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

Nine cases have been presented in detail to illustrate some of the varied causes of sudden neurological deficit in childhood: arteriovenous malformation, cryptic hamartoma, berry aneurysm, mycotic aneurysm, intraspinal arteriovenous malformation, brain tumor, migraine, arteritis, and multiple sclerosis. The Boston Children's Hospital experience with aneurysms and intracranial arteriovenous malformation has been summarized. It is noteworthy that a cutaneous hemangioma overlay one cranial and one intraspinal arteriovenous malformation. One small but deep cerebral arteriovenous malformation apparently destroyed itself after its second hemorrhage. Not only have multiple sclerosis and a brain tumor mimicked a vascular lesion, but a series of vascular accidents was misdiagnosed first as multiple sclerosis then as a thalamic tumor. The many possible causes of childhood strokes has been thoroughly cataloged in the Report of the Joint Committee for Stroke Facilities in 1973 (11). Children may be more susceptible to strokes because of congenital abnormalities such as congenital heart disease, hemophilia, and sickle cell anemia, or by diseases which more commonly occur in this age group, such as leukemia. The likelihood of brain abscess in cyanotic congenital heart disease is stressed. Arteriographic studies in our series have been safe; however, there have been reports of probable worsening of symptoms in children with multiple cerebral occlusive lesions in the presence of homocystinuria.
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PMID:Strokes in children. 98 45

Because massive pulmonary thromboembolism is a rarely described cause of sudden and unexpected death in the pediatric age group, a 50-year retrospective review of autopsy cases at the Hospital for Sick Children, Toronto, Canada, was conducted to determine the local incidence and clinicopathologic features of such patients. Only 8 cases (0.05%) from a total of approximately 17,500 autopsies were found. The ages ranged from 1 month to 13 years and predisposing factors included recent surgery, congenital heart disease, in-dwelling venous catheters, sepsis, an arteriovenous malformation, occult malignancy, and prolonged immobility. This study confirms that pulmonary thromboembolism is a diagnosis that must be considered in cases of sudden and unexpected death in the pediatric age group. Its incidence, however, appears to be extremely low even in a relatively high-risk population.
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PMID:Sudden and unexpected death in infancy and childhood due to pulmonary thromboembolism. An autopsy study. 230 30

Saline contrast echocardiography was performed in 889 children from June 1976 through February 1988. One-third of these studies were in postoperative patients. A patent foramen ovale was identified by finding right to left shunting on venous contrast injection in 37% of 127 children studied with a structurally normal heart. The incidence of such shunting was greater at younger ages (55% younger than 1 month versus 22% older than 1 month). In most patients with an atrial or ventricular septal defect, some right to left shunting was demonstrable. The technique was useful in distinguishing different forms of atrial septal defect and identifying muscular ventricular septal defects that were difficult to image directly. The technique was used in the catheterization laboratory to aid in the identification of congenital coronary artery fistulas and was diagnostic in two cases of pulmonary arteriovenous malformation. In patients with situs abnormalities, the technique was useful in identifying the systemic venous connections to the atria. Contrast echocardiography was also used in postoperative evaluations. The technique was useful in identifying patch leaks and residual defects after Senning, Mustard and Fontan operations, and after closure of atrial and ventricular septal defects. Most patients were found to have no superior vena cava obstruction by contrast echocardiography after the Senning or Mustard procedure. Contrast echocardiography continues to be a useful technique in the diagnosis of a wide spectrum of congenital heart disease, as well as in the postoperative evaluation of congenital heart surgery.
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PMID:Contrast two-dimensional echocardiography in congenital heart disease: techniques, indications and clinical utility. 291 74

Cyanosis is frequently encountered in the neonate or infant. Most often it is due to congenital heart disease or primary lung disease. Pulmonary arteriovenous malformation is an unusual cause of cyanosis. Polycythemia and clubbing are associated findings. The treatment is excision or embolization if the disease is not too extensive.
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PMID:Cyanosis due to pulmonary arteriovenous malformation. 305 75

