1/34. Congenital heart disease in pregnancy.The clinical cases of three patients affected respectively by Eisenmerger's syndrome, marfan syndrome, coarctation of the aorta are described. All patients belonged to NYHA class I or II. During pregnancy contact with cardiologists, anaesthetists, neonatologists was maintained and this allowed accurate management. Both pregnancy and delivery evolved without any complication and with a positive outcome for mother and newborn.- - - - - - - - - - ranking = 1keywords = pregnancy (Clic here for more details about this article) |
2/34. Surgical treatment for acute type A aortic dissection in pregnancy: a case of aortic root replacement just after cesarean section.A 25-year-old woman with marfan syndrome in the 37th week of pregnancy was referred for acute chest pain and dyspnea. An emergency cesarean section was performed because of fetal distress. Intraoperative echocardiography at the end of the cesarean section showed dilatation of the aortic root and dissection of the ascending aorta. The patient underwent replacement of the aortic root and the ascending aorta on the following day because of uterine bleeding. The postoperative course was uneventful for the mother and her baby.- - - - - - - - - - ranking = 0.83333333333333keywords = pregnancy (Clic here for more details about this article) |
3/34. Acute aortic dissection during pregnancy.We review the cases of two women with acute aortic dissection during their last trimester of pregnancy who underwent aortic root replacement. One patient with type A dissection had to undergo surgical intervention immediately, and required extracorporal membrane oxygenation for four days. The other patient with a pre-diagnosed Marfan's disease had suffered a type B dissection and had to undergo emergency operation after developing a type A dissection nine days later.- - - - - - - - - - ranking = 0.83333333333333keywords = pregnancy (Clic here for more details about this article) |
4/34. Anaesthetic management of a caesarean section in a patient with Marfan's syndrome and aortic dissection.This report describes a case of a Stanford Type B aortic dissection (originating distal to the left subclavian artery and extending to the aortic bifurcation and proximal left iliac artery) in a 31-year-old primigravid woman who was at 39 weeks gestation and had Marfan's syndrome. The dissection was managed conservatively. Caesarean section was performed under epidural anaesthesia with aggressive control of hypertension. Postoperatively, there was no extension of the dissection and no aneurysm formation. She was discharged from hospital two weeks after delivery and remained asymptomatic at six months. There are no plans for surgical intervention.- - - - - - - - - - ranking = 0.086715733362118keywords = gestation (Clic here for more details about this article) |
5/34. The marfan syndrome and pregnancy: a retrospective study in a Dutch population.OBJECTIVE: To assess the course and outcome of pregnancies in women with the marfan syndrome with the aim of developing guidelines for counseling. STUDY DESIGN: A retrospective study based on data collected from members of the Dutch association of Marfan patients. Pregnancies and neonatal outcomes of affected mothers were compared with those of non-affected mothers who delivered a Marfan infant. RESULTS: In a group of 44 affected women 78 pregnancies beyond 24 weeks of gestation were evaluated, compared with 51 in non-affected women. Obstetric course and neonatal outcome of pregnancy were similar in both groups. Aortic dissection was observed in five affected women, three of which were known to have an aorta diameter of 40 mm or more; two neurovascular events were recorded; all mothers survived. CONCLUSIONS: A preconceptional aortic diameter of 40 mm or more, progression of dilatation and decreased cardiac function are risk factors in pregnancy for women with the marfan syndrome. A multidisciplinary approach is recommended for the care of these patients and their infants.- - - - - - - - - - ranking = 1.0867157333621keywords = pregnancy, gestation (Clic here for more details about this article) |
6/34. Strategies for prenatal and preimplantation genetic diagnosis in marfan syndrome (MFS).marfan syndrome (MFS) is an autosomal dominant disorder with a prevalence of 2-3 per 10 000 individuals. Symptoms range from skeletal overgrowth, cutaneous striae to ectopia lentis and aortic dilatation leading to dissection. prenatal diagnosis was until recently mainly performed in familial cases by linkage analysis. However, mutation detection has become available with thorough screening methods. The phenotypic variability observed in MFS makes reproductive options difficult, as molecular diagnosis cannot predict clinical severity of the disease. Data are presented on 15 prenatal and/or preimplantation genetic diagnoses (PGD) in nine families, originating from belgium, the netherlands, spain and france. In four families data from linkage analysis were used, whereas in five other families the causative FBN1 mutation was characterised. Four PGD cycles in two couples led to one ongoing pregnancy. In addition, two amniocenteses and nine chorionic villus (CV) samplings were performed. In five pregnancies an affected fetus was diagnosed. In one of them, the couple chose to continue the pregnancy and an affected child was born, whereas the other four couples decided to terminate the pregnancy. It is expected that the greater availability of mutation testing of the FBN1 gene will increase requests for prenatal diagnosis. PGD appears to be an acceptable alternative for couples facing ethical reproductive dilemmas.- - - - - - - - - - ranking = 0.5keywords = pregnancy (Clic here for more details about this article) |
