Pregnancy in women with vascular Ehlers-Danlos syndrome: a case series from the registry of pregnancy and cardiac disease (ROPAC) III.

The Cardio-Obstetrics Patient and the Cardiothoracic Anesthesiologist

Pregnancy heart team: A lesion-specific approach

Heart Disease and Pregnancy: The Need for a Twenty-First Century Approach to Care…

Article, see p 806 Rheumatic heart disease (RHD) is a disease of poverty. Following the near eradication of rheumatic fever and RHD in the developed world and the emergence of competing large global epidemics such as AIDS, tuberculosis, and malaria, research and investment for the control of rheumatic fever and RHD worldwide declined.1 However, over the past decade, RHD has reemerged as a priority in global health. The World Health Organization and the World Heart Federation have called for a 25% reduction in mortality because of cardiovascular causes, including RHD, by 2025.2 Contemporary studies have begun to shed light on the enormous burden of RHD that persists in countries with emerging and developing economies. The highest prevalence of RHD has been reported in India, China, Pakistan, Indonesia, and the Democratic Republic of Congo—accounting for 73% of global cases. Within 20 countries where RHD remains endemic, the age-standardized prevalence is estimated at >1% and greatest in women of childbearing age.2 The economic impact of RHD in these regions is equally as unsettling. Disability-adjusted life years because of RHD is estimated at >10 million, accounting for 0.43% of any-cause disability-adjusted life years worldwide.2 In 2015, the first prospective global registry of RHD, from 12 African countries, India, and Yemen, provided a contemporary description of the presentation, complications, and treatment of RHD.2 The lower a country’s income category, the younger the median age and more advanced the disease at presentation.3 This epidemiological pattern has clear and direct implications for the maternal health of a country’s population. The World Health Organization set forth the goal of reducing the global maternal mortality ratio by three quarters between 1990 and 2015. …

Pregnancy outcomes in women with cardiovascular disease: evolving trends over 10 years in the ESC Registry Of Pregnancy And Cardiac disease (ROPAC).

BackgroundCardiovascular disease is the leading cause of death during pregnancy with thoracic aortic dissection being one of the main causes. Thoracic aortic disease is commonly related to hereditary disorders and...

OBJECTIVESHeart valve replacement during pregnancy is sometimes unavoidable, and the need for anticoagulation further complicates these procedures. Our study describes cases of valve replacement in pregnancy enrolled in the Registry Of Pregnancy And Cardiac disease (ROPAC) III and gives an overview of the published literature.METHODSWe performed a systematic review with new data from the ROPAC III and data available in the literature. ROPAC III is a global, prospective, observational registry that included pregnant women with 1 or more prosthetic valves between January 2018 and April 2023. Electronic databases were searched for studies enrolling pregnant women who underwent valve replacement during pregnancy with a fetus in utero. The primary outcomes were maternal and fetal death. Mixed-effect logistic regression models were used to identify predictors for maternal and fetal mortality.RESULTSA valve replacement was performed in 11 pregnancies. The mother and fetus died in 1 case, and in 2 cases, reversible postoperative complications occurred. We found 74 cases in the literature and calculated an overall maternal and fetal death rate of 9% and 34%, respectively. All maternal deaths occurred in women with a replacement of a prosthetic valve in mitral position. We found valve replacement in the 1st trimester (OR 10.0) and acute malfunctioning of an existing prosthetic valve (OR 19.7) as predictors for maternal mortality, and replacement of an existing prosthetic valve (OR 4.8) as predictor for fetal mortality.CONCLUSIONSValve replacement during pregnancy carries a high maternal and fetal death, especially in women who need a replacement of an existing prosthetic valve.

