Review of a rare, can't-miss infant diagnosis: ALCAPA (not alpaca!)
Learn how to identify this highly fatal diagnosis in the emergency department and get your patient to definitive surgical correction.
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ALCAPA = "anomalous left coronary artery from the pulmonary artery."
Normally, the LCA arises from the aorta to supply the LV. In ALCAPA, the LCA comes off the PA, so it fills with deoxygenated blood, leading to ischemia then infarction.
Img: researchfeatures.com/alcapa-adults-…
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As pulmonary vascular resistance nadirs at ~ 6 wks of age, the abnormal LCA experiences "steal" and blood flow becomes retrograde, resulting in infarction.
Img: nature.com/articles/jp201…
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ALCAPA is one of the many congenital heart diseases NOT detected on newborn congenital heart screening as pre/post ductal sats will not show a differential.
Img: Washinton State Dept of Health
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In infant ALCAPA, babies have ischemic periods during high myocardial demand (feeding, crying).
In the ED, you may see a "pink baby" with infant CHF (failure to thrive, tachypnea). You may also see a "gray baby" in cardiogenic shock with no perfusion.
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On exam, you will hear a systolic murmur at the apex radiating to the axilla, indicative of mitral regurgitation from an ischemic anterolateral papillary muscle (like an adult with a left sided MI). Ofc, hard to hear w/tachycardia & tachypnea.
Img: researchgate.net/publication/23…
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EKG will be grossly abnormal and show pathologic Q waves in lateral leads (I, aVL, V5-6). You may also see signs of ischemia in the anterolateral leads, superior axis deviation, or ventricular hypertrophy.
Img: emdocs.net/ecg-pointers-a…
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CXR will show cardiomegaly and pulmonary edema from CHF.
Img: aboutkidshealth.ca/article?conten…
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Labs may show increased BNP and elevated troponin if infarcted. Make sure to get pre-op labs and insure good IV access.
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ALCAPA is often misdiagnosed as myocarditis, "asthma," bronchiolitis, or other recurrent viral illnesses, congenital upper airway issues (laryngomalacia, tracheomalacia), or infant interstitial lung diseases.
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Diagnosis is made with EKG/Echo, but sometimes cath/cardiac MRI is needed. Consult Cardiology stat for admission to a CICU for definitive repair, which can lead to great quality of life.
Img: chop.edu/conditions-dis…
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In summary, ALCAPA:
* Presents at 2-3 mo of age in CHF or cardiogenic shock
* Has pathognomonic EKG findings of lateral Q waves (one of the few pediatric MI conditions)
* Is almost 100% fatal, but if dx & surgically corrected, pts can enjoy great quality of life
/End
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