Continuous Fetal Cardiac Monitoring During Fetoscopic Myelomeningocele Repair and Relationship to Spectral Doppler Changes.

INTRODUCTION: No evidence-based protocols exist for fetal cardiac monitoring during fetoscopic myelomeningocele (fMMC) repair and intraprocedural spectral Doppler data is limited. We determined the feasibility of continuous fetal echocardiography during fMMC repair and correlated Doppler changes with qualitative fetal cardiac function during each phase of fMMC repair. METHODS: Patients undergoing fMMC repair had continuous fetal echocardiography interpreted in real-time by pediatric cardiology. Fetal data included fetal heart rate (FHR), qualitative cardiac function, mitral and tricuspid valve inflow waveforms, and umbilical artery (UA), umbilical vein (UV), ductus arteriosus (DA) and ductus venosus (DV) Dopplers. RESULTS: UA abnormalities were noted in 14/25 patients, UV abnormalities were observed in two patients, and DV and DA abnormalities were each noted in 4 patients. Qualitative cardiac function was normal for all patients with the exception of one with isolated left ventricular dysfunction during myofascial flap creation, concurrent with an abnormal UA flow pattern. All abnormalities resolved by the first postoperative day. CONCLUSIONS: Continuous fetal echocardiography was feasible during all fMMC repairs. Spectral Doppler changes in the UA were common during fMMC procedures but qualitative cardiac dysfunction was rare. Abnormalities in the UV, DV and DA Dopplers, FHR, and cardiac function were less common findings.

Variation in prenatal surveillance and management of anti-SSA/Ro autoantibody positive pregnancies.

OBJECTIVE: To describe international surveillance and treatment strategies for managing anti-SSA/Ro autoantibody positive pregnancies. STUDY DESIGN: An electronic REDCap questionnaire was distributed to Fetal Heart Society and North American Fetal Therapy Network members which queried institution-based risk stratification, surveillance methods/frequency, conduction abnormality treatments, and postnatal anti-SSA/Ro pregnancy assessment. RESULTS: 101 responses from 59 centers (59% US, 17% international) were collected. Most (79%) do not risk stratify pregnancies by anti-SSA/Ro titer; those that do use varied cutoff values. Many pregnant rheumatology patients are monitored for cardiac abnormalities regardless of maternal anti-SSA/Ro status. Surveillance strategies were based on maternal factors (anti-SSA/Ro status 85%, titer 25%, prior affected child 79%) and monitoring durations varied. Most respondents treat 2° and 3° fetal atrioventricular block, commonly with dexamethasone and/or IVIG. CONCLUSIONS: Wide variation exists in current fetal cardiac surveillance and treatment for anti-SSA/Ro autoantibody positive pregnancies, highlighting the need for evidence-based protocols to optimize care.

Prenatal Predictors for Pulmonary Balloon Valvuloplasty in the Newborn.

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1-58) days and 11 (22%) underwent outpatient PBV at 51.8 (11-174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02-94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS.

Prospective Evaluation of High Titer Autoantibodies and Fetal Home Monitoring in the Detection of Atrioventricular Block Among Anti-SSA/Ro Pregnancies.

OBJECTIVE: This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti-SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second-degree atrioventricular block (AVB). METHODS: Women with anti-SSA/Ro autoantibodies by commercial testing were stratified into high and low anti-52-kD and/or 60-kD SSA/Ro titers applying at-risk thresholds defined by previous evaluation of AVB pregnancies. The high-titer group performed fetal heart rate and rhythm monitoring (FHRM) thrice daily and weekly/biweekly echocardiography from 17-26 weeks. Abnormal FHRM prompted urgent echocardiography to identify AVB. RESULTS: Anti-52-kD and/or 60-kD SSA/Ro met thresholds for monitoring in 261 of 413 participants (63%); for those, AVB frequency was 3.8%. No cases occurred with low titers. The incidence of AVB increased with higher levels, reaching 7.7% for those in the top quartile for anti-60-kD SSA/Ro, which increased to 27.3% in those with a previous child who had AVB. Based on levels from 15 participants with paired samples from both an AVB and a non-AVB pregnancy, healthy pregnancies were not explained by decreased titers. FHRM was considered abnormal in 45 of 30,920 recordings, 10 confirmed AVB by urgent echocardiogram, 7 being second-degree AVB, all <12 hours from normal FHRM and within another 0.75 to 4 hours to echocardiogram. The one participant with second/third-degree and two participants with third-degree AVB were diagnosed by urgent echocardiogram >17 to 72 hours from an FHRM. Surveillance echocardiograms detected no AVB when the preceding interval FHRM recordings were normal. CONCLUSION: High-titer antibodies are associated with an increased incidence of AVB. Anti-SSA/Ro titers remain stable over time and do not explain the discordant recurrence rates, suggesting that other factors are required. Fetal heart rate and rhythm (FHRM) with results confirmed by a pediatric cardiologist reliably detects conduction abnormalities, which may reduce the need for serial echocardiograms.

