Low-Cost Task Trainer for In Utero Fetal Stent Placement.

INTRODUCTION: Some fetal procedures such as intrauterine fetal stent placement remain rare, and simulation is needed to help learners and specialists in attaining and maintaining technical competence. We sought to design and assess a low-cost, easily assembled yet clinically relevant task trainer for fetal stent placement. METHOD: The simulator was constructed using 2 quart-sized freezer bags filled with ultrasound gel and sealed with clear packing tape. The bags were stacked vertically in a transparent plastic container with ultrasound gel applied between the bags when ultrasound was used. This task trainer was used to deploy in utero stents with or without the use of ultrasound. It has been used at the annual meeting of the Society for Maternal-Fetal Medicine since 2015, the annual meeting of the International Society of Ultrasound in Obstetrics and Gynecology in 2015 and 2016, and at regional Maternal-Fetal Medicine Fellow simulation workshops since 2016. Participants were asked to complete a 5-point Likert scale survey regarding the model's realism and usefulness in training. RESULTS: One hundred thirty-three course participants evaluated the task trainer. The median rating for realism of the ultrasound images, haptic feel of stent deployment, and usefulness in training was 5 (interquartile range, 4-5). Seven physicians participated in the timed assessment of model assembly, stent deployment, and model reassembly. The average times required for the freezer bag task trainer were 2.3 minutes (2.20-2.35), 1.0 minutes (0.70-1.93), and 0.1 minutes (0.08-0.10), respectively. For the porcine tissue-based model tested in parallel, the average times were 6.0 minutes (5.00-7.06), 3.7 minutes (3.63-3.75), and 3.3 minutes (3.00-3.70), respectively. CONCLUSIONS: This low-cost simulator was rated highly when used to practice in utero stent deployment and allows for numerous repetitions in each training session. It could be a valuable tool in training novice providers and allow more experienced providers to maintain competence in this low-volume procedure.

Fetal Doppler in monochorionic pregnancies complicated by twin-to-twin transfusion syndrome and selective in utero growth restriction.

BACKGROUND: Monochorionic (MC) twin pregnancies may be complicated by placental pathologies that impact fetal cardiac function, such as twin-twin transfusion syndrome (TTTS) and selective intrauterine growth-restriction (sIUGR). In the TTTS, the unbalanced blood flow through placental anastomoses lead a recipient volume overload, hypertension and hypertrophic cardiomyopathy and the donor twin experiences hypovolemia and hypertension due to increased placental resistance and poor renal perfusion. When MC pregnancies were complicated by sIUGR, the increase of placental resistances lead to complex fetal compensatory mechanisms with redistribution of cardiac output to vital organs. Increased placental vascular resistances, hypoxia and hemodynamic compensation mechanisms lead to higher pre and/or afterload for both ventricles, right cardiac failure and eventually left cardiac failure observed just before fetal death. OBJECTIVES: The purpose of this study was to describe the anomalies of umbilical, ductal and aortic isthmic Doppler as well as left and right myocardial performance index (MPI) across various clinical phenotypes of MC twin pregnancies, uncomplicated or complicated by TTTS or sIUGR, in order to help differentiating these conditions and to improve the understanding of TTTS and sIUGR pathophysiology. STUDY DESIGN: Aortic isthmic systolic index (ISI), umbilical artery pulsatility index (UAPI), ductus venosus pulsatility index (DVPI), and MPI were studied in uncomplicated MC twins (control group) and cases of sIUGR or TTTS. RESULTS: The measurements were obtained in 113 pregnancies (24 uncomplicated, 22 sIUGR, 51 TTTS). In comparison with controls, the sIUGR smaller twin sets had lower ISI and higher UAPI, and the larger twin had higher ISI. The TTTS donor and recipient had lower ISI, higher UAPI and DVPI. Compared to the co-twin, the ISI values were lower in the sIUGR smaller twin and the TTTS donor had lower ISI and MPI. Comparing TTTS and sIUGR, the recipient had higher DVPI and MPI than the sIUGR larger twin. CONCLUSIONS: The Doppler anomalies observed in the smaller twin reflected increased placental blood flow resistance, presumably due to abnormal feto-fetal transfusion in TTTS and to unequal placental sharing in sIUGR. Early hemodynamic changes suggestive of cardiac overload in the recipient twin may help to differentiate TTTS and sIUGR.

