Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Operation was carried out on 143 children (aged from 5 days to 16 years) for tracheobronchial obstruction caused by vascular rings. A double arch of the aorta was found in 50 patients, double arch of the aorta with atresia of the left arch--in 50 patients, right arch of the aorta with ligamentum arteriosum--in 30, left arch of the aorta with an aberrant right subclavian artery--in 10, and a pulmonary loop in 3 patients. Stridor was the main clinical manifestation of the vascular ring. The diagnosis was established by means of two-projection chest radiography and esophagography with a barium meal, bronchoscopy, and angiography. The surgical approach was gained through a posterolateral (left or right) thoracotomy. No deaths occurred during the operation. Two infants died in the postoperative period from a severe bacterial infection of the respiratory tract. On the basis of a rich clinical experience the authors came to the conclusion that the presence of a right aortic arch (except for cases with situs inversus) is always pathognomic because it either causes the appearance of a vascular ring without any intracardiac anomaly, or it may be combined with a congenital heart disease in which a vascular ring is usually not encountered (tetralogy of Fallot, truncus arteriosus communis, etc.). The authors never encountered atresia of a segment of the right aortic arch. The last-named was either patent or absent.
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PMID:[Vascular ring and pulmonary loop: experience with the surgical treatment]. 178 38

Forty four children underwent surgery for congenital vascular anomalies causing tracheal compression in Kardiocentrum--Motol during last ten years. Double aortic arch was encountered 18 times, tracheal compression by the innominate artery in 12 instances, right aortic arch with left ligamentum arteriosum and possibly aberrant left subclavian artery 7 times, pulmonary artery sling in 5, finally aberrant right subclavian artery twice. The age of the children was 2 weeks to 11 years (median 6 months). Stridor was present in 38 infants under 3 months, 17 children with severe dyspnoea had artificial ventilation or were resuscitated or hospitalized at the intensive care department. In four an associated cardiac defects was present. The diagnosis was made by oesophagography, echography, laryngotracheoscopy, in the majority of children it was confirmed by angiography of the sometime with tracheobronchography. The operation was performed in 41 children from left-sided thoracotomy, the vascular ring was released by resection or retraction of the compromising vessel and connective tissue from the trachea. Serious stenosis of the distal trachea in one patient found before operation persists one year after operation, the patient breathes through an individual tracheostomic cannula. Four children could not be extubated for prolonged periods after operation, they were re-operated, the annulus was released. Three patients died, one from bronchopneumonia during the postoperative period, the second one from hypoxic brain damage and the third one after operation of a complex heart disease. In 40 children satisfactory long-term results were achieved, the operation resolved respiratory complaints. The prerequisite of satisfactory results in an accurate diagnosis, early indication of the operation and a perfect surgical operation and good postoperative care.
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PMID:[Surgical treatment of congenital vascular anomalies causing tracheoesophageal compression]. 225 80

Favorable early results have been reported utilizing transthoracic diaphragmatic plication in symptomatic children with phrenic nerve injury. However, little has been published about the late functional results of this technique. Since 1976, 10 of 3,000 patients operated on for congenital heart disease have sustained phrenic nerve injury with subsequent respiratory embarrassment. An additional patient sustained phrenic nerve injury as a result of birth trauma. The diagnosis was confirmed by paradoxical diaphragmatic motion on fluoroscopy. All but 2 patients were less than 5 months old at the time of diaphragmatic plication, and the average weight was 5.4 kg. The indication for diaphragmatic plication was inability to wean from the ventilator in 8 of the 11 patients and persistent postoperative tachypnea, stridor, and CO2 retention in the remaining 3 patients. A more aggressive approach to diagnosis and operative treatment since 1980 has resulted in a substantially shorter duration of endotracheal intubation and a shorter stay in the intensive care unit. Diaphragmatic fluoroscopy 1 to 7 years postoperatively has demonstrated return of normal function in 6 of 6 patients studied.
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PMID:Long-term fate of the diaphragm surgically plicated during infancy and early childhood. 360 60

