Peripartum anesthetic management in patients with Ebstein anomaly: a case series.

Ebstein anomaly is a rare congenital cardiac condition associated with displacement and incompetence of the tricuspid valve. Peripartum anesthetic management can be particularly challenging since these patients are at high risk of cardiac failure and tachyarrhythmias. Risk stratification is important since it helps to identify high-risk patients who should deliver at a tertiary care center where a multidisciplinary team (obstetrics, cardiology, anesthesiology, and neonatology) is immediately available. We describe the peripartum anesthetic management of 9 patients with Ebstein anomaly who underwent 12 deliveries at our institution. All patients tolerated neuraxial anesthesia and analgesia well. No maternal or fetal deaths occurred.

Screening for peripartum cardiomyopathies using artificial intelligence in Nigeria (SPEC-AI Nigeria): Clinical trial rationale and design.

BACKGROUND: Artificial intelligence (AI), and more specifically deep learning, models have demonstrated the potential to augment physician diagnostic capabilities and improve cardiovascular health if incorporated into routine clinical practice. However, many of these tools are yet to be evaluated prospectively in the setting of a rigorous clinical trial-a critical step prior to implementing broadly in routine clinical practice. OBJECTIVES: To describe the rationale and design of a proposed clinical trial aimed at evaluating an AI-enabled electrocardiogram (AI-ECG) for cardiomyopathy detection in an obstetric population in Nigeria. DESIGN: The protocol will enroll 1,000 pregnant and postpartum women who reside in Nigeria in a prospective randomized clinical trial. Nigeria has the highest reported incidence of peripartum cardiomyopathy worldwide. Women aged 18 and older, seen for routine obstetric care at 6 sites (2 Northern and 4 Southern) in Nigeria will be included. Participants will be randomized to the study intervention or control arm in a 1:1 fashion. This study aims to enroll participants representative of the general obstetric population at each site. The primary outcome is a new diagnosis of cardiomyopathy, defined as left ventricular ejection fraction (LVEF) < 50% during pregnancy or within 12 months postpartum. Secondary outcomes will include the detection of impaired left ventricular function (at different LVEF cut-offs), and exploratory outcomes will include the effectiveness of AI-ECG tools for cardiomyopathy detection, new diagnosis of cardiovascular disease, and the development of composite adverse maternal cardiovascular outcomes. SUMMARY: This clinical trial focuses on the emerging field of cardio-obstetrics and will serve as foundational data for the use of AI-ECG tools in an obstetric population in Nigeria. This study will gather essential data regarding the utility of the AI-ECG for cardiomyopathy detection in a predominantly Black population of women and pave the way for clinical implementation of these models in routine practice. TRIAL REGISTRATION: Clinicaltrials.gov: NCT05438576.

Management of Complicated Monochorionic Twin Gestations: An Evidence-Based Protocol.

IMPORTANCE: Monochorionic (MC) twins are hemodynamically connected by vascular anastomoses within the single shared placenta. The transfer of fluid or blood from one fetus to the other may result in development of pathologic complications, such as twin-twin transfusion syndrome, twin anemia polycythemia sequence, selective intrauterine growth restriction, and twin reversed arterial perfusion sequence. Monoamniotic gestations, which comprise a small fraction of MC pregnancies, can also present with unique challenges, particularly antepartum umbilical cord entanglement. All these complications carry a high risk of fetal morbidity and mortality if not recognized and managed in a timely fashion. OBJECTIVE: The purpose of this article is to review evidence-based management of complicated MC twin gestations and propose a standardized approach to surveillance. EVIDENCE ACQUISITION: Monochorionic gestations account for the majority of complications that occur in twin pregnancies; however, there is unclear evidence on the appropriate surveillance for and management of specific complications associated with these pregnancies. RESULTS: This article summarizes management for each specific type of MC complication in a structured and clear manner. CONCLUSIONS: Early pregnancy ultrasound, ideally between 10 and 13 weeks' gestation, is critical for the diagnosis and characterization of twin pregnancies. To improve outcomes for MC twins, appropriate fetal surveillance should be initiated at 16 weeks' gestation and continued until delivery.