Impact of Selective Fetal Growth Restriction on Laser Therapy Outcomes in Twin-Twin Transfusion Syndrome.

INTRODUCTION: The aim of this study was to determine if outcomes of fetoscopic laser photocoagulation in isolated twin-twin transfusion syndrome (TTTS) differ from TTTS with concomitant selective fetal growth restriction (sFGR). METHODS: This is a retrospective cohort study of all cases of TTTS treated at the CHU Sainte-Justine between February 2006 and January 2020. Data were collected from maternal, obstetrical, and neonatal chart review. RESULTS: A total of 149 patients were included in our study. Forty-seven patients (31.5%) had a pregnancy complicated by TTTS and sFGR. Mean gestational age at diagnosis and at treatment was 20+4 weeks and 20+6 weeks for TTTS alone, and 20+5 weeks and 21+2 weeks with concomitant sFGR. The presence of concomitant sFGR negatively impacted survival. Double survival in the TTTS + sFGR was 48.9% (23/47) versus 68.6% (70/102) in the TTTS-only group (p = 0.021). Fetal donor survival was 59.6% (28/47) in the TTTS + sFGR group and 84.3% (86/102) in the TTTS-only group (p = 0.001). However, the survival of at least one twin did not differ between the two groups: 93.6% (44/47) in the TTTS + sFGR group versus 92.2% (94/102) in the TTTS-only group (p = 0.751). The presence of type 2-3 sFGR (OR = 0.56; 95% CI 0.32-0.96, p = 0.033) and gestational age at laser therapy (OR = 1.17; 95% CI 1.01 = 1.36, p = 0.036) were independently associated with dual survival. CONCLUSION: sFGR is independently associated with decreased double survivorship at the expense of the donor in TTTS undergoing laser therapy. Type 2 or 3 sFGR and early gestational age at treatment are especially at risk. A larger cohort is needed to validate our results.

Hands-on Simulation Workshop for Obstetric Ultrasound-Guided Invasive Procedures.

Introduction: Due to the introduction of cell-free DNA genetic testing options, the number of clinical ultrasound procedures has greatly diminished in recent years. With fewer real-life ultrasound-guided procedures being performed, it is difficult for OB/GYN and maternal-fetal medicine (MFM) trainees to achieve competency in doing them. Simulation can be utilized to address this issue and supplement a learner's real-life training. Methods: We developed a simulation workshop incorporating previously described ultrasound guidance task trainers and simulators of amniocentesis and chorionic villus sampling. The workshop had three parts: needle guidance basics, targeting task performance, and procedure-specific simulation. A form of this workshop has been held at the annual meeting of the Society for Maternal-Fetal Medicine since 2015 and as a regional course for MFM fellows since 2017. During the 2019 and 2020 courses, participants completed Likert-scale surveys evaluating the course. Results: Since the workshops began in 2015, approximately 300 people have participated. In 2019-2020, 41 MFM attending physicians, 136 MFM fellows, and three OB/GYN residents took our course and completed a postcourse survey. Participants rated the course highly and thought it was highly effective. Discussion: We created an introductory simulation workshop for obstetric ultrasound-guided invasive procedures that participants rated highly and thought was very effective. Objective clinical assessment of skill improvement after completion of this course is needed to verify its true impact. Repeated exposure to this introductory simulation and creation of more challenging workshops are needed to achieve a sustained high level of procedural skill.

Pulmonary hypertension associated with vein of Galen malformation. Fetal cardiac hemodynamic findings and physiological considerations.

