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

Among the causes of death of 43 scoliotics were 5 directly due to complications of congenital heart disease. Over half (57.9%) of the remaining 38 died of cardiac or respiratory causes. The paralytic scolitoics tended to die of pneumonia or respiratory failure, while the nonparalytic scoliotics died of cardiac failure. Right ventricular hypertrophy was present in 65% of the 17 subjects examined postmortem. Electrocardiographic evidence of right ventricular hypertrophy correlated well with the postmortem findings. The vital capacity was less than 1.75 liters in 84% of the dead subjects. The case records of a further 719 living scoliotics were examined for evidence of congenital heart disease. This was found in: 34 (4.5%) of the whole group of 762, 6.9% of the congenital ; 3.4% of the idiopathic scoliotics; 22.7% of those with Marfan's syndrome.
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PMID:Causes of death, right ventricular hypertrophy, and congenital heart disease in scoliosis. 15 77

A 14-year-old boy with an incomplete form of the Marfan syndrome developed an acute episode of severe congestive heart failure following exertion. The preoperative studies indicated a mitral insufficiency, and a floppy, thickened mitral valve was removed and replaced with a Starr-Edwards prosthesis. Microscopically, the valve showed increase in collagen with central myxomatous transformation. Seven months postoperatively the patient is doing well and has no symptoms of heart failure.
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PMID:Mitral insufficiency in an incomplete form of Marfan syndrome corrected with valve replacement. 112 90

We report the cases of eight children with Marfan syndrome. Seven (87.5%) were diagnosed with cardiopathy, everyone with auscultatory findings. Electrocardiographic patterns were nonspecific. Enlargement of the aortic root was present in two of the cases as seen by thorax x-rays. Echocardiography detected the presence of mitral valve prolapse in 87.5% of the patients (7 cases) and aortic enlargement in 75% of the patients (6 cases). Echocardiography also detected the presence of aortic dysplasia, tricuspid valve prolapse and right and left ventricular hypertrophy. During the follow-up period, no case had cardiac failure. There was no mortality. The aortic enlargement was progressive and was not modified by propranolol treatment. Surgical treatment was not needed. Family history related to this condition was present in a very small percentage (37.5%). We comment on one infantile form of Marfan syndrome with its own phenotype different from that of classical Marfan syndrome.
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PMID:[Marfan syndrome in childhood: cardiovascular manifestations. Echocardiographic changes]. 141 16

This report is concerned with results of surgical treatment for Marfan's syndrome combined with annulo-aortic ectasia (AAE) and mitral regurgitation (MR). Of the 23 patients with Marfan's syndrome who received Bentall's procedure during 14 year period, seven (30%) of these patients had both AAE and MR. The MR grade of seven patients by cardiac Doppler or left ventriculographic studies were grade 1 in 2, 2 in 1, 3 in 1, and 4 in 3. Atrial fibrillation was present in 4 patients. New York Heart Association Functional Class on admission in these 7 patients were II in 1, III in 4, and IV in 2. The mitral valve was replaced with mechanical valve in 4 patients by left atrial approach whose MR grade were over 3. In the 4 patients the mitral annuli were extremely dilated, both valve leaflets were massively redundant, and all chordae were elongated and turned chordae and vegetation were detected due to infective endocarditis. Only Bentall's procedure was performed in 3 patients whose MR were minimal. There were no early death, but two late deaths. One of them died of cardiac failure 2.3 years after Bentall's procedure because grade 2 MR was increased. Another one died from ventricular arrhythmia 1.6 years after MVR and Bentall's procedure. The remaining 5 patients are doing well for 3 months to 11.5 years after operation. For Marfan's syndrome combined with AAE and MR, early operation is recommended before left ventricular impairment. Mitral valve repair was not performed, both to save time and because anticoagulant therapy was need for aortic valve replacement. Concomitant MVR was to be done for moderate to severe MR.
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PMID:[Surgical treatment of Marfan's syndrome with annulo aortic ectasia and mitral regurgitation]. 158 63

We report a case of Marfan's syndrome with acute heart failure caused by a ruptured mitral chorda that was successfully treated by one operation of combined composite valve graft replacement of aortic root and mitral valve replacement (MVR). A 23-year-old man was admitted to our hospital presenting severe dyspnea and chest pain. Echocardiography and cardiac catheterization studies demonstrated marked annulo-aortic ectasia, aortic regurgitation and significant mitral regurgitation due to a ruptured chorda. In operation, it was found that a chorda of the mitral posterior leaflet had been torn, with the leaflet completely prolapsed to the left atrium, and that the aortic root was dilated to 90 mm in diameter. The ascending aorta was extensively resected leaving those areas of aortic tissue involving the coronary ostia. Then the mobilized coronary arteries were reattached to the composite graft. MVR was performed with preservation of the whole anterior and posterior mitral valve apparatus except for that small part with the torn chorda. Histopathological findings of the aortic wall and mitral valve were compatible with those of Marfan's syndrome.
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PMID:[Marfan's syndrome with annulo-aortic ectasia and ruptured mitral chorda--a case report of combined composite valve graft replacement of the aortic root and mitral valve replacement]. 177 94