Arteriovenous fistula is a relatively rare cause of severe congestive heart failure in the newborn. An intracranial arteriovenous malformation associated with an aneurysm of the great vein of Galen is the most frequent of such malformations and, although well known, it remains a difficult diagnosis in the neonate because the clinical picture first presents with findings suggesting cardiac disease. The newborn with a vein of Galen aneurysm has volume and pressure overload on his myocardium; cyanosis is often present due to persistent fetal circulation; peripheral pulses are generally decreased in amplitude except in those arteries near the fistula where they are bounding, as an expression of a hyperdynamic status. A continuous murmur may be heard over the scalp, but this highly suggestive sign is often not present. The majority of newborns with intracranial arteriovenous fistula die very soon. Unfortunately, most of these infants are at first considered to have congenital heart disease and are, therefore, subjected to cardiac catheterization and angiography, with their well-known risks, before a correct diagnosis is made. Cross-sectional echocardiography, by demonstrating normal intracardiac anatomy, eliminates the need for an invasive investigation in a very sick neonate. Ultrasonography of the head will then provide a rapid and accurate demonstration of the aneurysm of vein of Galen. Cerebral angiography will complete the diagnosis in those cases in which a neurosurgical intervention is contemplated.
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PMID:Congestive heart failure secondary to cerebral arterio-venous fistula. 330 85

Eight therapeutic embolisation procedures were performed by the transcutaneous catheter technique in seven patients with congenital heart disease. After surgical correction of tetralogy of Fallot (four patients), catheter embolisation was used to occlude two large aortopulmonary collaterals (one patient), three small aortopulmonary collaterals (one patient), and two Blalock-Taussig shunts (two patients). In two patients congenital coronary anomalies were occluded--a coronary arteriovenous malformation and a coronary artery/bronchial artery anastomosis. In one patient a pulmonary arteriovenous malformation was embolised. Detachable balloons were used to occlude six large arteries, the three small arteries were occluded with small gelfoam fragments, and the pulmonary arteriovenous malformation was occluded with multiple steel coils and large gelfoam pieces. Successful occlusion was achieved in all cases. No complications were encountered and the procedure was well tolerated even in the two patients receiving postoperative intensive care. Therapeutic embolisation in suitable cases is a safe and effective alternative to surgery and the detachable balloon technique is effective in occluding high flow vessels.
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PMID:Embolisation procedures in congenital heart disease. 401 28

A 10 years old school boy, who had been known to have a small ventricular septal defect from 2 months after birth, was admitted to us with a diagnosis of subarachnoid hemorrhage on October 24, 1978. On admission, neurological examination revealed mild right hemiparesis and miotic pupils in semicomatose state, and thrilling, that was equivalent to Levine III, pansystolic murmur and cyanosis of nails were noticed. By CT scan, intraventricular hemorrhage originated from hemorrhage in the basal ganglia was shown and the left CAG revealed an arteriovenous malformation in the left basal ganglia. Electrocardiogram showed mild cardiac failure and chest x-ray showed pulmonary congestion. Neurological symptoms and abnormal cardiopulmonary changes made well manageable after bilateral ventricular drainage. Twenty four cases of arteriovenous malformation associated with congenital heart disease were reviewed in the reported literature, and 20 cases were found to combine with Galenic aneurysm with congenital heart disease. All of those cases had been in a little pause after the birth and the most of congenital heart diseases were of patient ductus arteriosus. The rest of cases including our present case were 4 cases, who had rather small arteriovenous malformation, and the severity of the congenital heart disease was attributable to the patients mortality. In all of the report at present only our case was the single survival. Embryological investigation on arteriovenous malformation of the brain and congenital heart disease indicated that association of both conditions could be attributable to the vascular malformation in a very limited gestational stage, and that the incidence should be much less than reported. Our case showed the intracranial hypertension was found to create serious cardio-pulmonary dysfunction when congenital heart disease being accompanied, and it was presumably relating to the relatively higher incidence of Galenic aneurysm associated with ductus arteriosus.
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PMID:[Arteriovenous malformation associated with congenital heart disease, with a remark on accompanying cardiopulmonary dysfunction (author's transl)]. 724 22

The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplantation program, which began in 1982. Twenty pediatric patients (age range 3 to 18 years) have had heart-lung (n = 11), double lung (n = 8), and single lung (n = 1) transplantation procedures. The causes of end-stage lung disease were primary pulmonary hypertension (n = 7), congenital heart disease (n = 5), cystic fibrosis (n = 4), pulmonary arteriovenous malformation (n = 2), graft-versus-host disease (n = 1), and desquamative interstitial pneumonitis (n = 1). Four (20%) patients had thoracic surgical procedures before the transplantation operation. The survival was 80% at a mean follow-up of 2 years. Immunosuppressive drugs included cyclosporine (n = 9) or FK 506 (n = 11) based therapy with azathioprine and steroids. Children were followed up by means of spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection was 1.4 at 30 days, 0.5 at 30 to 90 days, and 1.4 at more than 90 days, and the first treated rejection episode occurred on average 28 days after the operation. Obliterative bronchiolitis developed in four (25%) of 16 patients surviving more than 100 days. Results of pulmonary function tests have remained good in almost all recipients. The greatest infectious risk was that of cytomegalovirus: one death and one case of pneumonia. Posttransplantation lymphoproliferative disease was diagnosed in two (12.5%) patients; both recovered. The most common complications were hypertension (25%) and postoperative bleeding (15%). Early results indicate that lung transplantation is a most promising therapy for children with severe vascular and parenchymal lung disease.
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PMID:Pediatric lung transplantation. The years 1985 to 1992 and the clinical trial of FK 506. 767 72

