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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of pharyngeal rhabdomyoma is reported with the unusual presenting symptom of haemoptysis. After complete excision of the lesion, the(71-year-old) patient remained tumour-free until his death from heart disease 19 months later. Seven other published cases of pharyngeal rhabdomyoma are reviewed together with 27 other reported extracardiac adult rhabdomyomas which, with a single exception, occurred in the head and neck region.
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PMID:Pharyngeal rhabdomyoma: an unusual presentation. 71 29

During a 14-year period, 8 cases of primary heart tumours were observed at Sainte-Justine Hospital. Three of these patients had a favourable course without any surgical treatment. The age of these patients was respectively 3 days, 7 weeks and 6 1/2 years. In what concerns the first two patients, the clinical and paraclinical pictures were suggestive of heart disease from the onset. Catheterization and angiocardiography confirmed the presence of a tumour deforming both ventricular cavities. In these two cases, an attempt at surgical resection proved to be impossible in view of the extent of the lesion. Biopsy demonstrated a rhabdomyoma in one of the patients and a diffuse fibroma in the other. Six and four years later, the patients were still alive, and an improvement of both the electrocardiogram and of the cardio-pulmonary X-ray pictures were noted. A second cardiac catheterization showed an almost complete disappearance of the pathological pictures. In what concerns the third patient, he was a 6-year old child with a classical Bourneville's tuberous sclerosis with a localized tumour at the junction of the superior vena cava and the right atrium. Three years later a control catheterization showed the tumour to have remained unchanged. Two conclusions might be drawn from these cases: 1 a surgical operation, although always indicated, should never entail a desperate attempt at tumour removal; 2 the prognosis should never be considered as lethal from the start.
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PMID:[Heart tumors in children. Report of 3 cases with favorable spontaneous courses]. 81 98

The cardiac manifestation usually associated with tuberous sclerosis is rhabdomyoma of the heart. We report a rare association with cyanotic congenital heart disease in the form of double outlet right ventricle with infundibular pulmonary stenosis.
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PMID:Bourneville's tuberous sclerosis associated with double outlet right ventricle and infundibular pulmonary stenosis. 148 31

Primary cardiac tumors are quite rare in the newborn period. Prior to surgery, cardiac catheterization and angiocardiography have been performed to confirm the two-dimensional echocardiographic findings. In this report a 2-day-old baby with the clinical impression of severe cyanotic congenital heart disease diagnosed by two-dimensional echocardiography as multiple rhabdomyoma and confirmed by surgery is presented. It is emphasized that two-dimensional echocardiography is a very useful technique for the diagnosis of primary cardiac tumors. By means of this method severely symptomatic newborns may be taken directly to surgery without prior cardiac catheterization, thus minimizing complications.
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PMID:Cardiac rhabdomyoma in a newborn two-dimensional echocardiographic diagnosis. 192 Aug 25

Fetal echocardiography was studied by PUMC Hospital Xi' an and Harbin Medical University in collaboration. 320 fetuses were examined with M-mode. 2D and pulsed Doppler echocardiograph during one year. 7 cases of congenital heart disease were found including four ASD, one VSD, one complex deformity (single atrium, left ventricle undeveloped and pulmonary arteria) and one heart tumor (rhabdomyoma). Normal fetal echocardiographic and Doppler parameters were detected in 313 normal fetuses. The characteristic of abnormal fetal echo are: (1) The diameter of the arterial and ventricular chambers increased with increasing fetal age. (2) Fetal RV/LV = 1.1, RA greater than or equal to LA. (3) The thickness of the RV free wall is same as that of the LV free wall and septum. The fetal Doppler echo revealed: (1) The peak velocity of pulmonary artery in the early systole. (2) Peak A/Peak E greater than 1 in mitral and tricuspid valves Doppler spectra. The characteristic of fetal echocardiography consists of prominence of the right ventricle and high pulmonary pressure in the fetus.
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PMID:[Clinical study of fetal echocardiography]. 262 May 78

The case presented is the first report of oncocytic cardiomyopathy associated with congenital heart disease. The clinical presentation was typical of hypoplastic left heart syndrome, and the oncocytic cardiomyopathy was an unexpected finding at autopsy. The associated lesions of oncocytic metaplasia in thyroid and pituitary gland have been previously reported. Maternal serology and viral cultures from the infant were negative, indicating that the oncocytic lesions were not secondary to congenital viral infection. We support the theory that oncocytic cardiomyopathy is a hamartomatous lesion, and propose as a paradigm the association between cardiac rhabdomyoma and tuberous sclerosis. Careful follow-up of surgically "cured" oncocytic cardiomyopathy should uncover oncocytomas in other organs later in life.
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PMID:Oncocytic cardiomyopathy syndrome. 318 57

