001). Conclusion Four-dimensional multiphase steady-state imaging with contrast enhancement MRI in pediatric congenital heart disease was feasible in a multicenter setting, shortened the examination time, and simplified the acquisition protocol, independently of disease complexity. Clinical trial registration no. NCT02752191 © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Roest and Lamb in this issue.

A "holistic" sonographic view on congenital heart disease: How automatic reconstruction using fetal intelligent navigation echocardiography eases unveiling of abnormal cardiac anatomy part II-Left heart anomalies.

Volume ultrasound has been shown to provide valid complementary information on fetal anatomy. Four-dimensional assessment (4D) of the fetal cardiovascular system using spatial-temporal image correlation (STIC) allows for detailed examination of a highly complex organ from the early second trimester onward. There is compelling evidence that this technique harbors quite a number of diagnostic opportunities, but manual navigation through STIC volume datasets is highly operator dependent. In fact, STIC is not incorporated yet into daily practice. Application of the novel fetal intelligent navigation echocardiography (FINE) considerably simplifies fetal cardiac volumetric examinations. This automatic technique applied on cardiac volume datasets reportedly has both high sensitivity and specificity for the detection of congenital heart defects (CHDs). Part I reviewed current data regarding detection rates of CHDs and illustrated the additional value of an automatic approach in delineating cardiac anatomy exemplified by congenital lesions of the right heart. In part II of this pictorial essay, we focused on left heart anomalies and aimed to tabulate recent findings on the quantification of normal and abnormal cardiac anatomy.

First-Trimester Maternal Folic Acid Supplementation Reduced Risks of Severe and Most Congenital Heart Diseases in Offspring: A Large Case-Control Study.

Background Maternal folic acid supplementation (FAS) reduces the risk of neural tube defects in offspring. However, its effect on congenital heart disease (CHDs), especially on the severe ones remains uncertain. This study aimed to assess the individual and joint effect of first-trimester maternal FAS and multivitamin use on CHDs in offspring. Methods and Results This is a case-control study including 8379 confirmed CHD cases and 6918 controls from 40 healthcare centers of 21 cities in Guangdong Province, China. Adjusted odds ratios (aORs) of FAS and multivitamin use between CHD cases (overall and specific CHD phenotypes) and controls were calculated by controlling for parental confounders. The multiplicative interaction effect of FAS and multivitamin use on CHDs was estimated. A significantly protective association was detected between first-trimester maternal FAS and CHDs among offspring (aOR, 0.69; 95% CI, 0.62-0.76), but not for multivitamin use alone (aOR, 1.42; 95% CI, 0.73-2.78). There was no interaction between FAS and multivitamin use on CHDs (P=0.292). Most CHD phenotypes benefited from FAS (aORs ranged from 0.03-0.85), especially the most severe categories (ie, multiple critical CHDs [aOR, 0.16; 95% CI, 0.12-0.22]) and phenotypes (ie, single ventricle [aOR, 0.03; 95% CI, 0.004-0.21]). Conclusions First-trimester maternal FAS, but not multivitamin use, was substantially associated with lower risk of CHDs, and the association was strongest for the most severe CHD phenotypes. We recommend that women of childbearing age should supplement with folic acid as early as possible, ensuring coverage of the critical window for fetal heart development to prevent CHDs.

Position paper concerning the competence, performance and environment required for the practice of ablation in children and in congenital heart disease.

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations.

Regionalization of Congenital Heart Surgery in the United States.

The objective of this study is to simulate regionalization of congenital heart surgery (CHS) in the United States and assess the impact of such a system on travel distance and mortality. Patients ≤18 years of age who underwent CHS were identified in 2012 State Inpatient Databases. Operations were stratified by the Risk Adjustment for Congenital Heart Surgery, version 1 (RACHS-1) method, with high risk defined as RACHS-1 levels 4-6. Regionalization was simulated by progressive closure of hospitals, beginning with the lowest volume hospital. Patients were moved to the next closest hospital. Analyses were conducted (1) maintaining original hospital mortality rates and (2) estimating mortality rates based on predicted surgical volumes after absorbing moved patients. One hundred fifty-three hospitals from 36 states performed 1 or more operation (19,064 operations). With regionalization wherein, all hospitals performed >310 operations, 37 hospitals remained, from 12.5% to 17.4% fewer deaths occurred (83-116/666), and median patient travel distance increased from 38.5 to 69.6 miles (P < 0.01). When only high-risk operations were regionalized, 3.9-5.9% fewer deaths occurred (26-39/666), and the overall mortality rate did not change significantly. Regionalization of CHS in the United States to higher volume centers may reduce mortality with minimal increase in patient travel distance. Much of the mortality reduction may be missed if solely high-risk patients are regionalized.

