Objective: To describe cardiac tomography findings of an apparently new, presumably congenital, left ventricular (LV) abnormality noted consistently in three patients. Patients: Three patients presenting with non-specific symptoms including fatigue, shortness of breath, or chest discomfort were evaluated with cardiac tomography for cardiac structure and function. Results: Findings from the three patients were very similar: a truncated and spherical LV with abnormal diastolic and systolic function, invagination of fatty material into the myocardium of the defective LV apex, origin of a complex papillary network in the anteroapical LV, and an elongated right ventricle wrapping around the deficient apex. Conclusions: Isolated LV apical hypoplasia is a unique, presumably congenital, cardiac anomaly that is an important condition to recognise.

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A 62-year-old woman presented with breathlessness and dysphagia. Routine chest x-ray showed a widening in the upper mediastinum that suggested a thoracic aorta enlargement. No cardiomegaly was found (A). Transthoracic echocardiography revealed normal left ventricular systolic function with diastolic dysfunction and aortic sclerosis. Magnetic resonance angiography demonstrated a double aortic arch forming a vascular ring, thus compressing the esophagus (B to D). This is a rare congenital cardiovascular disorder (1%), usually diagnosed at an early age. It is possible to reach middle to late adulthood with this pathology, as shown by this patient, and it can produce severe symptoms by compressing the trachea and esophagus. AA ϭ ascending aorta; E ϭ esophagus; LCCA ϭ left common carotid artery; LSA ϭ left subclavian artery; PA ϭ pulmonary artery; RCCA ϭ right common carotid artery; RSA ϭ right subclavian artery; T ϭ trachea.

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