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Case Reports
. 2004 May;90(5):552-5.
doi: 10.1136/hrt.2003.010637.

Isolated left ventricular apical hypoplasia: a new congenital anomaly described with cardiac tomography

M Fernandez-Valls  1 , M B Srichai, A E Stillman, R D White
Affiliations
Case Reports

Isolated left ventricular apical hypoplasia: a new congenital anomaly described with cardiac tomography

M Fernandez-Valls et al. Heart. 2004 May.

Abstract

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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Figures

Figure 1
Figure 1
Cardiac tomography of isolated left ventricular (LV) apical hypoplasia. Corresponding LV outflow tract and four chamber views from cine bright blood magnetic resonance imaging (MRI) in cases 1–3 (first and second rows) and corresponding four chamber views from multidetector computed tomography (MDCT) in cases 1 and 3 (third row) all show a spherical, truncated LV with bulging of the interventricular septum towards the right ventricle (RV), invagination of fatty material (arrows) contiguous with epicardial fat into the defective LV apex, origin of a complex papillary muscle network in the flattened anterior apex, and elongation of the RV, which wraps around the deficient LV apical region.
Figure 2
Figure 2
Comprehensive evaluation of isolated LV apical hypoplasia by cardiac MRI. Anatomical evaluation with dark blood imaging (A) without and then (B) with fat saturation confirms the fatty nature of the material (large arrows) contiguous with epicardial fat (small arrow) and extending into the truncated LV apex. Dynamic evaluation with (C, D) dynamic bright blood and (E, F) tissue tagged imaging shows the spherical shape of the restrictive LV in late diastole and lack of systolic myocardial deformation due to impaired contractility, especially in the interventricular septum.
See this image and copyright information in PMC

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