Ultra-High-Density Activation Mapping to Aid Isthmus Identification of Atrial Tachycardias in Congenital Heart Disease.

OBJECTIVES: A new electroanatomic mapping system (Rhythmia, Boston Scientific, Marlborough, Massachusetts) using a 64-electrode mapping basket is now available; we systematically assessed its use in complex congenital heart disease (CHD). BACKGROUND: The incidence of atrial arrhythmias post-surgery for CHD is high. Catheter ablation has emerged as an effective treatment, but is hampered by limitations in the mapping system's ability to accurately define the tachycardia circuit. METHODS: Mapping and ablation data of 61 patients with CHD (35 males, age 45 ± 14 years) from 8 tertiary centers were reviewed. RESULTS: Causes were as follows: Transposition of Great Arteries (atrial switch) (n = 7); univentricular physiology (Fontans) (n = 8); Tetralogy of Fallot (n = 10); atrial septal defect (ASD) repair (n = 15); tricuspid valve (TV) anomalies (n = 10); and other (n = 11). The total number of atrial arrhythmias was 86. Circuits were predominantly around the tricuspid valve (n = 37), atriotomy scar (n = 10), or ASD patch (n = 4). Although the majority of peri-tricuspid circuits were cavo-tricuspid-isthmus dependent (n = 30), they could follow a complex route between the annulus and septal resection, ASD patch, coronary sinus, or atriotomy. Immediate ablation success was achieved in all but 2 cases; with follow-up of 12 ± 8 months, 7 patients had recurrence. CONCLUSIONS: We demonstrate the feasibility of the basket catheter for mapping complex CHD arrhythmias, including with transbaffle and transhepatic access. Although the circuits often involve predictable anatomic landmarks, the precise critical isthmus is often difficult to predict empirically. Ultra-high-density mapping enables elucidation of circuits in this complex anatomy and allows successful treatment at the isthmus with a minimal lesion set.

[Hypnosis in transesophageal echocardiography. The experience in a Pediatric Cardiology and Congenital Heart Disease Unit]. / L'ipnosi nell'ecocardiografia transesofagea. L'esperienza di un Centro di Cardiologia Pediatrica e delle Cardiopatie Congenite.

BACKGROUND: The purpose of this study was to use hypnosis in patients with congenital heart disease undergoing transesophageal echocardiography (TEE). METHODS: From January 2016 to July 2017, 50 adult patients undergoing TEE were randomly assigned to two groups: TEE in hypnosis (n = 23), TEE in sedation (n = 27). Vital parameters (heart rate [HR], blood pressure [BP], oxygen saturation [SO2] before, during and after the procedure) and drug administration were recorded. The State-Trait Anxiety Inventory was performed before and after TEE, the memory and experience of TEE through a structured interview were assessed. RESULTS: All patients in the hypnosis group performed TEE without any sedation. As for anxiety before TEE, no significant differences were observed between groups; after TEE all patients were less anxious than at the beginning (p<0.001) with a greater decrease in patients of the hypnosis group (p<0.001). Before TEE, there were no significant differences also in HR, BP and SO2. During TEE in both groups a similar increase in HR and BP was found (p<0.001), whereas SO2 values remained stable. In the responses to the structured interview, 94% of patients in the sedation group remembered everything vs 36% of the hypnosis group (p<0.05). No differences were found in the other answers between the two groups. CONCLUSIONS: Hypnosis in TEE is useful to improve the emotional experience of patients with congenital heart disease.

An initial experience of high-density mapping-guided ablation in a cohort of patients with adult congenital heart disease.