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD. METHODS: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test. RESULTS: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m2 (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]). CONCLUSION: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.

The Resident Readiness Survey: A National Process for Program Directors to Provide Standardized Feedback to Medical Schools About Their Graduates.

Background The process for program directors (PDs) to provide feedback to medical schools about their graduates' readiness for postgraduate year 1 (PGY-1) training is burdensome and does not generate national benchmarking data. Objective The Association of American Medical Colleges (AAMC) tested the feasibility of administering a standardized Resident Readiness Survey (RRS) to PDs nationally about their PGY-1 residents' preparedness for residency. Methods In 2020 and 2021, the AAMC invited PDs via email to complete RRSs for their PGY-1s who graduated from participating schools; the AAMC provided schools with reports of identified RRS data for their graduates. Outcome measures included school participation rates, PD response rates, PGY-1s' coverage rates (RRSs completed/RRSs PDs invited to complete), RRS completion time (time-stamp difference: RRS opening-submission), and participating schools' feedback about the process collected via AAMC evaluation questionnaires sent to school leaders. Chi-square tested significance of differences between proportions. Results School participation increased from 43.8% (2020: 77 of 176) to 72.4% (2021: 131 of 181). PD response rates, similar in both years overall (2020: 1786 of 2847 [62.7%]; 2021: 2107 of 3406 [61.9%]; P=.48), varied by specialty (P<.001; range 65 of 154 [42.2%], neurology; 109 of 136 [80.1%], internal medicine-pediatrics, both years combined). PGY-1s' coverage rates were similar (P=.21) in 2020 (5567 of 10 712 [52.0%]) and 2021 (9688 of 18 372 [52.7%]). RRS completion time averaged less than 3 minutes. Numerous school leaders reported that RRS data stimulated new, or supported ongoing, curricular quality improvement. Conclusions Administration of a standardized RRS to PDs nationally about PGY-1s preparedness for residency is feasible and will continue.

Public and Population Health in U.S. Medical Education: A Review of Guidance in Extraordinary Times.

ABSTRACT: Generations of medical educators have recommended including public and population health (PPH) content in the training of U.S. physicians. The COVID-19 pandemic, structural racism, epidemic gun violence, and the existential threats caused by climate change are currently unsubtle reminders of the essential nature of PPH in medical education and practice. To assess the state of PPH content in medical education, the authors reviewed relevant guidance, including policies, standards, and recommendations from national bodies that represent and oversee medical education for physicians with MD degrees.Findings confirm that guidance across the medical education continuum, from premedical education to continuing professional development, increasingly includes PPH elements that vary in specificity and breadth. Graduate medical education policies present the most comprehensive approach in both primary care and subspecialty fields. Behavioral, quantitative, social, and systems sciences are represented, although not uniformly, in guidance for every phase of training. Quantitative PPH skills are frequently presented in the context of research, but not in relation to the development of population health perspectives (e.g., evidence-based medicine, quality improvement, policy development). The interdependence between governmental public health and medical practice, environmental health, and the impact of structural racism and other systems of oppression on health are urgent concerns, yet are not consistently or explicitly included in curricular guidance. To prepare physicians to meet the health needs of patients and communities, educators should identify and address gaps and inconsistencies in PPH curricula and related guidance.Re-examinations of public health and health care systems in the wake of the COVID-19 pandemic support the importance of PPH in physician training and practice, as physicians can help to bridge clinical and public health systems. This review provides an inventory of existing guidance (presented in the appendices) to assist educators in establishing PPH as an essential foundation of physician training and practice.

Physicians' continuing medical education activities and satisfaction with their ability to stay current in medical information and practice: A cross-sectional study.

