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

Two hundred nine children with congenital heart defects characterized by excessive pulmonary blood flow underwent pulmonary artery banding at The Hospital for Sick Children from January, 1972, through December, 1982. The indications for banding, rather than complete repair, varied with the type of cardiac defect as well as with the year of presentation to our hospital. A simplified method of estimating required band circumference has been developed for infants with ventricular septal defect, with or without major intracardiac mixing disorders. Infants with simple defects without intracardiac bidirectional mixing disorders receive a band at a circumference of 20 mm + 1 mm for each kilogram of body weight, whereas infants with bidirectional mixing disorders receive a band at a circumference of 24 mm + 1 mm for each kilogram of body weight. The overall operative mortality varies with the underlying cardiac defect and with associated medical conditions but is relatively low in the less-complicated cases. The use of a formula to predict a starting band circumference, with loosening only as required by cyanosis or bradycardia, allows predictable control of congestive symptoms and pulmonary hypertension in the majority of infants. The cumbersome measurement of pulmonary artery pressure and the unpredictable changes in pressure during anesthesia are avoided. Pulmonary artery banding remains an effective means of achieving satisfactory palliation in infants with congenital heart disease and excessive pulmonary blood flow.
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PMID:Pulmonary artery banding. 620 31

Children with cardiac disease who undergo noncardiac surgical procedures may encounter risks beyond those usually associated with surgical procedures. In this article, several complicating factors seen in children with heart disease are discussed, including arrhythmias, cyanosis, congestive heart failure, pulmonary hypertension, and subacute bacterial endocarditis.
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PMID:Evaluation of the pediatric surgical patient with congenital heart disease. 635 16

The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of non-dilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy. Twelve of the 14 infants who underwent left-heart catheterization showed substantial obstruction to left ventricular outflow (peak systolic pressure gradient greater than or equal to 35 mm Hg). However, unlike older patients with hypertrophic cardiomyopathy, infants with this condition commonly had marked obstruction to right ventricular outflow (35-106 mm Hg) (nine patients); in six patients, the magnitude of obstruction to right ventricular outflow was at least as great as that to left ventricular outflow. Asymmetric hypertrophy of the ventricular septum relative to the left ventricular free wall was present in the 16 patients who had echocardiographic or necropsy examination. Ventricular septal thickening was substantial in patients studied both before and after 6 months of age (mean 16 mm), indicating that in patients with hypertrophic cardiomyopathy, marked left ventricular hypertrophy may be present early in life and is probably congenital. The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure.
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PMID:Hypertrophic cardiomyopathy in infants: clinical features and natural history. 645 22

Four infants with Down syndrome developed cor pulmonale and heart failure in association with chronic upper airway obstruction. Features of the sleep apnea syndrome were conspicuous; namely, noisy breathing with retraction, cyanosis and frequent apnea during sleep, and daytime lethargy and somnolence. The clinical picture masqueraded as cyanotic congenital heart disease. Arterial blood gas analyses revealed alveolar hypoventilation, especially during sleep. The nature of the obstructive element was variable. Adenoidectomy provided partial relief in one patient, and tonsillectomy and adenoidectomy resulted in temporary improvement in two others. Three patients were markedly benefitted by tracheostomy. Functional inspiratory pharyngeal closure was demonstrated fluorographically in one patient. Infants with Down syndrome may be predisposed to upper airway obstruction by virtue of hypoplasia of facial and oropharyngeal structures and generalized hypotonia. Additional obstructive elements may be contributed by hypertrophied lymphoid tissue, excessive secretions, and glossoptosis. Removal of the obstructive element is helpful, but functional obstruction may only be relieved by tracheostomy.
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PMID:Alveolar hypoventilation and cor pulmonale associated with chronic airway obstruction in infants with Down syndrome. 645 3

Morgagni hernias during infancy are associated with significant respiratory symptoms as well as other congenital anomalies, particularly congenital heart disease. During the past 6 years we have had the opportunity to treat five infants less than 1 year of age with Morgagni hernias. A review of the literature revealed an additional seventeen detailed case reports of infants with Morgagni hernias. Each of our patients was symptomatic as were all but two of those previously reported. These patients presented with tachypnea, dyspnea, and cyanosis which was very similar to the presentation of Bochdalek hernias seen during infancy. Thirteen of 16 patients in whom detailed descriptions of associated anomalies were given including four of our own patients, had significant congenital anomalies. Nine involved the heart, including three infants with dextracardia, three with ventricular septal defects, and two with anomalous pulmonary venous return. Five infants were retarded, including three with Trisomy 21. Two of the previously reported patients had large omphaloceles and other stigmata of Cantrell's syndrome. All but one of the patients reported, including ours, had a hernia sac. The liver, colon, and small bowel were most commonly found in the hernia sac; however, the presence of the stomach and spleen have been reported. While both the transabdominal and transthoracic approaches to Morgagni hernias have been advocated and, indeed, repair of the defect can be accomplished through both approaches, the transthoracic approach allows better exposure of incarcerated viscera, particularly the frequently found anomalous left lobe of the liver.
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PMID:Morgagni hernias during infancy: presentation and associated anomalies. 648 84

