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

The authors have followed up 26 children suffering from severe scoliosis associated with congenital heart disease up to the end of puberty. The curve was usually a very severe idiopathic scoliosis developing early and requring surgical treatment. Two types may be distinguished: I. Scoliosis without excessive surgical risk, in patients in whom the heart disease is not associated with cyanosis or where the heart condition has already been treated surgically and in which there are no clinical, radiological or electrical signs of cardiac failure. 2. Scoliosis with considerable surgical risk because of heart disease with cyanosis not treated surgically, or with signs of heart failure or pulmonary hypertension. In such cases, the surgical treatment of the scoliosis is likely to endanger life.
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PMID:[Scoliosis and congenital cardiopathies]. 13 60

We described a 26-year-old man whose brother has a mottled fundus. Many maternal relatives suffer from myopia and heart disease, and one cousin has arachnodactylia. The patient is very tall and shows various abnormalities such as arachnodactylia, highly arched palate, funnel chest and scoliosis. He also shows hyperextensibility of joints, inguinal hernia, dissecting aneurysm of ascending aorta, dilatation of aortic ring, aortic and mitral insufficiency, myopia and striae distensae. These findings are that of Marfan's syndrome. At the same time, we noticed yellowish papules on the neck, axillae, inguinal region and inner aspect of thighs. Brown patches were found around umbilicus. These skin lesions as well as angioid streaks of fundus are typical for pseudoxanthoma elasticum. This case should be an association by chance of these two genetic affections.
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PMID:[Pseudoxanthoma elasticum associated with Marfan syndrome (author's transl)]. 49 13

The oto-vertebral syndrome is a rare combination of malformations of the ears, spinal anomalies, and congenital heart disease. The syndrome appears to be caused by early embryonic exogenous damage during the 6th or 7th week of embryonic development. The case is reported of a 28-year-old man with bilateral aplasia of the external ear, bilateral aplasia of the ear canal, hypoplasia of the mandibula, severe thoracic scoliosis, and ventricular septal defect with pulmonary hypertension. He was admitted with dyspnea on exertion, syncope, and severe cyanosis. Cardiac catheterization revealed severe pulmonary hypertension with moderate right-to-left and slightly left-to-right shunt (Eisenmenger syndrome). Right and left ventricular function, as evaluated by angiocardiography, was slightly reduced. Because of the severe hemodynamic alterations, symptomatic therapy with digitalis, repeated venesection, and anticoagulation was initiated.
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PMID:[The oto-vertebral syndrome]. 70 8

The basic features of Goldenhar syndrome are preauricular appendices, epibulbar dermoids, vertebral and cardiac anomalies, and hypoplasia of the lungs. The syndrome appears to be caused by early damage during the first or second month of embryonic development. Prognosis is most often determined by the underlying heart disease. In the present study a 24-year-old female student is reported who exhibited a preauricular appendix on the right side, severe thoracic scoliosis, aplasia of the middle and lower lobes of the right lung, dextropositio cordis and an atrial septal defect with severe pulmonary hypertension. Cineangiocardiography revealed a mitral valve prolapse with slight mitral regurgitation and a dilated right ventricle with severe tricuspid regurgitation. Left and right ventricular function was slightly to moderately reduced. Selective coronary arteriography revealed a coronary fistula from an atrial branch of the left coronary artery to the right atrium. Symptomatic therapy with digitalis, anticoagulation and repeated venesection was initiated because of the severe hemodynamic findings. However, the patient developed syncope at increasingly frequent intervals and died 3 months after the initial examination.
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PMID:[Goldenhar syndrome]. 76 Jan 85

Structural parameters of muscles in some diseases have been studied using X-ray diffraction methods. Fixed parts of muscles dissected in the operation correcting the vertebrate shape were used as samples in the case of patients III-IV stage of scoliosis muscles. The sarcomere protofibrillar lattice in muscles was found to be retained until the destruction of the muscle tissue; no differences between the muscles from the convex and concave sides of the vertebrate were observed. This seems to be an evidence of functional inertia of the diseased muscles and of their atrophy. Fixed parts of myocardial muscles taken during surgical correction were used as samples in investigating the Tetralogy of Fallot heart disease in children. A disturbance of packing parameters of the protofibrillar apparatus was observed in a significant part of the apparatus volume. In the case of critical experimental heart deficiency in dogs the protofibrillar lattice structure was retained, probably due to its resistance nature.
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PMID:[X-ray diffraction study of muscles in disease]. 111 26

An attempt has been made to determine the aetiological factors in infantile idiopathic scoliosis from a clinical, genetic and epidemiological survey of 134 infants, ninety-seven of whom developed a curve in the first six months of life. Plagiocephaly was present in all cases; mental retardation occurred in 13 per cent of males with progressive scoliosis; congenital dislocation of the hip occurred in 3-5 per cent of cases and congenital heart disease in 2-5 per cent; and inguinal hernia was found in 7-4 per cent of males. Approximately 3 per cent of parents and 3 per cent of sibs had the same deformity, thirty times the general population frequency for the Edinburgh area. Other positive findings included an excess of breech presentations and of premature, low birthweight males, and a preponderance of curves developing in the winter months. Infants with progressive scoliosis tended to have older mothers and to come from poorer families. Only three children all with resolving scoliosis, habitually lay prone in early infancy, in marked contrast to North American infants where this posture is usual. The almost complete absence of infantile idiopathic scoliosis in North America is noted and it is thought that the two facts may be related. The aetiology is likely to be multifactorial, with a genetic tendency to the deformity which is either "triggered off" or prevented by external factors.
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PMID:Infantile idiopathic scoliosis. Causative factors, particularly in the first six months of life. 114 Dec 79

