Long-term outcomes

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Coronary Artery Abnormalities - The Artery Attack

  • The most critical long-term complication of Kawasaki Disease, affecting 15-25% of untreated children.
  • Timely IVIG therapy dramatically reduces the risk to <5%.
  • Pathology involves necrotizing arteritis, destroying the tunica media and internal elastic lamina, leading to aneurysm formation.

Coronary artery aneurysms: types and complications

  • AHA Classification (by Z-score):
    • No involvement: Z-score < 2
    • Dilation only: Z-score 2 to < 2.5
    • Small aneurysm: Z-score ≥ 2.5 to < 5
    • Medium aneurysm: Z-score ≥ 5 to < 10
    • Giant aneurysm: Z-score ≥ 10 or absolute dimension > 8 mm

Giant aneurysms (>8 mm) have the worst prognosis, with a ~50% risk of thrombosis or stenosis, potentially leading to myocardial infarction.

  • Major Long-Term Risks:
    • Stenosis and occlusion from progressive myointimal proliferation.
    • Thrombosis within the aneurysm.
    • Myocardial infarction & sudden death.

Long-Term Management - The Lifelong Lookout

  • Goal: Prevent thrombosis & stenosis in affected coronary arteries.

  • Risk Stratification is key, based on echocardiography findings during the subacute phase.

  • Pharmacotherapy:

    • Low-Dose Aspirin: 3-5 mg/kg/day.
    • Dual Antiplatelet Therapy (DAPT): Aspirin + Clopidogrel, often post-stenting.
    • Anticoagulation: Warfarin (INR 2.0-2.5) or LMWH for giant aneurysms.

Exam Favourite: The highest risk of myocardial infarction is within the first year after onset, particularly in patients with giant aneurysms.

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Adverse Events & Prognosis - The Danger Zone

  • Primary Sequela: Coronary Artery Aneurysms (CAA).
    • Can lead to thrombosis, stenosis, & myocardial infarction (MI).
  • High-Risk Groups for CAA:
    • Treatment delay >10 days.
    • Age <1 year.
    • Incomplete/atypical presentation.
  • Prognosis by Aneurysm Size:
    • Small (<5 mm): High regression rate.
    • Medium (5-8 mm): Variable regression.
    • ⚠️ Giant (>8 mm): Lowest regression; ~50% risk of stenosis/thrombosis.

⭐ The most common cause of death in long-term follow-up is ischemic heart disease/MI from a thrombosed aneurysm.

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High‑Yield Points - ⚡ Biggest Takeaways

  • Coronary artery aneurysms (CAA) are the most feared long-term complication.
  • Risk of CAA is highest in infants <6 months and with delayed IVIG treatment (>10 days).
  • Giant aneurysms (>8 mm) carry the worst prognosis, with a high risk of thrombosis or stenosis.
  • Lifelong follow-up with echocardiography is mandatory for patients with coronary involvement.
  • Patients with giant aneurysms often require lifelong antiplatelet and/or anticoagulant therapy.
  • Increased lifetime risk of premature atherosclerotic cardiovascular disease.

Practice Questions: Long-term outcomes

Test your understanding with these related questions

A 24-year-old man presents with a complaint of breathlessness while jogging. He says that he recently started marathon training. He does not have any family history of asthma nor has any allergies. He currently takes no medication. The blood pressure is 120/80 mm Hg, and the heart rate is 67/min. With each heartbeat, he experiences pounding in his chest, and his head bobs. On physical examination, he has long fingers, funnel chest, and disproportionate body proportions with a decreased upper-to-lower segment ratio. On auscultation over the 2nd right intercostal space, an early diastolic murmur is heard, and 3rd and 4th heart sounds are heard. Echocardiography shows aortic root dilatation. The patient is scheduled for surgery. Which of the following is associated with this patient's condition?

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Flashcards: Long-term outcomes

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What vasculitis is treated with aspirin? _____

TAP TO REVEAL ANSWER

What vasculitis is treated with aspirin? _____

Kawasaki disease

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