AIMS: In the management of both ventricular and supraventricular tachycardia in patients with congenital heart disease (CHD) catheter ablation has now been recognized as one of the mainstays. METHODS AND RESULTS: We review our initial experience of using the Rhythmia mapping system in a cohort of 12 adult CHD patients presenting with multiple arrhythmia substrates. A total of 78 arrhythmia maps were attempted in a total of 15 procedures, but possible due to the dilatation of the target chamber only 44% of maps were able to reconstruct the entire arrhythmia. All patients underwent pre-procedure 3D imaging (either cardiac magnetic resonance or computed tomography), but image integration was suboptimal. A median of two maps per patient were finally analysed and acquisition took in median 22 min with a median number of 12 574 (8230-18 167) mapping points. Procedural data with a total duration amounting to in median 285 (194-403) min, with a median total fluoroscopy exposure of 7.5 (5.2-10.7) min. After a median of 1.5 procedures [median of 12 (8-16 months)], nine patients remained in stable sinus rhythm or atrial paced rhythm, while three patients had further sustained recurrences. One of these passed away in end-staged heart failure. CONCLUSION: This initial experience of using high-density mapping for arrhythmia management in patients with CHD allowed rapid acquisition of multiple maps with high accuracy to identify surgical scars and fibrosis, however, it was limited by large atrial volumes and a high percentage of incomplete maps resulting in modest clinical success.

State-of-the-art multimodality approach to assist ablations in complex anatomies-From 3D printing to virtual reality.

Imaging of the heart anatomy plays an important role, especially in catheter ablation for the treatment of arrhythmias in adults with congenital heart disease (ACHD). We present a comprehensive overview of the current state-of-the-art modalities available to plan and guide catheter ablation in an ACHD patient. In addition to the clinical assessment of the computed tomography and the integration of 3D reconstructions into the electroanatomical mapping system, 3D printing and virtual reality assessment showed its value in preprocedural planning of the intervention.

Use of autologous umbilical cord blood transfusion in neonates undergoing surgical correction of congenital cardiac defects: A pilot study.

BACKGROUND: Blood transfusion requirement during neonatal open heart surgeries is universal. Homologous blood transfusion (HBT) in pediatric cardiac surgery is used most commonly for priming of cardiopulmonary bypass (CPB) system and for postoperative transfusion. To avoid the risks associated with HBT in neonates undergoing cardiac surgery, use of autologous umbilical cord blood (AUCB) transfusion has been described. We present our experience with the use of AUCB for neonatal cardiac surgery. DESIGNS AND METHODS: Consecutive neonates scheduled to undergo cardiac surgery for various cardiac diseases who had a prenatal diagnosis made on the basis of a fetal echocardiography were included in this prospective observational study. After a vaginal delivery or a cesarean section, UCB was collected from the placenta in a 150-mL bag containing 5 mL of citrate-phosphate-dextrose-adenine-1 solution. The collected bag with 70-75 mL cord blood was stored at 2°C-6°C and tested for blood grouping and infections after proper labeling. The neonate's autologous cord blood was used for postcardiac surgery blood transfusion to replace postoperative blood loss. RESULTS: AUCB has been used so far at our institute in 10 neonates undergoing cardiac surgery. The donor exposure in age and type of cardiac surgery-matched controls showed that the neonates not receiving autologous cord blood had a donor exposure to 5 donors (2 packed red blood cells [PRBCs], including 1 for CPB prime and 1 for postoperative loss, 1 fresh frozen plasma, 1 cryoprecipitate, and 1 platelet concentrate) compared to 1 donor for the AUCB neonate (1 PRBC for the CPB prime). Postoperative blood loss was similar in both the groups of matched controls and study group. Values of hemoglobin, total leukocyte count, platelet counts, and blood gas parameters were also similar. CONCLUSIONS: Use of AUCB for replacement of postoperative blood loss after neonatal cardiac surgery is feasible and reduces donor exposure to the neonate. Its use, however, requires a prenatal diagnosis of a cardiac defect by fetal echo and adequate logistic and psychological support from involved clinicians and the blood bank.

Probabilistic tractography-based thalamic parcellation in healthy newborns and newborns with congenital heart disease.

