Noncompaction of the ventricular myocardium combined with polycystic kidney disease.
Noncompaction of the ventricular myocardium (NVM) is a rare cardiac abnormality of unknown etiology. The condition is characterized by prominent and excessive trabeculations in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Polycystic kidney diseases are characterized by the formation of multiple cysts in the kidneys and liver and, less frequently, in the pancreas. Cardiovascular abnormalities including hypertension, mitral valve prolapse, and intracranial aneurysms are also frequently recognized. However, polycystic kidney disease and isolated ventricular noncompaction have not previously been correlated. Here, we describe one case of isolated noncompaction of ventricular myocardium with polycystic kidney disease, coupled with a progressive worsening of heart failure. We confirmed these abnormalities using contrast echocardiography, abdominopelvic computed tomography, and cardiac magnetic resonance imaging.
Moon
Jae-Youn
JY
Cardiology Division, Yonsei Cardiovascular Hospital, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemun-ku, Seoul, 120-752, South Korea.
Chung
Namsik
N
Seo
Hye-Sun
HS
Choi
Eui-Young
EY
Ha
Jong-Won
JW
Rim
Se-Joong
SJ
D006332:Cardiomegaly / Q000000981:diagnostic imaging; D009202:Cardiomyopathies / Q000453:epidemiology* / Q000473:pathology*; D015897:Comorbidity; D003560:Cysts / Q000000981:diagnostic imaging / Q000453:epidemiology; D005260:Female; D006325:Heart Atria / Q000000981:diagnostic imaging / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D007690:Polycystic Kidney Diseases / Q000453:epidemiology* / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography