Association between pregnancy and long-term cardiac outcomes in individuals with congenital heart disease.

BACKGROUND: As early life interventions for congenital heart disease improve, more patients are living to adulthood and are considering pregnancy. Scoring and classification systems predict the maternal cardiovascular risk of pregnancy in the context of congenital heart disease, but these scoring systems do not assess the potential subsequent risks following pregnancy. Data on the long-term cardiac outcomes after pregnancy are unknown for most lesion types. This limits the ability of healthcare practitioners to thoroughly counsel patients who are considering pregnancy in the setting of congenital heart disease. OBJECTIVE: We aimed to evaluate the association between pregnancy and the subsequent long-term cardiovascular health of individuals with congenital heart disease. STUDY DESIGN: This was a retrospective longitudinal cohort study of individuals identifying as female who were receiving care in two adult congenital heart disease centers from 2014 to 2019. Patient data were abstracted longitudinally from a patient age of 15 years (or from the time of entry into the healthcare system) to the conclusion of the study, death, or exit from the healthcare system. The primary endpoint, a composite adverse cardiac outcome (death, stroke, heart failure, unanticipated cardiac surgery, or a requirement for a catheterized procedure), was compared between parous (at least one pregnancy >20 weeks' gestation) and nulliparous individuals. By accounting for differences in the follow-up, the effect of pregnancy was estimated based on the time to the composite adverse outcome in a proportional hazards regression model adjusted for the World Health Organization class, baseline cardiac medications, and number of previous sternotomies. Participants were also categorized according to their lesion type, including septal defects (ventricular septal defects, atrial septal defects, atrioventricular septal defects, or atrioventricular canal defects), right-sided valvular lesions, left-sided valvular lesions, complex cardiac anomalies, and aortopathies, to evaluate if there is a differential effect of pregnancy on the primary outcome when adjusting for lesion type in a sensitivity analysis. RESULTS: Overall, 711 individuals were eligible for inclusion; 209 were parous and 502 nulliparous. People were classified according to the World Health Organization classification system with 86 (12.3%) being classified as class I, 76 (10.9%) being classified as class II, 272 (38.9%) being classified as class II to III, 155 (22.1%) being classified as class III, and 26 (3.7%) being classified as class IV. Aortic stenosis, bicuspid aortic valve, dilated ascending aorta or aortic root, aortic regurgitation, and pulmonary insufficiency were more common in parous individuals, whereas dextro-transposition of the great arteries, Turner syndrome, hypoplastic right heart, left superior vena cava, and other cardiac diagnoses were more common in nulliparous individuals. In multivariable modeling, pregnancy was associated with the composite adverse cardiac outcome (36.4%% vs 26.1%%; hazard ratio, 1.83; 95% confidence interval, 1.25-2.66). Parous individuals were more likely to have unanticipated cardiac surgery (28.2% vs 18.1%; P=.003). No other individual components of the primary outcome were statistically different between parous and nulliparous individuals in cross-sectional comparisons. The association between pregnancy and the primary outcome was similar in a sensitivity analysis that adjusted for cardiac lesion type (hazard ratio, 1.61; 95% confidence interval, 1.10-2.36). CONCLUSION: Among individuals with congenital heart disease, pregnancy was associated with an increase in subsequent long-term adverse cardiac outcomes. These data may inform counseling of individuals with congenital heart disease who are considering pregnancy.

In-hospital outcomes and long-term survival of women of childbearing age with aortic dissection.

OBJECTIVE: In the present study, we defined the outcomes and effects of pregnancy in a cohort of women of childbearing age with acute aortic dissection (AAD). METHODS: We reviewed our database of AAD to identify all eligible female patients. Women aged <45 years were included. Data on pregnancy timing with respect to the occurrence of dissection, the demographic data, dissection extent, dissection treatment, dissection-related outcomes, overall maternal and fetal mortality, and genetic testing results were analyzed. RESULTS: A total of 62 women aged <45 years had presented to us with AAD from 1999 to 2017. Of the 62 women, 37 (60%) had had a history of pregnancy at AAD. Of these 37 patients, 10 (27%) had had a peripartum aortic dissection, defined as dissection during pregnancy or within 12 months postpartum. Of the 10 AADs, 5 were type A and 5 were type B. Three patients had presented with AAD during pregnancy (one in the second and two in the third trimester). Five patients (50%) had developed AAD in the immediate postpartum period (within 3 months) and two (20%) in the late postpartum period. For the immediate postpartum AADs (<3 months), four of the five patients delivered via cesarean section. Of these 10 peripartum AADs, 3 (30%) had occurred in patients with known Marfan syndrome. In-hospital mortality for those with peripartum AAD was 10% (1 of 10). Fetal mortality was 20% (2 of 10). CONCLUSIONS: The frequency of aortic dissection in women of childbearing age at our institution was low. However, pregnancy might increase the risk of those young women genetically predisposed to dissection events. From these data, this risk appears to be greatest in the immediate postpartum period, even for those who undergo cesarean section. Close clinical and radiographic surveillance is required for all women with suspected aortopathy, especially in the third trimester and early postpartum period.

Renal artery aneurysms in pregnancy-Management of an uncommon but serious vascular lesion.

Renal artery aneurysm (RAA) is defined as a localized saccular or fusiform dilation of the renal vasculature that exceeds 50% of the adjacent artery diameter. RAAs are rare in the general population and account for <1% of all peripheral aneurysms. Incidental diagnosis of RAA has increased due to the widespread clinical application of visceral duplex ultrasound scanning and computed tomography imaging. While the diagnosis of RAA before or during pregnancy is rare, pregnancy increases the risk of rupture significantly during the third trimester, with associated high mortality rates for both mother and fetus. The rarity of pregnancy-related RAAs contributes to our limited knowledge of their natural history, morphologic features, criteria for intervention, and treatment options. This review compiles opinions of published articles to provide an updated overview of RAA in pregnancy and aid clinicians in the management of this rare but serious vascular condition. An RAA 1.5 cm in diameter requires open or endovascular treatment in a woman planning to become pregnant or who is pregnant.

The Spectrum of Pregnancy-Associated Heart Failure Phenotypes: An Echocardiographic Study.

Compare echocardiographic phenotypes of women presenting with peripartum heart failure. A retrospective case-control study of pregnant women (n = 86) presenting with PP-HF symptoms (i.e., dyspnea, PND, orthopnea) and objective examination and laboratory findings (lung congestion, elevated JVP and/or HJR, elevated brain natriuretic peptide [BNP] and pulmonary edema on chest X-ray). Three distinct phenotypes based on echocardiographically-defined LVEF were identified: (a) PP-HF with preserved ejection fraction (PP HFpEF, LVEF: > 50%); (b) PP-HF with midrange ejection fraction (PP HFmrEF, LVEF: 40-50%); c) PP-HF with reduced ejection fraction (PP HFrEF, LVEF: < 40%); these were compared with 17 pregnant subjects without PP-HF symptoms/findings. Most patients were African American (n = 63; 73%), with low prevalence of hypertension (n = 15, 17%) or diabetes mellitus (n = 5, 5%); pre-eclampsia was highly prevalent (n = 52, 60%). Echocardiographically-defined phenotypes (HFpEF, n = 37; HFmrEF, n = 18; HFrEF, n = 31) showed progressively worse abnormalities in LV remodeling (LV enlargement, LV hypertrophy), LV diastolic function, and right ventricular function; the three PP-HF groups had comparable abnormalities in increased left atrial size and estimated peak tricuspid valve regurgitation velocity. Compared to controls, all three groups had significantly increased filling pressures, LV mass index and left atrial volume index. Peripartum women presenting with the clinical syndrome of heart failure exhibit a spectrum of echocardiographic phenotypes. Significant abnormalities in LV structure, diastolic function, LA size, peak TR velocity and RV function were identified in women with preserved and mid-range EFs, suggesting pregnancy-related cardiac pathophysiologic derangements.

Acute Stroke During Pregnancy and Puerperium.

