[Apical hypertrophic myocardiopathy with mid-ventricular obstruction and apical necrosis]

In a 41-year old man hospitalized for investigation of a systolic ejection murmur, echocardiography revealed an apical (17 mm) and, chiefly, a mid ventricular (38 mm) myocardial hypertrophy resulting in a true stricture at that level. During systole the mid ventricular area became obstructed, dividing the left ventricule into an apical akinetic chamber and a proximal hyperkinetic chamber. Left ventriculography confirmed this abnormality and catheterization showed a left intraventricular pressure gradient of 60 mmHg. Although left coronary arteriography proved normal, myocardial radionuclide scanning at exercise displayed apical necrosis which resulted in a peculiar haemodynamic behaviour well demonstrated by doppler ultrasound. The apex of the left ventricle was filled in two stages: first, during atrial contraction, giving at doppler (mid ventricular sample) a positive flow with a speed of 1m/sec during 120 ms; then, during the isovolumetric period, giving a positive flow with a speed of 1m/sec during 60 ms. The apex drained during systole and protodiastole, giving a negative mid ventricular obstruction flow with a speed of 4 m/sec with protosystolic and mesodiastolic reinforcement.