Forty children (aged 1 to 18 years, 27 female and 13 male) have undergone heart-lung (21), double lung (17), and single lung (2) transplant procedures at our center from 1985 through April 1994. The indications for transplantation have been diverse, primary pulmonary hypertension (10), cystic fibrosis (11), congenital heart disease (10), arteriovenous malformation (3), emphysema (1), graft-versus-host disease (1), rheumatoid lung (1), cardiomyopathy (1), desquamative interstitial pneumonitis (1), and Proteus syndrome (1). The actuarial 1-year survival was 73% (mean follow-up 2 years). One-year actuarial survival for disease groups ranged from 60% for cystic fibrosis to 90% for congenital heart disease. We have identified six issues critical to the patient and programatic survival of pediatric lung transplantation. Our experience and management strategies in these areas are reviewed. Cytomegalovirus: Cytomegalovirus disease developed in six of eight patients with cytomegalovirus mismatching (donor +/recipient-) and in seven of 32 patients who survived more than 30 days (23%). All but cytomegalovirus donor -/recipient- patients were treated with ganciclovir for 4 weeks after transplantation. Obliterative bronchiolitis: Obliterative bronchiolitis developed in seven of 32 (25%) patients who survived more than 30 days. Obliterative bronchiolitis was manifest within the first posttransplantation year as a rapid decline in small airway function. Aggressive augmentation of immunosuppression has been used with little success. Posttransplantation lymphoproliferative disease: Posttransplantation lymphoproliferative disease developed in five of 32 (15%) patients who survived more than 30 days developed. One patient died (17% mortality) despite retransplantation. In four patients the disease resolved with reduction in immunosuppression alone, and one required the addition of interferon alfa. Cystic fibrosis: We have changed our management strategies to avoid triple drug immunosuppression, perioperative blood and bronchial cultures, aggressive antimicrobial therapy, and exclusion of patients with panresistant organisms; this has resulted in elimination of infectious mortalities thus far in the pediatric cystic fibrosis group. Airways: In 21 heart-lung recipients with tracheal anastomoses we have had no airway complications. The double and single lung transplant recipients accounted for 34 bronchial and one tracheal anastomoses. Three (9%) bronchial stenoses developed. Two were treated with silicone stents and one with balloon dilation.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Critical issues in pediatric lung transplantation. 781 8

The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplant program, which began in 1982. Thirty-two pediatric patients (age range 1 to 18 years) have undergone heart-lung (n = 16), double-lung (n = 14), and single-lung (n = 2) transplantation procedures. The cause of end-stage lung disease was primary pulmonary hypertension (n = 7), congenital heart disease (n = 7), cystic fibrosis (n = 9), pulmonary arteriovenous malformation (n = 2), desquamative interstitial pneumonitis (n = 2), graft-versus-host disease (n = 1), emphysema (n = 1), rheumatoid lung (n = 1), cardiomyopathy (n = 1), and Proteus syndrome (n = 1). Six patients (19%) underwent pretransplantation thoracic surgical procedures. The survival rate was 78% at a mean follow-up of 1.8 years. The survival rate in the 23 recipients without cystic fibrosis was 87% (95% since 1985). The actuarial 1-year survival rate in the nine recipients with cystic fibrosis was 55%. Immunosuppression was cyclosporine (n = 9) or FK 506 (n = 23)-based therapy with azathioprine and steroids. Children were followed up by spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection per patient in the groups treated with cyclosporine and FK 506, respectively, was 1.0 and 1.2 at 30 days, 0.67 and 0.38 at 30 to 90 days, and 2.33 and 0.46 at greater than 90 days (p < 0.001, Fisher exact test).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pediatric lung transplantation: expanding indications, 1985 to 1993. 831 44


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