MOFFITT RENAL REPORT PEARLS 11/18/16: V-fib Arrest and Renal Failure!

Hi Everyone! Thanks to Rabih for presenting the case of a young man with sudden V-fib arrest and anuric renal failure. It was unclear whether pre-existing CKD might have contributed to his cardiac arrest or if all of the renal injury was a consequence of the arrest. Pearls below!

***************************************

Top pearls:

  • Think about sudden cardiac death in terms of structural and non-structural cardiac causes, as well as non-cardiac causes.
  • Structural: HOCM, DCM, valve disease, scar, congenital coronary abnormalities
  • Non-structural: Ischemia, channelopathies (QT, Brugada), myocarditis
  • Non-cardiac: PE, intracranial hemorrhage, medications/drugs

***************************************

For those who want more info:

See these pearls from earlier in the year about sudden cardiac death in a young patient and ARVD:

https://ucsfmed.wordpress.com/2016/06/07/sudden-cardiac-death-and-arvd/

 

The most common cause of renal injury after cardiac arrest is ATN due to renal hypoperfusion. In this case, there was a suggestion that the patient may have had pre-existing CKD.

We also discussed some congenital abnormalities that could lead to cardiac and renal problems:

  • LCAT deficiency
  • Fabry disease
  • Tuberous sclerosis

 

LCAT deficiency (autosomal recessive) leads to very low HDL, severe corneal opacities (“fish eye syndrome”), normocytic anemia and target cells on blood smear, as well as proteinuria and CKD. Premature CAD can result but is unusual.

Fabry disease (X-linked recessive) is the 2nd most common lysosomal storage disease (after Gaucher disease). Defect is in alpha-Gal A which results in accumulation of Gb3 within lysosomes in the vascular endothelium and smooth muscle, renal cells (glomerular, tubular, and interstitial), as well as cardiac muscle cells and conduction fibers (among others). Severe neuropathic pain and telangiectasias/angiokeratomas are commonly seen early in life. Cardiac, renal, cerebral, and dermatologic involvement is common in adulthood.

Tuberous sclerosis (autosomal dominant) is caused by mutations in TSC1 or TSC2, usually characterized by benign tumors in multiple organs and skin manifestations. Most have epilepsy and cognitive deficits. Benign cardiac rhabdomyomas and renal angiomyolipomas are common. CKD in the absence of renal tumors is possible, even progressing to ESRD.

 

Evernote: https://www.evernote.com/shard/s272/sh/2ee327ab-94cf-499c-892e-a61630f32352/948bbcb4ab39664c94177dade7977fbf

Have a great day everyone!

SamMy

 

Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s