Worsening kidney function (WKF) is common in patients with acute heart failure (AHF) syndromes. Although WKF has traditionally been associated with worse outcomes on a population level, serum creatinine concentrations vary greatly during episodes of worsening heart failure, with substantial individual heterogeneity in terms of their clinical meaning. Consequently, interpreting such changes within the appropriate clinical context is essential to unravel the pathophysiology of kidney function changes and appropriately interpret their clinical meaning. This article aims to provide a critical overview of WKF in AHF aiming to provide physicians with some tips and tricks to appropiately interpret kidney function changes in the context of AHF.
Fabry disease is a rare X-linked inherited lysosomal storage disorder caused by
the absence or reduction of alfa-galactosidase A activity in lysosomes, resulting
in accumulation of glycosphingolipids in various tissues. The main organ affected
is the heart, which frequently manifests as left ventricular hypertrophy and can
ultimately lead to cardiac fibrosis, heart failure, valve disease, cardiac
conduction abnormalities and sudden cardiac death. Today we know that myocyte
damage starts before these signs and symptoms are detectable on routine studies,
during the designated pre-clinical phase of Fabry disease. The initiation of
specific therapy for Fabry disease during the early stages of the disease has a
great impact on the prognosis of these patients avoiding progression to
irreversible fibrosis and preventing cardiovascular complications. Cardiac
imaging has become an essential tool in the management of Fabry disease as it can
help physicians suspect the disorder, diagnose patients in the early stages and
improve outcomes. The recent development of novel imaging techniques makes
necessary an update on the subject. This review discusses the role of multimodal
imaging in the diagnosis, staging, patient selection for treatment and prognosis
of Fabry disease and discusses recent advances in imaging techniques that provide
new insights into the pathogenesis of the disorder and the possibility of novel
treatment targets.
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