BACKGROUND: Acute stroke during pregnancy or within 6 weeks of childbirth is devastating for the mother and her family, yet data regarding incidence and contemporary trends are very limited. OBJECTIVES: This study sought to investigate the incidence and outcomes of acute stroke and transient ischemic attack during pregnancy or within 6 weeks of childbirth in a large database. METHODS: The National Inpatient Sample was queried to identify women age ≥18 years in the United States with pregnancy-related hospitalizations from January 1, 2007, to September 30, 2015. Temporal trends in acute stroke (ischemic and hemorrhagic)/transient ischemic attack incidence and in-hospital mortality were extracted. RESULTS: Among 37,360,772 pregnancy-related hospitalizations, 16,694 (0.045%) women had an acute stroke. The rates of acute stroke did not change (42.8 per 100,000 hospitalizations in 2007 vs. 42.2 per 100,000 hospitalizations in 2015; ptrends = 0.10). Among those with acute stroke, there were increases in prevalence of obesity, smoking, hyperlipidemia, migraine, and gestational hypertension. Importantly, in-hospital mortality rates were almost 385-fold higher among those who had a stroke (42.1 per 1,000 pregnancy-related hospitalizations vs. 0.11 per 1,000 pregnancy-related hospitalizations; p < 0.0001). The rates of in-hospital mortality among pregnant women with acute stroke decreased (5.5% in 2007 vs. 2.7% in 2015; ptrends < 0.001). CONCLUSIONS: In this contemporary analysis of pregnancy-related hospitalizations, acute stroke occurred in 1 of every 2,222 hospitalizations, and these rates did not decrease over approximately 9 years. The prevalence of most stroke risk factors has increased. Acute stroke during pregnancy and puerperium was associated with high maternal mortality, although it appears to be trending downward. Future studies to better identify mechanisms and approaches to prevention and management of acute stroke during pregnancy and puerperium are warranted.

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge / Gravidez em Portadoras de Cardiopatias Congênitas Complexas: Um Constante Desafio

Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

Resumo Fundamento: A contínua habilidade na conduta das cardiopatias congênitas complexas (CCC) tem permitido o alcance da idade fértil. Contudo, a heterogeneidade das lesões cardíacas na idade adulta limita a estimativa do prognóstico da gravidez. Objetivo: Estudar a evolução materno-fetal das gestantes portadoras de CCC e analisar as variáveis presumíveis de prognóstico. Método: No período de 10 anos, 435 gestantes portadoras de cardiopatias congênitas foram consecutivamente incluídas no Registro do Instituto do Coração (Registro-InCor). Dentre elas, foram selecionadas 42 gestações em 40 mulheres com CCC (24,5 ± 3,4 anos) que haviam sido desaconselhadas a engravidar. As cardiopatias de base distribuíram-se em: transposição das grandes artérias, atresia pulmonar, atresia tricúspide, ventrículo único, dupla via de saída de ventrículo direito, dupla via de entrada de ventrículo esquerdo e outras lesões estruturais. As cirurgias realizadas foram Rastelli, Fontan, Jatene, Senning, Mustard e outros procedimentos combinados, como tunelização, Blalock Taussing e Glenn. Oito pacientes (20%) não haviam sido operadas, e 19 (47,5%) apresentavam hipoxemia. O protocolo de atendimento incluiu: registro da saturação de oxigênio, hemoglobina sérica, hematócrito, ajuste das medicações, anticoagulação individualizada e hospitalização a partir de 28 semanas de gestação, em face da gravidade do quadro clínico e obstétrico. Na análise estatística, o nível de significância adotado foi de 0,05. Resultado: Somente 17 gestações (40,5%) não tiveram complicações maternas nem fetais. Houve 13 problemas maternos (30,9%) e 2 mortes (4,7%) causadas por hemorragia pós-parto e pré-eclâmpsia grave, ambas em pacientes que apresentavam hipoxemia. Houve 7 perdas fetais (16,6%), 17 bebês prematuros (40,5%) e 2 recém-nascidos (4,7%) com cardiopatia congênita. As complicações materno-fetais foram significativamente maiores em pacientes que apresentavam hipoxemia (p < 0,05). Conclusão: O alcance da idade reprodutiva em pacientes com CCC é crescente; contudo, a má evolução materno-fetal desaconselha a gravidez, particularmente nas pacientes que apresentam hipoxemia.

Poor Outcome of Surgical Management of Acute Malfunctioning Mechanical Mitral Valve During Pregnancy. Should Centers with Limited Resources Find Different Options?