Fetal hydrops and possible congenital heart disease with atrioventricular block was diagnosed one day before birth in a male infant of 35 weeks' gestation. Echocardiography and angiocardiography soon after birth revealed a cardiac tumor. The child died three days after birth. Necropsy showed tuberous sclerosis involving the heart (type-I rhabdomyoma), kidneys, retina, and central nervous system.
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PMID:Cardiac rhabdomyoma presenting as fetal hydrops. 354 73

Six cases of primary cardiac tumour have been operated upon in a 7 year period from 1 June 1979 until 1 June 1986. All patients were under 6 months of age at the time of operation and two of the patients were in their first week of life. The principal indication for surgery was obstruction mainly at the right or left ventricular outflow tract level. More recently echo evaluation alone has been adequate to define the problem prior to surgery. Surgical excision has been performed without mortality or significant complications. In most cases resection has been complete, although in one case residual tumour has been left because of attachment of the tumour to vital structures. Follow-up of this case has not resulted in further surgery being required because of regrowth of the tumour. In one case, with co-existent congenital heart disease, the tumour was brought to notice after palliative systemic to pulmonary artery shunt had been performed. From the cardiac view point gratifying results have been obtained both in the short and long term following surgical resection. However, for patients with rhabdomyoma, later development of symptomatic tuberosclerosis should be anticipated in 50% of cases.
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PMID:Surgical excision of primary cardiac tumours in infancy. 368 46

The object of our investigation was to evaluate the potential of transvaginal echocardiography for a complete anatomic study of the fetal heart, and for detection of congenital heart diseases in low-risk pregnant patients between 13 and 15 weeks' gestation. High-frequency (6.5 MHz and 7.5 MHz) transvaginal probes were employed for fetal echocardiographic assessment. Six hundred and sixty patients considered at low risk for congenital heart disease underwent in utero ultrasonographic studies, neonatal records, postnatal imaging studies, and pathologic examination. The four-chamber view was obtained in 100% of the fetuses, while the extended fetal heart examination was completed in 98%. Six fetuses with cardiac defects were diagnosed during the study: three had major heart defects (aortic atresia, tetralogy of Fallot, and persistent truncus arteriosus), all detected at the early transvaginal scan; three cardiac anomalies escaped our early diagnosis; two fetuses had a minor ventricular septal defect diagnosed only postnatally; and one had multiple cardiac rhabdomyoma diagnosed in the third trimester. Our results demonstrate that between 13 and 15 weeks' gestation transvaginal echocardiographic assessment of the fetal heart in the low-risk population is feasible. Moreover, some severe anomalies may be detected at such an early gestational age.
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PMID:Transvaginal echocardiographic examination of the fetal heart between 13 and 15 weeks' gestation in a low-risk population. 782 40

The clinical and pathological findings of three patients with hamartomas of mature cardiac myocytes resembling localized hypertrophic cardiomyopathy are presented. Hypertrophic cardiomyopathy is manifest by a poorly demarcated area of cardiac hypertrophy, microscopically demonstrating myofiber disarray and intramural coronary thickening. Localized, nonencapsulated masses of hypertrophied cardiac myocytes in locations other than the left ventricle or ventricular septum have not been reported. The clinical and pathological data of three patients with localized hamartomas were retrospectively retrieved. The patients were 9, 22, and 28 years old, respectively; none had a known family history of heart disease or cardiomyopathy. Two patients had cardiac arrhythmias: one patient died suddenly, and one patient had the Wolff-Parkinson-White syndrome. The third patient was asymptomatic. Two patients treated surgically had single masses in the right atrium and right ventricle, respectively. The patient who died suddenly had multiple discrete masses throughout the atrial and ventricular myocardium, including the left ventricular free wall. None of the three patients had septal asymmetry suggestive of hypertrophic cardiomyopathy. Histologically, there were discrete but unencapsulated nodules of marked myocyte hypertrophy with disorganization, focal scarring, and thickened intramural arteries. There was no myocyte vacuolization suggestive of cardiac rhabdomyoma. Ultrastructurally, the myocytes showed abundant and disorganized myofilaments and normal intercellular junctions. Hamartoma of mature cardiac myocytes is a previously undescribed cardiac tumor that shares some features of hypertrophic cardiomyopathy and rhabdomyoma, but is currently best considered a separate entity.
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PMID:Hamartoma of mature cardiac myocytes. 974 5


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