Aortic Dissection In Pregnancy Research Articles

MANAGEMENT OF TYPE A DISSECTION IN PREGNANCY: SITE AND TEAM CAN MAKE THE DIFFERENCE

Introduction Aortic dissection is rare in pregnancy but associated with poor maternal and neonatal outcomes. We present a case of delayed diagnosis of type A aortic dissection in pregnancy in a previously healthy woman to raise awareness and highlight the value of an integrated, multidisciplinary approach. Methods A 38-year-old, G1P0 woman was transferred to our hospital with a type A aortic dissection diagnosed at 30 weeks of pregnancy. The patient had no significant medical history and her pregnancy was uneventful. At 29 weeks gestation, however, she developed central chest pain. Her local hospital excluded a diagnosis of pulmonary embolism and discharged her home. She represented 3 days later with shortness of breath and orthopnoea. On the second day of this admission, Type A aortic dissection was diagnosed on computed tomography of the chest. She was transferred to our hospital for access to multiple specialist services including cardiothoracic surgery and high-risk obstetrics, neonatal and adult critical care. On arrival, an ad hoc MDT convened in the emergency department by a senior team of cardiothoracic surgeons, obstetricians, anaesthetists and neonatologists agreed immediate intervention was required. She went directly to theatres for delivery of the baby and dissection repair. Awake establishment of invasive blood pressure monitoring was followed by an opiate-based cardiostable induction of anaesthesia and intubation in a head-up position. The caesarian section was uneventful and exceptional measures to minimise blood loss were not required. The ascending aorta and hemi-arch were repaired on cardio-pulmonary by-pass with circulatory arrest time of 19 minutes without intraoperative complications. The baby was electively intubated and transferred to neonatal ICU in stable condition. Mother and baby were both extubated the following day and discharged home after a week. Discussion Acute aortic dissection should always be included in the differential of acute chest pain in pregnant women. It most commonly occurs during the third trimester or the early postpartum period and it can sometimes present in women with no previous history of aortopathy. High clinical suspicion, careful history taking and examination, CT, MRI or echocardiography can establish the diagnosis. Optimum management to ensure the survival of both mother and fetus varies with gestational age and planning to ensure this requires a complex balance of expertise between specialities. In the third trimester, emergency delivery followed by surgical aortic repair probably offers the best survival chances for both. Institutions which offer senior input from multiple specialities are crucial for the timely management of these complex cases. This need for urgent intervention from outside cardiothoracic surgery in cases with multiple issues must be considered when regional care models for the management of aortic dissection cases are being designed.

Acute Aortic Dissection in Third Trimester Pregnancy with Well Controlled Hypertension

We describe a 32-year-old pregnant woman at 31 + 6 weeks of gestation who presented to a tertiary-care hospital with headache and back pain. She was found to have a sign of tear about 1.3 cm away from the aortic valve, and the torn intima extended upward to the ascending aorta and the descending part of the aortic arch. She delivered a live male neonate via cesarean delivery. She had a history of hypertension with oral Labetalol 50 mg twice times daily. At the very beginning of the onset, physicians in the emergency room wouldn’t be aware of the symptoms of aortic dissections, as well as atypical presentations which include anterior chest pain or chest pain radiating to the back. She was only received anti-hypertension management, until her symptoms of back pain aggravated, and an aortic dissection was confirmed by ultrasound and CT angiography. Then she received a mechanical aortic valve replacement. After 51 days of treatment, she was discharged and recovered without any complications. Aortic dissection in pregnancy is a rare disease. We recommend transthoracic echocardiography every 1 to 2 months to monitor the diameter of the ascending aorta during pregnancy in which patients with hypertension problems or other risk factors of aneurysm and perform the CT angiography scan to confirm aortic dissection in a pregnant or postpartum woman with suspicious pain symptoms. It is essential that multidisciplinary approach in which teams must urgently collaborate to ensure the protection life of both mother and baby.