BACKGROUND: Rheumatic heart disease (RHD) is the most common cardiac disease in pregnancy in developing countries with the mitral valve being the most affected. In this study, the results of surgical intervention in pregnant women presented with acute malfunctioning mechanical mitral valve were discussed. METHODS: All patients underwent emergency redo mitral valve replacement due to acute malfunctioning mechanical mitral valve during pregnancy in a single center between January 2005 and December 2017. These patients were retrospectively analyzed. Additionally, anticoagulation strategy before the event and outcomes for the mother and the fetus were outlined. RESULTS: Between 2005 and 2017, 16 pregnant women arrived in a single tertiary center with acute malfunctioning mechanical mitral valves. The mean gestational age at the time of presentation was 13.19 ± 2.6 weeks. Fifteen out of the 16 patients changed their anticoagulation regimen either with or without medical advice. After replacing the valve, cardiopulmonary bypass successfully was weaned in 12 patients, who were transferred to the ICU sedated and ventilated with variable doses of chemical Inotropes. The remaining 4 patients died on the table after failure of weaning from bypass. In one case, the patient developed immediate postoperative stroke with the Glasgow Coma Scale (GCS) of 7, CT brain revealed massive infarction, her fetus was not viable, she remained sedated and ventilated, and she passed away on post-operative Day 12, due to pneumonia and sepsis. Another patient, with a viable fetus, passed away on post-operative Day 1, due to low cardiac output. CONCLUSION: Acute malfunctioning MHV during pregnancy represents a real dilemma to patients and caregivers. It carries high fetal and maternal morbidity and mortality, especially in centers with limited resources. We believe that an alternative plane must be formulated for such patients to avoid devastating complications, including maternal and fetal deaths.

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge.

BACKGROUND: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. OBJECTIVES: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. METHODS: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. RESULTS: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. CONCLUSIONS: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

Cardiomyopathy and pregnancy.

Cardiomyopathy is a group of disorders in which the heart muscle is structurally and functionally abnormal in the absence of other diseases that could cause observed myocardial abnormality. The most common cardiomyopathies are hypertrophic and dilated cardiomyopathy. Rare types are arrhythmogenic right ventricular, restrictive, Takotsubo and left ventricular non-compaction cardiomyopathies. This review of cardiomyopathies in pregnancy shows that peripartum cardiomyopathy is the most common cardiomyopathy in pregnancy. Peripartum cardiomyopathy develops most frequently in the month before or after partum, whereas dilated cardiomyopathy often is known already or develops in the second trimester. Mortality in peripartum cardiomyopathy varies from <2% to 50%. Few reports on dilated cardiomyopathy and pregnancy exist, with only a limited number of patients. Ventricular arrhythmias, heart failure, stroke and death are found in 39%-60% of high-risk patients. However, patients with modest left ventricular dysfunction and good functional class tolerated pregnancy well. Previous studies on >700 pregnancies in 500 women with hypertrophic cardiomyopathy showed that prognosis was generally good, even though three deaths were reported in high-risk patients. Complications include different types of supraventricular and ventricular arrhythmias, heart failure and ischaemic stroke. Recent studies on 200 pregnancies in 100 women with arrhythmogenic right ventricular cardiomyopathy have reported symptoms, including heart failure in 18%-33% of pregnancies. Ventricular tachycardia was found in 0%-33% of patients and syncope in one patient. Information on rare cardiomyopathies is sparse and only presented in case reports. Close monitoring by multidisciplinary teams in referral centres that counsel patients before conception and follow them throughout gestation is recommended.

"Ikigai", Subjective Wellbeing, as a Modifier of the Parity-Cardiovascular Mortality Association　- The Japan Collaborative Cohort Study.

BACKGROUND: Nulliparity is associated with an excess risk of cardiovascular disease (CVD). "Ikigai", subjective wellbeing in Japan, is associated with reduced risk of CVD. The impact of ikigai on the association between parity and the risk of CVD, however, has not been reported.Methods and Results:A total of 39,870 Japanese women aged 40-79 years without a history of CVD, cancer or insufficient information at baseline in 1988-1990, were enrolled and followed until the end of 2009. They were categorized into 7 groups according to parity number 0-≥6. Using Cox regression hazard modeling, the associations between parity and mortality from stroke, coronary artery disease, and total CVD were investigated. During the follow-up period, 2,121 total CVD deaths were documented. No association was observed between parity and stroke and CVD mortality in women with ikigai, but there was an association in those without ikigai. The multivariable hazard ratios of stroke and total CVD mortality for nulliparous women without ikigai vs. those with 1 child were 1.87 (95% CI: 1.15-3.05) and 1.46 (95% CI: 1.07-2.01), respectively, and that for stroke mortality in high parity women without ikigai was 1.56 (95% CI: 1.00-2.45). CONCLUSIONS: Nulliparous or high parity women without ikigai had higher mortality from stroke and/or total CVD, suggesting that ikigai attenuated the association between parity and CVD mortality in Japanese women.

