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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Surgery has changed the prognosis of the Marfan's syndrome. Cardiovascular manifestations (aneurysmal dilatation of the aortic root and its risk of dissection, aortic insufficiency, mitral insufficiency due to valvular anomalies) represent the vital risk of the disease and reduce the life expectancy in one-third of these patients during the first 32 years. Twenty seven patients (22 to 53 years of age, mean 38) surgically treated have been followed up to 14 years (mean 6.5 years). 12 had aortic valve and ascending aorta replacement with reimplantation of coronary arteries, 9 had mitral valve replacement and 6 had simultaneous correction of aortic and mitral lesions. All patients survived the operation and late mortality was 18.5% to 14 years, with a long term survival of 90% at 5 and 70% at 10 years. Echocardiographic studies permit nowadays to measure the aortic root diameter, to appreciate the left ventricular function, to reveal a mitral prolapse and to precise valvular insufficiency, in young patients with Marfan's syndrome and during family investigation. Indication of surgical repair can be stated at the right time to prevent dissection, aortic rupture or cardiac failure with excellent and durable long term results.
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PMID:[Cardiovascular complications of Marfan's syndrome: long-term results of surgical treatment]. 263 61

A patient with typical Marfan's syndrome was monitored by echocardiography, which detected (in 1978) a prolapse of the mitral valve complicated by severe cardiac insufficiency, necessitating valve replacement. Seven years later an aneurysm of the ascending aorta (60 mm) was detected which caused prolapse of the aortic valve with valvular insufficiency, combined with prolapse of the tricuspid valve and a calcified foramen ovale the permeability of which was verified by peripheral injection of contrast medium. Echocardiography, notably two-dimensional, is an excellent examination for regular and atraumatic monitoring of Marfan's syndrome and detection of cardiovascular complications.
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PMID:[Echocardiographic monitoring of Marfan's syndrome. Triple valvular prolapse and calcified permeable foramen ovale]. 374 Jul 76

From 1965 to 1979, 59 patients with the Marfan syndrome and cardiovascular complications underwent aortic root and aortic valve surgery. The hospital mortality was 12% (6.5% over the past 5 years). At follow-up an average of 5.1 years later, 2 patients had postoperative heart block, and late complications required reoperation in 4. There was 8 late deaths (overall mortality 25%). Survival at 6 years was 77% and improvement was noted in the functional class of 32 (74%) of the 43 survivors. 3 patients with abnormal preoperative ventricular function have continued in cardiac failure; 3 have moderate mitral regurgitation and 2 have aortic regurgitation. Aneurysms of the abdominal aorta and pulmonary artery have developed in 2 patients. Initially patients underwent surgery when their condition deteriorated despite medical treatment. Echocardiographic assessment of the aortic root has improved diagnosis and management; a large, progressively dilating aorta and evidence of ventricular dilatation were indications for surgery in 11 symptom-free patients. Although patients with Marfan syndrome are prone to other cardiovascular manifestation which affect survival, elective prophylactic surgery to the ascending aorta can be done with a low mortality and expectation of improved lifespan.
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PMID:Management of cardiovascular complications in Marfan syndrome. 610 79

Marfan's syndrome in its complete and incomplete forms takes very often a course with cardiac complications, predetermining the fate of such patients. The incomplete forms often present difficulties for the timely etiological elucidation of the cardiac changes leading to a retardation in the prescribing a rational regime to the patients and timeliness of the operation treatment. The authors' team own observations are reported on 10 patients with Marfan's syndrome, one with complete form and nine--incomplete forms, all of them with cardiac complications; two with mitral insufficiency, three with aortic insufficiency, four with aortic and mitral insufficiency and one with aortic, mitral and tricuspidal insufficiency; seven of the patients had aneurysm at the initial part of the aorta, manifested to various degrees and one--dysplasia of aorta. Rhythm disturbances were found in one of the patients. One of the patients underwent operation--prosthesis of mitral and aortic valve--with good results. Three of them died of severe total cardiac insufficiency.
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PMID:[Cardiovascular changes in the Marfan syndrome]. 711 87

Left ventricular assist device (LVAD) was utilized for the treatment of postcardiotomy heart failure in two patients with Marfan's syndrome. Patient 1 (a 22-year-old) with annuloaortic ectasia (AAE) and DeBakey type II dissection had been supported by LVAD for 87h after composite graft replacement of the ascending aorta and aortic valve. Patient 2 (a 52-year-old) with AAE and DeBakey type I dissection had been supported by LVAD for 91 h after aortic valve replacement. During the assist, both patients complicated bleeding from the fragile left atria near the sites of cannulation. Patient 1 died of multiple organ failure on the 62nd postoperative day, but patient 2 returned to work after surgery.
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PMID:Mechanical left ventricular support in patients with Marfan's syndrome: a report of two cases. 767 48

