Korean J Radiol.  2013 Dec;14(6):874-877. 10.3348/kjr.2013.14.6.874.

Isolated Left Ventricular Apical Hypoplasia with Infundibular Pulmonary and Aortic Stenosis: a Rare Combination

• Moon JI
• Jeong YJ
• Lee G
• Choi JH
• Lee JW

• KMID: 1711452
• DOI: http://doi.org/10.3348/kjr.2013.14.6.874

Abstract

Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.

Keyword

Isolated left ventricular apical hypoplasia; Pulmonary subvascular stenosis; Aortic valve stenosis; Magnetic resonance imaging; Congenital heart disease

MeSH Terms

Adult
Aortic Valve Stenosis/*complications/diagnosis
Diagnosis, Differential
Echocardiography
Electrocardiography
Humans
Hypoplastic Left Heart Syndrome/*complications/diagnosis
Magnetic Resonance Imaging, Cine
Male
Pulmonary Valve Stenosis/*complications/diagnosis

Figure

• 

  Fig. 1 Thirty-three-old male was diagnosed with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. Echocardiographic images of patient (A-C). A. Apical four-chamber view demonstrates spherically truncated LV with elongated RV wrapped around deficient LV apex. B. Three-dimensional midesophageal transesophageal echocardiographic images of aortic stenosis. Parasternal shortaxis and three-chamber views show aortic calcification with moderate to severe stenosis. At rest, mean pressure gradient was 44 mm Hg and peak velocity was 4.2 m/sec. Calculated aortic orifice is 1.1 cm2. C. Parasternal short-axis view taken during diastole, showing pulmonary valve (white arrowhead), demonstrates thickened muscular infundibulum (black arrowhead). LV = left ventricle, RV = right ventricle. Cardiac MR findings of patient (D-H). D. Four-chamber cardiac MR image taken during diastole shows truncated, spherical LV with rightward bulging of interventricular septum and elongation of RV. Papillary muscles originate from flattened anterior apex (arrowheads). E. Transverse HASTE dark-blood MR image outlines fat within myocardium at LV apex (arrowhead). F. Transverse HASTE dark-blood MR image with fat saturation shows loss of high-signal intensity of deficient LV apex consistent with fatty infiltration, which is characteristic of dysplasia affecting this area (arrowhead). G. Transverse HASTE dark-blood MR image taken during end-systole shows infundibular pulmonary stenosis (arrowhead). H. Static image from three-chamber cardiac MRI examination performed with true fast imaging with steady-state precession shows systolic jet flow, suggestive of aortic stenosis (arrowhead). LV = left ventricle, RV = right ventricle, HASTE = half-fourrer-acquisition single-short turbo spin-echo

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