Risk factors for perinatal cardiac complications in pregnancy with pulmonary hypertension.

OBJECTIVE: To analyze the risk factors for complications in pregnancy associated with pulmonary hypertension (PH) and to develop a logistic regression model to predict cardiac outcomes. METHODS: A retrospective analysis was performed on 249 women with PH, who were followed at the Beijing Anzhen Hospital, Affiliated to the Capital Medical University, from January 2012 to March 2015. All pregnancies were recorded. Overall, 214 cases of pulmonary arterial hypertension were identified. Univariate analysis and multivariate analysis were performed to determine the risk factors occurring during or after pregnancy in women with PH. Ultimately, six independent risk factors for cardiac events were determined. RESULTS: There were 70 cases of cardiac complications (28.1%) with PH, including 7 cases of maternal death (2.81%). Independent risk factors were rapid progression of symptoms [OR=3.044, 95%CI (1.042-8.895), P<0.05], brain natriuretic peptide (BNP) plasma levels ≥300pg/mL [OR=5.543, 95%CI (1.403-21.896), P<0.05], severe pulmonary hypertension (PAP≥80mmHg, 1mmHg=0.133kPa) [OR=6.769, 95%CI (2.748-16.677), P<0.05], World Health Organization functional class (WHO-FC) III-IV [OR=6.053, 95%CI (2.638-13.886), P<0.05], PH pre-pregnancy [OR=5.434, 95%CI (1.298-22.738), P<0.05], and delivery ≥28weeks gestation [OR=10.876, 95%CI (3.957-29.893), P<0.05]. CONCLUSIONS: Early advice on contraception for patients with PH, and the need for patients to undergo a comprehensive assessment of cardiac function pre-pregnancy are suggested from the results of the present study.

[Maternal death by stroke. Results from the French enquiry into maternal deaths, 2010-2012]. / Morts maternelles par accident vasculaire cérébral. Résultats de l'ENCMM, FRANCE 2010-2012.

Stroke is a rare event during pregnancy (10/100,000) and can be ischemic (24%), hemorrhagic (74%) or both (2%). Pregnancy probably increases the risk even if it is discussed for arteriovenous malformation (AVM), aneurismal subarachnoid hemorrhage (SAH) and cavernomas. Between 2010 and 2012, 31 maternal deaths were associated with stroke. In 22 cases, stroke was the direct cause of death giving a maternal mortality ratio of 0,9/100,000 witch is not different from the former report (2007-2009). There were 2 cerebral thrombophlebitis, 2 ischemic strokes and 18 hemorrhagic strokes (4 SAH, 2 AVM). These deaths occurred during ongoing pregnancy in 5 cases (25%), after miscarriage in 1 case (5%) and in the post-partum period in 14 cases (70%). In this last situation, stroke occurred before delivery in 4 cases and during the post-partum period in 9 cases (1 to 9 days) (1 unknown). There were 7 vaginal deliveries (50%) and 6 emergency cesarean sections (43%) (1 unknown). Most of those deaths were considered to be unavoidable (13/17, 76%). Four deaths were considered by the experts as being possibly avoidable (delay diagnosis, diagnostic error, inadequate treatment, lake of interruption of the pregnancy). Analyzing those deaths remind that any sudden, severe and unusual headache must be explored and that pregnancy does not contraindicate any of the diagnostic examinations (TDM, angio-TDM, MRI) or invasive treatments (surgery, arterio-embolization, fibrinolysis) necessary for its management. Furthermore, the diagnosis of postdural puncture headache should not be establishedwithout imaging when the symptomatology is not absolutely typical.

Epidemiology of venous thromboembolism in Africa: a systematic review.

