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

During the last 25 years, 20 patients with cirrhosis of liver with severe cyanosis and gross clubbing simulating congenital cyanotic heart disease were subjected to cardiac catheterization and angiography, splenography, liver function tests, and liver biopsy. No portopulmonary fistulas could be demonstrated. The cyanosis and clubbing were secondary to right to left intrapulmonary shunting across multiple tiny pulmonary arteriovenous fistulas. In 15 cases, selective pulmonary angiography revealed discrete arteriovenous fistulas. In five cases, the angiogram did not reveal any convincing evidence of pulmonary arteriovenous fistulas. In two of these five cases, peripheral vein contrast echocardiography demonstrated right to left intrapulmonary shunting and seems a sensitive investigation. Open lung biopsy in one case showed evidence of pulmonary arteriovenous fistulas.
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PMID:Cirrhosis of the liver simulating congenital cyanotic heart disease. 279 83

Right ventricular hypoplasia without additional congenital heart disease is an uncommon abnormality. It is characterized by partial absence of right ventricular inflow tract, both tricuspid and pulmonary valves being normally shaped. We report three cases; a newborn, an infant and a twelve years old boy. All of them had cyanosis and right atrial enlargement and both of the younger patients had electrocardiographic signs suggesting right ventricular hypoplasia. Right atrial mean pressure were raised. A differential diagnosis should be stablished with all sorts of right ventricular hypoplasia with cyanosis and with restrictive myocardiopathyes. Surgery for symptomatic patient should be palliative during first months of life and corrective from age one year onwards.
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PMID:[Isolated hypoplasia of the right ventricle. Study of 3 cases]. 293 60

Twenty-five cases of double-inlet left ventricle, 23 in atrial situs solitus and 2 with dextroisomerism are described. Twenty had both atrioventricular valves and in 3 a common atrioventricular valve connected to the left ventricle. In rest, the left atrioventricular valve was straddling over the left-sided right ventricle in about 15%. The position of the rudimentary right ventricle varied from superior, anterior and to the right of left ventricle (6 with transposition and 9 with concordant ventriculo arterial connection), to superior anterior and to the left of left ventricle (10 cases, all with transposition). Five cases had stenosis of the left atrioventricular valve and 2 stenosis of the right one. In 11, the clinical presentation was dominated by cyanosis, reduced pulmonary blood flow and right to left shunt. The rest had cyanosis and congestive heart failure. Five patients with right ventricle on the left had complete atrioventricular block, 9 right AQRS orientation and all of them findings of left ventricle hypertrophy. Three cases showed initial Q wave in VI and one left bundle branch block. Cases with right ventricle on the right, had left QRS orientation, left ventricle hypertrophy and 2 complete atrioventricular block. Cross-sectional echoes showed in 13, two atrioventricular valves committed to the left ventricle; in 3 of them the right ventricle was visualized. In 22 cases angiography determined the type of atrioventricular connection. Double inlet left ventricle is one of the most challenging diagnosis in congenital heart disease. Considering its lack of clinical specificity and according to our results, the electrocardiogram, echocardiogram and angiographic findings are of great help for its identification.
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PMID:[Double inlet left ventricle]. 294 47

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

A seven-month-old child with complex cyanotic heart disease desaturated dramatically following induction of anaesthesia. While a degree of hypoxaemia would have been acceptable in this infant, pulse oximetry detected an abrupt desaturation prompting the anaesthetist to consider other less common causes of cyanosis. This episode of desaturation subsided with the removal of a 12 french oesophageal stethoscope which had been inserted following induction. Further attempts to re-insert this oesophageal probe led to repeated episodes of desaturation. The most likely cause of this desaturation was a reduction in pulmonary blood flow due to compression by the oesophageal probe of an aorto-pulmonary collateral posterior to the oesophagus.
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PMID:Hypoxaemia produced by an oesophageal stethoscope: a case report. 316 34

