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.

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.

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.

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).

Antepartum nonobstetrical surgery at ≥23 weeks' gestation and risk for preterm delivery.

OBJECTIVE: We sought to describe the influence of antepartum nonobstetrical surgical procedures performed at viable fetal gestational ages (GAs) on incidence of preterm delivery. STUDY DESIGN: This was a retrospective case series of patients requiring nonobstetrical surgery at ≥23 weeks' gestation at the Mayo Clinic during the interval 1992 through 2014. Data were abstracted for maternal demographic variables, operative procedure, anesthetic type, whether intraoperative fetal monitoring was employed, and both GA and method of delivery. RESULTS: In all, 111 patients underwent 121 operative procedures at a mean GA of 29.2 weeks (range, 23-37 weeks). The majority of procedures were completed under general anesthesia (88/121, 73%), with intraoperative fetal monitoring performed in 14 cases (14/121, 12%); fetal loss occurred during a single unmonitored procedure. Outcome data were available for the majority of patients (86/111, 78%) with preterm delivery occurring in 41% (35/86) at a mean GA of 36.9 weeks (range, 25-41 weeks). Mean interval from procedure to delivery was 7.7 weeks, with 9 patients (9/86, 10%) delivering within 1 week of surgery. Neither procedures requiring entry into the abdominal cavity (P = .65) nor GA at time of procedure (P = 1.0) statistically influenced the risk of preterm delivery. CONCLUSION: Nonobstetrical surgical procedures performed at or beyond fetal viability increased the incidence of preterm delivery regardless of surgical site or timing of procedure, however the risk of intraoperative or immediate postoperative obstetrical complications was relatively low.

Refractory hypotension during spinal anesthesia for Cesarean delivery due to undiagnosed pheochromocytoma.

Profound hypotension and resistance to conventional vasopressor therapy following administration of spinal anesthesia for Cesarean delivery occurred in a multiparous parturient. Postpartum evaluation for secondary hypertension showed a diagnosis of pheochromocytoma. Pheochromocytoma was mistaken for preeclampsia with significant vasopressor requirement to treat hypotension from spinal anesthesia. If pheochromocytoma is diagnosed during pregnancy and Cesarean delivery is required, spinal anesthesia may not be the optimal choice of anesthesia.

Podocyturia predates proteinuria and clinical features of preeclampsia: longitudinal prospective study.

Podocyturia, the shedding of live podocytes, is present at delivery in women with preeclampsia. The aim of this study was to test whether podocyturia is present earlier in pregnancy and predicts for preeclampsia. We also aimed to compare test characteristics of podocyturia with those of angiogenic factors previously implicated in the pathogenesis of this disorder. We prospectively enrolled 315 women who provided blood and urine samples at the end of the second trimesters of their pregnancies (median, 27 gestational weeks) and within 24 hours of their deliveries (median, 39.5 gestational weeks). Blood samples were analyzed for angiogenic markers, including placental growth factor, the soluble receptor fms-like tyrosine kinase receptor-1 for vascular endothelial growth factor, and endoglin. The urine sediments were analyzed for podocytes, identified by staining for podocin after culturing the urinary sediments for 24 hours. This analysis included all women who developed preeclampsia (n=15), gestational hypertension (n=15), and a subsample of women who remained normotensive throughout pregnancy (n=44), matched for maternal age and number of previous pregnancies to those who developed preeclampsia. At the second trimester collection, all women who developed preeclampsia had podocyturia, compared with none of those who remained normotensive or were diagnosed with gestational hypertension. Podocyturia in the second trimester had a significantly greater sensitivity and specificity for the subsequent diagnosis of preeclampsia than any single angiogenic marker or a combination thereof. Screening for podocyturia at the end of the second trimester may allow for accurate identification of pregnant women at risk for preeclampsia.

Transabdominal and transcervical chorionic villus sampling models to teach maternal-fetal medicine fellows.

OBJECTIVE: Many fellowship programs in maternal-fetal medicine (MFM) lack sufficient exposure and training in chorionic villus sampling (CVS). We describe a novel training model of transabdominal and transcervical CVS. METHODS: A porcine heart simulated a 12-week human uterus with a cervical canal created at the apex of the heart. A piglet was placed within a water-filled condom, which was placed inside the "uterus." A second water-filled condom simulated a maternal bladder. Fresh human placenta was placed between the condom and uterine wall. A zipper-sealed freezer bag with ultrasonic gel simulated an abdominal wall. Transabdominal ultrasound was utilized with this model to perform CVS. RESULTS: The design involved four MFM specialists and three fellows. Twenty-three faculty MFM specialists and eight MFM fellows endorsed the fidelity of both models. One hundred percent of attendees of a procedural workshop agreed that these models could be used to teach proper technique to fellows and faculty. CONCLUSION: We report a novel training model for transabdominal and transcervical CVS to teach proper technique in a nonclinical setting.