Essentials Venous thromboembolism (VTE) is among the three main causes of cardiovascular disease worldwide. This review is the first to summarize the epidemiology of VTE in African populations. The prevalence of VTE in Africa is high following surgery, in pregnancy and post-partum. At least one quarter of patients at risk of VTE in Africa are not receiving prophylaxis. SUMMARY: Background Venous thromboembolism (VTE) is among the three leading causes of cardiovascular disease worldwide. Despite its high burden, there has been no previous study summarizing the epidemiology of VTE in African populations. Hence, we conducted this systematic review to determine the prevalence, incidence and mortality associated with VTE, and to evaluate the use of VTE prophylaxis in Africa. Methods We searched PubMed, Scopus and African Journals Online to identify articles published on VTE in Africa from inception to November 19, 2016, without language restriction. The reference list of eligible articles were further scrutinized to identify potential additional studies. Results Overall, we included 21 studies. The great majority of the studies yielded a moderate risk of bias. The prevalence of deep vein thrombosis (DVT) varied between 2.4% and 9.6% in postoperative patients, and between 380 and 448 per 100 000 births per year in pregnant and postpartum women. The prevalence of pulmonary embolism (PE) in medical patients varied between 0.14% and 61.5%, with a mortality rate of PE between 40% and 69.5%. The case-fatality rate after surgery was 60%. Overall, 31.7-75% of the patients were at risk of VTE, and between 34.2% and 96.5% of these received VTE prophylaxis. Conclusion The prevalence of VTE and associated mortality are high following surgery, and in pregnant and postpartum women in Africa. At least one-quarter of patients who are at risk for VTE in Africa are not receiving prophylaxis. These results are generated from studies with small sample size, highlighting an urgent need for well-designed studies with larger sample size to evaluate the true burden of VTE in Africa.

Treatment options for severe pulmonary embolism during pregnancy and the postpartum period: a systematic review.

Essentials The evidence on how to manage life-threatening pregnancy-related pulmonary embolism (PE) is scarce. We systematically reviewed all available cases of (sub)massive PE until December 2016. Thrombolysis in such severe PE was associated with a high maternal survival (94%). The major bleeding risk was much greater in the postpartum (58%) than antepartum period (18%). SUMMARY: Background Massive pulmonary embolism (PE) during pregnancy or the postpartum period is a rare but dramatic event. Our aim was to systematically review the evidence to guide its management. Methods We searched Pubmed, Embase, conference proceedings and the RIETE registry for published cases of severe (submassive/massive) PE treated with thrombolysis, percutaneous or surgical thrombectomy and/or extracorporeal membrane oxygenation (ECMO), occurring during pregnancy or within 6 weeks of delivery. Main outcomes were maternal survival and major bleeding, premature delivery, and fetal survival and bleeding. Results We found 127 cases of severe PE (at least 83% massive; 23% with cardiac arrest) treated with at least one modality. Among 83 women with thrombolysis, survival was 94% (95% CI, 86-98). The risk of major bleeding was 17.5% during pregnancy and 58.3% in the postpartum period, mainly because of severe postpartum hemorrhages. Fetal deaths possibly related to PE or its treatment occurred in 12.0% of cases treated during pregnancy. Among 36 women with surgical thrombectomy, maternal survival and risk of major bleeding were 86.1% (95% CI, 71-95) and 20.0%, with fetal deaths possibly related to surgery in 20.0%. About half of severe postpartum PEs occurred within 24 h of delivery. Conclusions Published cases of thrombolysis for massive PE during pregnancy and the postpartum period suggest a high maternal and fetal survival (94% and 88%). In the postpartum period, given the high risk of major bleeding with thrombolysis, other therapeutic options (catheter [or surgical] thrombectomy, ECMO) may be considered if available.

Comparison of clinical outcomes in peripartum cardiomyopathy and age-matched dilated cardiomyopathy: A 15-year nationwide population-based study in Asia.

