Yonsei Med J.  2007 Dec;48(6):1052-1055. 10.3349/ymj.2007.48.6.1052.

A Case of Noncompaction of the Ventricular Myocardium Combined with Situs Ambiguous with Polysplenia

• Cho YH
• Jin SJ
• Je HC
• Yoon YW
• Hong BK
• Kwon HM
• Kim TH
• Rim SJ

• KMID: 1786225
• DOI: http://doi.org/10.3349/ymj.2007.48.6.1052

Abstract

A 33-year-old man was admitted to our hospital with chest pain and exertional dyspnea. Two-dimensional echocardiography showed prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Thoracoabdominal CT and cardiac magnetic resonance imaging (CMR) revealed situs ambiguous with polysplenia and noncompaction of the left ventricular myocardium. CMR also demonstrated delayed enhancement of the trabeculations located at the apical portion of the left ventricle. The coronary angiogram was normal. This is the first case of noncompaction of the ventricular myocardium associated with situs ambiguous with polysplenia.

Keyword

Noncompaction of the ventricular myocardium (NVM); situs ambiguous with polysplenia

MeSH Terms

Abnormalities, Multiple/*pathology
Adult
Echocardiography
Heart Ventricles/abnormalities
Humans
Magnetic Resonance Imaging
Male
Myocardium/*pathology
Spleen/*abnormalities
Syndrome
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Echocardiography revealing prominent trabeculations and deep intertrabecular recesses. The noncompacted areas mainly involve the anterior and inferolateral left ventricular segments.

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  Fig. 2 Chest CT showing the two-layered structure of a thin, compacted epicardial layer and an extremely thick endocardial layer with prominent trabeculations and deep recesses.

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  Fig. 3 Chest CT showing bilateral, bilobed lungs, where the main bronchi is seen below the pulmonary artery. Minor fissures are absent, and double superior vena cava and left superior vena cava connect to the coronary sinus.

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  Fig. 4 Abdominal CT showing right-sided stomach and multiple spleens in the right abdomen, persistent accessory azygous drainage to the intrahepatic vein and IVC segment, and interruption of the inferior vena cava.

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  Fig. 5 Delayed enhancement CMR showing the two-layered structure of NVM and hyperenhancement of the trabeculations located at the apical portion of the left ventricle, suggesting areas of fibrosis.

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