Antisynthetase Syndrome with Severe Interstitial Lung Disease in Pregnancy.

Antisynthetase syndrome is a rare multisystem autoimmune disorder which clinically manifests with myositis, arthritis, interstitial lung disease, Raynaud phenomenon, and skin hyperkeratosis. Lung involvement represents the most severe form of disease and has rarely been reported in pregnancy. We present the case of a 22-year-old woman with antisynthetase syndrome and severe restrictive pulmonary disease who experienced a successful pregnancy and delivery. We discuss anesthetic considerations and highlight the importance of a multidisciplinary team approach in caring for parturients with multifactorial medical conditions.

Timing of delivery with coronavirus disease 2019 pneumonia requiring intensive care unit admission.

Approximately 4% of pregnant patients with coronavirus disease 2019 require intensive care unit admission. Given the practical implications of advanced ventilatory and circulatory support techniques, urgent or emergent delivery for nonreassuring fetal status frequently presents a logistical impossibility. This article proposes a protocol for obstetrical management of patients in these situations, emphasizing coordinated preparation among obstetrical, anesthesiology, and intensivist teams for planned preterm delivery at gestational ages when neonatal outcomes are likely to be favorable.

Detecting cardiomyopathies in pregnancy and the postpartum period with an electrocardiogram-based deep learning model.

AIMS: Cardiovascular disease is a major threat to maternal health, with cardiomyopathy being among the most common acquired cardiovascular diseases during pregnancy and the postpartum period. The aim of our study was to evaluate the effectiveness of an electrocardiogram (ECG)-based deep learning model in identifying cardiomyopathy during pregnancy and the postpartum period. METHODS AND RESULTS: We used an ECG-based deep learning model to detect cardiomyopathy in a cohort of women who were pregnant or in the postpartum period seen at Mayo Clinic. Model performance was evaluated using the area under the receiver operating characteristic curve (AUC), accuracy, sensitivity, and specificity. We compared the diagnostic probabilities of the deep learning model with natriuretic peptides and a multivariable model consisting of demographic and clinical parameters. The study cohort included 1807 women; 7%, 10%, and 13% had left ventricular ejection fraction (LVEF) of 35% or less, <45%, and <50%, respectively. The ECG-based deep learning model identified cardiomyopathy with AUCs of 0.92 (LVEF ≤ 35%), 0.89 (LVEF < 45%), and 0.87 (LVEF < 50%). For LVEF of 35% or less, AUC was higher in Black (0.95) and Hispanic (0.98) women compared to White (0.91). Natriuretic peptides and the multivariable model had AUCs of 0.85 to 0.86 and 0.72, respectively. CONCLUSIONS: An ECG-based deep learning model effectively identifies cardiomyopathy during pregnancy and the postpartum period and outperforms natriuretic peptides and traditional clinical parameters with the potential to become a powerful initial screening tool for cardiomyopathy in the obstetric care setting.

Detection of distal ureteral stones in pregnancy using transvaginal ultrasound.

AIMS: To determine the performance of transvaginal ultrasound for the visualization of distal ureteral stones in pregnant patients with renal colic and to evaluate the diagnostic value of secondary findings suggestive of obstructing ureteral stone disease. METHODS: We retrospectively identified 129 pregnant patients with a total of 142 encounters with both abdominal and transvaginal ultrasound. Ultrasound images for each patient were reviewed recording the presence of stone with location, hydronephrosis, resistive indices (RI), and status of the ureteral jets. Patients were subcategorized into two groups based on the visualization of distal ureteral stone. RESULTS: The transvaginal technique identified 94% (N = 16/17) of sonographically detected stones in the distal ureter/urethra, while the transabdominal technique identified 29% (N = 5/17). The combined imaging for initial assessment of renal colic in pregnancy demonstrated a sensitivity of 89%, specificity 100%, and negative predictive value (NPV) of 98%. The frequency of hydronephrosis was statistically greater in the visualized stone group (94% vs 51%). Mean RI was identical in both groups however the delta RI was significantly elevated in those patients with distal ureteral stones with a mean delta RI value of 0.05. The rate of absence of ureteral jets was not statistically significant. CONCLUSION: The present data would suggest a utility of transvaginal ultrasound for the evaluation of the pregnant patient with 94% of distal stones being detected transvaginal versus 29% transabdominally. Additionally, there was significantly increased hydronephrosis and elevated RIs in patients with distal ureteral stones.

