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 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a series of 9 children with neonatal Marfan syndrome. The diagnosis was made on a striking facial dysmorphia associated with arachnodactyly type skeletal anomalies. They all had cardiovascular anomalies: aortic dilatation 9 times, and mitral and/or tricuspid valve prolapse 8 times. Three children underwent ascending aorta replacement with the Yacoub technique. There was one operative death in a young infant and the two survivors required further surgery for gross mitral insufficiency and an aneurysm of the horizontal aorta. Three children died before the age of 1 year from cardiac insufficiency linked to massive leaks of all the valves. The three youngest children in the series did not undergo any procedure and were followed up for a slowly growing dilatation of the initial aorta. The prognosis of neonatal Marfan syndrome is weighted with a heavy mortality. The timing of surgical intervention in case of excessive dilatation of the aortic root is difficult to determine. It depends not only on the aortic diameter but also on the aortic valve function and the existence of other mitral or tricuspid lesions. Surgical treatment for lesions of the initial aorta and the atrioventricular valves improves the short-term prognosis but the risk of re-intervention is higher.
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PMID:[Marfan syndrome in the newborn and infants less than 4 months: a series of 9 patients]. 1208 46

The present study determines the effect of surgical treatment of cardiovascular manifestations of Marfan syndrome in 72 patients by 114 operations, during 34-year period. This therapy resulted in aortic root repair, aortic arch replacement, or both in 78, mitral valve repair in 9, descending thoracic aortic replacement in 14, thoracoabdominal aortic replacement in 10, and abdominal aortic replacement in 6, including total aortic replacement in 4 and nearly total aortic replacement in 4 patients. Fusiform aneurysms were present in the the ascending aorta in 37, the aortic arch in 2, the thoracoabdominal aorta in 2, and the abdominal aorta in 6 patients. Aortic dissection occurred in 40 (55.6%), including type A aortic dissection in 29 patients. Aortic root repair included separate valve-graft in 8, Bentall composite valve-graft in 25, composite valve-graft with button technique in 26, composite valve-graft with interposition graft technique in 10, and valve sparing procedure in 5 patients. The overall early (30-day) mortality was 7.9%. The early survival was 75% in separate valve-graft procedure and 99.2% in composite valve-graft procedure. Late coronary dehiscence did not occur in the patients with Bentall technique in which the reattachments of coronary ostia were performed in 2 layers, but occurred in 50% of patients with the coronary anastomoses in 1 layer. Aortic valve regurgitation relapsed in 2 of the 5 patients with valve sparing procedure. Event free rate for the patients with composite valve-graft using button technique was 81.1% at 10 years. There were 14 late deaths; dissection or rupture of the residual aorta, composite graft endocarditis and cardiac failure were the principle causes of late deaths. In conclusion, Marfan patients with cardiovascular diseases can undergo surgical treatment with a low operative risk and low morbidity. Although late endocarditis remains a serious problem, we believe that Marfan syndrome is a contraindication for valve sparing procedure. Because of the potential for late dissection or aneurysm in other areas of the aorta, patients with Marfan syndrome should have serial computed tomographic scans indefinitely.
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PMID:[Surgical treatment of cardiovascular manifestations of Marfan syndrome]. 1217 52

Neonatal Marfan syndrome caused by an exon 25 mutation of the Fibrillin-1 gene: We describe a male infant with severe arachnodactyly, hypermobility of the fingers, flexion contractures of elbows, wrists, hips, and knees, microretrognathia, crumpled ears, rockerbottom feet, loose redundant skin, and lens dislocations. Cardiac valve insufficiency and aortic dilatation resulted in cardiac failure, decompensated with digitalisation and death occurred at the age of 4 months. This case represents the severe end of the clinical spectrum of Marfan syndrome, namely neonatal Marfan syndrome. Molecular diagnostic analyses confirmed a de novo exon 25 mutation in the FBN1 gene.
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PMID:Neonatal Marfan syndrome caused by an exon 25 mutation of the fibrillin-1 gene. 1528 23