BACKGROUND: Given the central role of the thalamus in motor, sensory, and cognitive development, methods to study emerging thalamocortical connectivity in early infancy are of great interest. PURPOSE: To determine the feasibility of performing probabilistic tractography-based thalamic parcellation (PTbTP) in typically developing (TD) neonates and to compare the results with a pilot sample of neonates with congenital heart disease (CHD). STUDY TYPE: Institutional Review Board (IRB)-approved cross-sectional study. MODEL: We prospectively recruited 20 TD neonates and five CHD neonates (imaged preoperatively). FIELD STRENGTH/SEQUENCE: MRI was performed at 3.0T including diffusion-weighted imaging (DWI) and 3D magnetization prepared rapid gradient-echo (MPRAGE). ASSESSMENT: A radiologist and trained research assistants segmented the thalamus and seven cortical targets for each hemisphere. Using the thalami as seeds and the cortical labels as targets, FSL library tools were used to generate probabilistic tracts. A Hierarchical Dirichlet Process algorithm was then used for clustering analysis. A radiologist qualitatively assessed the results of clustering. Quantitative analyses were also performed. STATISTICAL TESTS: We summarized the demographic data and results of clustering with descriptive statistics. Linear regressions covarying for gestational age were used to compare groups. RESULTS: In 17 of 20 TD neonates, we identified five connectivity-determined clusters, which correlate with known thalamic nuclei and subnuclei. In four neonates with CHD we observed a spectrum of abnormalities including fewer and disorganized clusters or small supernumerary clusters (up to seven per thalamus). After covarying for differences in corrected gestational age (cGA), the fractional anisotropy (FA), volume, and normalized thalamic volume were significantly lower in CHD neonates (P < 0.01). DATA CONCLUSIONS: Using PTbTP clusters, correlating well with the location and connectivity of known thalamic nuclei, were identified in TD neonates. Differences in thalamic clustering outputs were identified in four neonates with CHD, raising concern for disordered thalamic connectivity. PTbTP is feasible in TD and CHD neonates. Preliminary findings suggest the prenatal origins of altered connectivity in CHD. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 4 J. Magn. Reson. Imaging 2018;47:1626-1637.

Supplementation with the Methyl Donor Betaine Prevents Congenital Defects Induced by Prenatal Alcohol Exposure.

BACKGROUND: Despite decades of public education about dire consequences of prenatal alcohol exposure (PAE), drinking alcohol during pregnancy remains prevalent. As high as 40% of live-born infants exposed to alcohol during gestation and diagnosed with fetal alcohol syndrome have congenital heart defects that can be life-threatening. In animal models, the methyl donor betaine, found in foods such as wheat bran, quinoa, beets, and spinach, ameliorated neurobehavioral deficits associated with PAE, but effects on heart development are unknown. METHODS: Previously, we modeled a binge drinking episode during the first trimester in avian embryos. Here, we investigated whether betaine could prevent adverse effects of alcohol on heart development. Embryos exposed to ethanol (EtOH) with and without an optimal dose of betaine (5 µM) were analyzed at late developmental stages. Cardiac morphology parameters were rapidly analyzed and quantified using optical coherence tomography. DNA methylation at early stages was detected by immunofluorescent staining for 5-methylcytosine in sections of embryos treated with EtOH or cotreated with betaine. RESULTS: Compared to EtOH-exposed embryos, betaine-supplemented embryos had higher late-stage survival rates and fewer gross head and body defects than seen after alcohol exposure alone. Betaine also reduced the incidence of late-stage cardiac defects such as absent vessels, abnormal atrioventricular (AV) valves, and hypertrophic ventricles. Furthermore, betaine cotreatment brought measurements of great vessel diameters, interventricular septum thickness, and AV leaflet volumes in betaine-supplemented embryos close to control values. Early-stage 5-methycytosine staining revealed that DNA methylation levels were reduced by EtOH exposure and normalized by co-administration with betaine. CONCLUSIONS: This is the first study demonstrating efficacy of the methyl donor betaine in alleviating cardiac defects associated with PAE. These findings highlight the therapeutic potential of low-concentration betaine doses in mitigating PAE-induced birth defects and have implications for prenatal nutrition policies, especially for women who may not be responsive to folate supplementation.

A congenital diverticulum of the left ventricular apex manifested by stroke and recurrent ventricular tachycardia.

Ventricular outpouchings include acquired abnormalities (aneurysms and pseudoaneurysms) and congenital ventricular diverticula (CVD). CVD represent rare cardiac pathologies. Although CVD is often associated with other cardiac and extracardiac congenital anomalies, it can also be incidentally observed in otherwise healthy subjects. CVD may lead to significant morbidity and even have lethal consequences. We describe a case of arrhythmogenic left ventricle (LV) apical CVD revealed by cardiac magnetic resonance imaging (CMRI) after being initially overlooked by echocardiography. The paper includes the review of the literature also. This clinical case highlights the possible association of this pathology with recurrent ventricular tachycardia and stroke, and illustrates the importance of multimodal imaging approach in differential diagnosis.