Acute Pancreatitis in a Pregnant Patient With Coronavirus Disease 2019 (COVID-19).

BACKGROUND: Pregnant women with coronavirus disease 2019 (COVID-19) infection are at risk for a variety of COVID-19 complications. CASE: We report a case of acute pancreatitis in a pregnant patient hospitalized for COVID-19 pneumonia. Comprehensive evaluation ruled out other etiologies of acute pancreatitis. Preterm labor developed at 33 5/7 weeks of gestation, and the patient delivered a liveborn male neonate; neonatal severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) screening was negative. The patient improved significantly postpartum and was discharged home on postpartum day 3. CONCLUSION: Coronavirus disease 2019 may present in pregnancy with a myriad of clinical symptoms other than respiratory. Acute pancreatitis represents an infrequent complication of primary COVID-19 infection.

Rare Case of Fetal Permanent Junctional Reciprocating Tachycardia Refractory to Prenatal Antiarrhythmic Therapy.

Permanent junctional reciprocating tachycardia (PJRT) is a rare form of atrioventricular reentrant tachycardia that is commonly resistant to most antiarrhythmic medication therapy and over an extended duration can result in tachycardia-induced cardiomyopathy. The prenatal presentation of PJRT is typically similar to that of other types of fetal supraventricular tachycardia (SVT), making it difficult to distinguish from other forms of SVT in utero by fetal echocardiography. Surface electrocardiography after delivery is typically required to make a definitive diagnosis of PJRT. We report a case of fetal SVT at 19 weeks' gestation refractory to maternal transplacental treatment with digoxin, amiodarone, flecainide, sotalol, metoprolol, intraumbilical amiodarone, and fetal intramuscular digoxin over the course of 12 weeks. Repeat cesarean delivery was performed at 30 2/7 weeks' gestation for tachycardia-induced cardiomyopathy with hydrops fetalis. Postnatal electrocardiogram and continuous rhythm monitoring confirmed the diagnosis of PJRT. Combined neonatal treatment with amiodarone, digoxin, and propranolol was successful in reestablishment of sinus rhythm, with radiofrequency ablation planned if medical therapy eventually fails or once early childhood is reached. To our knowledge, this is the first described case of fetal PJRT refractory to multiple standard in utero antiarrhythmic modalities and highlights the importance of inclusion in the differential diagnosis.

Association of Pregnancy With Recurrence of Spontaneous Coronary Artery Dissection Among Women With Prior Coronary Artery Dissection.