Fifty-one patients under the age of 18 with histologic-proven malignant urinary tract tumors were encountered at the Department of Pediatrics of National Taiwan University Hospital from 1979 to 1989. There were 47 tumors arising from the kidney, 2 from the bladder, and 2 from the urethra. For upper urinary tract tumors, abdominal mass (92%) and abdominal distension (86%) were the most common symptoms and signs. For lower urinary tract tumors, painless hematuria, bladder distension and urinary difficulty were most frequently encountered. Associated anomalies were found in one-fifth of our patients, including kyphosis, undescended testes, hypospadias, inguinal hernia, intersex and congenital heart disease. Diagnosis was aided by plain X ray, abdominal ultrasonography, intravenous pyelography, computed tomography, or angiography in upper urinary tract tumors, and cystoscopy, cystography and computed tomography in lower urinary tract tumors. Multimodal treatment including total excision, multiagent chemotherapy, and radiotherapy were performed. Postoperative complications included infection (22%), ascites (19%), ileus, pleural effusion, scoliosis, neuropathies, and growth retardation. The local recurrence rate was 32%, and the metastatic rate was 34%. The overall mortality rate was 53% for an average follow-up period of 38 months. The causes of death included sepsis, respiratory failure and hepatic failure. The two-year survival rate was 47.6%. The important prognostic factors included histology, staging and vascular invasion, in addition to combined chemotherapy and radiotherapy.
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PMID:Malignant urinary tract tumor in childhood. 168 60

In order to evaluate the difficulties of treatment of associated scoliosis and congenital heart disease (C.H.D.), 44 patients who have been treated between 1970 and 1988 were reviewed. The scoliosis was idiopathic in 30 cases, congenital in 11, neurologic in 3. There were 27 females and 17 males. Twenty one patients had a cyanotic C.H.D. (twelve tetralogies of Fallot). No relation was found between the side of the cardiac approach and the side of the thoracic curves. Three cases of right aortic arch with two left thoracic scolioses were noted. Associated anomalies were encountered in 60 p. 100 of cases. The analysis of old chest X-rays, revealed that the scoliosis was already present an average of 5 years before the first orthopaedic consultation. Eight patients had an orthopaedic treatment; in ten cases surgery was decided but not performed. Twenty six patients were operated. (22 posterior and six anterior procedures). The average angulation was 55 degrees pre-operatively, 30 degrees post-operatively and 40 degrees at a 40 months follow-up. In five cases a pseudarthrosis led to re-intervention. One patient died during a posterior procedure, an other post-operatively. Two patients had a severe complication (one heart arrest; one gaz-embolism) but recovered. The surgical prognosis can be evaluated on an original scale including different cardiac parameters, and the importance of the planned surgical procedure. Under a limited mark, no serious complications occurred. In conclusion, a closed collaboration between pediatricians and the orthopaedic team, should lead to more precocious screening of scoliosis in cardiologic children and before the orthopaedic procedure, to a more precise risk factors evaluation.
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PMID:[Scoliosis and congenital heart diseases in children. Apropos of 44 cases]. 183 22

We defined the natural history of scoliosis in congenital heart disease (CHD) with respect to the risk of progression and indications for treatment. We reviewed radiographs of 48 children with CHD and scoliosis. Thirty-seven had developmental scoliosis (77%), and 11 (23%) had congenital scoliosis. Children with congenital scoliosis can be managed in the same way as children without CHD. Developmental curves less than 30 degrees in children aged greater than 10 years showed no progression. Developmental curves greater than 30 degrees with onset in children aged less than 10 years and progression greater than 9 degrees per year require treatment.
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PMID:Natural history of scoliosis in congenital heart disease. 198 77

A 10-year-old boy with idiopathic scoliosis presented with exertional dyspnea, lower leg edema since two weeks prior to admission. Physical examination revealed neck venous engorgement, moist rales, mild hepatomegaly, ascites and lower leg edema in addition to the scoliosis. Chest x-ray films showed cardiomegaly, pulmonary congestion and pneumonic infiltration of both lower lobes with bilateral pleural effusion. Electrocardiograms showed right axis deviation and right atrial hypertrophy. Two-dimensional echocardiography revealed dilatation of the right heart chambers and pulmonary artery. He was treated initially with mechanical ventilation, antibiotics, bronchodilators, diuretics, vasodilators, and digoxin with improvement. Unfortunately the patient became comatose after an episode of cardiopulmonary arrest. He did not recover from this tragedy and was discharged against medical advice in comatose state. Scoliosis may be complicated with pneumonia and cor pulmonale in children. Early surgical correction of the scoliosis may prevent the development of cor pulmonale. Combined medical and surgical managements should be carried out aiming at scoliotic heart disease.
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PMID:[Idiopathic scoliosis and pneumonia complicated with cor pulmonale. Report of one case]. 227 31


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