Current obstetric outcomes in Jamaican women with sickle hemoglobinopathy - a balance of risks for aspirin?

OBJECTIVES: Sickle cell disease (SCD) occurs in 2.8 % of our Jamaican antenatal population with homozygous HbSS being most associated with adverse maternal and perinatal outcomes. METHODS: A retrospective comparative analysis of HbSS, HbSC and HbSßThal pregnancy outcomes at the University Hospital of the West Indies (UHWI) between January 2012 and December 2022 was conducted. RESULTS: Of 120 patients (138 pregnancies), obesity occurred in 36 % (20/56) of the 'non-HbSS' group, i.e. HbSßThal (55 %, 5/9) and HbSC (32 %, 15/47) combined vs. 9.7 % of the HbSS (8/82). HbSS patients had more crises requiring transfusions, acute chest syndrome (ACS), maternal 'near-misses' (OR=10.7, 95 % 3.5-32.3; p<0.001), hospitalizations (OR 7.6, 95 % CI 3.4-16.9; p<0.001), low birth weight (LBW) neonates (OR 3.1, 1.1-8.9; p=0.037) and preterm birth (OR=2.6, 1.2-5.8; p=0.018) compared to HbSC and HbSßThal. Low dose aspirin was prescribed in 43 %. Logistic regression showed those NOT on aspirin (n=76) had more miscarriages (22 v. 2 %), were LESS likely to have a live birth (75 v. 95 % (0.2, 0.04-0.57, p=0.005)), but surprisingly had fewer painful crises (28 v. 46 % (0.5, 0.03-0.9, p=0.03)). CONCLUSIONS: HbSS women had a 10-fold excess of maternal near-misses. Additional research may further clarify the effects of aspirin on pregnancy outcomes as related to SCD genotypes.

Experience with SGLT2 Inhibitors in Patients with Single Ventricle Congenital Heart Disease and Fontan Circulatory Failure.

Heart failure is the leading cause of morbidity and mortality in patients with Fontan circulation. Sodium-glucose-cotransporter 2 inhibitors (SGLT2i) have become a mainstay of heart failure therapy in adult patients, however, there remains a paucity of literature to describe its use in pediatric heart failure patients, especially those with single ventricle physiology. We describe our early experience using SGLT2i in patients with single ventricle congenital heart disease surgically palliated to the Fontan circulation. We conducted a single-center retrospective chart review of all patients with Fontan circulation who were initiated on an SGLT2i from January 1, 2022 to March 1, 2023. Patient demographics, diagnoses, clinical status, and other therapies were collected from the electronic medical record. During the study period, 14 patients (median age 14.5 years, range 2.0-26.4 years) with Fontan circulation were started on a SGLT2i. Mean weight was 54 kg (range 11.6-80.4 kg). Median follow-up since SGLT2i initiation was 4.1 months (range 13 days-7.7 months). Four patients had a systemic left ventricle and 10 had a systemic right ventricle. Half the patients had Fontan Circulatory Failure with reduced Ejection Fraction (FCFrEF) of the systemic ventricle and the other half had Fontan Circulatory Failure with preserved Ejection Fraction (FCFpEF) of the systemic ventricle. In addition, 3 patients experienced Protein Losing Enteropathy (PLE) and 2 patients had plastic bronchitis, one of whom also was diagnosed with chylothorax. There were no genitourinary infections, hypoglycemia, ketoacidosis, hypotension or other significant adverse effects noted in our patient population. One patient experienced significant diuresis and transient acute kidney injury. Patients with FCFrEF showed a decrease in natriuretic peptide levels. Given the lack of proven therapies, demonstrated benefits of SGLT2i in other populations, and some suggestion of efficacy in Fontan circulation, further study of SGTLT2i in patients with Fontan circulation is warranted.

Patient and parent-reported outcomes in paediatric ventricular assist device support: a multi-center ACTION learning network feasibility and pilot experience.