The management of newborns with vein of Galen aneurysmal malformation (VGAM) is clinically challenging for neonatologists and cardiologists. Hemodynamic profiles in four fetuses diagnosed with VGAM who subsequently developed neonatal cardiac failure and pulmonary hypertension were studied using two-dimensional and Doppler echocardiography. All four had an increased cardiothoracic ratio due to right ventricular dilatation on antenatal ultrasound. Doppler studies of the aortic isthmus were abnormal with retrograde flow starting in mid systole and throughout diastole. Left and right ventricular outputs were significantly increased. Net pulmonary flow was highly abnormal with a flow rate almost four times higher than normal. After a short period of clinical stability, all neonates developed cardiac failure and pulmonary hypertension. This article reviews VGAM pathophysiology and the potential relationship between pulmonary hypertension and VGAM, supporting early post-natal treatment of pulmonary hypertension and right ventricular failure.

Perinatal and cardiac outcomes of women with hypertrophic cardiomyopathy.

RATIONALE: Pregnancy causes important physiologic stress for women with hypertrophic cardiomyopathy. Data regarding the impact of this condition on obstetrical outcomes is missing. OBJECTIVES: Our objective was to report obstetrical and cardiac outcomes in pregnant women with hypertrophic cardiomyopathy and to assess the possible adverse effects of left ventricular outflow tract obstruction in pregnancy. STUDY DESIGN: This was a retrospective cohort study of pregnant women diagnosed with HCM and followed at single tertiary center between 1995 and 2019. Demographic, medical and surgical data, echocardiographic parameters, and pregnancy outcomes were abstracted through extensive chart review. Patients were divided into 2 groups: obstructive (maximal left ventricular outflow tract gradient over 30 mmHg) versus non-obstructive hypertrophic cardiomyopathy. Outcomes between groups were compared with t-test, Mann-Whitney and Fisher's exact tests when appropriate. RESULTS: Eighteen women with 27 pregnancies were included. The study population was formed of 18 women with a total of 27 pregnancies that reached at least 20 weeks of gestation: 12 pregnancies in women with obstructive hypertrophic cardiomyopathy and 15 pregnancies in women with non-obstructive hypertrophic cardiomyopathy. Among the non-obstructive hypertrophic cardiomyopathy, 5 of them had been treated for their obstruction. One patient with obstructive hypertrophic cardiomyopathy had a medical termination of pregnancy for uncontrolled arrhythmia at 21 weeks. There were no maternal deaths. Left ventricular outflow tract obstruction was associated with increased cardiac events including arrhythmias and heart failure (5/12 versus 0/15; p = .006). Preterm birth occurred in more than 50% of cases, resulting from induced delivery for a maternal (40%) or fetal reason (60%). Most deliveries were late preterm between 34 and 36 6/7 weeks. In both groups, birthweight was mainly distributed below the 50th percentile (89%) and 35% of neonates were born small for gestational age defined as a birthweight below the 10th percentile. Most severe cases of small for gestational age (birthweight under the 5th percentile) were found in patients with treated obstructive hypertrophic cardiomyopathy. CONCLUSION: Hypertrophic cardiomyopathy is associated with prematurity and small for gestational age. Left ventricular outflow tract obstruction is associated with adverse cardiac events including arrythmias or heart failure. Treated obstructive cardiomyopathy constitutes a sub-group of patients at high risk of severe small for gestational age and deserves a close surveillance. Therefore, fetal growth surveillance with ultrasound, early in the third trimester and doppler studies to assess the utero-placental perfusion in the second and third trimesters are warranted in all patients with hypertrophic cardiomyopathy regardless of the severity of their condition.

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.

Prenatal presentation of glutaric aciduria type II: A case report with radiologic, clinical, biochemical, molecular, and pathological phenotyping.

We know that glutaric aciduria type II is an inborn metabolism. This case report highlights that polycystic kidneys with hepatomegaly in prenatal ultrasound are suggestive of glutaric aciduria type II and it identifies a new variant as pathogenic.