Background and Aims: Little is known about physicians' approaches to continuing medical education (CME) for continuing professional development despite the rapid evolution of CME offerings. We sought to identify the extent to which demographic, career, and experiential CME-activity variables were independently associated with physicians' satisfaction with their ability to stay current on medical information and practice. Methods: Using the 2019 Association of American Medical Colleges' National Sample Survey of Physicians data, we ran multivariable logistic regression models examining demographic, career, and experiential (participation in 11 CME activities in the past year) variables for their associations with physicians' satisfaction (satisfied vs. not satisfied/neutral) with their ability to stay current. Results: Of 5926 respondents, 90% (5341/5926) were satisfied with their ability to stay current. Significant (each two-sided p < 0.05) predictors of respondents who were satisfied included (among others) a surgery specialty (vs. primary care; adjusted odds ratio [AOR] = 1.41, 95% confidence interval [CI] = 1.06-1.88), an academic affiliation (vs. none; AOR = 1.35, 95% CI = 1.10-1.66), and participation (vs. no participation) in each of professional meetings (AOR = 1.31, 95% CI = 1.07-1.60) and journal-based CME (AOR = 1.29, 95% CI = 1.07-1.56). Respondents who self-identified as a race/ethnicity underrepresented in medicine (vs. white; AOR = 0.68, 95% CI = 0.48-0.97) and were between ages 40 and 49 years (vs. 50-59; AOR = 0.74; 95% CI = 0.58-0.94) were less likely to be satisfied. Gender, board certification status, and medical degree type did not independently predict satisfaction (each p > 0.05). Conclusion: We observed independent associations between physicians' satisfaction with their ability to stay current in medical information and practice and each specialty, academic affiliation, race/ethnicity, age, and CME activity type (for 2 of 11 examined). Findings may be relevant to organizations and institutions designing and implementing CME activities in the current context of COVID-19 pandemic-related in-person activity limitations and can inform targeted interventions addressing differences in the satisfaction we observed to better support physicians' CME.

Amniotic Fluid microRNA in Severe Twin-Twin Transfusion Syndrome Cardiomyopathy-Identification of Differences and Predicting Demise.

Twin-twin transfusion syndrome (TTTS) is a rare but serious cause of fetal cardiomyopathy with poorly understood pathophysiology and challenging prognostication. This study sought a nonbiased, comprehensive assessment of amniotic fluid (AF) microRNAs from TTTS pregnancies and associations of these miRNAs with clinical characteristics. For the discovery cohort, AF from ten fetuses with severe TTTS cardiomyopathy were selected and compared to ten normal singleton AF. Array panels assessing 384 microRNAs were performed on the discovery cohort and controls. Using a stringent q < 0.0025, arrays identified 32 miRNAs with differential expression. Top three microRNAs were miR-99b, miR-370 and miR-375. Forty distinct TTTS subjects were selected for a validation cohort. RT-PCR targeted six differentially-expressed microRNAs in the discovery and validation cohorts. Expression differences by array were confirmed by RT-PCR with high fidelity. The ability of these miRNAs to predict clinical differences, such as cardiac findings and later demise, was evaluated on TTTS subjects. Down-regulation of miRNA-127-3p, miRNA-375-3p and miRNA-886 were associated with demise. Our results indicate AF microRNAs have potential as a diagnostic and prognostic biomarker in TTTS. The top microRNAs have previously demonstrated roles in angiogenesis, cardiomyocyte stress response and hypertrophy. Further studies of the mechanism of actions and potential targets is warranted.

Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD. METHODS: A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019. The left ventricle was assessed in the first 48 hours after birth using two-dimensional, Doppler-derived, six-segment global and segmental longitudinal strain and circumferential strain (CS) and dyssynchrony indices (the SD of time-to-peak strain and a novel global dyssynchrony index [DI], calculated as [peak segmental average - peak global average]/peak segmental average). RESULTS: Neonates with EA/TVD demonstrated reduced combined cardiac index (4.2 ± 1.5 L/min/m2 vs 6.5 ± 2.2 L/min/m2 in control subjects, P < .001), impaired LV CS (-15.4 ± 6.9 vs -26.2 ± 5.8, P < .001), and increased circumferential dyssynchrony (CS DI 0.20 ± 0.16 vs 0.09 ± 0.04 [P = .019]; SD of time-to-peak CS 63 ± 25 vs 40 ± 15 [P = .003]). Transplantation-free survival occurred in 20 of 32 patients (63%) at 6 months. Increased CS DI and absence of pulmonary valve flow (PVF) were most predictive of mortality; CS DI > 0.2 was associated with 25% survival in subjects without PVF, whereas all patients with CS DI < 0.1 survived. CONCLUSIONS: In neonates with EA/TVD and absence of PVF, there is abnormal LV deformation and compromised cardiac output in association with increased dyssynchrony. Increased CS DI is associated with increased risk for mortality in EA/TVD with no forward PVF.