BACKGROUND: Patient- and proxy-reported outcomes (PROs) are an important indicator of healthcare quality and can be used to inform treatment. Despite the widescale use of PROs in adult cardiology, they are underutilised in paediatric cardiac care. This study describes a six-center feasibility and pilot experience implementing PROs in the paediatric and young adult ventricular assist device population. METHODS: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a collaborative learning network comprised of 55 centres focused on improving clinical outcomes and the patient/family experience for children with heart failure and those supported by ventricular assist devices. The development of ACTION's PRO programme via engagement with patient and parent stakeholders is described. Pilot feasibility, patient/parent and clinician feedback, and initial PRO findings of patients and families receiving paediatric ventricular assist support across six centres are detailed. RESULTS: Thirty of the thirty-five eligible patients (85.7%) were enrolled in the PRO programme during the pilot study period. Clinicians and participating patients/parents reported positive experiences with the PRO pilot programme. The most common symptoms reported by patients/parents in the first month post-implant period included limitations in activities, dressing change distress, and post-operative pain. Poor sleep, dressing change distress, sadness, and fatigue were the most common symptoms endorsed >30 days post-implant. Parental sadness and worry were notable throughout the entirety of the post-implant experience. CONCLUSIONS: This multi-center ACTION learning network-based PRO programme demonstrated initial success in this six-center pilot study experience and yields important next steps for larger-scale PRO collection, research, and clinical intervention.

Use of Cine Loops and Structural Landmarks in Ultrasound Image Processing Improves Reliability and Reduces Error in the Assessment of Foot and Leg Muscles.

OBJECTIVES: Foot and leg muscle strength and size are crucial to proper function. It is important to assess these characteristics reliably. Our primary objective was to compare the measurement of still images to cine loops. The secondary purpose was to determine interoperator and intraoperator reliability between operators of different experience levels using video clips and internal and external landmarks. METHODS: Twelve healthy volunteers participated in our study. Internal (navicular tuberosity) and external (lateral leg length at 30% and 50% from the knee joint line) landmarks were used. Two operators each captured and later measured still and cine loop images of selected foot and leg muscles. RESULTS: The 12 participants included 8 male and 4 female volunteers (mean age ± SD, 23.5 ± 1.9 years). Good to excellent intraoperator and interoperator reliability was seen (intraclass correlation coefficient range of 0.946-0.998). The use of cine loops improved the intraclass correlation coefficients for both intraoperator and interoperator reliability (0.5%-4% increases). The use of cine loops decreased the intraoperator standard error of the measurement and limits of agreement of the novice operator (decreases of 45%-73% and 24%-51%, respectively), and these became comparable to those of experienced operators using still images. The interoperator standard errors of the measurement dropped by 42% to 53%, whereas the limits of agreement dropped by 27% to 40%. No substantial changes were noted in the tibialis anterior across reliability metrics. CONCLUSIONS: Improved protocols that take advantage of using internal bony landmarks and cine loops during both the image-gathering and measurement processes improve the reliability of research examining muscle size changes in the lower leg or foot associated with muscle changes due to exercise, injury, disuse, or disease.

Current practices in the prediction and prevention of preterm birth in patients with higher-order multiple gestations.

OBJECTIVE: We sought to determine the interventions utilized by maternal-fetal medicine specialists in the prediction and prevention of preterm labor in higher-order multiple (HOM) gestations. STUDY DESIGN: Online questionnaires and email surveys were sent to all the maternal-fetal medicine specialists in Canada (n=122). Questionnaire items included interventions physicians routinely recommended for HOM gestations including: (1) bed rest; (2) cervical length measurement on transvaginal ultrasound; (3) corticosteroids use; (4) cerclage; and (5) tocolytic therapy. RESULTS: Response rate was 66% (81/122), with 68% of respondents in practice for >10 years. Of physicians, 91% did not routinely recommend bed rest (95% confidence interval [CI], 84.7-97.2). In all, 82% (95% CI, 73.63-90.4%) recommended routine cervical length assessment with 32.3% (95% CI, 20.7-43.2) and 37.1% (95% CI, 25.3-48.6) of this group suggesting assessment at 16-18 and 19-21 weeks, respectively. Frequency of assessment varied from biweekly (53.3%; 95% CI, 40.9-65.0), to monthly (23.3%; 95% CI, 12.8-33.1), to a single measurement repeated only if abnormal (12.5%; 95% CI, 4.5-20.8). In all, 28% (95% CI, 18.2-37.8) recommended routine administration of corticosteroids for lung maturation. Timing of administration varied, with 24% initiating steroids between 24-26 weeks, 59% between 27-28 weeks, and 17% after 28 weeks. None reported routine cerclage placement. However, 71% (95% CI, 61.1-80.8) would perform cerclage based on history or ultrasound. Of respondents, 81% (95% CI, 72.4-89.5) would consider using tocolytic agents for threatened preterm labor including calcium channel blockers (94%), nonsteroidal antiinflammatory drugs (5%), and nitroglycerin transdermal patch (24%). CONCLUSION: The variable practice guidelines and paucity of data for management of HOM pregnancy places the onus on individual practitioners to develop their own management schemes. This results in heterogeneous management, which is based on conflicting international guidelines, studies, expert opinion, or past experience.

