No abstract

Over an average period of 1 year, almost half of patients showed signs of clinical deterioration despite specific vasodilator therapy. Parameters of right ventricular morphology and function had prognostic value in these patients.

Although imaging methods to investigate Ebstein's malformation have continuously evolved, standardization of assessment protocols by 3D echocardiographic imaging, speckle tracking imaging, and cardiac magnetic resonance imaging is required.

Patients with ES have a more hypertrophied RV free wall, better RV performance as assessed by RVFAC and RV free wall strain and increased COi compared to other types of PH. Furthermore, RV performance appears to be less dependent on the level of pressure overload. These findings could contribute to the better survival profile of patients with ES.

Subaortic stenosis caused by an accessory mitral valve is an exceedingly rare finding. We report the case of an asymptomatic 14-year-old patient, in whom transthoracic echocardiography revealed an accessory mitral valve in the left ventricular outflow tract, producing mild subaortic stenosis. Except for an aneurysm of the interventricular septum, with no shunt, there were no other anomalies. Transesophageal echocardiography provided details about the morphology and location of the accessory valve. Being asymptomatic and having only a mild gradient, antibiotic prophylaxis for infective endocarditis and follow-up were recommended. After 2 years the patient is asymptomatic, with a similar echocardiographic gradient.