Are ECG abnormalities in Noonan syndrome characteristic for the syndrome?
| Autor: | Cees Noordam, J. A. Noonan, R. Raaijmakers, C.J.A.M. van der Burgt, Jos M. T. Draaisma, Ellen A. Croonen |
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| Rok vydání: | 2008 |
| Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Health aging / healthy living [IGMD 5] Adolescent Heart disease Cardiomyopathy Genomic disorders and inherited multi-system disorders [IGMD 3] Cohort Studies Hospitals University Electrocardiography Heart Conduction System Internal medicine Humans Medicine Pediatrics Perinatology and Child Health cardiovascular diseases Child Netherlands medicine.diagnostic_test Endocrinology and reproduction [UMCN 5.2] business.industry Noonan Syndrome Infant Newborn Hypertrophic cardiomyopathy Infant Cardiomyopathy Hypertrophic medicine.disease United States Precordium Surgery Pulmonary Valve Stenosis Stenosis Mitochondrial medicine [IGMD 8] Phenotype medicine.anatomical_structure Child Preschool Pediatrics Perinatology and Child Health Cardiology Left axis deviation Noonan syndrome Female business |
| Zdroj: | European Journal of Pediatrics, 167, 1363-7 European Journal of Pediatrics, 167, 12, pp. 1363-7 |
| ISSN: | 1432-1076 0340-6199 |
| DOI: | 10.1007/s00431-008-0670-9 |
| Popis: | Contains fulltext : 69906.pdf (Publisher’s version ) (Open Access) Of all patients with Noonan syndrome, 50-90% have one or more congenital heart defects. The most frequent occurring are pulmonary stenosis (PS) and hypertrophic cardiomyopathy. The electrocardiogram (ECG) of a patient with Noonan syndrome often shows a characteristic pattern, with a left axis deviation, abnormal R/S ratio over the left precordium, and an abnormal Q wave. The objective of this study was to determine if these ECG characteristics are an independent feature of the Noonan syndrome or if they are related to the congenital heart defect. A cohort study was performed with 118 patients from two university hospitals in the United States and in The Netherlands. All patients were diagnosed with definite Noonan syndrome and had had an ECG and echocardiography. Sixty-nine patients (58%) had characteristic abnormalities of the ECG. In the patient group without a cardiac defect (n = 21), ten patients had a characteristic ECG abnormality. There was no statistical relationship between the presence of a characteristic ECG abnormality and the presence of a cardiac defect (p = 0.33). Patients with hypertrophic cardiomyopathy had more ECG abnormalities in total (p = 0.05), without correlation with a specific ECG abnormality. We conclude that the ECG features in patients with Noonan syndrome are characteristic for the syndrome and are not related to a specific cardiac defect. An ECG is very useful in the diagnosis of Noonan syndrome; every child with a Noonan phenotype should have an ECG and echocardiogram for evaluation. |
| Databáze: | OpenAIRE |
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