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[Major cardiomegalies of the newborn].

Massive enlargement of the heart measured by cardio-thoracic ratio (C.T.R. greater than or equal to 0,75) was found in 17 neonates in the first ten days of their life. The diseases causing this rare anomaly (5% of all newborns with cardiac malformations) were: Ebstein's disease (6 cases), cerebral arterio-venous aneurism (6 cases), primary endocardial fibro-elastosis (2 cases), septic pericarditis, transposition of great arteries and pulmonary atresia with intact ventricular septum (1 case of each). A diagnosis scheme is proposed in order to recognize early tractable diseases and to avoid inadequate surgical indications.

Cardiomegaly↗

[Successful treatment of atrial fibrillation by resection of a congenital aneurysm of the left heart atrium].

Atrial fibrillation occurred in a 27-year-old patient with a history of globular cardiac enlargement since childhood. Because of the probable causal relationship between the preexisting heart disease-which was supposed to be an enlargement of the left atrium-and the rhythm disturbance, we recommended a surgical intervention. Cardiac surgery revealed a congenital aneurysm of the left atrial appendage which could be resected without any complication. Postoperatively, atrial fibrillation had returned to regular sinus rhythm. The bad prognosis with a high risk of systemic embolism is the reason why early cardiac surgery should be performed after diagnosis of this rare anomaly (20 reported cases) of the left atrium.

Adult↗

[Fibroelastoma of the tricuspid valve presenting with syncope].

Cardiac fibroelastomas are rare benign tumours. Initially observed as a chance finding at autopsy or during cardiac surgery, the diagnosis has become more common since the introduction of echocardiography. These tumours are usually asymptomatic. When the left heart valves are involved, embolic complications may be threatening. When the right heart valves are affected they are usually asymptomatic. The authors report the case of a fibroelastoma of the tricuspid valve responsible for several syncopal attacks. The tumour was excised with preservation of the native valve. There has been no recurrence of syncope since surgery. The authors believe this to be the second reported case of fibroelastoma of the tricuspid valve presenting with syncope.

Aged↗

[A rare cause of myocardial infarction: papillary fibroelastoma of the aortic valve].

Papillary fibroelastoma is a rare, benign endocardial tumour usually located on the cardiac valves. Before echocardiography, these tumours were chance findings either at surgery or at autopsy. With the advent of echocardiography, the diagnosis has become commoner and they are often the cause of systemic embolism justifying surgical ablation. In this case, an aortic valve papillary fibroelastoma presented with myocardial infarction in a 78 year old woman with normal coronary angiography. The diagnosis was strongly suspected at echocardiography and confirmed by histological analysis of the surgically excised tumour.

Aged↗

Incidental disclosure of asymptomatic coronary embolic occlusion related to mitral valve papillary fibroelastoma: an unusual finding and a review of the literature.

Cardiac papillary fibroelastoma (CPF) located on mitral and aortic valves are known to produce systemic embolism mainly represented by strokes, whereas myocardial infarction and sudden death usually result from specific locations around LVOT. Coronary artery embolic occlusion originating from a mitral location has not yet been reported. The case is reported of a 42-year-old man referred for surgical treatment of a mitral valve papillary fibroelastoma disclosed after transitory and completely regressive left hemicorporeal deficiency and previous myocardial infarct. Due to the left chamber location, surgery was scheduled and complete removal of the mass achieved. These findings emphasize the potential life-threatening complications of CPF and, independent of risk factors, the need to perform systematic coronary angiography before surgical excision is considered.

Adult↗

[Doppler-echocardiographic examination of patients with pulmonary tuberculosis].

Echocardiography of 110 newly diagnosed patients with pulmonary tuberculosis was undertaken to find anatomic changes and certain blood flow indices in the right heart. These changes were represented by an enlarged chamber of the left ventricle in over half of the patients and that of the left atrium in 1/3 of them (including myocardial hypertrophy also detected most commonly in the ventricular septum in 1/3 of the cases); fibroplastic changes in the anterior wall and ventricular septum; and induration of the cusps and semilunums of the tricuspid and pulmonary valves with subvalvular structures in 1/5 of the patients. In addition to hypertrophy of the ventricular septum, an enlarged chamber of the right ventricle and the left atrium was most frequently revealed in patients with primary tuberculous process. Fibroplastic changes in the myocardium plus calcinosis were traced in the majority of the patients with disseminated tuberculosis. Higher blood pressure in the outlet chamber of the right ventricle was mostly observed in patients with intrathoracic lymph nodes and disseminated tuberculosis, elevated pressure largely coinciding with right ventricle and pulmonary artery dilatation.

Adolescent↗

[A rare cause of obstruction of mitral valve prosthesis: periannular fibrosis. Diagnostic by transesophageal echocardiography].

The authors report a case of dysfunction of a double hemi-disc mitral valve prosthesis due to peri-annular fibrous overgrowth on the atrial side of the prosthesis. Conventional transthoracic Doppler echocardiography showed signs of thrombosis of the prosthesis. Transoesophageal echocardiography allowed a precise diagnosis of the lesions, resulting in immediate surgical referral.

Aortic Valve↗

Familial occurrence in cardiovascular diseases. Familial cardiovascular diseases (1).

Current knowledge and assumptions about inherited cardiovascular diseases are reported in this review. They are examined from two different points of view. In the first section (familial cardiovascular diseases) discussion will center on the main cardiovascular diseases that have a definite clinical and pathophysiological feature in which familial occurrence has been extensively demonstrated. The genetic aspects of the primary cardiomyopathies, mitral valve prolapse, arrhythmias and conduction disturbances, long QT syndromes and abnormalities of ventricular repolarization, cardiovascular malformations, coronary artery disease, essential hypertension and rheumatic fever will be examined. In the second section (cardiovascular involvement in genetic disorders) discussion will be confined to the inherited multisystem disorders involving the cardiovascular system that most frequently occur in clinical practice. Currently known cardiovascular findings in relationship to chromosomal aberrations, connective tissue disorders, metabolic and enzymatic disorders, neuromuscular disorders and other rarer syndromes will be reported.

Adult↗