Peripartum cardiomyopathy (PPCM), often classified as a form of dilated cardiomyopathy (DCM), is the myocardial dysfunction that occurs in late pregnancy and through the first few postpartum months.The aim of this study is to investigate the differences in the clinical outcomes of PPCM and DCM.Electronic medical records from 1997 to 2011 were retrieved from the Taiwan National Health Insurance Research Database. Patients with PPCM were compared with age- and clinical characteristics-matched patients with DCM. Primary outcomes were 1- and 3-year heart failure (HF) readmission, cardiac death, all-cause mortality, and major adverse cardiovascular events. Secondary outcomes were myocardial infarction, new onset of dialysis, heart transplant, and cerebrovascular accident. Follow-up period was divided into "within the first year" and "after the first year."A total of 527,979 patients (253,166 females) were hospitalized with a principal diagnosis of HF during 1997 to 2011 period. After excluding patients aged <18 and >50 years, patients with other forms of HF, and those with a history of cerebrovascular accidents or coronary artery disease, 797 patients with PPCM and 1267 patients with DCM were evaluated. Propensity score matching yielded 391 patients in each group. Patients with DCM had a significantly worse prognosis compared to those with PPCM for all primary and secondary outcomes at the 1- and 3-year follow-ups. After 1 year, the HF readmission rate did not significantly differ between the 2 diseases, suggesting that HF medications should be aggressively instituted in patients with PPCM.This is the first study to directly compare the clinical outcomes between age-matched patients with PPCM and DCM. Patients with PPCM had a significantly better prognosis across all cardiovascular endpoints compared to patients with DCM.

Myocardial Damage Detected by Late Gadolinium Enhancement Cardiac Magnetic Resonance Is Uncommon in Peripartum Cardiomyopathy.

BACKGROUND: In peripartum cardiomyopathy, the prevalence of focal myocardial damage detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance is important to elucidate mechanisms of myocardial injury and cardiac dysfunction. LGE equates irreversible myocardial injury, but LGE prevalence in peripartum cardiomyopathy is uncertain. METHODS AND RESULTS: Among 100 women enrolled within the Investigations of Pregnancy Associated Cardiomyopathy cohort, we recruited 40 women at 13 centers to undergo LGE cardiovascular magnetic resonance, enrolled within the first 13 weeks postpartum. Follow-up scans occurred at 6 months postpartum, and death/transplant rates at 12 months. Baseline characteristics did not differ significantly in the parent cohort according to cardiovascular magnetic resonance enrollment except for mechanical circulatory support. LGE was noted only in 2 women (5%) at baseline. While left ventricular dysfunction with enlargement was prevalent at baseline cardiovascular magnetic resonance scans (eg, ejection fraction 38% [Q1-Q3 31-50%], end diastolic volume index=108 mL/m2 [Q1-Q3 83-134 mL/m2]), most women demonstrated significant improvements at 6 months, consistent with a low prevalence of LGE. LGE was not related to baseline clinical variables, ejection fraction, New York Heart Association heart failure class, or mortality. Neither of the 2 women who died exhibited LGE. LGE was inversely associated with persistent left ventricular ejection fraction at 6 months (P=0.006). CONCLUSIONS: Factors other than focal myocardial damage detectable by LGE explain the initial transient depressions in baseline left ventricular ejection fraction, yet focal myocardial damage may contribute to persistent myocardial dysfunction and hinder recovery in a small minority. Most women exhibit favorable changes in ventricular function over 6 months. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01085955.

Pregnancy-Related Spontaneous Coronary Artery Dissection: A Case Series and Literature Review.

BACKGROUND: Cardiac emergencies during pregnancy are rare but have significant associated morbidity and mortality when they do occur. The emergency physician must not only be aware of potentially life-threatening conditions in the pregnant woman, but also know the emergent management and treatment of these conditions to avoid worsening of the underlying condition. Pregnancy-related spontaneous coronary artery dissection has been described in the cardiology literature, but is not well-known in emergency medicine literature. CASE SERIES: We present a case series of six previously healthy women ages 27 to 39 years who presented 1 to 75 days after delivery with spontaneous coronary artery dissection. The left main coronary was involved in 5 of 6 cases. One patient died, 5 survived. Two survivors maintained significant long-term disability. The patient that died had the diagnosis made on autopsy, the others were diagnosed with coronary angiography. Two patients were treated with stents, 2 with coronary artery bypass surgery, and 2 with medical management. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergent coronary catheterization is indicated if this diagnosis is suspected. However, emergency care teams must also understand how and why management including coronary artery catheterization can exacerbate the underlying condition. The role of coronary artery computed tomography remains unknown, although it exposes the fetus to significant radiation if the woman is still pregnant at presentation. Medical management is indicated with diffuse or distal disease as pregnancy-related coronary artery dissections often resolve with time. Localized discrete lesions may be stented. Coronary artery bypass graft surgery may be considered if the left main artery is involved or there are multiple proximal lesions. Cardiac transplantation is indicated rarely.

Pregnancy and the Risk of Spontaneous Coronary Artery Dissection: An Analysis of 120 Contemporary Cases.