Importance: Spontaneous coronary artery dissection (SCAD) is a notable cause of acute coronary syndrome in women of childbearing age. Objective: To test the hypothesis that pregnancy after SCAD is associated with recurrent SCAD. Design, Setting, and Participants: Three study designs were implemented: a case series of women with pregnancy after SCAD; a nested case-control study comparing patients with recurrent SCAD to matched controls without recurrent SCAD; and a cohort study. Women with SCAD who were of childbearing potential and enrolled into the Mayo Clinic SCAD Registry from August 30, 2011, to April 4, 2019, were included in the study. Patients with coronary dissections associated with iatrogenesis, trauma, or atherosclerosis were not enrolled. Exposures: Pregnancy after SCAD. Main Outcomes and Measures: The primary outcome was SCAD recurrence, defined as an acute coronary syndrome or cardiac arrest due to new SCAD. Other demographic measures collected included age, year of SCAD occurrence, and comorbidities. Results: The cohort included 636 women of childbearing potential. Twenty-three of those women had a total of 32 pregnancies after SCAD. The median (interquartile range) age of women with pregnancy after SCAD was 38 years (34-40 years), and 20 (87%) were White. In the nested case-control study, 92 cases of recurrent SCAD were matched to 158 controls. There was no significant difference in exposure to subsequent pregnancies in the women with recurrent SCAD as compared with matched controls (2 of 92 [2%] vs 13 of 158 [8%]; P = .06). In the overall cohort of 636 patients, recurrent SCAD was present in 122 patients with a Kaplan-Meier 5-year SCAD recurrence estimate of 14.8%. The Cox analysis showed no significant association between subsequent pregnancy and SCAD recurrence with a nonsignificant hazard ratio of 0.38 (95% CI, 0.09-1.6) when controlling for age at first SCAD, year of first SCAD, and fibromuscular dysplasia. Conclusions and Relevance: This study found that most women tolerated pregnancy and lactation after SCAD without evidence for increased risk of SCAD recurrence when compared with women with a history of SCAD who did not experience pregnancy. Although this study is reassuring and indicates complex contributors to SCAD recurrence, the results need to be interpreted prudently because of study selection bias and the small total number of women who became pregnant after SCAD. The notable hemodynamic changes that occur with pregnancy and severe presentation of pregnancy-associated SCAD are reasons for concern when considering pregnancy after SCAD.

Pregnancy-Associated Myocardial Infarction: Prevalence, Causes, and Interventional Management.

Pregnancy-associated myocardial infarction is a primary contributor to maternal cardiovascular morbidity and mortality. Specific attention to the cause of myocardial infarction, diagnostic evaluation, treatment strategies, and postevent care is necessary when treating women with pregnancy-associated myocardial infarction. This review summarizes the current knowledge, consensus statements, and essential nuances.

Spontaneous Coronary Artery Dissection: JACC State-of-the-Art Review.

Over the past decade, spontaneous coronary artery dissection (SCAD) has emerged as an important cause of myocardial infarction, particularly among younger women. The pace of knowledge acquisition has been rapid, but ongoing challenges include accurately diagnosing SCAD and improving outcomes. Many SCAD patients experience substantial post-SCAD symptoms, recurrent SCAD, and psychosocial distress. Considerable uncertainty remains about optimal management of associated conditions, risk stratification and prevention of complications, recommendations for physical activity, reproductive planning, and the role of genetic evaluations. This review provides a clinical update on the diagnosis and management of patients with SCAD, including pregnancy-associated SCAD and pregnancy after SCAD, and highlight high-priority knowledge gaps that must be addressed.

Outcomes of Pregnancy in Patients With Prior Right Ventricular Outflow Interventions.

Background The purpose of this study was to compare the incidence of pregnancy-related adverse outcomes ( PRAO ) between patients with versus without hemodynamically significant right ventricle outflow tract ( RVOT) . Methods and Results This was a retrospective cohort study of all pregnant patients with isolated RVOT lesions undergoing evaluation at the Mayo Clinic, 1990 to 2017. Hemodynamic significance was defined as ≥moderate pulmonary/conduit stenosis (≥3 m/s) and/or ≥moderate regurgitation. Patients with concomitant significant left heart disease were excluded. PRAO was defined as cardiovascular, obstetric, and/or neonatal complications occurring during the pregnancy through 6 weeks postpartum. A total of 224 pregnancies in 114 patients with RVOT lesions were identified; 38 pregnancies occurred in 24 patients with hemodynamically significant RVOT . Forty-eight (21%) pregnancies ended in spontaneous abortion. Of the 173 completed pregnancies, median gestational age at delivery was 38 (35-40) weeks and median birth weight 2965 (2065-4122) g. Seven pregnancies (4%) were complicated by cardiovascular events, 14 (8%) by obstetric complications, with adverse neonatal outcomes occurring in 38 (22%). There were no maternal deaths. The incidence of spontaneous abortion and PRAO were similar in both the RVOT and hemodynamically significant RVOT groups. As an isolated condition, Tetralogy of Fallot-pulmonary atresia was associated with spontaneous abortion and neonatal complications. Conclusions The risk of cardiovascular complications was low in patients with isolated RVOT lesions, and hemodynamically significant RVOT lesions were not associated with either cardiovascular complications or PRAO . Further studies are required to explore the factors responsible for PRAO in patients with Tetralogy of Fallot-pulmonary atresia.