Expectant Management of Monochorionic-Triamniotic Triplets Complicated by Selective In Utero Growth Restriction: Report of 2 Cases.

The optimal management of monochorionic-triamniotic (MCTA) triplet pregnancies is not clearly established, and there is no literature to guide management of MCTA complicated with selective intrauterine growth restriction (sIUGR). This gap in knowledge and the concern for higher risk of severe complications have led some medical societies to recommend selective termination of nontrichorionic triplet pregnancies. We sought to report the favourable outcomes of two MCTA complicated by sIUGR expectantly managed at Sainte-Justine Hospital, Montreal, Canada. The first case is of a 42-year-old woman with spontaneous MCTA triplets diagnosed at 18 weeks with type II sIUGR who opted for expectant management. The second patient was a 22-year-old woman with a spontaneous MCTA triplet pregnancy diagnosed at 18 weeks with type III sIUGR. Our experience shows that close serial ultrasounds could potentially allow physicians to foresee fetal deterioration. In our opinion, expectant management should be considered as a management option for MCTA complicated by sIUGR.

Electronic Algorithm Is Superior to Hospital Discharge Codes for Diagnoses of Hypertensive Disorders of Pregnancy in Historical Cohorts.

OBJECTIVES: To develop and validate criteria for the retrospective diagnoses of hypertensive disorders of pregnancy that would be amenable to the development of an electronic algorithm, and to compare the accuracy of diagnoses based on both the algorithm and diagnostic codes with the gold standard, of physician-made diagnoses based on a detailed review of medical records using accepted clinical criteria. PATIENTS AND METHODS: An algorithm for hypertensive disorders of pregnancy was developed by first defining a set of criteria for retrospective diagnoses, which included relevant clinical variables and diagnosis of hypertension that required blood pressure elevations in greater than 50% of readings ("the 50% rule"). The algorithm was validated using the Rochester Epidemiology Project (Rochester, Minnesota). A stratified random sample of pregnancies and deliveries between January 1, 1976, and December 31, 1982, with the algorithm-based diagnoses was generated for review and physician-made diagnoses (normotensive, gestational hypertension, and preeclampsia), which served as the gold standard; the targeted cohort size for analysis was 25 per diagnosis category according to the gold standard. Agreements between (1) algorithm-based diagnoses and (2) diagnostic codes and the gold standard were analyzed. RESULTS: Sensitivities of the algorithm for 25 normotensive pregnancies, 25 with gestational hypertension, and 25 with preeclampsia were 100%, 88%, and 100%, respectively, and specificities were 94%, 100%, and 100%, respectively. Diagnostic code sensitivities were 96% for normotensive pregnancies, 32% for gestational hypertension, and 96% for preeclampsia, and specificities were 78%, 96%, and 88%, respectively. CONCLUSION: The electronic diagnostic algorithm was highly sensitive and specific in identifying and classifying hypertensive disorders of pregnancy and was superior to diagnostic codes.

The Current State of Ultrasound Training in Obstetrics and Gynecology Residency Programs.

OBJECTIVES: We evaluated the current state of ultrasound training in obstetrics and gynecology (OB-GYN) residency programs across the United States. METHODS: An electronic survey was sent to OB-GYN residency program directors and OB-GYN residents. Responses were obtained in September 2016. Program directors and residents were asked to reflect on their current ultrasound curriculum. RESULTS: A total of 93 program directors and 437 residents responded. Respondents were mostly from university programs located in tertiary centers. Ultrasound curricula varied: 11% of program directors and 23% of residents did not have any ultrasound-related didactics; of those who did, 27% of program directors and 40% of residents had it yearly or less. Three-quarters had mandatory ultrasound rotations, and few offered ultrasound electives (program directors, 52%; residents, 28%). Most residents were required to perform ultrasound examinations daily or weekly (98%). Most stated that the main focus of the rotation was OB only. Skill was evaluated mainly subjectively by direct observation. Although most program directors stated that residents were satisfactory/excellent in ultrasound, 22% would not treat patients on the basis of ultrasound examinations performed by their senior residents. Similarly, of all postgraduate year 4 respondents (n = 86), 76% stated that they will require additional training to be able to perform or read ultrasound examinations independently, and 43% would not treat a patient on the basis of their own ultrasound examinations without further confirmation. Residents believed that the biggest obstacle in ultrasound training is lack of dedicated faculty time (41%). CONCLUSIONS: Recognizing the lack of clearly defined milestones in ultrasound training in OB-GYN residency, this study confirms the substantial heterogeneity in curricula between programs, highlighting a need for a standardized ultrasound curriculum.

