Korean J Radiol.  2016 Feb;17(1):79-82. 10.3348/kjr.2016.17.1.79.

Three-Dimensional Endo-Cardiovascular Volume-Rendered Cine Computed Tomography of Isolated Left Ventricular Apical Hypoplasia: A Case Report and Literature Review

• Hong SH
• Kim YM
• Lee HJ

• KMID: 2351166
• DOI: http://doi.org/10.3348/kjr.2016.17.1.79

Abstract

We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy.

Keyword

Isolated left ventricular apical hypoplasia; Multidetector computed tomography; Magnetic resonance imaging; Cardiomyopathies; Congenital heart disease; Three-dimensional rendering

MeSH Terms

Adult
Cardiomyopathy, Dilated/*radiography
Female
Heart Ventricles/*radiography
Humans
Hypoplastic Left Heart Syndrome/*radiography
Imaging, Three-Dimensional/*methods
Multidetector Computed Tomography/*methods
Myocardium/pathology

Figure

• 

  Fig. 1 Isolated left ventricular apical hypoplasia in 34-year-old woman. A. Echocardiogram demonstrates spherical and truncated LV with echo-bright apex (arrow), bulging of interventricular septum towards right ventricle, and elongated RV wrapping around deficient LV apex in parasternal long-axis view. B. Four-chamber view from CMR during diastole and systole shows spherical, truncated LV at apical level with prominent fat (arrows). Papillary muscles originate from flattened anterior apex. RV is elongated and wraps around deficient LV apex. Late gadolinium enhancement revealed no enhancement in apex and ventricular septum of LV. C. Four-chamber view from cardiac MDCT more clearly visualizes extent of fatty material within apical myocardium, interventricular septum and inferior LV wall. Almost all fatty material was confined within myocardium, although small portion of apical fat was contiguous with epicardial fat (arrow). MDCT clearly shows cardiac layers and fat distribution. Fatty tissue is confined within thin myocardial layers of apex and interventricular septum. Note pericardium (arrowheads) and epicardial fat. CMR = cardiac magnetic resonance, LA = left atrium, LV = left ventricle, MDCT = multidetector computed tomography, RV = right ventricle, VR = volume-rendering

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