Cardiac findings in 31 patients with Noonan's syndrome

AUTOR(ES)

Bertola, Débora Romeo, Chong, Ae Kim, Sugayama, Sofia M. M., Albano, Lilian Maria José, Wagenführ, Jaqueline, Moysés, Regina Lúcia, Gonzalez, Claudette Hajaj

FONTE

Arquivos Brasileiros de Cardiologia

DATA DE PUBLICAÇÃO

2000-11

RESUMO

OBJECTIVE: To evaluate cardiac findings in 31 Noonan syndrome patients. METHODS: Thirty-one (18 males and 13 females)patients from 26 families affected with Noonan's syndrome were evaluated from the cardiac point of view with electrocardiography and echodopplercardiography. RESULTS: Twenty patients had some type of cardiac abnormality. The most frequent was pulmonary valve stenosis followed by hypertrophic myocardiopathy, commonly associated with valve defects. Upper deviation of the QRS axis was observed in 80% of these patients. CONCLUSION: In view of the high frequency and diversity of cardiac abnormalities present in Noonan syndrome, cardiac evaluation with electrocardiography and echocardiography should be performed in all patients diagnostically suspected of having this disease.

Documentos Relacionados

• Cardiovascular malformations in Turner's and Noonan's syndrome.
• Cardiomyopathy in Noonan's syndrome. Report of 3 cases.
• A case of Noonan's syndrome and hypoparathyroidism presenting with epilepsy
• Chiari (type 1) malformation and syringomyelia in a patient with Noonan's syndrome.
• Noonan's syndrome with hydrocephalus, hindbrain herniation, and upper cervical intracord cyst.