Case Report: Obstetric and COVID-19-Related Morbidity and Mortality in Three Patients with Sickle Hemoglobinopathy.

Approximately 3% of pregnant women have sickle cell disease (SCD). COVID-19, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), became a global pandemic in March 2020, resulting in more than 3,500 deaths in Jamaica by April 2023. Sickle cell disease is an immunocompromised state; therefore, contracting COVID-19 may result in adverse maternal/neonatal outcomes. Current literature focusing on individuals of Afro-Caribbean descent is limited. Our objective was to describe the obstetric and neonatal outcomes of pregnant patients with SCD who contracted COVID-19. A retrospective case series was conducted at the University Hospital of the West Indies (Jamaica) from 2020 to 2022. We describe the maternal and neonatal outcomes of three patients with COVID-19 and SCD (including two with hemoglobin SC disease and one with hemoglobin SS disease), with complications including the demise of a mother and a newborn. Vaso-occlusive crisis was the more common presentation. Two patients required ventilatory support. Although previous reports have shown similar clinical sequelae in pregnant and nonpregnant patients with SCD and COVID-19, maternal and neonatal deaths remain possible.

Pregnancy outcomes in systemic lupus erythematosus: experience from a Caribbean center.

BACKGROUND: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune multi-system disorder frequently affecting black women of childbearing age. No published data exist on the obstetric outcomes in a Caribbean population. OBJECTIVE: We analyzed pregnancy outcomes in an Afro-Caribbean cohort of women with SLE at a tertiary university hospital. METHODS: A retrospective cohort study was performed of all pregnant women with SLE prior to pregnancy from January 1990 to December 2021 at the University Hospital of the West Indies (UHWI), Jamaica. Maternal rheumatologic, obstetric, fetal/neonatal data were analyzed. Descriptive statistical analyses were performed. To determine if outcomes were associated with various factors, Spearman's rho was followed by logistic regression analysis to estimate unadjusted odds ratios with statistical significance at p < 0.05. RESULTS: A total of 56 pregnancies in 47 women were identified with SLE. Live births were 87.5%, with 10.7% spontaneous miscarriages and no neonatal deaths. Prednisone was the most used drug in 67.9% of patients. 85% of women had an adverse outcome with an adverse fetal outcome occurring in 55% of cases. Prednisone was associated with an adverse fetal/neonatal outcome (Spearman's rho = 0.38; p = .004). CONCLUSION: In this first Caribbean series on SLE in pregnancy, reasonably successful pregnancy outcomes are achievable in Afro-Caribbean women managed in multidisciplinary centers.

Pregnancy outcomes of women with cardiac disease.

OBJECTIVE: To determine and predict the maternal and neonatal outcomes of pregnancies occurring in patients with cardiac disease. METHOD: This retrospective review included 147 pregnancies identified from antenatal, delivery, and nursery records. Information concerning the nature and severity of the pre-existing cardiac disease, comorbidities, risk scores, obstetric or cardiac complications, and pregnancy outcomes were collected. The data were analyzed using SPSS Windows version 22. RESULTS: In all, 111 (73.5%) of the cohort had acquired heart disease and 4 (2.7%) of patients belonged to WHO class IV, in which pregnancy is not recommended. Additionally, 12 (8.1%) were categorized as being at significant risk of having a cardiac complication. The proportion of patients that had maternal and perinatal mortality was 6 (4.0%) and 7 (4.8%), respectively. The WHO and CARPREG scoring systems were reliably able to predict cardiac events (P < 0.01). Mothers who received preconception counseling had significantly fewer occurrences of cardiac and obstetric events than those who did not. CONCLUSION: Cardiac disease in pregnancy in women managed at our center was most often an acquired disease. The baseline risk assessment scores accurately predicted the likelihood of adverse cardiac outcomes.

