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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1997-6-10
pubmed:abstractText
We retrospectively studied the experience of our institution with isolated dysplasia of one or both atrio-ventricular valves in 22 newborn infants. All patients with associated cardiac malformations were excluded. Ten patients exhibited isolated tricuspid valve dysplasia. One patient had tricuspid valve dysplasia and a dysplastic pulmonary valve. In 10 patients, both atrio-ventricular valves were affected. Finally, mitral valve dysplasia was associated with pulmonary valve stenosis in 1 case. Associated syndromes and/or chromosomal anomalies were: Down syndrome (n=2), trisomy 18 (n=1), Noonan syndrome (n=1), Marfan syndrome (n=3), Ehlers-Danlos and Cutis laxa (n=2). Mortality was 27.2% during follow-up (mean 51 months): 3 patients with chromosomal aneuploidies, 2 patients with severe neonatal Marfan syndrome and 1 with Ehlers-Danlos. Complications were: sustained supra-ventricular tachycardia in 3, neonatal staphylococcal tricuspid valve endocarditis in 1, persistent significant valvular disease in 8. In the remaining 9 survivors, the dysplasia of the atrio-ventricular valves persists with absent or mild incompetence. Beside obvious chromosomal anomalies, newborn infants with dysplastic valves should be investigated for manifestations of connective tissue disorders. This may help to identify new pleiotropic syndromes which include valvular dysplasia as one manifestation.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0167-5273
pubmed:author
pubmed:issnType
Print
pubmed:day
18
pubmed:volume
59
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
113-8
pubmed:dateRevised
2006-7-12
pubmed:meshHeading
pubmed-meshheading:9158161-Aneuploidy, pubmed-meshheading:9158161-Chromosomes, Human, Pair 18, pubmed-meshheading:9158161-Connective Tissue Diseases, pubmed-meshheading:9158161-Cutis Laxa, pubmed-meshheading:9158161-Down Syndrome, pubmed-meshheading:9158161-Ehlers-Danlos Syndrome, pubmed-meshheading:9158161-Endocarditis, Bacterial, pubmed-meshheading:9158161-Follow-Up Studies, pubmed-meshheading:9158161-Humans, pubmed-meshheading:9158161-Infant, Newborn, pubmed-meshheading:9158161-Marfan Syndrome, pubmed-meshheading:9158161-Mitral Valve, pubmed-meshheading:9158161-Mitral Valve Insufficiency, pubmed-meshheading:9158161-Noonan Syndrome, pubmed-meshheading:9158161-Pulmonary Valve, pubmed-meshheading:9158161-Pulmonary Valve Stenosis, pubmed-meshheading:9158161-Retrospective Studies, pubmed-meshheading:9158161-Staphylococcal Infections, pubmed-meshheading:9158161-Survival Rate, pubmed-meshheading:9158161-Tachycardia, Supraventricular, pubmed-meshheading:9158161-Tricuspid Valve, pubmed-meshheading:9158161-Tricuspid Valve Insufficiency, pubmed-meshheading:9158161-Trisomy
pubmed:year
1997
pubmed:articleTitle
Atrio-ventricular valve dysplasia in 22 newborn infants.
pubmed:affiliation
Service de Cardiologie Pédiatrique, Hôpital Necker/Enfants Malades, Paris, France.
pubmed:publicationType
Journal Article