Cardiopulmonary bypass during pregnancy.

BACKGROUND: Cardiac surgery during pregnancy carries significant maternal and fetal risk and is typically considered after failure of medical therapy. We sought to determine the maternal and neonatal outcomes of cardiopulmonary bypass during pregnancy. METHODS: Twenty-one pregnant patients undergoing cardiothoracic surgery were identified from the Mayo Clinic surgical database (1976 to 2009). Maternal and neonatal outcomes were reviewed. RESULTS: Operations included 8 aortic valve replacements, 6 mitral valve repair-replacements, 2 myxoma excisions, 1 patent foramen ovale closure, 1 myectomy, 2 aortic aneurysm repairs, and 1 prosthetic aortic valve thrombectomy. Median cardiopulmonary bypass time was 53 minutes (range 16 to 185). Twelve patients (57%) required emergent surgery with a median gestational age (GA) of 25 weeks (range 7 to 35.5). Seven patients underwent cesarean section immediately prior to sternotomy delivering viable infants (median GA 31 weeks). In the remaining patients, three additional preterm births occurred, all in operations performed at an early GA (13 to 15 weeks). Median follow-up was 16 months (range 3 to 305). All patients improved to New York Heart Association functional class I or II. One early maternal death occurred 2 days after emergent mechanical aortic valve thrombectomy and 3 late maternal deaths occurred 2, 10, and 19 years postoperatively. Three fetal deaths occurred in mothers with additional medical comorbidities. CONCLUSIONS: In the current era, cardiothoracic surgery can be performed with relative safety during pregnancy. Fetal complications (prematurity and death) are associated with urgent, high-risk surgery, maternal comorbidity, and early GA. Emergent surgery appears to confer a higher risk of maternal death.

Expectant management of uterine incarceration from an anterior uterine myoma: a case report.

BACKGROUND: Uterine incarceration is an infrequent complication of pregnancy in the early second trimester. Although imaging can be confirmatory, the diagnosis is made primarily on clinical grounds, and definitive treatment involves manual reduction to restore the proper anatomic position. Except for preexisting uterine retroversion, often this event is idiopathic. CASE: A 30-year-old primigravida presented at 15 weeks' gestation with uterine incarceration. Manual replacement was unsuccessful. Spontaneous resolution occurred at 20 weeks, followed by uneventful pregnancy. The patient underwent a classical cesarean section at term due to fetal malpresentation. CONCLUSION: Uterine incarceration may be managed conservatively, with a favorable outcome.

Non-immune hydrops fetalis: prenatal diagnosis and perinatal outcomes.

PURPOSE: To describe the perinatal outcomes after a prenatal diagnosis of non-immune hydrops fetalis (NIHF). METHODS: Retrospective case series. University of Mississippi ultrasound database from July 2000 to January 2003 was reviewed for cases of NIHF. Records abstracted for maternal demographics and neonatal outcomes. RESULTS: Thirty-one cases of NIHF were identified and full data was available for 29 cases. Median gestational age at diagnosis was 17.3 weeks. Structural fetal malformations were noted in 22 cases (76%); most common were cystic hygroma (N = 17, 59%), followed by cardiac (N = 10, 34%). Amniocentesis was performed in 20 cases and 10 were aneuploid. Only 10 fetuses (34%) were liveborn and only five (17%) survived the neonatal period. CONCLUSIONS: Antepartum diagnosis of NIHF conveys a grave perinatal prognosis. Associated anomalies can be expected in approximately 75% of cases. Even for those fetuses achieving viability, survival beyond the neonatal period is rare.

Perinatal outcomes with the antepartum diagnosis of cystic hygroma.

OBJECTIVE: To describe antenatal diagnosis and clinical outcomes of fetal cystic hygroma. STUDY DESIGN: Retrospective case series derived from ultrasound database. Diagnoses were made from July 2000 through January 2003. RESULTS: Twenty-five fetuses were identified with cystic hygroma. Median gestational age at diagnosis was 17.3 weeks. Posterior nuchal cystic hygromas accounted for 21 (84%) cases, while two (8%) were axillary, and one each in the anterior neck and buttock. Additional abnormalities were noted in twenty (80%) fetuses; most common were non-immune hydrops (12/25, 48%) and cardiac defects (5/25, 20%). Eighteen patients (72%) underwent amniocentesis and ten were aneuploid. Six fetuses (25%) sustained intrauterine fetal demise and nine (38%) patients sought therapeutic termination. Nine infants (38%) were liveborn, but only six (25%) survived the neonatal period. CONCLUSIONS: Antenatal diagnosis of fetal cystic hygroma heralds an extremely poor perinatal prognosis. Associated abnormalities were noted in 80% of fetuses with a significant incidence of aneuploidy. Targeted ultrasonography and extensive counseling of parents is recommended.