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

다국어 초록 (Multilingual 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.

참고문헌 (Reference)

1 Tonkin IL, "The definition of cardiac malpositions with echocardiography and computed tomography" 157-87, 1984

2 Richardson P, "Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies" 93 : 841-842, 1996

3 Dusek J, "Postnatal persistence of spongy myocardium with embryonic blood supply" 99 : 312-7, 1975

4 Peoples WM, "Polysplenia:a review of 146 cases" 4 : 129-37, 1983

5 Ichida F, "Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome" 103 : 1256-1263, 2001

6 Moon JY, "Noncompaction of the ventricular myocardium combined with polycystic kidney disease" 21 : 195-198, 2006

7 Oechslin EN, "Long-term follow-up of 34 adults with isolated left ventricular noncompaction:a distinct cardiomyopathy with poor prognosis" 36 : 493-500, 2000

8 Stllberger C, "Left ventricular hypertrabeculation/noncompaction" 17 : 91-100, 2004

9 Chin TK, "Isolated noncompaction of left ventricular myocardium" 82 : 507-13, 1990

10 Moon JC, "Images in cardiovascular medicine. Myocardial fibrosis in glycogen storage disease type III" 107 : e47-, 2003

11 Jenni R, "Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction:a step towards classification as a distinct cardiomyopathy" 86 : 666-671, 2001

12 Sedmera D, "Developmental patterning of the myocardium" 258 : 319-337, 2000

13 Korcyk D, "Contrast-enhanced cardiac magnetic resonance in a patient with familial isolated ventricular non-compaction" 6 : 569-576, 2004

14 Bellet S, "Congenital heart disease with multiple cardiac anomalies:report of a case showing aortic atresia,fibrous scar in myocardium and embryonal sinusoidal remains" 183 : 458-65, 1932

15 Feldt RH, "Anomalous ventricular myocardial patterns in a child with complex congenital heart disease" 23 : 732-4, 1969