MOFFITT CARDIOLOGY REPORT PEARLS 5/2/17: Kawasaki Disease and Coronary Aneurysms!

Hey Everyone! Thanks to Jin and Alayn for presenting the case of a young man with childhood Kawasaki disease coming in with progressive chest pain and dyspnea, and found to have a coronary aneurysm! He will undergo a cardiac stress test to figure out whether the symptoms are related to the aneurysm. Pearls below!

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Top Pearls:

  1. Kawasaki disease is usually self-limited, but cardiac complications are common.
  2. Coronary aneurysms are the most common complication.
  3. Treat early with IVIG and aspirin to prevent these complications.

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For those who want more info:

Kawasaki disease (KD) is rare in adults and is typically self-limited with an acute course of (average) 12 days. There DOES seem to be a genetic component to Kawasaki disease (increased frequency among family members of an index case and increased frequency in Asian and Asian-American patients).

*Cardiovascular complications include:

  • Coronary aneurysms (this is the major one, see below for more info!)
  • Heart failure
  • MI
  • Arrhythmias
  • PAD

*Diagnostic criteria: Fever lasting at least 5 days without other explanation, AND at least 4 of:

  • Bilateral conjunctivitis [>75%]
  • Oral mucous membrane changes (fissures, injection, strawberry tongue) [90%]
  • Cervical lymphadenopathy [25-70%]
  • Peripheral erythema or edema (palms/soles) [50-85%]
  • Polymorphous rash [70-90%]

*Treatment: IVIG (single dose), ASA during acute phase until inflammatory markers resolve. ASA is continued if aneurysms persist.

*Coronary artery aneurysms:

  • 30-40% of patients with acute Kawasaki disease!

 

  • 10-20% persist beyond one month (highest mortality is 15-45 days after disease onset)
  • May cause late ischemic heart disease
  • ECG and TTE for all patients with KD (CTA or MRA if TTE cannot image arteries)
  • Aneurysms most commonly found in proximal LAD, often at arterial branch points
  • “Giant” aneurysms >8 mm have high risk of morbidity/mortality (up to 50%), often anticoagulated in addition to aspirin
  • Who gets PCI? Ischemic symptoms + reversible ischemia on stress testing + >75% stenosis. CABG recommended if severe LV dysfunction or stenosis not amenable to PCI.

IN SUMMARY: If someone has suspected KD, treat early with IVIG and aspirin to prevent cardiac complications. Do ECG and TTE (and possibly CTA/MRA) in the acute phase looking for coronary aneurysms.

*Pearl: KD is not the only disease associated with coronary aneurysms. Other associations include PAN, Ehlers-Danlos, Loeys-Dietz, and other familial syndromes.

 

Evernote: https://www.evernote.com/shard/s272/sh/30ae8e3e-2d24-4074-b914-615906dac5ed/05ae900b26ab74cdec5d1c9a7d9a6bea

 

Have a great day everyone!

SamMy

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