title: Noncompaction of the Ventricular Myocardium and Polycystic Kidney Disease: A Case Report.
abstract: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary disorders, characterized by the formation of multiple cysts in the kidneys and other organs, as well as noncystic manifestations such as cerebral aneurysm. The most common cardiovascular disorders associated with ADPKD include valvular abnormalities and aortic aneurysm. An association between ADPKD and impaired left ventricular function has occasionally been reported. We describe a 74-year-old woman with ADPKD and exertional dyspnea. Impaired left ventricular function resulting from noncompaction of the ventricular myocardium (NVM) and secondary left ventricular aneurysm were diagnosed. Cardiac sarcoidosis and ischemic heart disease were ruled out. Myocardial ischemia resulting from NVM was the presumptive cause of the ventricular aneurysm. To our knowledge, this is the first report of concurrent isolated NVM and left ventricular aneurysm in a patient with ADPKD. ADPKD and various cardiomyopathies, including NVM, are all reported to involve mutations of sarcomere genes, suggesting a possible link between the conditions.
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan. Electronic address: fukinok@gmail.com.
authors.forename: Keiko
authors.initials: K
authors.lastname: Fukino
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Junpei
authors.initials: J
authors.lastname: Ishiwata
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Shinohara
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Oshima
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Tsunashi
authors.initials: T
authors.lastname: Kozaki
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Masayasu
authors.initials: M
authors.lastname: Ikutomi
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Amaki
authors.affiliation: Department of Cardiovascular Medicine, Teikyo University Chiba Medical Center, Ichihara, Chiba, Japan.
authors.forename: Fumitaka
authors.initials: F
authors.lastname: Nakamura
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000175:diagnosis / Q000209:etiology*; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications*
keywords: Autosomal dominant polycystic kidney disease (ADPKD); PKD2; cardiomyopathy; left ventricular aneurysm; microcirculation; non-compaction of the left ventricular myocardium (NVM); sarcomere genes