The neural crest is involved in the differentiation of the cardiac outflow tract and branchial arches. Branchial arches one and two give rise to the eustachian tubes. Anatomically different eustachian tubes (e.g., short length) are found in persons prone to otitis media. We hypothesized a relationship between outflow tract anomalies and eustachian tube anomalies. The medical records of 238 children with a catheterization diagnosis of congenital heart disease were reviewed for otitis diagnoses. Children with syndromes known to be associated with recurring otitis media were excluded. Recurrent otitis media was recorded in 32.2% of those with outflow tract anomaly, significantly (P less than 0.03) higher than the 18.9% rate in children with a cardiac anomaly not involving the outflow tract. Neither race, sex, nor cyanosis seemed to account for the differing rates of otitis. These data may support the concept of a field defect arising from neural crest cells that influences the development of the cardiac outflow tract and eustachian tubes.
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PMID:Congenital cardiac outflow tract anomalies and otitis media. 323 84

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

Ischemic myocardial and papillary muscle dysfunction has considerable implication in newborn infants and children with normal or malformed hearts. Papillary muscle dysfunction in adults primarily involves coronary artery occlusion and ischemic necrosis in the left ventricle and papillary muscles. Infants and children rarely develop coronary artery occlusion. Their myocardial dysfunction and injury occurs with nearly equal frequency in both ventricles as a result of underperfusion from a wide range of causes, including severe birth asphyxia, congenital heart disease, and complications of premature delivery. A history of cardiogenic shock, acute congestive heart failure with cyanosis and atrioventricular murmur, or persistent fetal circulation in a newborn without congenital heart disease should alert the pathologist to the possibility of ischemic myocardial necrosis (IMN). Older infants with ventricular hypertrophy, persistent pulmonary hypertension (PPHN), bronchopulmonary dysplasia (BPD), and those with malformed hearts involving severe ventricular hypertension due to outflow obstruction or pulmonary hypertension may have IMN, fibrosis, or dystrophic calcification alone or in combination. Animal models of adult ischemic cardiac injury may not be suitable for study of the newborn.
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PMID:Ischemic myocardial necrosis and papillary muscle dysfunction in infants and children. 333 39

Serum erythropoietin levels were measured by radioimmunoassay in 146 children and young adults with congenital heart disease to assess the relationship between erythropoietin and clinical factors (heart failure, anemia, cyanosis) and hemodynamic variables affecting oxygen delivery and utilization. Erythropoietin values were in the normal range (10 to 30 microU/mL) in 73% (58 of 80) of the patients with and 82% (54 of 66) of those without cyanosis. Elevated erythropoietin values in cyanotic patients were associated with lower mixed venous oxygen saturation and tension than in cyanotic patients with normal erythropoietin levels, even though the degree of polycythemia was similar. In contrast, most of the acyanotic patients who had elevated erythropoietin levels were anemic. Of the blood oxygen measurements, mixed venous oxygen saturation and tension had the closest inverse correlation with erythropoietin values. The normal erythropoietin values in most patients are in accord with other observations that show that an elevation in erythropoietin level in response to hypoxia will be transient if it results in a rise in hemoglobin concentration "appropriate" to the degree of hypoxia. Persistent elevation of erythropoietin in patients with congenital heart disease may indicate harmful impairment of hemoglobin production that is potentially correctable.
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PMID:Serum erythropoietin levels in patients with congenital heart disease. 355 1

We describe a neurologically compromised infant with tetralogy of Fallot who was having multiple paroxysmal episodes of hypotonia, eye rolling, stiffening, and loss of consciousness. Simultaneous electroencephalography with video monitoring was used to determine if these episodes were a primary epileptic phenomenon or the result of hypoxic central nervous system involvement from paroxysms of hyperpnea and cyanosis. The findings would suggest that the paroxysmal episodes were not primarily epileptic. The electroencephalographic findings during the spell were similar to those found with hypoxia due to other causes. This may have physiological and therapeutic significance and be useful in following other infants with congenital heart disease who have similar episodes, especially when the infants have significant risk factors for seizures.
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PMID:EEG monitoring during paroxysmal hyperpnea of tetralogy of Fallot: an epileptic or hypoxic phenomenon? 359 45


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