Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012:2012:978170.
doi: 10.1155/2012/978170. Epub 2012 Nov 22.

An unusual cardiac manifestation in autosomal dominant polycystic kidney disease

Affiliations

An unusual cardiac manifestation in autosomal dominant polycystic kidney disease

Fausta Catapano et al. Case Rep Nephrol. 2012.

Abstract

Autosomal dominant polycystic kidney disease is a common hereditary disorder characterized by renal and extrarenal, cystic and noncystic manifestations. Connective tissue defects, including cerebral aneurysm, meningeal diverticula, abdominal wall hernias, intestinal diverticula, and cardiac valvular abnormalities, are widely known manifestations. Instead intracardiac aneurysms have never been reported in adults with autosomal dominant polycystic kidney disease. We describe a 65-year-old patient with end-stage renal disease due to autosomal dominant polycystic kidney disease and an atrial septum aneurysm associated with platypnoea-orthodeoxia syndrome.

PubMed Disclaimer

Figures

Figure 1
Figure 1
(a) Chest X-ray showing the elevation of the right diaphragm; (b) abdomen multislices CT-scan view showing the hepatic and kidney cysts.
Figure 2
Figure 2
Transesophageal echocardiogram demonstrating: (a) the aneurysm of the atrial septum (white arrow) and (b) the size of patent foramen ovale (0.8 cm) with right-to-left shunting (black arrow) after agitated saline contrast injection. RA, right atrium; LA, left atrium.

References

    1. Ecder T, Fick-Brosnahan GM, Schrier RW. Polycystic kidney disease. In: Schrier RW, editor. Diseases of the Kidney and Urinary Tract. 8th edition. Philadelphia, Pa, USA: Lippincott Williams & Wilkins; 2007. pp. 502–539.
    1. Chapman AB, Schrier RW. Pathogenesis of hypertension in autosomal dominant polycystic kidney disease. Seminars in Nephrology. 1991;11(6):653–660. - PubMed
    1. Ecder T, Schrier RW. Hypertension in autosomal-dominant polycystic kidney disease: early occurrence and unique aspects. Journal of the American Society of Nephrology. 2001;12(1):194–200. - PubMed
    1. Chapman AB, Johnson AM, Rainguet S, Hossack K, Gabow P, Schrier RW. Left ventricular hypertrophy in autosomal dominant polycystic kidney disease. Journal of the American Society of Nephrology. 1997;8(8):1292–1297. - PubMed
    1. Angelini P, Wilansky S, Gaos C, Montazavi A, Boncompagni E, Cooley DA. Prolapsing large aneurysm of the atrial septum simulating a right atrial mass. Catheterization and Cardiovascular Diagnosis. 1992;26(2):122–126. - PubMed

LinkOut - more resources