This paper analyzes the results of the use of enoxaparin for anticoagulant therapy in reconstructions on the ascending aorta (AA) as compared to unfractionated heparin applied previously in the control group. Between 1986 and 2003 a total of 30 patients with AA aneurysms were operated on at the clinic. Insufficiency of the aortic valve with degree II-III regurgitation was present in 25 (83.3%) cases. Chronic dissection of the AA was identified in 10 (33.3%) cases. The patient's age varied from 24 to 52 years (mean 39 years). The etiological factors of AA aneurysm were: Marfan's syndrome (46.7% of cases), Erdheim's syndrome (26.7%), atherosclerosis (10.0% of cases); previous chest traumas were recognized in 16.6% of patients. All the patients were operated on under extra-corporeal circulation and moderate hypothermia. The patients were distributed into two groups. In the control group, eighteen patients were operated on. Anticoagulant therapy was carried out using unfractionated heparin i. v. in a daily dose 10-15 thousand units. Heparin injection was initiated on the first postoperative day and continued for 6.5 days on the average, with a progressive change over to the use of indirect anticoagulants. In the basic group, twelve operated patients were administered the anticoagulant enoxaparin s.c. in a daily dose 0.7-1.0 mg/kg bw. Enoxaparin therapy was initiated from the first postoperative day and continued for 8.9 days on the average, with a progressive change over to indirect anticoagulants. The postoperative lethality in the control group accounted for 22.2% (4 patients). In two cases, it was induced by heart failure and in two cases, by hemorrhagic complications. In the basic group, the beneficial results were achieved in 91.7%; no hemorrhagic complications were recorded. The data obtained allow the conclusion that the use of enoxaparin significantly facilitates the postoperative management of patients with AA aneurysms, providing for a controllable and safe anticoagulant effect.
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PMID:[The use of low-molecular heparins in reconstructive surgery of ascending aortic aneurysms]. 1562 90

A 32-year-old woman with Marfan syndrome was diagnosed as annuloaortic ectasia and acute aortic dissection at 20 weeks of gestation. At 24 weeks of gestation, she got heart failure. Bentall's procedure was performed under cardiopulmonary bypass. Anesthesia was given using sevoflurane, midazolam and fentanyl. Normothermic high-flow high-pressure non-pulsatile perfusion was performed during cardiopulmonary bypass. We used dopamine, dobutamine, milrinone and nitroglycerin for weaning of cardiopulmonary bypass. Fetal heart rate was monitored, and was stable at 120 bpm throughout the operation. At 33 weeks of pregnancy a healthy baby was delivered by cesarian section.
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PMID:[Open heart operation for a pregnant patient with Marfan syndrome]. 1591 53

Aneurysms of the sinus of Valsalva (ASVs) are rare. They can be congenital or acquired through infection, trauma, or degenerative diseases. They frequently co-occur with ventricular septal defects, aortic valve dysfunction, or other cardiac abnormalities. Although unruptured ASVs are usually asymptomatic, ruptured ASVs often cause symptoms similar to those of heart failure and produce a continuous, mechanical-sounding murmur. Transsternal or transesophageal echocardiography is usually effective in detecting ASVs. Because symptomatic ASVs pose significant risks for the patient, and because the repair of asymptomatic ASVs generally produces excellent outcomes, surgery is indicated in most cases. The primary goals of surgical repair are to close the ASV securely, remove or obliterate the aneurysmal sac, and repair any associated defects. Operative mortality is generally low except in patients with concomitant bacterial endocarditis or other infections. Late events are uncommon and tend to be related to aortic valve prothesis or Marfan syndrome.
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PMID:Aneurysm of the sinus of valsalva. 1663 63

The coincidence of Marfan syndrome and pregnancy means a high risk for mother and child, as it is associated with cardiovascular and obstetric complications. We report our experience of four pregnancies with the Marfan syndrome. The course of pregnancy, the peripartum management and both the maternal and neonatal outcomes of four pregnant women with the Marfan syndrome, who were treated in our department between 1995 and 2005, were retrospectively analysed. The pregnancies of two women were complicated by premature rupture of membranes (36 (th) gestational week) and premature uterine contractions with cervical incompetence (30 (th) gestational week), respectively. One patient developed class 3 (NYHA) heart failure in the 3 (rd) trimenon. Two out of four women had mild cardiovascular disease and could deliver vaginally. In the other two cases a primary Caesarean section was performed at the 36 (th) week of gestation because of severe cardiovascular morbidity. No patient had a progressive aortic dilatation, dissection or rupture. The neonatal outcome was uneventful in all cases. Three newborns underwent a genetic evaluation for the Marfan syndrome, in two of them mutations in the fibrillin 1 gene were detected. Women with the Marfan syndrome should be counselled pre-conception and observed by an interdisciplinary team during pregnancy. If the aortic root diameter is < 40 mm, without progression in pregnancy, and in the absence of severe valve insufficiency, then pregnancy is in most cases well tolerated and vaginal delivery can be performed.
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PMID:[Marfan syndrome in pregnancy: presentation of four cases and discussion]. 1732 91