Subsequent Pregnancy Outcomes in Patients With Peripartum Cardiomyopathy.

OBJECTIVE: To describe cardiac and obstetric outcomes in subsequent pregnancies of patients with peripartum cardiomyopathy and to report demographic and clinical characteristics of index pregnancies. METHODS: We conducted a retrospective cohort study of all pregnant patients with prior peripartum cardiomyopathy seen at the Mayo Clinic from January 2000 through March 2017. Maternal and neonatal outcome data of index and all subsequent pregnancies were abstracted, and all echocardiography examinations were individually reviewed. RESULTS: Twenty-five patients with prior peripartum cardiomyopathy were included; all except one had recovered left ventricular (LV) function (LV ejection fraction 50% or greater) before the subsequent pregnancy. Forty-three subsequent pregnancies were identified: six (14.0%) miscarriages, four (9.3%) terminations, and 33 (76.7%) live births. The rate of peripartum cardiomyopathy relapse was 20.9%; median LV ejection fraction nadir in patients with relapse was 43% (range 35-45%). None had LV ejection fraction decline to the level of their index pregnancy. No cardiac arrests or deaths were observed, and all patients with relapse recovered LV function. Median gestational age at delivery for all live births in subsequent pregnancies was 39.0 weeks (range 36 6/7-41 3/7 weeks). CONCLUSION: Patients with a history of peripartum cardiomyopathy who recover LV function are at risk for a transient minor decrease in LV ejection fraction during future pregnancies, but obstetric and neonatal outcomes are often favorable.

Use of Genetic Testing after Abnormal Screening Ultrasound: A Descriptive Cohort Study.

BACKGROUND/AIMS: The study aimed to characterize the use of genetic testing after abnormal screening ultrasound. METHODS: We performed a retrospective review of patients undergoing genetic testing after abnormal ultrasound. Genetic evaluation consisted of noninvasive prenatal screening (NIPS) or amniocentesis. Classification of ultrasound findings, type of genetic testing, and results were collected. RESULTS: A total of 139 subjects underwent genetic evaluation after abnormal screening ultrasound. Screening via NIPS was pursued by 61 (44%) patients while 78 (56%) proceeded directly to amniocentesis. Patients electing for amniocentesis had more cardiac, neurologic, and gastrointestinal malformations while soft markers for aneuploidy prompted more NIPS screening. Results were negative in 85% of the NIPS group compared to 60% of the amniocentesis group. Only 8% of patients who underwent NIPS proceeded to diagnostic testing. CONCLUSION: Patients pursuing NIPS after abnormal ultrasound had more soft markers of aneuploidy. Patients pursuing diagnostic testing were more likely to have major structural malformations and more total abnormalities identified. Patients who proceeded directly to amniocentesis were more likely to have abnormal genetic testing.