Pregnancy and delivery in women with congenital heart disease remain inadvisable for patients at high risk due to the following congenital diseases: (1.) Severe left ventricular outflow tract obstruction (increase of pressure gradient); (2.) Eisenmenger syndrome (increase of right to left shunt with worsening of cyanosis and fetal growth retardation); (3.) Marfan syndrome with enlarged aortic root (risk of aortic dissection). In women suffering from cyanotic congenital heart disease the main risk is fetal complications. These are correlated to oxygen saturation and to the type of maternal disease, particularly if a palliative shunt operation has never been performed. Women with left to right shunt, right outflow tract obstruction or previous correction of the disease can go through pregnancy with low risk of mortality. The complications (mainly heart failure and arrhythmias) can be well managed with medical treatment, and the fetal outcome is similar to that in the general population.
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PMID:Pregnancy in patients with congenital heart disease. 787 10

We describe here the identification of defined mutations in both alleles of the fibrillin gene (FBN1) in a compound-heterozygote Marfan syndrome (MFS) child who had a very severe form of MFS resulting in death from cardiac failure at the age of 4 mo. The nonconsanguineous parents were both affected with MFS. The father's heterozygous point mutation has earlier been reported to result in W217G substitution, the mother was here shown to carry a heterozygous point mutation resulting in G2627R substitution, and the child had inherited both these mutations. The mutant FBN1 alleles were demonstrated to be transcribed with equal efficiency compared with the normal alleles, but metabolic labeling of fibroblast cultures from the child and both parents showed reduced biosynthesis and secretion of profibrillin. Also, the respective amounts of fibrillin in cell-culture media and extracellular-matrix extracts were markedly diminished, particularly in the cell cultures from father and child. In addition, immunofluorescence analysis of the cell cultures of all three family members revealed a drastically reduced amount of microfibrils, and virtually no visible fibrils could be seen in the case of the compound-heterozygote child. These findings demonstrate incomplete dominance of fibrillin mutations and underline the fatal consequences of the complete absence of normal fibrillin molecules in the microfibrils.
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PMID:A compound-heterozygous Marfan patient: two defective fibrillin alleles result in a lethal phenotype. 797 66

Left ventricular pulsus alternans developed immediately after Bentall's operation in a 37-year-old patient with Marfan's syndrome accompanied by severe left heart failure due to annuloaortic ectasia. Echocardiographic examination suggested that this symptom was caused by alternating contractility based on left ventricular myocardial failure. Left heart failure disappeared early after operation, but pulsus alternans persisted until 7 months after operation, when EDVI decreased from 225 ml/m2 to 113 ml/m2.
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PMID:[A case who developed mechanical pulsus alternans after Bentall's operation for annuloaortic ectasia]. 809 66

The authors present the clinical history of a patient with Marfan's syndrome (MS) and aortic insufficiency, who expired at 22 years of age, in order to illustrate the clinical evolution and review the management of this problem. It is known that patients with MS and heart failure due to dilatation of the aortic annulus have an early mortality of almost 100%. The authors conclude that echocardiographic follow up of these patients is vital in their medical and surgical management and that the use of beta blockers is the best medical therapy, and that prophylactic surgery with composite graft (aortic valve and ascending aorta) gives the best survival rate.
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PMID:[The cardiovascular manifestations of Marfan's syndrome. A case report and review of the management]. 814 46

A 34-year-old woman with Marfan's syndrome had severe heart failure due to annulo-aortic ectasia and aortic insufficiency, which was accompanied also by a dissecting aneurysm (DeBakey type IIIb) that was demonstrated by aortography. 4 days before the operation, sudden progression of the aneurysm to a DeBakey type II, and finally DeBakey type I dissecting aneurysm was seen. The first operation was an extended aortic resection with replacement from the aortic valve to the descending thoracic aorta (level of the 7th thoracic vertebra) using selective cerebral perfusion. The second operation was a replacement of the residual dissecting aorta from the level of the 8th thoracic vertebra to the celiac artery with partial extracorporeal circulation. The postoperative course of the patient was uneventful. Dissecting aneurysm with Marfan's syndrome should be operated as extensively as possible if necessary.
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PMID:[A case of staged operation for a dissecting aneurysm (DeBakey type IIIb+II) with Marfan's syndrome]. 817 4


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