Consensus-Driven Priorities for Firearm Injury Education Among Medical Professionals.

PURPOSE: Firearm injury is a leading cause of morbidity and mortality in the United States. However, many medical professionals currently receive minimal or no education on firearm injury or its prevention. The authors sought to convene a diverse group of national experts in firearm injury epidemiology, injury prevention, and medical education to develop consensus on priorities to inform the creation of learning objectives and curricula for firearm injury education for medical professionals. METHOD: In 2019, the authors convened an advisory group that was geographically, demographically, and professionally diverse, composed of 33 clinicians, researchers, and educators from across the United States. They used the nominal group technique to achieve consensus on priorities for health professions education on firearm injury. The process involved an initial idea-generating phase, followed by a round-robin sharing of ideas and further idea generation, facilitated discussion and clarification, and the ranking of ideas to generate a prioritized list. RESULTS: This report provides the first national consensus guidelines on firearm injury education for medical professionals. These priorities include a set of crosscutting, basic, and advanced learning objectives applicable to all contexts of firearm injury and all medical disciplines, specialties, and levels of training. They focus on 7 contextual categories that had previously been identified in the literature: 1 category of general priorities applicable to all contexts and 6 categories of specific contexts, including intimate partner violence, mass violence, officer-involved shootings, peer (nonpartner) violence, suicide, and unintentional injury. CONCLUSIONS: Robust, data- and consensus-driven priorities for health professions education on firearm injury create a pathway to clinician competence and self-efficacy. With an improved foundation for curriculum development and educational program-building, clinicians will be better informed to engage in a host of firearm injury prevention initiatives both at the bedside and in their communities.

Successful Treatment of Placental Chorangiomas by Ultrasound-Guided Percutaneous in utero Microcoil Embolization.

Placental chorangiomas can cause a high-output fetal state and increase neonatal morbidity and mortality. There is a paucity of data published describing the optimal treatment of these cases, and methods for occlusion to date include placement of vascular clips, bipolar cautery, injection of alcohol or surgical glue, interstitial laser, and microcoil embolization. We report 2 cases of prenatally diagnosed chorangiomas that caused a high-output fetal state and were successfully treated with microcoil embolization. This case series describes our technique and supports microcoil embolization as a potentially safe and effective antenatal treatment option in symptomatic chorangiomas.

Discontinuation of the USMLE Step 2 Clinical Skills Examination: Studying the Past to Define the Future.

The United States Medical Licensing Examination (USMLE) Step 2 Clinical Skills (CS) was discontinued in January 2021, marking a significant milestone in assessment of clinical skills. In this commentary, the authors trace the history of the Step 2 CS exam-beginning with its early roots in the 1960s up to its discontinuation in 2021. In this new era, the medical education community is replete with opportunities for advancing methodology and content associated with clinical skills assessment. The authors propose 3 main lessons gleaned from this rich history and modern evolution, which are aimed at defining a future that includes creative collaboration toward development of comprehensive, equitable, student-focused, and patient-centered clinical performance assessment. First, as it has done throughout history, the medical education community should continue to innovate, collaborate, and improve upon methods of clinical skills assessment. Second, medical educators should continue to shift to more complex and student-driven approaches of assessment, that is, assessments that provide an unstructured environment, are realistic with respect to the natural conditions, and do not limit students to lists of options or force them to take a certain path of reasoning. Third, medical educators should continue to rethink the role of assessment and ensure that all assessments, regardless of stakes or type, provide sufficient feedback for the student to identify areas of strength and weakness.

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow-up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1-30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Impact of Socioeconomic Status, Race and Ethnicity, and Geography on Prenatal Detection of Hypoplastic Left Heart Syndrome and Transposition of the Great Arteries.

BACKGROUND: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. METHODS: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. RESULTS: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. CONCLUSIONS: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Fetal Unguarded Mitral Valve Orifice, Aortic Atresia, and Severe Left Heart Enlargement.

Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with normal segmental cardiac anatomy, severe left ventricular dilatation and dysfunction, aortic atresia, and atrial flutter. (Level of Difficulty: Advanced.).