To assess the etiology of depressed ventricular function in cyanotic congenital heart disease, an experimental model in immature puppies was devised in which the left atrial appendage is directly anastamosed to the distally banded pulmonary artery. The preparation results in significant cyanosis during a 3-month study period (PO2 = 91 +/- 4 vs 43 +/- 3, hematocrit = 33 +/- 3 vs 55 +/- 5). Compared to age- and weight-matched unoperated controls, cyanotic animals developed significant depression of biventricular ejection fraction (P less than 0.05), increased heart weight indexed for body weight (P less than 0.05), increased right ventricular weight (P less than 0.02), and increased right ventricular free wall thickness (P less than 0.05). There was no alteration in ventricular volumes. In addition, mean myocardial stores of high-energy phosphate compounds were unaltered. The model allows a reproducible level of hypoxemia to be produced in young animals, utilizes no prosthetic materials, and allows future experiments to be performed to ascertain the metabolic response of the cyanotic myocardium to exercise or surgically induced ischemia.
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PMID:A model of cyanotic heart disease: functional, pathological, and metabolic sequelae in the immature canine heart. 648 24

A 10.5 year old girl with liver cirrhosis due to AAT-deficiency (Pi type ZZ) developed cyanosis and clubbing of finger and toes. Clinical aspect of a cyanotic heart disease appeared with 10 years, 7 years after diagnosis of cirrhosis. By contrast echocardiography existence of intrapulmonary arterio-venous shunts was demonstrated. When determined during the first year of life, serum-alpha-1-globulin-fraction of the patient was found to be normal. The result indicates, that even in severe AAT-deficiency of Pi type ZZ direct determination of AAT is necessary for diagnosis of the disease.
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PMID:[Liver cirrhosis due in alpha-1-antitrypsinin deficiency and development of an arteriovenous shunts of the lungs]. 660 21

The authors experienced with four cases of brain abscess, one of which ruptured into the lateral ventricle. Two cases were treated conservatively and the remaining two were treated surgically. All cases recovered satisfactorily. Case 1: A 30-year-old man with congenital cyanotic heart disease was admitted on Oct. 4, 1979, because of convulsion. Physical examination revealed motor aphasia, right hemihypesthesia, cyanosis, clubbed finger and continuous heart murmur. CT scan showed a ring-enhance mass lesion in the left temporal lobe. Although transient deterioration of the clinical course was observed, he improved satisfactorily after medical treatment of 20 days. Case 2: A 54-year-old man with left homonymous upper quadrantanopia and systolic heart murmur was admitted on Sept. 17, 1979. alpha-streptococcus was detected in the culture of his arterial blood. Vegetation near the mitral valve was revealed by ultrasonic cardiogram. CT scan showed an irregular and ill-shaped ring-enhanced mass in the left temporoparietal region. A saccular aneurysm in the insular portion of the posterior parietal artery was seen on the left carotid angiogram. He was also treated medically and the abscess was gradually reduced and the aneurysm disappeared. Case 3: A 28-year-old man complaining of headache, nausea and vomiting was admitted on Dec. 1, 1979. Physical examination revealed nuchal stiffness, right homonymous hemianopia and bilateral choked disc.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Brain abscess: successful treatment of 4 cases including one with ventricular perforation]. 666 53

A newborn with massive tricuspid regurgitation, atrial flutter, congestive heart failure, and a high serum lithium level is described. This is the first patient to initially manifest tricuspid regurgitation and atrial flutter, and the 11th described patient with cardiac disease among infants exposed to lithium compounds in the first trimester of pregnancy. Sixty-three percent of these infants had tricuspid valve involvement. Lithium carbonate may be a factor in the increasing incidence of congenital heart disease when taken during early pregnancy. It also causes neurologic depression, cyanosis, and cardiac arrhythmia when consumed prior to delivery.
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PMID:Tricuspid valve regurgitation and lithium carbonate toxicity in a newborn infant. 679 56

Four cases of "absent pulmonary valve" (APV) are described. This congenital heart disease consists of aplasia or extreme hypoplasia of pulmonary semilunar cusps and is always combined with aneurysmatic dilatation of pulmonary artery. In two of them the diagnosis was confirmed at autopsy. The remaining two are clinical reports surgically confirmed. One of them was studied by single cristal and two-dimensional echocardiography. In three cases APV was associated with tetralogy of Fallot, while in one case there was an intact ventricular septum. Review of literature allowed us to select 149 cases of APV anatomically confirmed, besides our ownes. It is stressed on that APV is usually associated with dextroposition of the aorta and ventricular septal defect by conoventricular malallignment. It is suggested that pathogenesis of this malformation is a consequence of an anomalous development of mesenchimal tissue of pulmonary cusps rather than an asymmetrical truncal sepimentation. We favour the hypothesis that aneurysmatic dilatation of pulmonary artery is caused by altered hemodynamics acting both in foetal and extrauterine life, even if differently expressed. Pathophysiologic and diagnostic value of cyanosis, dyspnea, and systo-diastolic murmur are discussed. Some outlines of the most important diagnostic procedures are reviewed and particularly echocardiography, which shows aortic overriding and dilatation of right ventricular outflow tract and pulmonary artery separated by a restricted pulmonary annulus. Prognosis and therapy are also mentioned.
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PMID:[Absent pulmonary valve. Report of 4 cases, echocardiographic assessment and review of the literature (author's transl)]. 700 35


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