Cardiac findings in 31 patients with Noonan's syndrome.

作者信息

Bertola D R, Kim C A, Sugayama S M, Albano L M, Wagenführ J, Moysés R L, Gonzalez C H

机构信息

Unidade de Genética, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

出版信息

Arq Bras Cardiol. 2000 Nov;75(5):409-12. doi: 10.1590/s0066-782x2000001100005.

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 Doppler echocardiography.

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.