AN UNUSUAL ASPECT OF HYPERTROPHIC

CARDIOMYOPATHYN. Mouine, F. Oussibla, R . Amri, M. Cherti

Cardiology B departmentIBN SINA Rabat,

MOROCCO

INTRODUCTION� Left ventricular hypertrophic cardiomyopathy is a

rare congenital cardiomyopathy that affects both children and adults.

� Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular hypertrophy and establish prognostic factors

CASE REPORT�We report a 13 year’s old girl with Noonan syndrome,

admitted for effort dyspnea.�Clinical examination showed ejectional systolic murmur in

aortic area, electrocardiogram showed an axe deviated in lead aVR. �Transthoracic echocardiography showed atypical aspect of

hypertrophic cardiomyopathy with a large spiral recessus of infero septal and inferior wall of left ventricle with diastolic dysfunction, due to both abnormal relaxation and reduced compliance.�She was previewed for septal myectomy but she died few

days after diagnosis.

A spiral aspect of infero septal wall

A spiral aspect of infero septal wall

DISCUSSION [1]�Hypertrophic cardiomyopathy is a heart disease instigated

by a variety of causes, such as genetic syndromes, e.g., Noonan syndrome and Costello syndrome, metabolic diseases, such as long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and adenosine triphosphate (ATP) synthase deficiency, familial history, maternal diabetes mellitus, steroid use and etc. These can also be caused by a variety of reasons; heart and other organs are often accompanied by other malformations .� According to a study performed by Oh et al incidences of

primary hypertrophic cardiomyopathy are very rare.

DISCUSSION [2]�Here, we describe a hypertrophic cardiomyopathy with

Noonan syndrome. Compared to children with hypertrophic cardiomyopathy, those with left ventricular hypertrophy in the setting of Noonan syndromes show more ventricular hypertrophy and diastolic dysfunction, due to both abnormal relaxation and reduced compliance, in our case, it was atypical aspect of hypertrophy with a large spiral recessus. They also exhibit an increased prevalence of obstruction of the left ventricular outflow tract. �We considered performing a septal myectomy or

transplantation, despite the small chance of success. However, our patient was died few days after diagnosis.

CONLUSION

These morphological and functional findings could explain the different symptoms and clinical events, and potentially define the more appropriate therapeutic options in children with left ventricular hypertrophy of different etiology.