Intraoperative fetal heart monitoring for non-obstetric surgery: A systematic review.

Limited data are available on fetal monitoring during non-obstetric surgery in pregnancy. We performed a systematic review to evaluate the incidence of emergent cesarean delivery performed for non-reassuring fetal heart rate patterns during non-obstetric surgery. Electronic databases were searched from their inception until October 2018 without limit for language. We included studies evaluating at least five cases of intraoperative fetal heart rate monitoring -either with ultrasound or cardiotocography- during non-obstetric surgery in pregnant women at ≥22 weeks of gestation. The primary outcome was the incidence of intraoperative cesarean delivery performed for non-reassuring fetal heart rate monitoring. Non-reassuring fetal heart rate monitoring was defined by attendant personnel, meeting NICHD criteria for category II or III patterns. Data extracted regarded type of study, demographic characteristics, maternal and perinatal outcomes. Statistical analysis was performed for continuous outcomes by calculating mean and standard deviations for appropriate variables. Of 120 studies identified, 4 with 41 cases of intraoperative monitoring met criteria for inclusion and were analyzed. Most (66%) surgeries were indicated for neurological or abdominal maternal issues and were performed under general anesthesia (88%) at a mean gestational age of 28 weeks. Minimal or absent fetal heart variability was noted in most cases and a 10-25 beats per minutes decrease in fetal heart rate baseline was observed in cases with general anesthesia. No intraoperative cesarean deliveries were needed. The incidence of non-reassuring fetal heart rate monitoring was 4.9% (2/41) and were limited to fetal tachycardia during maternal fever. Two (4.9%) cases of non-reassuring fetal heart rate monitoring were noted within the immediate 48 h after surgery, necessitating cesarean delivery. A single case of intrauterine fetal demise occurred four days postoperatively in a woman who had neurosurgery and remained comatose. In conclusion, limited data exist regarding the clinical application of fetal heart rate monitoring at viable gestational ages during non-obstetric surgical procedures. Fetal heart rate monitoring during non-obstetric surgery at ≥22 weeks was not associated with need for intraoperative cesarean delivery, but two (4.9%) cesarean deliveries were performed for non-reassuring fetal heart rate monitoring within 48 h after surgery.

Venous thromboembolic and bleeding complications among pregnant women with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a vascular malformation overgrowth syndrome characterized by capillary malformation, venous malformation, and limb overgrowth, with or without lymphatic malformation. Patients are at an increased risk of hemorrhage and venous thromboembolism (VTE). Consequently, women with this condition often are counseled to avoid pregnancy, but minimal data are available on the relationship between pregnancy, VTE, and bleeding risk. OBJECTIVE: To review the risk of VTE and bleeding in pregnant and nulligravid women with KTS. METHODS: A retrospective medical record review was performed of women with KTS, aged ≥18 years, evaluated at Mayo Clinic Rochester, Minnesota, from August 1945 to April 2018. RESULTS: We identified 75 women with ≥1 pregnancy and 64 nulligravid women. VTE prevalence was 14 of 70 (20%) for women with a history of pregnancy and 16 of 64 (25%) for nulligravid women (P = .93). Among the 70 women with a history of pregnancy, 7 of 18 VTE events (39%) occurred in association with pregnancy, with VTE affecting 7 of 151 pregnancies (4.6%). Significant bleeding prevalence was 6 of 70 (8.6%) for women with a history of pregnancy and 6 of 64 (9.4%) for nulligravid women (P = .54). LIMITATIONS: This was a retrospective review. CONCLUSION: The prevalence of VTE and bleeding was similar in patients with KTS, irrespective of pregnancy status.

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.