Coronary artery aneurysms

Coronary artery aneurysms

Coronary artery aneurysms

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Kawasaki Disease - The Fiery Vasculitis

  • Acute febrile illness causing medium-vessel vasculitis, primarily in children < 5 years.
  • Diagnosis: Fever for ≥ 5 days plus ≥ 4 of 5 clinical criteria.
  • 📌 Mnemonic: CRASH & Burn
    • Conjunctivitis (bilateral, non-exudative)
    • Rash (polymorphous)
    • Adenopathy (cervical, >1.5 cm)
    • Strawberry tongue, lip fissures
    • Hand/foot erythema & edema
  • Most serious complication: Coronary artery aneurysms.
  • Treatment: IV Immunoglobulin (IVIG) + high-dose Aspirin.

⭐ IVIG (2 g/kg) given within 10 days of fever onset significantly reduces the risk of coronary artery aneurysms.

Echocardiogram: Left Main Coronary Artery Aneurysm

Diagnosis - Cracking the CRASH Code

  • Primary Criterion: Fever ≥ 5 days (high-spiking, unresponsive to antibiotics).
  • Principal Clinical Criteria (≥ 4 of 5): 📌 CRASH Mnemonic
    • Conjunctivitis: Bilateral, non-exudative, limbic-sparing.
    • Rash: Polymorphous (maculopapular, morbilliform), non-vesicular. Perineal erythema & desquamation is characteristic.
    • Adenopathy: Cervical, usually unilateral, >1.5 cm.
    • Strawberry Tongue & Lip Changes: Erythema, fissuring of lips, oropharyngeal erythema.
    • Hand & Foot Changes: Acute erythema/edema; subacute periungual desquamation.

Kawasaki Disease: CRASH & BURN Mnemonic and Diagnostic Flow

⭐ In suspected cases not meeting full criteria (Incomplete Kawasaki), persistent unexplained fever with ↑CRP/ESR warrants echocardiography to rule out coronary artery changes.

Coronary Aneurysms - The Heart of the Matter

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  • Most feared complication, leading to thrombosis, stenosis, & myocardial infarction (MI).
  • Risk ↑ with treatment delay >10 days, male sex, age <1 yr.
  • Incidence: 15-25% (untreated) vs. <5% (treated with IVIG).
  • Diagnosis & monitoring via Echocardiography at baseline, 1-2 weeks, and 4-6 weeks after treatment.
  • Classified by z-scores (internal diameter):
    • Small: z ≥2.5 to <5
    • Medium: z ≥5 to <10
    • Giant: z ≥10 or >8 mm diameter.

⭐ Giant aneurysms have the highest risk of thrombosis/stenosis (~50%) and mandate long-term anticoagulation.

Management & Follow-up - Dousing the Flames

  • Primary Goal: Prevent Coronary Artery Aneurysms (CAA).
  • Acute Phase (within 10 days):
    • IVIG: Single dose of 2 g/kg over 10-12 hrs.
    • Aspirin: High dose (80-100 mg/kg/day) until afebrile for 48-72 hrs.
  • Convalescent Phase:
    • Low-dose Aspirin (3-5 mg/kg/day) for 6-8 weeks if no CAA. Lifelong if CAA present.

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⭐ In Kawasaki disease, high-dose aspirin is a cornerstone of initial therapy. Its anti-inflammatory effect is prioritized over the risk of Reye's syndrome, especially in febrile children.

High-Yield Points - ⚡ Biggest Takeaways

  • Kawasaki Disease is the leading cause of acquired heart disease in children in most developed nations.
  • The most feared complication is coronary artery aneurysms (CAA), seen in 15-25% of untreated children.
  • Risk is highest in infants <1 year, those with incomplete presentation, and with delayed IVIG treatment.
  • Echocardiography is the gold standard for diagnosis and serial monitoring of coronary arteries.
  • Giant aneurysms (>8 mm or Z-score ≥10) carry the highest risk of thrombosis and stenosis.

Practice Questions: Coronary artery aneurysms

Test your understanding with these related questions

A 6-year-old boy presents to his pediatrician accompanied by his mother for evaluation of a rash. The rash appeared a little over a week ago, and since that time the boy has felt tired. He is less interested in playing outside, preferring to remain indoors because his knees and stomach hurt. His past medical history is significant for an upper respiratory infection that resolved uneventfully without treatment 2 weeks ago. Temperature is 99.5°F (37.5°C), blood pressure is 115/70 mmHg, pulse is 90/min, and respirations are 18/min. Physical exam shows scattered maroon macules and papules on the lower extremities. The abdomen is diffusely tender to palpation. There is no cervical lymphadenopathy or conjunctival injection. Which of the following will most likely be found in this patient?

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Flashcards: Coronary artery aneurysms

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What demographic is classically affected by Kawasaki disease? _____

TAP TO REVEAL ANSWER

What demographic is classically affected by Kawasaki disease? _____

Asian children < 4 years old

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