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Three female patients with Danon disease presenting with predominant cardiac phenotype: a case series.


ABSTRACT:

Background

Danon disease is a rare X-linked multisystemic disorder that has primarily been described in male patients.

Case summary

We present three female patients with Danon disease with a predominantly cardiac phenotype in whom disease onset and expression was very different from that of male patients. Case 1 was first admitted for acute heart failure and then readmitted a few months later for cardiac shock, necessitating mechanical support, and heart transplantation. Case 2 had complex arrhythmias for which many antiarrhythmic drugs were tried with only limited success. Her disease accelerated after her first pregnancy, and she showed reduced left ventricular function and dilated cardiomyopathy. Case 3 was referred for near syncope and ablated for an accessory pathway; s

SUBMITTER: Mulder BA 

PROVIDER: S-EPMC6764575 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature