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)

Congenital complete atrioventricular block is uncommon, and the outlook is usually regarded as favourable. Thirty-five patients with congenital heart block are presented. There was no obvious sex difference and their ages ranged from 12 days to 85 years, though most were under the age of 20 years when first seen. Accompanying heart disease was noted in six, but presentation with symptoms in early infancy was a more serious risk factor in our experience. Permanent pacing was required in 21, three of whom were neonates. One of the 14 unpaced patients died unexpectedly at the age of 5 years. Long term supervision is necessary, as most will require permanent pacing before their 50th birthday.
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PMID:Complete congenital heart block. Report of 35 cases. 710 15

Congenital complete atrioventricular block (CCAVB) is a rare potentially lethal disease with an estimated incidence of 1 every 15.000 to 20.000 live born infants.IN STRUCTURALLY NORMAL HEARTS TWO KINDS OF CONGENITAL HEART BLOCK CAN BE IDENTIFIED: one usually diagnosed in utero associated with the circulating maternal anti-SS-A/Ro and anti-SS-B/La antibodies, the other kind is detected later in the neonatal period or during the infancy or childhood and present no clear relation with maternal antibodies. Nowadays, the diagnosis can be made in utero as early as between week 16 and 28 of gestation by foetal echocardiography.The mortality in isolated CCAVB is estimated between 8 and 16% and between 4 and 8% in children and adults. The mortality and morbidity of patients diagnosed outside the neonatal period is significantly lower than those with a in utero diagnosis.Risk factors for worse outcome in CCAVB are the foetal diagnosis, the presence of hydrops fetalis, delivery at 32 weeks gestation, and a ventricular rate <55 beats/min in early pregnancy (13,18).Aim of this review is to delineate the current knowledge on CCAVB presenting in children without structural heart disease including aetiology, outcome and management. Also outlined in this review are some of the problems still debated in this issue.
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PMID:Congenital complete atrioventricular block in the early pediatric population. 2197 44