7/34. Cardiovascular manifestations in marfan syndrome.INTRODUCTION: Cardiovascular complications are the major cause of morbidity and mortality in marfan syndrome (MS), a common connective tissue disorder. Currently it is considered that the prognosis and morphologic characteristics in infantile marfan syndrome may be quite different from those reported in older patients. The objective of this study was to analyze the cardiovascular manifestations and evolution of the patients with marfan syndrome followed at our pediatric cardiac unit. methods: The authors reviewed the clinical files of all the patients that fulfilled the diagnostic criteria for MS according to De Paepe et al. (1996). We analyzed the following parameters: gender, age at referral, race, family history, clinical examination, diagnostic exams, therapy and evolution. The patients were divided into two groups according to the age at diagnosis: infants (group 1) and older patients (group 2). RESULTS: Group 1 included 3 infants, two boys and one girl, sporadic cases, presenting congestive heart failure. The major cardiac diagnoses were aortic dilatation (1/3) and mitral valve prolapse with severe mitral regurgitation (2/3). Congenital heart disease was associated in two cases (patent ductus arteriosus and atrial septal defect). Two needed cardiac surgery at an early age and one was recently proposed for surgery. There were no deaths. Group 2 included 20 patients, 14 boys and 6 girls, first seen at a mean age of 8 years. Ten had a positive family history and none presented cardiac symptoms. The major cardiac manifestations were mitral valve prolapse (18/20) and aortic dilatation (17/20). There was no significant progression of the cardiac lesions, except for one case, during the 12 years of follow-up. CONCLUSION: Infantile MS presented high morbidity; mitral regurgitation was severe in two cases. All patients presented heart failure, two needing early operations. In classic MS evolution was favorable, and the commonest cardiac lesions were mitral valve prolapse and aortic dilatation. We emphasize the need for beta-blockers to prevent progression of aortic dilatation. The decision for surgery rests upon the severity of valve regurgitation and the rate of progression of aortic dilatation. It is important to inform patients and family about physical exercise, prevention of endocarditis, risks associated with pregnancy and genetic counseling.- - - - - - - - - - ranking = 0.16666666666667keywords = pregnancy (Clic here for more details about this article) |
8/34. Functional pulmonary atresia in neonatal Marfan's syndrome: successful treatment with inhaled nitric oxide.Functional pulmonary atresia is characterized by a structurally normal pulmonary valve not opening during right ventricular ejection. We report this rare condition in a premature newborn of a twin pregnancy, in which fetal echocardiography findings were consistent with critical pulmonary stenosis. After birth, features of neonatal Marfan's syndrome were noted. echocardiography showed a morphologically normal but immobile pulmonary valve with continuous regurgitation. Right ventricular pressure was subsystemic. In this case, initial treatment with nitric oxide, followed by pharmacological duct closure, was successful. Differentiating between anatomic and functional pulmonary valve atresia may be difficult. The echocardiographic criteria are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = pregnancy (Clic here for more details about this article) |
9/34. Prenatal ultrasound findings in a fetus with congenital contractural arachnodactyly.Congenital contractural arachnodactyly (CCA) or Beals-Hecht syndrome is an autosomal dominant disorder caused by mutations in the fibrillin-2 (FBN2) gene. The principal features of CCA are a marfanoid habitus, multiple congenital contractures, camptodactyly, arachnodactyly, kyphoscoliosis, muscular hypoplasia, and external ear malformations. Our case is the first that shows typical sonographic signs in a fetus at 25 weeks' gestation with molecular genetically verified CCA in a large family with many members affected over four generations. This demonstrates that CCA can be detected prenatally by non-invasive ultrasonography. The importance of confirmation of CCA by means of dna sequence analysis of the FBN2 gene is stressed.- - - - - - - - - - ranking = 0.086715733362118keywords = gestation (Clic here for more details about this article) |
10/34. The marfan syndrome and pregnancy.The patient described had the marfan syndrome complicated by a 22-week pregnancy. Because pregnancy potentiates the cardiovascular complications of aortic aneurysm or dissection and there is a 50% risk of having a child afflicted with the disorder, this pregnancy was electively terminated by hysterotomy along with a bilateral tubal ligation. The risks of pregnancy to a woman with the marfan syndrome are reviewed. The rationale for the method of pregnancy termination chosen is discussed.- - - - - - - - - - ranking = 1.5keywords = pregnancy (Clic here for more details about this article) |
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