Significant obstruction of the right and left ventricular outflow tract in a patient with biventricular hypertrophic cardiomyopathy

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Abstract

Echocardiography demonstrated pronounced asymmetric left ventricular (LV) hypertrophy and thickened right ventricular muscular components in a 54-year-old woman with a history of dyspnoea (NYHA III), and recurrent syncopes. Left ventricular outflow peak gradient was 80 mmHg at rest and 125 mmHg during Valsalva manoeuvre. Cardiac cine and gadolinium-enhanced T1 weighted magnetic resonance imaging (MRI) provided striking images of a right ventricular outflow tract obstruction and a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis. Right and left heart catherization, with simultaneous measurement of aortic and LV pressures revealed a 150 mmHg subaortic gradient and a 130 mmHg subpulmonic gradient at rest. Impediment to right ventricular (RV) outflow was due to massive hypertrophy of the crista supraventricularis with an 'hour-glass' deformity. A surgical intervention with LV septal myotomy-myectomy and RV ventriculotomy was performed successfully. Hypertrophic obstructive cardiomyopathy with significant RV and LV outflow tract obstruction is a very rare finding.Echocardiography and MRI can be used in combination for non-invasive evaluation of morphological and haemodynamic information because mechanisms of obstruction are different in each ventricle. © The Author 2008.

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Butz, T., Horstkotte, D., Langer, C., Esdorn, H., Kleikamp, G., Körfer, R., & Faber, L. (2008). Significant obstruction of the right and left ventricular outflow tract in a patient with biventricular hypertrophic cardiomyopathy. European Journal of Echocardiography, 9(2), 344–345. https://doi.org/10.1093/ejechocard/jen018

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