Pregnancy after ascending aorta surgery in women with Marfan syndrome

Prophylactic surgery of the ascending aorta to prevent the risk of type A aortic dissection is recommended prior to pregnancy in women with Marfan syndrome (MFS) and FBN1 mutation when the aortic diameter is greater than 45 mm or earlier in related syndromes. To assess the risk of aortic dissection in pregnancy after replacement of the ascending aorta in this population. Our study consists of a retrospective analysis of data collected at the national reference centre for Marfan and related syndromes, concerning women with MFS or related syndromes who have undergone prophylactic ascending aorta surgery before the age of 45 years and who have had at least one full-term pregnancy. The primary endpoint was the occurrence of aortic dissection in the peripartum period. In our cohort, 23 women with MFS according to the modified Gent criteria and genetically confirmed have a history of prophylactic aortic surgery followed by pregnancy. In total, there were 49 pregnancies: 35 postoperative and 31 full term. Two postpartum aortic dissections occurred: – one type A on day 3 in a patient with a SMAD3 mutation (dissection of the distal segment of ascending aorta), – one type B at day 46 in a patient with an FBN1 mutation who died following descending aortic replacement surgery 1.5 years later. No aortic dissection was observed in pre-surgical pregnancies. Women with Marfan syndrome or related conditions who have had prophylactic ascending aortic replacement can experienced aortic dissection during pregnancy, however this risk is low (2/31; 6%).

Management and Outcomes of Aortic Dissection in Pregnancy with Marfan Syndrome: A Systematic Review.

In Marfan Syndrome (MFS), aortic dilatation is one of the main cardiovascular manifestations which deteriorate due to the physiological changes during pregnancy. We aimed to assess the up-to-date management and outcomes of aortic root dilation and dissection (AoD) in pregnancy with MFS. A systematic review was conducted and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Original studies published between January 1, 2001 and December 31, 2018 and which described the management and/or outcomes of AoD during or after pregnancy in women with MFS were included. Literature searches were conducted. The PubMed search was performed using terms "Marfan Syndrome" [Mesh] and "Pregnancy" [Mesh] whereas the Google Scholar search was for "Marfan" and "Pregnancy", all words anywhere in the article. The literature search yielded 177 articles on PubMed and 13,900 articles on Google Scholar. Assessment of full-text articles for eligibility after removal of duplicates from both databases yielded 12 eligible studies to be included in the final review. Women with MFS are at high risk of aortic dissection during pregnancy and women with aortic root 41-45 mm should consider avoiding pregnancy. Guideline-specific management of aortic aneurysms in pregnancy will reduce the risk of dissection. Diagnosis and Management of MFS need a multidisciplinary approach and team that should start working early in pregnancy. Further studies are needed to optimize medical and surgical approaches in addition to preconception counselling in highrisk subjects.

Common Maternal Genetic Syndromes II: Marfan Syndrome

Marfan syndrome is an autosomal dominant syndrome that affects various connective tissues including the aorta and skeletal system. It represent a major cause of aortic dissection in individuals with seemingly unremarkable past medical history, and is the most common cause of aortic dissection in pregnancy. Prompt and accurate diagnosis can be lifesaving. Careful physical examination and detailed personal and family history is vital for clinical evaluation. Genetic testing is often needed for accurate diagnosis but result interpretation might be challenging and genetic counseling is always required. Established guidelines can help navigate the challenges in obstetric management, which may include major surgical interventions during or after pregnancy. This review contains 6 figures, 4 tables, and 40 references. Keywords: Marfan syndrome, FBN1, aortic dissection, dilatation, connective tissue, ectopia lentis, pectus, systemic score, Ghent diagnostic criteria, genetic testing.

Common Maternal Genetic Syndromes II: Marfan Syndrome

Marfan syndrome is an autosomal dominant syndrome that affects various connective tissues including the aorta and skeletal system. It represent a major cause of aortic dissection in individuals with seemingly unremarkable past medical history, and is the most common cause of aortic dissection in pregnancy. Prompt and accurate diagnosis can be lifesaving. Careful physical examination and detailed personal and family history is vital for clinical evaluation. Genetic testing is often needed for accurate diagnosis but result interpretation might be challenging and genetic counseling is always required. Established guidelines can help navigate the challenges in obstetric management, which may include major surgical interventions during or after pregnancy. This review contains 6 figures, 4 tables, and 40 references. Keywords: Marfan syndrome, FBN1, aortic dissection, dilatation, connective tissue, ectopia lentis, pectus, systemic score, Ghent diagnostic criteria, genetic testing.

