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A 68 year old woman was referred to our cardiology department for a preoperative evaluation after the finding of a systolic ejection murmur and an abnormal ECG. She had a history of diabetes mellitus, hypertension, and hypercholesterolaemia. She denied any history of chest pain, palpitations, or dyspnoea on exertion. Except for a raised blood pressure of 165/70 mm Hg and a systolic murmur with the punctum maximum at the second right intercostal space, physical examination revealed no further abnormalities. The ECG showed a sinus rhythm with T wave inversion in the inferior and anterolateral leads with giant negative T waves in leads V3–V6 (panel A). The echocardiogram raised the suspicion of a hypertrophic cardiomyopathy (HCM); however, this could not be demonstrated with fundamental echocardiography (panel B). Transthoracic contrast enhanced echocardiography demonstrated the diagnosis of apical HCM, showing a typical spade-like lumen of the left ventricle (panel C). Magnetic resonance imaging confirmed the myocardial thickening of the apex of the left ventricle (panel D).
Although more common in Japan, apical HCM is a relatively rare form of hypertrophic cardiomyopathy in western countries. Giant negative T waves on the ECG in asymptomatic patients should raise the suspicion of apical HCM. As contrast enhanced echocardiography improves visualisation of the myocardial wall, administration of an ultrasound contrast agent in patients with these typical ECG abnormalities is recommended, especially in patients with suboptimal and/or non-diagnostic windows.
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