We describe a male neonate with severe arachnodactyly, hypermobility of the fingers, flexion contractures of elbows, wrists, hips, and knees, micrognathia, crumpled ears, rockerbottom feet, loose redundant skin, and ocular abnormalities. Severe cardiac valve insufficiency and aortic dilatation resulted in cardiac failure and death 20 hours after birth. This case represents the severe end of the clinical spectrum of Marfan syndrome. As similar patients have been reported, they may represent a separate mutation.
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PMID:Neonatal Marfan syndrome with congenital arachnodactyly, flexion contractures, and severe cardiac valve insufficiency. 185 34

A case of the elder PDA with aneurysm of the main pulmonary artery is reported. The patient was a 59-year-old female who had been suffering from severe heart failure. The PDA was closed directly from inside of the pulmonary artery using balloon catheter under usual cardiopulmonary bypass with limited circulatory arrest, and the aneurysm was repaired by aneurysmorrhaphy. The postoperative course was uneventful and she was discharged after 34 postoperative days. The pathological finding of the aneurysm of the main pulmonary artery is a cystic mucoid degeneration like a finding of Marfan syndrome. The etiology of the aneurysm of the main pulmonary artery seems to be extended Ao-PA shunt besides somewhat congenital change of the pulmonary artery.
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PMID:[Surgical treatment of PDA in the elderly patient accompanied with aneurysm of the main pulmonary artery]. 188 21

An infant girl with arachnodactyly, spontaneously resolving contractures, dolichostenomelia, iridodonesis, and mitral and tricuspid incompetence died in cardiac failure. We confirm that congenital contractural arachnodactyly may exhibit serious cardiovascular and ophthalmic complications like Marfan's syndrome. The presence of iridodonesis further obscures the differentiation between classical Marfan's syndrome and congenital contractural arachnodactyly.
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PMID:Contractural arachnodactyly with mitral regurgitation and iridodonesis. 227 Oct 65

Since the clinical, 2-dimensional and Doppler echocardiographic and pathologic findings in infantile Marfan syndrome have not been documented in detail, a study of 9 such infants was performed. The previously reported 64 cases were reviewed and the salient findings in 22 additional cases were discussed. The age at diagnosis in our 9 cases ranged from birth to 12 months (mean 2.7 months). Mitral valve prolapse was demonstrated in all, with mitral regurgitation in 8. Tricuspid valve prolapse was present in 8, with tricuspid regurgitation in 6. Marked aortic root dilatation was present in all, and was progressive. The aortic root assumed a "clover leaf" appearance in the parasternal short-axis view. Aortic regurgitation was documented initially in 1 patient, and developed during follow-up in 4 of 7 infants. Dilation of the pulmonary arterial root and pulmonary regurgitation were found in 3 of 7 infants. Severe heart failure associated with mitral or tricuspid regurgitation was present in 7 of the 9 patients. Four infants died during the first year of life. The salient pathologic features were myxomatous thickening and redundancy of the mitral and tricuspid leaflets, marked elongation of chordae tendineae and prominent dilatation of the aortic and pulmonary roots. Histologically, the collagen and elastic fibers were severely disrupted, disarrayed and fragmented with increased interstitial ground substance. These data document that infantile Marfan syndrome is characterized by clinical and morphologic features that are distinctly different from the classic syndrome seen in adolescents and adults. The aforementioned findings should facilitate early clinical and echocardiographic diagnosis of infantile Marfan syndrome.
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PMID:Two-dimensional and Doppler echocardiographic and pathologic characteristics of the infantile Marfan syndrome. 233 33

The authors report the case of a neonate who died early of refractory heart failure and presented with all the characteristics of Marfan's syndrome in its early form with articular retractions. There was clinical evidence of mitral, tricuspid and major aortic regurgitation. Two-dimensional and doppler echocardiography confirmed the prolapse of highly dystrophic valves and the quadrivalvar regurgitation. Typical lesions of the connective-elastic tissue were found at autopsy. The occurrence of malignant Marfan's syndrome in neonates seems to be exceptional; the syndrome may take the rare "en contractures" form. Death is not always ascribable to the cardiac pathology, this pathology being often less generalized than in our case which seems to be the first one where such diffuse and massive valval lesions have been observed. This particular form was comparable to the "congenital polyvalvular disease" described by Bahrati and Lev, except that the skeletal and visceral abnormalities of Marfan's syndrome are absent in that disease.
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PMID:[Malignant quadrivalvular dysplasia of Marfan syndrome in a neonate]. 250 Jan 5


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