404. A nationwide prospective study of aortic dissection in pregnancy using the UK Obstetric Surveillance System (UKOSS)

Background Aortic dissection (AD) in pregnancy is a life-threatening event and 50% of cases in women aged 40 years occur during pregnancy.1 Patients with AD present with a wide array of symptoms and the condition may be missed and patients treated for other diseases.2 This study estimated the UK incidence of AD in pregnancy and provided insight into presentation and management practices. Methods A national prospective population-based study was undertaken across 229 hospitals over a 2-year period. Participants were diagnosed (a) using suitable imaging (echocardiography, CT or MRI) or (b) at surgery/postmortem. Results The estimated UK incidence of AD in pregnancy was 1.31 per 100,000 maternities (95% CI 0.69–1.93 per 100,000) with 17 cases confirmed by diagnostic criteria. The mean [SD] age of women was 32.5[7.2]years. Fourteen cases were due to type-A dissection (involving ascending aorta), the remainder type-B (no involvement of ascending aorta). Nine women died, all of whom with type-A dissection, giving a case fatality rate of 53%. We compared the UKOSS data for survivors to information available for 21 women with confirmed AD who died (during and just after the UKOSS study period). There were no differences in age, BMI or ethnicity but increased prevalence of pre-existing co-morbidities in women who died. On initial presentation all survivors complained of anterior chest pain (compared to 66% who died). Survivors were more likely to have recorded bilateral BP and pulse readings, prompt imaging (87.5% underwent echocardiography + either CT/MRI aorta). In the survivor group (n = 8), 5 women successfully underwent surgical aortic root replacement (for type-A dissection) after appropriate BP control usually β -blockade. 3 were conservatively managed for type-B dissection. Discussion AD is a rare but serious illness. Thorough physical examination and prompt imaging with quick access to surgery (type-A) or conservative management (typeB) could prevent mortality from AD.

Acute Type A Aortic Dissection in Pregnancy—Diagnostic and Therapeutic Challenges in a Multidisciplinary Setting

Acute Type A Aortic Dissection in Pregnancy—Diagnostic and Therapeutic Challenges in a Multidisciplinary Setting

Acute Type-B Aortic Dissection in Pregnancy: Therapeutic Challenges in a Multidisciplinary Setting

Acute Type-B Aortic Dissection in Pregnancy: Therapeutic Challenges in a Multidisciplinary Setting

769: Aortic dissection in pregnancy- a texas population-based study

769: Aortic dissection in pregnancy- a texas population-based study

Aortic Dissection in Pregnancy: Management Strategy and Outcomes

Aortic dissection in pregnancy is a rare but lethal catastrophe. Clinical experiences are limited. We report our experience in 25 patients focusing on etiology, management strategies, and outcomes. Between June 1998 and February 2015, we treated 25 pregnant women (mean age, 31.6 ± 4.7 years) in whom aortic dissection developed at a mean of 28 ± 10 gestational weeks (GWs). Type A aortic dissection (TAAD) was present in 20 (80%) and type B (TBAD) in 5 (20%). Marfan syndrome was seen in 17 (68%). Management strategy was based on dissection type and GWs. TAADs were managed surgically in 19 (95.0%) and medically in 1 (5.0%). Maternal and fetal mortalities were, respectively, 14.3% (1 of 7) and 0 (0 of 7) in the "delivery first" group (7 of 20), 16.7% (1 of 6) and 33.3% (2 of 6) in "single-stage delivery and aortic repair" group (6 of 20), 16.7% (1of 6) and 66.7% (4 of 6) in "aortic repair first" group (6 of 20), and100% (1 of 1) and 100% (1 of 1) in the "medical management" group (1 of 20). TBADs were managed surgically in 60% (3 of 5) and endovascularly and medically in 20% each (1 of 5). No maternal deaths occurred. Fetal mortality was 100% in the surgical group and 0% in the other groups. During late follow-up, which was complete in 95.2% (20 of 21), 3 maternal and 2 fetal deaths occurred in the TAAD group. Overall maternal survival was 68.6% at 5 years. Marfan syndrome predominates among women with aortic dissection in pregnancy. For TAADs, after 28 GWs, delivery followed by surgical repair can achieve maternal and fetal survival adequately; before 28 GWs, maternal survival should be prioritized given the high risk of fetal death. For TBADs in pregnancy, nonsurgical management is preferred.