BACKGROUND: Because of the rarity of this condition, information on pregnancy-associated spontaneous coronary artery dissection is limited. We reviewed a large number of contemporary pregnancy-associated spontaneous coronary artery dissection cases in an attempt to define the clinical characteristics and provide management recommendations. METHODS AND RESULTS: A literature search for cases of pregnancy-associated spontaneous coronary artery dissection reported between 2000 and 2015 included 120 cases; 75% presented with ST-segment-elevation myocardial infarction, and 80% had anterior myocardial infarction. Left anterior descending coronary artery was involved in 72% of cases, left main segment in 36%, and 40% had multivessel spontaneous coronary artery dissection. Ejection fraction was reduced to <40% in 44% of cases. Percutaneous coronary intervention was successful in only 50% of cases. Coronary artery bypass surgery was performed in 44 cases because of complex anatomy, hemodynamic instability, or failed percutaneous coronary intervention. Maternal complications included cardiogenic shock (24%), mechanical support (28%), urgent percutaneous coronary intervention (28%), urgent coronary artery bypass surgery (27.5%), maternal mortality (4%), and fetal mortality (2.5%). During follow-up for 305±111 days, there was a high incidence of symptoms because of persistent or new spontaneous coronary artery dissections, and 5 women needed heart transplantation or ventricular assist device implantation. CONCLUSIONS: Pregnancy-associated spontaneous coronary artery dissection is commonly associated with left anterior descending, left main, and multivessel involvement, which leads to a high incidence of reduced ejection fraction, and life-threatening maternal and fetal complications. Percutaneous coronary intervention is associated with low success rate and high likelihood of complications, and coronary artery bypass surgery is often required. Recurrent ischemic events because of persistent or new spontaneous coronary artery dissection are common during long-term follow-up.

Pregnancy in Women with Cardiovascular Diseases.

Patients with cardiovascular disease represent a significant cohort at risk for complications during pregnancy. The normal physiologic changes of pregnancy could further compromise the hemodynamics of various cardiovascular conditions, resulting in clinical deterioration and even death. The fetus of a gravida with cardiovascular disease also has an increased risk of morbidity, including an increased risk of inherited cardiac genetic disorders, fetal growth restriction, and premature delivery. These complications also increase the risk for antenatal and perinatal mortality. Ideally, the management of a patient with cardiac disease who is considering pregnancy should start with pre-conception counseling that outlines the maternal and fetal complications associated with her particular cardiac disorder. The pregnancy is best managed by a dedicated team of specialists in maternal-fetal medicine, cardiology, cardiovascular surgery, anesthesiology, and neonatology, preferably in a tertiary care center.

International Registry of Patients Carrying TGFBR1 or TGFBR2 Mutations: Results of the MAC (Montalcino Aortic Consortium).

BACKGROUND: The natural history of aortic diseases in patients with TGFBR1 or TGFBR2 mutations reported by different investigators has varied greatly. In particular, the current recommendations for the timing of surgical repair of the aortic root aneurysms may be overly aggressive. METHODS AND RESULTS: The Montalcino Aortic Consortium, which includes 15 centers worldwide that specialize in heritable thoracic aortic diseases, was used to gather data on 441 patients from 228 families, with 176 cases harboring a mutation in TGBR1 and 265 in TGFBR2. Patients harboring a TGFBR1 mutation have similar survival rates (80% survival at 60 years), aortic risk (23% aortic dissection and 18% preventive aortic surgery), and prevalence of extra-aortic features (29% hypertelorism, 53% cervical arterial tortuosity, and 27% wide scars) when compared with patients harboring a TGFBR2 mutation. However, TGFBR1 males had a greater aortic risk than females, whereas TGFBR2 males and females had a similar aortic risk. Additionally, aortic root diameter prior to or at the time of type A aortic dissection tended to be smaller in patients carrying a TGFBR2 mutation and was ≤45 mm in 6 women with TGFBR2 mutations, presenting with marked systemic features and low body surface area. Aortic dissection was observed in 1.6% of pregnancies. CONCLUSIONS: Patients with TGFBR1 or TGFBR2 mutations show the same prevalence of systemic features and the same global survival. Preventive aortic surgery at a diameter of 45 mm, lowered toward 40 in females with low body surface area, TGFBR2 mutation, and severe extra-aortic features may be considered.