Neonatal outcomes of preterm twins according to mode of birth and presentation.

PURPOSE: To compare neonatal outcomes of twins delivered <33 weeks' gestation in tertiary centers in Canada according to the mode of birth and presentation. MATERIALS AND METHODS: This retrospective cohort from the Canadian Neonatal Network database studied preterm twins born from 24 + 0 to 32 + 6 weeks' gestation between 2005 and 2012. Twins were grouped by the mode of birth: both vaginal, combined vaginal/cesarean section (CS), and both CS. Additionally, twins were grouped by the mode of presentation: both vertex, vertex/breech, breech/vertex, and both breech. The primary outcome was a composite of mortality or severe neonatal morbidity (severe neurological injury [intraventricular hemorrhage grade 3/4 or periventricular leukomalacia], bronchopulmonary dysplasia, retinopathy of prematurity, and necrotizing enterocolitis). RESULTS: Of the 6636 twins, 1934 (29%) were delivered vaginally, 418 (6%) by combined vaginal birth/CS, and 4284 (65%) were born by CS. The composite did not differ between the groups. However, severe neurological injury was decreased (adjusted odds ratio [AOR], 0.77; 95% confidence interval [CI], 0.61-0.98) and respiratory distress syndrome (AOR, 1.34; 95%CI, 1.15-1.56) was increased when both the twins were delivered by CS. CONCLUSIONS: Preterm twin infants born via CS experienced less severe neurological injury when compared to those delivered vaginally, but had an increase in respiratory distress syndrome.

Four Residents' Narratives on Abortion Training: A Residency Climate of Reflection, Support, and Mutual Respect.

The decision on the part of obstetrics and gynecology residents to opt in or out of abortion training is, for many, a complex one. Although the public debate surrounding abortion can be filled with polarizing rhetoric, residents often discover that the boundaries between pro-choice and pro-life beliefs are not so neatly divided. We present narratives from four residents, training at a 32-resident program in the Northeast, who have a range of views surrounding abortion. Their stories reveal how some struggle with the real-life experience of providing abortions, while others feel angst over lacking the skills to terminate a life-threatening pregnancy. These residents have found that close relationships with coworkers from all sides of this issue, along with a residency program that encourages open conversation, have fostered understanding. Their narratives demonstrate that reasonable providers can disagree fundamentally and still work effectively with one another and that the close relationships formed in residency can allow both sides to see beyond the black and white of the public abortion debate. Our objectives in this commentary are to encourage a more nuanced discussion of abortion among obstetrician-gynecologists, to describe the aspects of our residency program that facilitate open dialogue and respect across diverse viewpoints, and to demonstrate that the clear distinction between being pro-life and pro-choice often breaks down when one is immediately responsible for the care of pregnant women.

Clinical significance of the umbilical cord twist.

OBJECTIVE: The objective of this study was to determine the clinical significance of the umbilical cord twist direction. STUDY DESIGN: Two hundred singleton third-trimester placentas with a right umbilical cord twist and 200 placentas with a left umbilical cord twist, which was determined by pathologic examination, were included. Maternal and neonatal outcomes were compared with the use of Fisher's exact and Mann Whitney U tests; a probability value of <.05 considered statistically significant. RESULTS: Placenta previa was more common in patients with a right umbilical cord twist compared with a left umbilical cord twist (6.0% vs 1.5%; P<.05). There was a trend towards an increased incidence of single umbilical artery in patients with a right umbilical cord twist (2.5% vs 0%; P=.06). The incidence of fetal demise, intrauterine growth restriction, chromosomal abnormalities, congenital anomalies, preterm delivery, infant gender, birth weight, maternal age, and parity were similar between the 2 groups. CONCLUSION: Placenta previa is associated with a right umbilical cord twist.