Type I Aortic Dissection on Pregnant Woman

Acute aortic dissection is rare in young women. Half of the dissections in women younger than 40 years old occur during pregnancy, typically in third trimester and in postpartum period. The reasons for increased rate of aortic dissection in pregnancy are known to be increased sex hormones, degeneration of elastic tissue in aorta, and pressure of the uterus on aorta and distal iliac arteries causing pathological alterations in the artery walls by increasing the resistance to distal flow. Aortic dissection is a condition that can be presented with different symptoms and can be fatal for both mother and the fetus if the diagnosis is missed or late. Despite the diagnosis tools like computerized tomography, echocardiography, magnetic resonance imaging or aortography, suspicion of aortic dissection is still the basis of diagnosis. Treatment objective is to provide the security of the mother and the fetus. In this case presentation, approach to pregnancy-induced aortic dissection was discussed by presenting a case in which a 41-year-old woman in 34th week presented with swelling in the legs, pain in the epigastric region and blurry vision complaints, developed type A aortic dissection and died during the follow-up. J Med Cases. 2016;7(3):105-108 doi: http://dx.doi.org/10.14740/jmc2437w

Type I Aortic Dissection on Pregnant Woman

Acute aortic dissection is rare in young women. Half of the dissections in women younger than 40 years old occur during pregnancy, typically in third trimester and in postpartum period. The reasons for increased rate of aortic dissection in pregnancy are known to be increased sex hormones, degeneration of elastic tissue in aorta, and pressure of the uterus on aorta and distal iliac arteries causing pathological alterations in the artery walls by increasing the resistance to distal flow. Aortic dissection is a condition that can be presented with different symptoms and can be fatal for both mother and the fetus if the diagnosis is missed or late. Despite the diagnosis tools like computerized tomography, echocardiography, magnetic resonance imaging or aortography, suspicion of aortic dissection is still the basis of diagnosis. Treatment objective is to provide the security of the mother and the fetus. In this case presentation, approach to pregnancy-induced aortic dissection was discussed by presenting a case in which a 41-year-old woman in 34th week presented with swelling in the legs, pain in the epigastric region and blurry vision complaints, developed type A aortic dissection and died during the follow-up. J Med Cases. 2016;7(3):105-108 doi: http://dx.doi.org/10.14740/jmc2437w

Aortic dissection in pregnancy in England: an incidence study using linked national databases

ObjectivesTo conduct the first population-level incidence study of aortic dissection in pregnancy using linked hospital-based data in England.SettingHospital-based data (Hospital Episode Statistics (HES) linked with mortality data from the Office...

Aortic dilatation and dissection in Turner syndrome: what we know, what we are unclear about and what we should do in clinical practice?