Cardiovascular disease is the leading cause of death in developed countries (40% of all causes of mortality). Heart disease is common in the working age population and thus it contributes to a decline in employees' fitness for work. In Israel about 80% of patients recuperating from myocardial infarction (MI) return to work. However, long term employment may be as low as 50% and its patterns are associated predominantly with patient age and job characteristics, as compared to measures of illness severity or the method of coronary revascularization. The need for clinical guidelines in the management of return to work after myocardial infarction has recently led to the initiation of a joint committee of the Israeli National Heart and Occupational Societies. These clinical guidelines have been published and are summarized in this issue. For most common cardiac disease, including heart failure, valve disease and angina, patients can exert themselves up to onset of symptoms. Therefore, patients with functional capacity I and II, as estimated by New York Heart Association (NYHA) criteria, can return to their previous work. Timing of return to work for patients with asymptomatic uncomplicated cardiac disease: Post MI within 4 weeks, CABG within 4-8 weeks and percutaneous interventions within 1 week. For patients with a strenuous job or in NYHA functional capacity III or IV, a few weeks of delay and exercise or other functional testing may be needed. There are a few exceptions including patients with strenuous work or specific cardiac diseases as hypertrophic cardiomyopathy, severe aortic stenosis and Marfan's syndrome. In such cases, cardiological and occupational specialist advice should be sought.
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PMID:[Return to work with heart disease]. 1735 80

Angiotensin-converting enzyme inhibitors (ACE-I) and angiotensin receptor blockers (ARB) posses multiple beneficial effects such as cardioprotection, cerebroprotection, nephroprotection which provide opportunity to select the most suitable drug for the target vascular bed (e.g. coronary, or cerebral circulation). In some clinical settings, combined therapy ACE-I with ARB (double blockage of the renin-angiotensin-aldosteron system) may appear the most effective. These drugs (especially ARB) may successfully prevent atrial fibrillation and play a protective role in metabolic syndrome. Recently, it has been demonstrated that losartan is able to inhibit vasodilatation of the aorta in Marfan syndrome, which might prevent sudden death due to aorta rupture. An increasing role of ARB is most beneficial in hipotensive therapy (inhibition/regression of hypertension-related organ damage). With particular interest, results of the ONTARGET study are being awaited. This study is focused on the effect of double blockage (ramipril and telmisartan) on reduction of the occurrence of myocardial infarction, stroke, and heart failure.
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PMID:[New therapeutic targets for ACE inhibitors and angiotensin receptor blockers]. 1772 75

Patients with Marfan syndrome (n=44) and ascending aorta aneurism combined with aortic insufficiency were followed up for 1 month - 16 years after graft repair of the ascending aorta aneurysm and replacement of aortic valve. Patients were divided into two groups: with dissecting aneurism (n=25) and chronic nondissecting aneurism (n=19). In remote postoperative period 11 patients had 13 complications (2 patients had 2 complications each - graft dysfunction and arterial thromboembolism). Repetitive surgery was carried out in 5 patients after 67.2 +/- 19.4 months because of expansion of dissection to the abdominal aorta, dysfunction of mitral valve prosthesis. In remote period of follow up 15 patients (34.1%) died. Causes of death were graft dysfunction, extension of aortic dissection, myocardial failure. Total survival was 80, 54 and 46% for 1 month, 10 and 15 years, respectively.
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PMID:[Survival and complications in remote period of follow-up in patients with Marfan syndrome after correction of aneurism of the ascending aorta and aortic insufficiency]. 1826 Sep 13


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