Transthoracic diaphragmatic plication is a simple and effective means of treatment for phrenic nerve injury and resultant diaphragmatic paralysis, and should be undertaken as soon as the diagnosis of diaphragmatic paralysis is established in a child with substantial respiratory difficulty. Seven of more than 1,500 patients sustained phrenic nerve injury during operation for congenital heart disease at our institution over the past five years. All but 1 patient were less than 5 months old, and the average weight was 3.8 kg. Five of the 7 could not be weaned from the ventilator, and the other 2 had persistent postoperative tachypnea and stridor. Before 1980, 3 patients were intubated for an average of 16 days prior to diagnosis by fluoroscopy. Since then, 4 patients have been intubated for an average of 7.8 days before diagnosis. All 7 underwent transthoracic diaphragmatic plication and were extubated by 6 days after operation (mean, 3.7 days). Six patients survived and are doing well. The 1 death occurred in 1976 in a premature infant with multiple congenital cardiac lesions; diagnosis was delayed until the twenty-eighth day after intubation and transthoracic diaphragmatic plication, until the sixty-second day. This infant died of pneumonia one month after extubation. These data support our policy of establishing an early diagnosis of phrenic nerve injury and early treatment utilizing transthoracic diaphragmatic plication in symptomatic children.
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PMID:Aggressive treatment of acquired phrenic nerve paralysis in infants and small children. 728 17

Over a period of 13 years 21 patients between the ages of three weeks and 13 years presenting to this centre had a vascular ring. A double aortic arch was present in 11 cases, a right aortic arch with a retroesophageal left subclavian artery in five cases, a right aortic arch with a normal left innominate artery, a Kommerell's diverticulum and a left ligamentum arteriosum encircling the trachea and oesophagus occurred in two cases and an aberrant left pulmonary artery arising from the right pulmonary artery occurred in three cases. The majority (19/21) presented with a history of chronic stridor, although other significant associated symptoms included: recurrent respiratory infection, wheeze, cyanotic episodes and difficulty or pain on swallowing. The barium swallow was the most useful method of investigation and was diagnostic in all of the 95% of cases in which it was performed. Nineteen patients underwent surgery uneventfully although stridor often remained a problem for some time following repair. Six patients (28%) had associated congenital heart disease. Three (14%) patients who did not have surgery died.
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PMID:A review of vascular rings 1980-1992. 786 Feb 61

Symptomatic coronary artery fistulas (CAF) are associated with significant morbidity and mortality. With the advent of high-resolution 2-dimensional and color Doppler echocardiography, the detection rate of clinically silent CAF has increased, but their clinical significance and outcome have not been defined. The clinical, echocardiographic, electrocardiographic, and angiographic findings and documented follow-up of 31 patients with an echocardiographic finding of a clinically silent coronary artery fistula from 1986 to 1997 were analyzed. Mean age at diagnosis was 7.2+/-8.4 years. Indications for echocardiography were murmur (n = 23), congenital heart disease (n = 2), cardiomegaly (n = 2), chest pain (n = 1), stridor (n = 1), syncope (n = 1), and chest trauma (n = 1). CAF were detected with color Doppler flow mapping in all patients. The origin of the fistula was from the left coronary artery system (n = 27), right coronary artery system (n = 3), and bilateral (n = 1). The exit sites were the pulmonary artery (n = 18), right ventricle (n = 8), right atrium (n = 2), and left ventricle (n = 3). Global and regional left ventricular function were normal in all patients at presentation and follow-up. Spontaneous closure of the fistula was documented in 7 patients (23%) at mean follow-up of 2.6+/-2.0 years. In 23 patients the fistula persisted without intervention. All patients remained asymptomatic, without adverse clinical events or evidence of ischemia at a mean age at follow-up of 9.3+/-9.1 years (range 4 months to 42.0). Based on this experience, there is no evidence that clinically silent CAF diagnosed incidentally by color Doppler echocardiography are associated with adverse clinical outcome in childhood and adolescence. Conservative management with continued follow-up of these patients appears to be appropriate.
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PMID:Prognostic significance of clinically silent coronary artery fistulas. 1007 33

Down syndrome (DS) is the most common chromosomal anomaly in humans. Numerous congenital malformations associated with DS have been described. However, there are insufficient data available about airway anomalies. Our objective was to characterize the clinical presentation, frequency, and type of airway anomalies in a population of patients with DS. A retrospective evaluation of flexible bronchoscopies performed in 24 DS patients due to significant respiratory morbidity was compared to the findings in 324 non-DS patients during the same time period. The procedure was carried out under sedation, using an Olympus BF3C20 bronchoscope. The main indications for the procedure were atelectasis of the right upper lobe (12/24) and stridor (7/24). The most common associated conditions were congenital heart disease (12/24) and reactive airways disease (12/24). The most important endoscopic findings were: laryngomalacia (12/24), tracheomalacia (8/24), tracheal bronchus (5/24), and bronchomalacia (5/24). Only six patients had a normal examination. Multiple airway anomalies (>/=2) were a common finding in this series. We conclude that patients with DS and respiratory symptoms have a high incidence of airway anomalies compared to non-DS patients. The clinician should have a high index of suspicion for airway anomalies in DS patients with respiratory symptoms.
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PMID:Airway anomalies in children with Down syndrome: endoscopic findings. 1283 93

Compression of the paediatric airway is a relatively common and often unrecognized complication of congenital cardiac and aortic arch anomalies. Airway obstruction may be the result of an anomalous relationship between the tracheobronchial tree and vascular structures (producing a vascular ring) or the result of extrinsic compression caused by dilated pulmonary arteries, left atrial enlargement, massive cardiomegaly, or intraluminal bronchial obstruction. A high index of suspicion of mechanical airway compression should be maintained in infants and children with recurrent respiratory difficulties, stridor, wheezing, dysphagia, or apnoea unexplained by other causes. Prompt diagnosis is required to avoid death and minimize airway damage. In addition to plain chest radiography and echocardiography, diagnostic investigations may consist of barium oesophagography, magnetic resonance imaging (MRI), computed tomography, cardiac catheterization and bronchoscopy. The most important recent advance is MRI, which can produce high quality three-dimensional reconstruction of all anatomic elements allowing for precise anatomic delineation and improved surgical planning. Anaesthetic technique will depend on the type of vascular ring and the presence of any congenital heart disease or intrinsic lesions of the tracheobronchial tree. Vascular rings may be repaired through a conventional posterolateral thoracotomy, or utilizing video-assisted thoracoscopic surgery (VATS) or robotic endoscopic surgery. Persistent airway obstruction following surgical repair may be due to residual compression, secondary airway wall instability (malacia), or intrinsic lesions of the airway. Simultaneous repair of cardiac defects and vascular tracheobronchial compression carries a higher risk of morbidity and mortality.
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PMID:Cardiovascular causes of airway compression. 1471 76

The anomalous origin of the left pulmonary branch known as pulmonary artery sling (PAS) is a rare form of vascular ring, a congenital heart disease in which the left pulmonary artery originates from the right pulmonary artery and runs posterior to the trachea, encircling it, and goes leftwards between the trachea and oesophagus to reach the left pulmonary hilum. The clinical outcome depends on the associated tracheal lesions and cardiac anomalies. The majority of patients (90%) have respiratory symptoms in the first year of life, due to tracheal stenosis (chronic stridor and wheezing), and with a high mortality rate if there is no surgical intervention. Other major congenital heart diseases are present in up to 50% of patients with this rare vascular ring. We present a case of this rare disease in an asymptomatic 3-year-old girl, without any associated congenital heart disease and the findings in the echocardiography and tomography that made the non-invasive diagnosis possible.
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PMID:[Asymptomatic pulmonary artery sling: Noninvasive diagnosis]. 2011 64

Vitamin D deficiency, once thought to be eradicated, is becoming a frequent occurence in children, caused mainly by dietary insufficiency. The classical manifestation is rickets, but in infants severe hypocalcaemia may present as stridor, tetany, seizures or, rarely, heart disease. Here, we describe four infants who presented with complications of severe hypocalcaemia secondary to nutritional vitamin D deficiency. (1) Female, 4 months old, several spasms. (2) Male, 8 days old, generalised tonic-clonic seizure. (3) Male, 9 months old, tetany. (4) Male, 4 months old, cardiogenic shock. The cases highlight the importance of child vitamin D supplementation from birth and throughout childhood. We also note that the vitamin D state should be evaluated by the 25(OH)-D value and not the 1,25(OH)2-D.
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PMID:Hypovitaminosis D and severe hypocalcaemia: the rebirth of an old disease. 2372 99


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