Spontaneous Coronary Artery Dissection Associated With Pregnancy.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) is the most common cause of pregnancy-associated myocardial infarction and remains poorly characterized. OBJECTIVES: This study sought to assess presentation, clinical factors, and outcomes of pregnancy-associated spontaneous coronary artery dissection (P-SCAD) compared with spontaneous coronary artery dissection not associated with pregnancy (NP-SCAD). METHODS: A Mayo Clinic registry was established in 2010 to include comprehensive retrospective and prospective SCAD data. Records were reviewed to identify women who were pregnant or ≤12 weeks postpartum at time of SCAD. Complete records were available for 323 women; 54 women met criteria for P-SCAD (4 during pregnancy) and they were compared with 269 women with NP-SCAD. RESULTS: Most events occurred within the first month postpartum (35 of 50). Compared with NP-SCAD, P-SCAD patients more frequently presented with ST-segment elevation myocardial infarction (57% vs. 36%; p = 0.009), left main or multivessel SCAD (24% vs. 5%; p < 0.0001; and 33% vs. 14%; p = 0.0027, respectively), and left ventricular function ≤35% (26% vs. 10%; p = 0.0071). Among women with imaging of other vascular territories, P-SCAD was less likely with a diagnosis of fibromuscular dysplasia and extracoronary vascular abnormalities (42% vs. 64%; p = 0.047; and 46% vs. 77%; p = 0.0032, respectively). Compared with U.S. birth data, women with P-SCAD were more often multiparous (p = 0.0167), had a history of infertility therapies (p = 0.0004), and had pre-eclampsia (p = 0.001). On long-term follow-up (median 2.3 years) recurrent SCAD occurred in 51 patients, with no difference in the Kaplan Meier 5-year recurrence rates (10% vs. 23%; p = 0.18). CONCLUSIONS: P-SCAD patients had more acute presentations and high-risk features than women with NP-SCAD did. The highest frequency of P-SCAD occurred during the first postpartum month and P-SCAD patients less often had extracoronary vascular abnormalities. Hormonal, hemodynamic variations, and yet-undefined mechanisms might be significant contributors to P-SCAD. (The "Virtual" Multicenter Spontaneous Coronary Artery Dissection [SCAD] Registry [SCAD]; NCT01429727; Genetic Investigations in Spontaneous Coronary Artery Dissection [SCAD]; NCT01427179).

Longitudinal characterization of renal proximal tubular markers in normotensive and preeclamptic pregnancies.

Glomerular damage is common in preeclampsia (PE), but the extent and etiology of tubular injury are not well understood. The aim of this study was to evaluate tubular injury in patients with PE and to assess whether it predates clinical disease. We performed a prospective cohort study of 315 pregnant women who provided urine samples at the end of the second trimester and at delivery. This analysis included women who developed PE (n = 15), gestational hypertension (GH; n = 14), and normotensive controls (NC; n = 44). Urinary markers of tubular injury, α1-microglobulin (A1M), retinol-binding protein (RBP), kidney-injury molecule-1 (KIM1), complement C5b-9, tissue inhibitor metalloproteinase-2 (TIMP-2), and insulin-like growth factor binding protein-7 (IGFBP-7) were measured by enzyme-linked immunosorbent assay (ELISA) and reported in relation to urine creatinine concentration. Second-trimester concentrations of all markers were similar among groups. At delivery, A1M concentrations were higher in the PE group than in the GH and NC groups as an A1M/creatinine ratio >13 (66.7, 8.3, and 35%, respectively, P = 0.01). Concentrations of C5b-9 were higher in the PE group than in the GH and NC groups (medians 9.85, 0.05, and 0.28 ng/mg, respectively, P = 0.003). KIM1, RBP, TIMP-2, and IGFBP-7 concentrations did not differ among groups at delivery. In conclusion, proximal tubular dysfunction, as assessed by A1M and C5b-9, developed during the interval between the end of the second trimester and delivery in patients with PE. However, this was not matched by abnormalities in markers previously associated with tubular cell injury (KIM-1, IGFBP-7, and TIMP-2).

Peripartum Cardiomyopathy Presenting as Bradycardia.

Peripartum cardiomyopathy (PPCM) is a disease that typically affects young otherwise healthy women. As PPCM is associated with significant mortality, timely diagnosis is necessary to ensure appropriate care. To our knowledge, this represents the first reported case of PPCM presenting as symptomatic bradycardia. We describe the patient's clinical presentation and relevant findings and review the potential etiology and ramifications of bradycardia in patients with PPCM.