Aortic dilatation and aortic dissection are increasingly recognised in patients with Turner syndrome (TS). Risk factors for aortic dissection include aortic dilatation, bicuspid aortic valves, coarctation of aorta and pregnancy. The risk of death due to aortic dissection in pregnancy in TS is 2%, which is approximately 100 times higher than the general population, as maternal mortality is extremely low. Ongoing cardiovascular monitoring is recommended, although there remain several unanswered questions in relation to cardiovascular imaging especially the choice of modality for detection of vascular, valvular abnormalities and measurements of aortic dimensions. Due to the relative short stature of patients with TS, aortic dimensions need to be defined by aortic measurements adjusted for body surface area, known as aortic sized index (ASI). The relationship of ASI and other risk factors with aortic dissection is only beginning to be clarified. Clinical management and monitoring of such patients should be delivered by a group of clinicians familiar with the issues unique to TS patients in a multidisciplinary fashion. All clinicians including the non-specialists need to have a low threshold of suspecting aortic dissection in these adolescents and young adults. This up to date review, including a summary of all 122 published cases of TS patients with aortic dissection, aims to provide a summary of recent publications on characteristics of aortic dissection and aortic dilatation in TS to highlight gaps in knowledge and propose possible clinical monitoring pathway of cardiovascular health in children and adults with TS. Cardiovascular assessment and risk counselling is especially crucial during the period of transition of adolescents with TS, although life long monitoring by expert cognizant to the issues specific in TS is essential.

Marfan's syndrome and other aortopathies in pregnancy.

Aortopathies, or disease affecting the aorta, are associated with a significant mortality risk for the mother and foetus during pregnancy because of an increased rate of aortic dissection. The hereditary aortopathies; Marfan's syndrome, bicuspid aortic valve, Loeys-Dietz syndrome, Ehlers-Danlos (type IV) syndrome, Turner's syndrome and nonsyndromic familial thoracic aortic aneurysm and dissection are all associated with an increased risk of aortic dissection particularly during the third trimester and early postpartum period. Maternal outcome in pregnancy depends on the underlying disorder and the aortic dimensions prior to pregnancy. The foetus has up to 50% chance of inheriting the underlying genetic defect. Vasculitis, particularly Takayasu's arteritis may also be a problem in pregnancy and predispose to aortic dissection. Prepregnancy review, including careful assessment of the aorta and prophylactic aortic surgery for an aortic aneurysm may reduce the risk of aortic dissection in pregnancy for some of the aortopathies but for women with Marfan's syndrome, Loeys-Dietz syndrome and Ehlers-Danlos (vascular type IV) who have had surgery, the risk of death remains high. A subgroup of women with Marfan's syndrome or a bicuspid aortic valve and normal aortic dimensions prepregnancy should do well in a pregnancy. Multidisciplinary pregnancy care with agreement on pregnancy follow-up, delivery and postpartum care with a crisis plan for an aortic dissection can improve pregnancy outcome and ensure prompt management of an aortic dissection should it occur.

Spontaneous Acute Aortic Dissection in Pregnancy

Acute aortic dissection in pregnancy is a rare condition with a potentially catastrophic outcome. The low rate of incidence together with variable presenting features in such circumstances makes diagnosis elusive. A high degree of clinical suspicion and diagnostic acumen is especially required in pregnant women without any history of connective tissue disorder, congenital aortic valvular malformation, or chronic hypertension. In this report, we present a case of aortic dissection with multiple aortic intimal tears in a medicolegal autopsy of a young pregnant woman who developed circulatory collapse and died during labor.

Type A Aortic Dissection in Pregnancy

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Peripartum acute aortic dissection in a single centre: Experience in 9 patients

Objective: Spontaneous acute aortic dissection in pregnancy is a rare, life-threatening condition for both the mother and the fetus. Most commonly, it is associated with connective tissue disorders, cardiac valve variants, hypertension or trauma. In order to increase the awareness of this hazardous condition, we have summarized findings from 9 cases from our center.

A fatal case of dissecting aortic root aneurysm due to undiagnosed Marfan syndrome in the puerperium

Aortic dissection in pregnancy is a lethal cardiovascular complication in women with Marfan syndrome. The course of the treatment would be decided upon by the size of aortic root dilation. We report a case where the unawareness of the diagnosis of Marfan syndrome antenatally led to disastrous post partum consequences even after an uneventful childbirth. DOI: http://dx.doi.org/10.4038/sljog.v34i2.4829 Sri Lanka Journal of Obstetrics and Gynaecology 2012; 34 : 55-57