The vascular type of Ehlers–Danlos syndrome (EDS), EDS type IV (Online Mendelian Inheritance in Man [MIM] #130050) is characterized by thin, translucent skin, easy bruising, and arterial, intestinal, and/or uterine fragility during pregnancy, which may lead to sudden death. It is an autosomal dominant inherited disorder caused by type III procollagen gene (COL3A1: MIM #120180) mutations. Approximately 50% of the COL3A1 mutations are inherited from an affected parent, and 50% are de novo mutations. Each child of an affected individual has a 50% chance of inheriting the mutation and developing the disorder. Pregnant women with vascular EDS are at an increased risk of uterine and arterial rupture during the peripartum period, with high maternal morbidity and mortality rates. We report the first case of an asymptomatic 35-year-old woman at a risk of complications of vascular EDS who underwent presymptomatic evaluation during pregnancy. The sequencing results of both her brother and mother had a one-base-pair deletion, resulting in Glutamate at position 730 changing to Lysine and causing a frame shift and premature termination codon at 61 amino acids from the mutation position (p. Glu730Lysfs*61) on exon 32 of COL3A1. This deletion caused frameshift, leading to a premature termination codon (TAG) at 181 nucleotides downstream in exon 35, which could not be detected by previous total RNA (ribonucleic acid) method. Thus, she was at risk of complications of vascular EDS, and diagnostic testing was employed at 8 weeks of pregnancy to minimize the risk of developing vascular EDS-related complications. The negative presymptomatic diagnostic result allowed the patient to choose normal delivery at term. Vascular EDS is a serious disorder, with high mortality, especially in high-risk women with vascular EDS during pregnancy. The presymptomatic genetic testing of vascular EDS during pregnancy for a high-risk family can help with the early establishment of preventive measures.

Hypertensive disorders of pregnancy in women with structural heart disease: data from the ESC EORP Registry of Pregnancy and Cardiac disease (ROPAC)

BackgroundThe prevalence of ischemic heart disease (IHD) in women of child‐bearing age is rising. Data on pregnancies however are scarce. The objective is to describe the pregnancy outcomes in these women.Methods and ResultsThe European Society of Cardiology‐EURObservational Research Programme ROPAC (Registry of Pregnancy and Cardiac Disease) is a prospective registry in which data on pregnancies in women with heart disease were collected from 138 centers in 53 countries. Pregnant women with preexistent and pregnancy‐onset IHD were included. Primary end point were maternal cardiac events. Secondary end points were obstetric and fetal complications. There were 117 women with IHD, of which 104 had preexisting IHD. Median age was 35.5 years and 17.1% of women were smoking. There was no maternal mortality, heart failure occurred in 5 pregnancies (4.8%). Of the 104 women with preexisting IHD, 11 women suffered from acute coronary syndrome during pregnancy. ST‐segment‒elevation myocardial infarction were more common than non‒ST‐segment‒elevation myocardial infarction, and atherosclerosis was the most common etiology. Women who had undergone revascularization before pregnancy did not have less events than women who had not. There were 13 women with pregnancy‐onset IHD, in whom non‒ST‐segment‒elevation myocardial infarction was the most common. Smoking during pregnancy was associated with acute coronary syndrome. Caesarean section was the primary mode of delivery (55.8% in preexisting IHD, 84.6% in pregnancy‐onset IHD) and there were high rates of preterm births (20.2% and 38.5%, respectively).ConclusionsWomen with IHD tolerate pregnancy relatively well, however there is a high rate of ischemic events and these women should therefore be considered moderate‐ to high‐risk. Ongoing cigarette smoking is associated with acute coronary syndrome during pregnancy.

Vascular Ehlers-Danlos syndrome (vEDS) is a hereditary connective tissue disorder associated with an elevated risk of vascular, uterine and digestive complications. Managing pregnancy in this context can be a challenge. To systematically review the literature data on the complications in pregnancy associated with vEDS. We searched the Pubmed Medline and Embase databases for articles using the following terms "vascular Ehlers-Danlos syndrome" or "vEDS" AND "pregnancy". Women with vEDS. We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS. A total of 121 publications were screened, with six (accounting for 412 pregnancies) included in our review. Of the women included in this sample, 30% were infertile. The miscarriage rate was 13.8% (57/412) and 8.8% of the live births were premature. Obstetric anal sphincter injuries occurred in 11.3% (23/203) of the deliveries. The maternal mortality rate per pregnancy was 5.7%. Women with vEDS present an elevated risk of uterine rupture, vascular events, digestive events and death during pregnancy. Women appear to be most at risk during the peripartum period; to avoid expulsive efforts, a caesarean section should be scheduled at 37 weeks of gestation.

Mortalité maternelle chez les patientes atteintes de syndromes de Marfan ou d’Ehlers-Danlos vasculaire en France, 2001–2012

Aortic coarctation in pregnancy: outcomes and predictors for cardiovascular and hypertensive complications. Data from the ESC Registry of Pregnancy and Cardiac disease (ROPAC)

ObjectiveEbstein’s anomaly is a rare congenital cardiac condition and data regarding pregnancy outcomes in this patient group are scarce. We evaluated the maternal and perinatal risks of pregnancy in 81...

Trends in pregnancy outcomes in patients with pulmonary hypertension: still a long way to go.