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A 45 year old man was admitted because of dyspnoea on effort and palpitations. He had no familial history of heart disease. An ECG showed left ventricular hypertrophy. Radiographs of the chest revealed mild cardiomegaly. Short axis transthoracic echocardiography indicated the existence of two orifices in the mitral valve. With the apical four chamber view, double left ventricular inflow jets were obtained by colour Doppler at the diastolic phase. Colour Doppler echocardiography also showed mild mitral regurgitation from both orifices. To define double orifice mitral valve and evaluate the subvalvar apparatus in detail, a transoesophageal echocardiograph was performed. Double orifices were clearly visible from the ring of the valve, and each orifice had its own subvalvar apparatus separately at the free part of each leaflet. An abnormality of the left ventricle was also detected. The left ventricle was dilated and diffusely hypokinetic (end diastolic/end systolic dimension 58/48 mm, ejection fraction 30%). The left ventricular wall was thickened, especially at the apex, and appeared sponge-like. There were numerous, excessively prominent trabeculations associated with deep intertrabecular recesses. The contrast entered into the intertrabecular recesses. We diagnosed non-compaction of left ventricular myocardium. Treatment with an angiotensin converting enzyme inhibitor and diuretics proved effective. After the patient's symptoms disappeared, β blocker treatment with metoprolol was initiated; six months later, the patient's left ventricular dimension was reduced and left ventricular wall motion was improved (end diastolic/end systolic dimension 53/32 mm, ejection fraction 52%).